Congenital Heart Disease

PHOENIX – A prosthetic conduit that contains a porcine valve showed excellent intermediate-term durability for repairing the right ventricular outflow tract in 100 teenagers and young adults at a single U.S. center.

“The Carpentier-Edwards xenograft for right ventricular outflow tract [RVOT] reconstruction provides excellent freedom from reoperation and valve dysfunction, as well as sustained improvement in right-ventricular chamber size at intermediate-term follow-up,” Dr. Heidi B. Schubmehl said at the Society of Thoracic Surgeons annual meeting.

Mitchel L. Zoler/Frontline Medical News

Dr. Schubmehl reported a 92% rate of freedom from valve dysfunction with follow-up out to about 10 years, and significant reductions in right ventricular size at follow-up, compared with baseline, as measured by both echocardiography and by MRI.

The Carpentier-Edwards porcine valve and conduit “seemed to hold up better than a lot of other [prosthetic] valves,” said Dr. George M. Alfieris, director of pediatric cardiac surgery at the University of Rochester (N.Y.), and senior author for the study. In addition to the valve’s durability over approximately the first 10 years following placement, the results also showed the positive impact the valve had on right ventricular size, an important result of the repair’s efficacy, Dr. Alfieris said.

“It’s a mistake to allow the right ventricle to be under high pressure or to reach a large volume. We now focus on preserving the right ventricle,” he said in an interview. “I’ve become very concerned about preventing right ventricular dilation and preserving right ventricular function.”

Dr. Alfieris noted that his prior experience using other types of valves in the pulmonary valve and RVOT position showed those valves “did great for the first 10 years and then failed. What’s different in this series is that after 10 years, we have not seen the same dysfunction as with the prior generation of valves. I will be very interested to see what happens to them” as follow-up continues beyond 10 years. He also expressed dismay that recently the company that had been marketing the valve and conduit used in the current study, the Carpentier-Edwards, stopped selling them. He expects that as his supply of conduits runs out he’ll have to start using a different commercial valve and conduit that he believes will not perform as well or create his own conduits with a porcine valve from a different supplier.

The series of 100 patients comprised individuals aged 17 or older who received a pulmonary artery and had RVOT reconstruction at the University of Rochester during 2000-2010, Dr. Schubmehl reported. The series included 78 patients with a history of tetralogy of Fallot, 8 patients born with transposition of their great arteries, 8 patients with truncus arteriosus, and 6 patients with other congenital heart diseases. Their median age at the time they received the RVOT conduit was 24 years, 59% were men, and 99 had undergone a prior sternotomy. At the time they received the conduit, 55 had pulmonary valve insufficiency, 30 had valve stenosis, and 15 had both. Follow-up occurred an average of 7 years after conduit placement.

Two recipients died: One death occurred perioperatively in a 41-year old who had a massive cerebrovascular event, and the second death was in a 39-year old who died 2.6 years after conduit placement from respiratory failure. Two additional patients required a reintervention during follow-up, said Dr. Schubmehl, a general surgeon at the University of Rochester. One reintervention occurred after 11 years to treat endocarditis, and the second after 11 years to perform balloon valvuloplasty because of valve stenosis.

The results reported by Dr. Schubmehl for echocardiography examinations showed that the patients had a statistically significant reduction in their RVOT pressure gradient from baseline to 1-year follow-up that was sustained through their intermediate-term follow-up. Seventy-seven patients had pulmonary valve insufficiency at baseline that resolved in all patients at 1-year follow-up and remained resolved in all but one patient at extended follow-up. Nineteen patients underwent additional imaging with MRI at an average follow-up of 7 years, and these findings confirmed the echo results.

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On Twitter @mitchelzoler

PHOENIX – A prosthetic conduit that contains a porcine valve showed excellent intermediate-term durability for repairing the right ventricular outflow tract in 100 teenagers and young adults at a single U.S. center.

“The Carpentier-Edwards xenograft for right ventricular outflow tract [RVOT] reconstruction provides excellent freedom from reoperation and valve dysfunction, as well as sustained improvement in right-ventricular chamber size at intermediate-term follow-up,” Dr. Heidi B. Schubmehl said at the Society of Thoracic Surgeons annual meeting.

Mitchel L. Zoler/Frontline Medical News

Dr. Schubmehl reported a 92% rate of freedom from valve dysfunction with follow-up out to about 10 years, and significant reductions in right ventricular size at follow-up, compared with baseline, as measured by both echocardiography and by MRI.

The Carpentier-Edwards porcine valve and conduit “seemed to hold up better than a lot of other [prosthetic] valves,” said Dr. George M. Alfieris, director of pediatric cardiac surgery at the University of Rochester (N.Y.), and senior author for the study. In addition to the valve’s durability over approximately the first 10 years following placement, the results also showed the positive impact the valve had on right ventricular size, an important result of the repair’s efficacy, Dr. Alfieris said.

“It’s a mistake to allow the right ventricle to be under high pressure or to reach a large volume. We now focus on preserving the right ventricle,” he said in an interview. “I’ve become very concerned about preventing right ventricular dilation and preserving right ventricular function.”

Dr. Alfieris noted that his prior experience using other types of valves in the pulmonary valve and RVOT position showed those valves “did great for the first 10 years and then failed. What’s different in this series is that after 10 years, we have not seen the same dysfunction as with the prior generation of valves. I will be very interested to see what happens to them” as follow-up continues beyond 10 years. He also expressed dismay that recently the company that had been marketing the valve and conduit used in the current study, the Carpentier-Edwards, stopped selling them. He expects that as his supply of conduits runs out he’ll have to start using a different commercial valve and conduit that he believes will not perform as well or create his own conduits with a porcine valve from a different supplier.

The series of 100 patients comprised individuals aged 17 or older who received a pulmonary artery and had RVOT reconstruction at the University of Rochester during 2000-2010, Dr. Schubmehl reported. The series included 78 patients with a history of tetralogy of Fallot, 8 patients born with transposition of their great arteries, 8 patients with truncus arteriosus, and 6 patients with other congenital heart diseases. Their median age at the time they received the RVOT conduit was 24 years, 59% were men, and 99 had undergone a prior sternotomy. At the time they received the conduit, 55 had pulmonary valve insufficiency, 30 had valve stenosis, and 15 had both. Follow-up occurred an average of 7 years after conduit placement.

Two recipients died: One death occurred perioperatively in a 41-year old who had a massive cerebrovascular event, and the second death was in a 39-year old who died 2.6 years after conduit placement from respiratory failure. Two additional patients required a reintervention during follow-up, said Dr. Schubmehl, a general surgeon at the University of Rochester. One reintervention occurred after 11 years to treat endocarditis, and the second after 11 years to perform balloon valvuloplasty because of valve stenosis.

The results reported by Dr. Schubmehl for echocardiography examinations showed that the patients had a statistically significant reduction in their RVOT pressure gradient from baseline to 1-year follow-up that was sustained through their intermediate-term follow-up. Seventy-seven patients had pulmonary valve insufficiency at baseline that resolved in all patients at 1-year follow-up and remained resolved in all but one patient at extended follow-up. Nineteen patients underwent additional imaging with MRI at an average follow-up of 7 years, and these findings confirmed the echo results.

[email protected]

On Twitter @mitchelzoler

PHOENIX – A prosthetic conduit that contains a porcine valve showed excellent intermediate-term durability for repairing the right ventricular outflow tract in 100 teenagers and young adults at a single U.S. center.

“The Carpentier-Edwards xenograft for right ventricular outflow tract [RVOT] reconstruction provides excellent freedom from reoperation and valve dysfunction, as well as sustained improvement in right-ventricular chamber size at intermediate-term follow-up,” Dr. Heidi B. Schubmehl said at the Society of Thoracic Surgeons annual meeting.

Mitchel L. Zoler/Frontline Medical News

Dr. Schubmehl reported a 92% rate of freedom from valve dysfunction with follow-up out to about 10 years, and significant reductions in right ventricular size at follow-up, compared with baseline, as measured by both echocardiography and by MRI.

The Carpentier-Edwards porcine valve and conduit “seemed to hold up better than a lot of other [prosthetic] valves,” said Dr. George M. Alfieris, director of pediatric cardiac surgery at the University of Rochester (N.Y.), and senior author for the study. In addition to the valve’s durability over approximately the first 10 years following placement, the results also showed the positive impact the valve had on right ventricular size, an important result of the repair’s efficacy, Dr. Alfieris said.

“It’s a mistake to allow the right ventricle to be under high pressure or to reach a large volume. We now focus on preserving the right ventricle,” he said in an interview. “I’ve become very concerned about preventing right ventricular dilation and preserving right ventricular function.”

Dr. Alfieris noted that his prior experience using other types of valves in the pulmonary valve and RVOT position showed those valves “did great for the first 10 years and then failed. What’s different in this series is that after 10 years, we have not seen the same dysfunction as with the prior generation of valves. I will be very interested to see what happens to them” as follow-up continues beyond 10 years. He also expressed dismay that recently the company that had been marketing the valve and conduit used in the current study, the Carpentier-Edwards, stopped selling them. He expects that as his supply of conduits runs out he’ll have to start using a different commercial valve and conduit that he believes will not perform as well or create his own conduits with a porcine valve from a different supplier.

The series of 100 patients comprised individuals aged 17 or older who received a pulmonary artery and had RVOT reconstruction at the University of Rochester during 2000-2010, Dr. Schubmehl reported. The series included 78 patients with a history of tetralogy of Fallot, 8 patients born with transposition of their great arteries, 8 patients with truncus arteriosus, and 6 patients with other congenital heart diseases. Their median age at the time they received the RVOT conduit was 24 years, 59% were men, and 99 had undergone a prior sternotomy. At the time they received the conduit, 55 had pulmonary valve insufficiency, 30 had valve stenosis, and 15 had both. Follow-up occurred an average of 7 years after conduit placement.

Two recipients died: One death occurred perioperatively in a 41-year old who had a massive cerebrovascular event, and the second death was in a 39-year old who died 2.6 years after conduit placement from respiratory failure. Two additional patients required a reintervention during follow-up, said Dr. Schubmehl, a general surgeon at the University of Rochester. One reintervention occurred after 11 years to treat endocarditis, and the second after 11 years to perform balloon valvuloplasty because of valve stenosis.

The results reported by Dr. Schubmehl for echocardiography examinations showed that the patients had a statistically significant reduction in their RVOT pressure gradient from baseline to 1-year follow-up that was sustained through their intermediate-term follow-up. Seventy-seven patients had pulmonary valve insufficiency at baseline that resolved in all patients at 1-year follow-up and remained resolved in all but one patient at extended follow-up. Nineteen patients underwent additional imaging with MRI at an average follow-up of 7 years, and these findings confirmed the echo results.

[email protected]

On Twitter @mitchelzoler

ORLANDO – Bystander CPR was provided in 49% of U.S. cases of pediatric out-of-hospital cardiac arrest during 2013-2014, a major improvement over the 35% rate in a prior study 15 years ago, Dr. Maryam Y. Naim reported at the American Heart Association scientific sessions.

She presented an analysis of 2,176 out-of-hospital cardiac arrests (OHCA) in patients up to age 18 years who were included in the Cardiac Arrest Registry to Enhance Survival (CARES), the nation’s largest OHCA registry. Patients with traumatic OHCA and those whose bystander CPR (BCPR) was provided by a health care professional weren’t included.

Bruce Jancin/Frontline Medical News

Overall, the rate of neurologically favorable survival in pediatric recipients of BCPR was 11%, compared with 7% when BCPR wasn’t provided. But the results were far more impressive in the 14% of cardiac arrests that occurred outside the home, where the rate of neurologically favorable survival in BCPR recipients was 34%, more than twice the 15% figure for nonrecipients, according to Dr. Naim, a pediatrician and cardiac intensivist at Children’s Hospital of Philadelphia and the University of Pennsylvania.

Infants accounted for 47% of all pediatric OHCA, and in these youngest patients BCPR was of no benefit.

“The most common etiology of cardiac arrest in infants is sudden infant death syndrome. These are children who are found unresponsive in their cribs, and sometimes they’ve been dead a long time. We need to find something different for this population: perhaps developing a monitor to signal when an infant stops breathing or the heart rate goes down,” she said.

The fact that the BCPR rate in pediatric OHCA has climbed to 49% speaks well for public health efforts to improve education and awareness. Of those who received BCPR during 2013 and 2014, half got compression-only CPR, suggesting increasing adherence to the 2010 AHA guidelines for CPR and emergency cardiovascular care, which emphasized compression-only CPR as a viable alternative to conventional CPR, Dr. Naim added.

Her study highlighted a racial disparity in the application of BCPR in children and adolescents: Sixty percent of white youths with OHCA received BCPR, compared with 42% of blacks and 48% of Hispanics.

“About 70% of all bystander CPR was provided by a family member at home. So there’s really an opportunity there, especially in minority communities, to further increase education and awareness about bystander CPR, teaching family members to do it and also how to call 911 to start the chain of response,” she said.

Dr. Naim reported having no financial conflicts regarding her study.

[email protected]

ORLANDO – Bystander CPR was provided in 49% of U.S. cases of pediatric out-of-hospital cardiac arrest during 2013-2014, a major improvement over the 35% rate in a prior study 15 years ago, Dr. Maryam Y. Naim reported at the American Heart Association scientific sessions.

She presented an analysis of 2,176 out-of-hospital cardiac arrests (OHCA) in patients up to age 18 years who were included in the Cardiac Arrest Registry to Enhance Survival (CARES), the nation’s largest OHCA registry. Patients with traumatic OHCA and those whose bystander CPR (BCPR) was provided by a health care professional weren’t included.

Bruce Jancin/Frontline Medical News

Overall, the rate of neurologically favorable survival in pediatric recipients of BCPR was 11%, compared with 7% when BCPR wasn’t provided. But the results were far more impressive in the 14% of cardiac arrests that occurred outside the home, where the rate of neurologically favorable survival in BCPR recipients was 34%, more than twice the 15% figure for nonrecipients, according to Dr. Naim, a pediatrician and cardiac intensivist at Children’s Hospital of Philadelphia and the University of Pennsylvania.

Infants accounted for 47% of all pediatric OHCA, and in these youngest patients BCPR was of no benefit.

“The most common etiology of cardiac arrest in infants is sudden infant death syndrome. These are children who are found unresponsive in their cribs, and sometimes they’ve been dead a long time. We need to find something different for this population: perhaps developing a monitor to signal when an infant stops breathing or the heart rate goes down,” she said.

The fact that the BCPR rate in pediatric OHCA has climbed to 49% speaks well for public health efforts to improve education and awareness. Of those who received BCPR during 2013 and 2014, half got compression-only CPR, suggesting increasing adherence to the 2010 AHA guidelines for CPR and emergency cardiovascular care, which emphasized compression-only CPR as a viable alternative to conventional CPR, Dr. Naim added.

Her study highlighted a racial disparity in the application of BCPR in children and adolescents: Sixty percent of white youths with OHCA received BCPR, compared with 42% of blacks and 48% of Hispanics.

“About 70% of all bystander CPR was provided by a family member at home. So there’s really an opportunity there, especially in minority communities, to further increase education and awareness about bystander CPR, teaching family members to do it and also how to call 911 to start the chain of response,” she said.

Dr. Naim reported having no financial conflicts regarding her study.

[email protected]

ORLANDO – Bystander CPR was provided in 49% of U.S. cases of pediatric out-of-hospital cardiac arrest during 2013-2014, a major improvement over the 35% rate in a prior study 15 years ago, Dr. Maryam Y. Naim reported at the American Heart Association scientific sessions.

She presented an analysis of 2,176 out-of-hospital cardiac arrests (OHCA) in patients up to age 18 years who were included in the Cardiac Arrest Registry to Enhance Survival (CARES), the nation’s largest OHCA registry. Patients with traumatic OHCA and those whose bystander CPR (BCPR) was provided by a health care professional weren’t included.

Bruce Jancin/Frontline Medical News

Overall, the rate of neurologically favorable survival in pediatric recipients of BCPR was 11%, compared with 7% when BCPR wasn’t provided. But the results were far more impressive in the 14% of cardiac arrests that occurred outside the home, where the rate of neurologically favorable survival in BCPR recipients was 34%, more than twice the 15% figure for nonrecipients, according to Dr. Naim, a pediatrician and cardiac intensivist at Children’s Hospital of Philadelphia and the University of Pennsylvania.

Infants accounted for 47% of all pediatric OHCA, and in these youngest patients BCPR was of no benefit.

“The most common etiology of cardiac arrest in infants is sudden infant death syndrome. These are children who are found unresponsive in their cribs, and sometimes they’ve been dead a long time. We need to find something different for this population: perhaps developing a monitor to signal when an infant stops breathing or the heart rate goes down,” she said.

The fact that the BCPR rate in pediatric OHCA has climbed to 49% speaks well for public health efforts to improve education and awareness. Of those who received BCPR during 2013 and 2014, half got compression-only CPR, suggesting increasing adherence to the 2010 AHA guidelines for CPR and emergency cardiovascular care, which emphasized compression-only CPR as a viable alternative to conventional CPR, Dr. Naim added.

Her study highlighted a racial disparity in the application of BCPR in children and adolescents: Sixty percent of white youths with OHCA received BCPR, compared with 42% of blacks and 48% of Hispanics.

“About 70% of all bystander CPR was provided by a family member at home. So there’s really an opportunity there, especially in minority communities, to further increase education and awareness about bystander CPR, teaching family members to do it and also how to call 911 to start the chain of response,” she said.

Dr. Naim reported having no financial conflicts regarding her study.

[email protected]

Concerns with delayed right ventricle dysfunction have offset the early survival advantages after Norwood procedure with right ventricle to pulmonary artery conduit (NW-RVPA) over the Norwood with Blalock-Taussig shunt (NW-BT) in newborns with left ventricular outflow tract obstruction, but a recent report provides evidence that RV function between the two procedures is comparable for up to six years.

Reporting in the January issue of the Journal of Thoracic and Cardiovascular Surgery (J Thorac Cardiovasc Surg. 2015 Dec;150:1440–52), investigators for the Congenital Heart Surgeons’ Society (CHSS) trial found that NW-RVPA has better overall six-year survival and superior right ventricle function in the short term after surgery than NW-BT. The study involved 454 newborns with critical left ventricular outflow tract obstruction (LVOTO) in the CHSS database who had Norwood stage-1 from 2005 to 2014. Propensity matching paired 169 NW-RVPA patients with the same number of NW-BT patients for comparison. CHSS along with the Hospital for Sick Children in Toronto provided funding for the study.

“For neonates with critical LVOTO and similar baseline characteristics undergoing a Norwood stage-1 operation, the six-year overall survival and transplant-free survival were significantly better after NW-RVPA vs. NW-BT,” said Dr. Travis J. Wilder and his colleagues from the Hospital for Sick Children in Toronto.

Key questions the study sought to answer involved the clinical implications of the small variations in RV function between the two procedures, as well as the association between Norwood procedures and tricuspid valve regurgitation (TR) and overall survival.

Overall six-year survival was 70% for the NW-RVPA group vs. 55% for the NW-BT group. Right ventricle dysfunction rates three months after the procedure were lower for the NW-RVPA group, 6% vs. 16%, but rates of late RV dysfunction were less than 5% for both groups. Likewise, rates of moderate or greater TR at two years were lower in the NW-RVPA group: 11% vs. 16%.

Rates of Fontan operation after six years were higher among the NW-RVPA group (54% vs. 49%), as were transplantation rates (6% vs. 2%). Overall, 2% converted to a biventricular repair, but only after NW-RVPA; and seven patients who had NW-RVPA underwent a tricuspid valve repair, compared with four in the NW-BT group.

“For all survivors not undergoing transplantation or biventricular repair, the prevalence of late moderate or greater RV dysfunction and TR were similar between NW-BT and NW-RVPA at six years, without evidence of increased RV dysfunction for patients who underwent NW-RVPA,” Dr. Wilder and his colleagues said.

Consistent with previous studies, the CHSS study showed an early risk of death after a Norwood stage-1 operation, which may be due to a greater prevalence of significant RV dysfunction as the operation transitions from stage 1 to stage 2, Dr. Wilder and his coauthors said. “Although causation between these two time-related events cannot directly be made, it suggests that poor RV function contributes to early hazard for death,” they said.

The authors acknowledge a number of limitations with their study: the variation in the quality of echocardiogram reports from the multiple institutions involved, the inability of propensity matching to account for unmeasured factors and the influence of center and surgeon volume among participating sites. They also said that the ventriculotomy the NW-RVPA involves can lead to late aneurysm, arrhythmias, and ventricular failure. The adverse effects of ventriculotomy on long-term RV function “may not become apparent for years,” Dr. Wilder and his coauthors said.

Dr. Wilder presented a report of the original results at the 2015 American Association for Thoracic Surgery Annual meeting.

The authors had no relationships to disclose.

Concerns with delayed right ventricle dysfunction have offset the early survival advantages after Norwood procedure with right ventricle to pulmonary artery conduit (NW-RVPA) over the Norwood with Blalock-Taussig shunt (NW-BT) in newborns with left ventricular outflow tract obstruction, but a recent report provides evidence that RV function between the two procedures is comparable for up to six years.

Reporting in the January issue of the Journal of Thoracic and Cardiovascular Surgery (J Thorac Cardiovasc Surg. 2015 Dec;150:1440–52), investigators for the Congenital Heart Surgeons’ Society (CHSS) trial found that NW-RVPA has better overall six-year survival and superior right ventricle function in the short term after surgery than NW-BT. The study involved 454 newborns with critical left ventricular outflow tract obstruction (LVOTO) in the CHSS database who had Norwood stage-1 from 2005 to 2014. Propensity matching paired 169 NW-RVPA patients with the same number of NW-BT patients for comparison. CHSS along with the Hospital for Sick Children in Toronto provided funding for the study.

“For neonates with critical LVOTO and similar baseline characteristics undergoing a Norwood stage-1 operation, the six-year overall survival and transplant-free survival were significantly better after NW-RVPA vs. NW-BT,” said Dr. Travis J. Wilder and his colleagues from the Hospital for Sick Children in Toronto.

Key questions the study sought to answer involved the clinical implications of the small variations in RV function between the two procedures, as well as the association between Norwood procedures and tricuspid valve regurgitation (TR) and overall survival.

Overall six-year survival was 70% for the NW-RVPA group vs. 55% for the NW-BT group. Right ventricle dysfunction rates three months after the procedure were lower for the NW-RVPA group, 6% vs. 16%, but rates of late RV dysfunction were less than 5% for both groups. Likewise, rates of moderate or greater TR at two years were lower in the NW-RVPA group: 11% vs. 16%.

Rates of Fontan operation after six years were higher among the NW-RVPA group (54% vs. 49%), as were transplantation rates (6% vs. 2%). Overall, 2% converted to a biventricular repair, but only after NW-RVPA; and seven patients who had NW-RVPA underwent a tricuspid valve repair, compared with four in the NW-BT group.

“For all survivors not undergoing transplantation or biventricular repair, the prevalence of late moderate or greater RV dysfunction and TR were similar between NW-BT and NW-RVPA at six years, without evidence of increased RV dysfunction for patients who underwent NW-RVPA,” Dr. Wilder and his colleagues said.

Consistent with previous studies, the CHSS study showed an early risk of death after a Norwood stage-1 operation, which may be due to a greater prevalence of significant RV dysfunction as the operation transitions from stage 1 to stage 2, Dr. Wilder and his coauthors said. “Although causation between these two time-related events cannot directly be made, it suggests that poor RV function contributes to early hazard for death,” they said.

The authors acknowledge a number of limitations with their study: the variation in the quality of echocardiogram reports from the multiple institutions involved, the inability of propensity matching to account for unmeasured factors and the influence of center and surgeon volume among participating sites. They also said that the ventriculotomy the NW-RVPA involves can lead to late aneurysm, arrhythmias, and ventricular failure. The adverse effects of ventriculotomy on long-term RV function “may not become apparent for years,” Dr. Wilder and his coauthors said.

Dr. Wilder presented a report of the original results at the 2015 American Association for Thoracic Surgery Annual meeting.

The authors had no relationships to disclose.

Concerns with delayed right ventricle dysfunction have offset the early survival advantages after Norwood procedure with right ventricle to pulmonary artery conduit (NW-RVPA) over the Norwood with Blalock-Taussig shunt (NW-BT) in newborns with left ventricular outflow tract obstruction, but a recent report provides evidence that RV function between the two procedures is comparable for up to six years.

Reporting in the January issue of the Journal of Thoracic and Cardiovascular Surgery (J Thorac Cardiovasc Surg. 2015 Dec;150:1440–52), investigators for the Congenital Heart Surgeons’ Society (CHSS) trial found that NW-RVPA has better overall six-year survival and superior right ventricle function in the short term after surgery than NW-BT. The study involved 454 newborns with critical left ventricular outflow tract obstruction (LVOTO) in the CHSS database who had Norwood stage-1 from 2005 to 2014. Propensity matching paired 169 NW-RVPA patients with the same number of NW-BT patients for comparison. CHSS along with the Hospital for Sick Children in Toronto provided funding for the study.

“For neonates with critical LVOTO and similar baseline characteristics undergoing a Norwood stage-1 operation, the six-year overall survival and transplant-free survival were significantly better after NW-RVPA vs. NW-BT,” said Dr. Travis J. Wilder and his colleagues from the Hospital for Sick Children in Toronto.

Key questions the study sought to answer involved the clinical implications of the small variations in RV function between the two procedures, as well as the association between Norwood procedures and tricuspid valve regurgitation (TR) and overall survival.

Overall six-year survival was 70% for the NW-RVPA group vs. 55% for the NW-BT group. Right ventricle dysfunction rates three months after the procedure were lower for the NW-RVPA group, 6% vs. 16%, but rates of late RV dysfunction were less than 5% for both groups. Likewise, rates of moderate or greater TR at two years were lower in the NW-RVPA group: 11% vs. 16%.

Rates of Fontan operation after six years were higher among the NW-RVPA group (54% vs. 49%), as were transplantation rates (6% vs. 2%). Overall, 2% converted to a biventricular repair, but only after NW-RVPA; and seven patients who had NW-RVPA underwent a tricuspid valve repair, compared with four in the NW-BT group.

“For all survivors not undergoing transplantation or biventricular repair, the prevalence of late moderate or greater RV dysfunction and TR were similar between NW-BT and NW-RVPA at six years, without evidence of increased RV dysfunction for patients who underwent NW-RVPA,” Dr. Wilder and his colleagues said.

Consistent with previous studies, the CHSS study showed an early risk of death after a Norwood stage-1 operation, which may be due to a greater prevalence of significant RV dysfunction as the operation transitions from stage 1 to stage 2, Dr. Wilder and his coauthors said. “Although causation between these two time-related events cannot directly be made, it suggests that poor RV function contributes to early hazard for death,” they said.

The authors acknowledge a number of limitations with their study: the variation in the quality of echocardiogram reports from the multiple institutions involved, the inability of propensity matching to account for unmeasured factors and the influence of center and surgeon volume among participating sites. They also said that the ventriculotomy the NW-RVPA involves can lead to late aneurysm, arrhythmias, and ventricular failure. The adverse effects of ventriculotomy on long-term RV function “may not become apparent for years,” Dr. Wilder and his coauthors said.

Dr. Wilder presented a report of the original results at the 2015 American Association for Thoracic Surgery Annual meeting.

The authors had no relationships to disclose.

Most children and young adult patients with congenital heart disease can and should engage in some form of physical activity and should avoid a sedentary lifestyle, according to a task force scientific statement from the American Heart Association and the American College of Cardiology (AHA/ACC).

This recommendation comes despite the fears of sudden cardiac death (SCD) in young athletes, which formed the initial impetus of the entire series of task force reports.

The recommended level of sports participation for patients with treated or untreated congenital heart defect, however, should consider the training and the competitive aspects of the sport itself and must be individualized to the patient. This means taking into account the patient’s current functional status, history of surgery, and the presence of implanted cardiac devices, according to the report by Dr. George F. Van Hare of Washington University, St. Louis, and his colleagues, which was published online in the Journal of the American College of Cardiology.

© Jody Dingle/Fotolia.com

The report breaks down its specific recommendations based upon the various types of congenital heart defect (CHD). Full details and nuances of the recommendations and their specific levels of evidence for each individual condition and the many variants can be found in the online publication. Below is a brief and selected summary for some of the most common defects and some of those most pertinent to sudden cardiac death in young athletes.

Simple shunting lesions (atrial septal defect, ventricular septal defect, patent ductus arteriosus): Treated and untreated

In addressing the three most common subtypes of CHD – ventricular septal defect (VSD, 34%), atrial septal defect (ASD, 13%), and patent ductus arteriosus (PDA, 10%) – the committee found no data that children with these lesions are related to acknowledged episodes of sudden cardiac death (SCD). This applied whether the defects were closed or remained open. “With rare exceptions, patients with hemodynamically insignificant CHD such as VSD, ASD, and PDA may participate competitively in all sports,” it concluded. These recommendations fall under class I; level of evidence C for almost all of these patients, according to the writing committee.

Congenital coronary anomalies: Treated and untreated

Anomalies of coronary arteries are the second-most commonly identified structural causes of SCD in competitive athletes, accounting for about 17% of such deaths in the United States, according to the report. The vast majority of sudden deaths associated with coronary anomalies occur during or shortly after exercise. Despite being less commonly represented in patients, among athletes who have died suddenly, anomalous origin of the left main or left anterior descending coronary artery from the right sinus of Valsalva is far more prevalent. In addition, SCDs are most strongly associated with the pattern in which the anomalous left coronary artery passes between the aorta and main pulmonary artery. Recommended return to intense athletic activities is only to be permitted at least 3 months after surgery, and with a demonstration of the absence of ischemia on postoperative stress testing, with evidence levels depending on the type of anomaly. Of note, in contrast, the committee indicated that athletes with an anomalous origin of a right coronary artery from the left sinus of Valsalva should simply be evaluated by an exercise stress test, and for those without symptoms or a positive exercise stress test, permission to compete can be considered after adequate counseling (class IIa; level of evidence C).

Pulmonary valve stenosis: Treated and untreated

The committee determined that athletes with mild pulmonary stenosis (PS) and normal right ventricular (RV) function can participate in all competitive sports, although annual reevaluation also is recommended (class I; level of evidence B). In addition, athletes treated by operation or balloon valvuloplasty who have achieved adequate relief of PS (gradient less than 40 mm Hg by Doppler) can participate in all competitive sports (class I; level of evidence B). Other patients should be restricted to low-intensity sports, according to the committee.

Aortic valve stenosis: Treated and untreated

Children and adolescents with aortic stenosis (AS) are differentiated between those with mild, moderate, and severe AS by physical examination, ECG, and Doppler echocardiography. In all cases, regardless of the degree of stenosis, patients with a history of fatigue, light-headedness, dizziness, syncope, chest pain, or pallor on exercise deserve a full evaluation. Annual re-evaluation is required for all patients with AS because the disease can progress. Patients with severe AS are at risk of sudden death, particularly with exercise. The committee determined that athletes with mild AS can participate in all competitive sports (class I; level of evidence B), but that athletes with severe AS should be restricted from all competitive sports, with the possible exception of low-intensity sports (class III; level of evidence B).

Coarctation of the aorta: Treated and untreated

Before a decision is made regarding exercise participation, a detailed evaluation should be conducted, including a physical examination, ECG, chest radiograph, exercise testing, transthoracic echocardiographic evaluation of the aortic valve and aorta, and either magnetic resonance imaging or computed tomography angiography, according to the committee. The determination as to the level of sports participation permitted requires a complex assessment of these various test results and can range from full participation in the case of the least affected to restrictions to low-intensity sports in those more severely affected.

Cyanotic CHD, including tetralogy of Fallot

Full clinical assessment, including laboratory and exercise testing, should be considered before any physical activity because this population is at very high risk of sudden death, according to the committee. Recommendations are complex and depend on the level of repair and its success, but, in general, significant restrictions are recommended for all but the most effectively treated patients.

Transposition of the great arteries after atrial switch (Mustard or Senning operation)

This is a population highly at risk, according to the committee. They appear to have a unique response to exercise with reports that a high proportion of sudden death events occur during exertion. In addition, evidence of exercise-induced arrhythmias on routine clinical testing has not been shown to reliably predict exercise-induced SCD events. Although recommendations vary, including strong restrictions for many, at best the most successful of these patients should only be considered for low- to moderate-intensity competitive sports, according to the committee.

Other conditions assessed and evaluated by the committee included congenitally corrected TGA, TGA after the arterial switch, Fontan procedure, elevated pulmonary vascular resistence in CHD, ventricular dysfunction after CHD surgery, and Ebstein anomaly of the tricuspid valve.

In all cases, complete physical assessment of these patients is recommended, especially due to the often highly individualized nature of the patient’s presentation of these conditions and the variety and variability of interventions that may have been performed. Such differentials make recommendations regarding sports participation a complex calculus, which the committee attempts to provide, listing whatever evidence is available.

The majority of these patients, however, will not be considered for the highest levels of competitive sports participation. Although, in almost all cases, the need for physical activity as a contributor to patient health and well-being is stressed at whatever level of performance is possible.

The report ”Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology,” was prepared by Dr. Van Hare and his colleagues on behalf of the American Heart Association Electrocardiography and Arrhythmias Committee of the Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular and Stroke Nursing, Council on Functional Genomics and Translational Biology, and the American College of Cardiology (doi: 10.1016/j.jacc.2015.09.032).

This report is one of the assessments and recommendations of 15 task forces on eligibility and disqualification recommendations for young athletes, nine of which are disease or multidisease related. The other six task forces focus on a variety of relevant topics and issues regarding the risks of young athletes on the field, including screening, the use of automated external defibrillators on the field, the use of dietary supplements and performance-enhancing drugs, sudden death, and the medical-legal perspectives involved.

All 15 task force reports were simultaneously published online in the Journal of the American College of Cardiology and the journal Circulation.

Dr. Van Hare and all but one member of the writing group had no disclosures. One member disclosed consultant/advisory committee associations with a variety of medical device companies.

[email protected]

Most children and young adult patients with congenital heart disease can and should engage in some form of physical activity and should avoid a sedentary lifestyle, according to a task force scientific statement from the American Heart Association and the American College of Cardiology (AHA/ACC).

This recommendation comes despite the fears of sudden cardiac death (SCD) in young athletes, which formed the initial impetus of the entire series of task force reports.

The recommended level of sports participation for patients with treated or untreated congenital heart defect, however, should consider the training and the competitive aspects of the sport itself and must be individualized to the patient. This means taking into account the patient’s current functional status, history of surgery, and the presence of implanted cardiac devices, according to the report by Dr. George F. Van Hare of Washington University, St. Louis, and his colleagues, which was published online in the Journal of the American College of Cardiology.

© Jody Dingle/Fotolia.com

The report breaks down its specific recommendations based upon the various types of congenital heart defect (CHD). Full details and nuances of the recommendations and their specific levels of evidence for each individual condition and the many variants can be found in the online publication. Below is a brief and selected summary for some of the most common defects and some of those most pertinent to sudden cardiac death in young athletes.

Simple shunting lesions (atrial septal defect, ventricular septal defect, patent ductus arteriosus): Treated and untreated

In addressing the three most common subtypes of CHD – ventricular septal defect (VSD, 34%), atrial septal defect (ASD, 13%), and patent ductus arteriosus (PDA, 10%) – the committee found no data that children with these lesions are related to acknowledged episodes of sudden cardiac death (SCD). This applied whether the defects were closed or remained open. “With rare exceptions, patients with hemodynamically insignificant CHD such as VSD, ASD, and PDA may participate competitively in all sports,” it concluded. These recommendations fall under class I; level of evidence C for almost all of these patients, according to the writing committee.

Congenital coronary anomalies: Treated and untreated

Anomalies of coronary arteries are the second-most commonly identified structural causes of SCD in competitive athletes, accounting for about 17% of such deaths in the United States, according to the report. The vast majority of sudden deaths associated with coronary anomalies occur during or shortly after exercise. Despite being less commonly represented in patients, among athletes who have died suddenly, anomalous origin of the left main or left anterior descending coronary artery from the right sinus of Valsalva is far more prevalent. In addition, SCDs are most strongly associated with the pattern in which the anomalous left coronary artery passes between the aorta and main pulmonary artery. Recommended return to intense athletic activities is only to be permitted at least 3 months after surgery, and with a demonstration of the absence of ischemia on postoperative stress testing, with evidence levels depending on the type of anomaly. Of note, in contrast, the committee indicated that athletes with an anomalous origin of a right coronary artery from the left sinus of Valsalva should simply be evaluated by an exercise stress test, and for those without symptoms or a positive exercise stress test, permission to compete can be considered after adequate counseling (class IIa; level of evidence C).

Pulmonary valve stenosis: Treated and untreated

The committee determined that athletes with mild pulmonary stenosis (PS) and normal right ventricular (RV) function can participate in all competitive sports, although annual reevaluation also is recommended (class I; level of evidence B). In addition, athletes treated by operation or balloon valvuloplasty who have achieved adequate relief of PS (gradient less than 40 mm Hg by Doppler) can participate in all competitive sports (class I; level of evidence B). Other patients should be restricted to low-intensity sports, according to the committee.

Aortic valve stenosis: Treated and untreated

Children and adolescents with aortic stenosis (AS) are differentiated between those with mild, moderate, and severe AS by physical examination, ECG, and Doppler echocardiography. In all cases, regardless of the degree of stenosis, patients with a history of fatigue, light-headedness, dizziness, syncope, chest pain, or pallor on exercise deserve a full evaluation. Annual re-evaluation is required for all patients with AS because the disease can progress. Patients with severe AS are at risk of sudden death, particularly with exercise. The committee determined that athletes with mild AS can participate in all competitive sports (class I; level of evidence B), but that athletes with severe AS should be restricted from all competitive sports, with the possible exception of low-intensity sports (class III; level of evidence B).

Coarctation of the aorta: Treated and untreated

Before a decision is made regarding exercise participation, a detailed evaluation should be conducted, including a physical examination, ECG, chest radiograph, exercise testing, transthoracic echocardiographic evaluation of the aortic valve and aorta, and either magnetic resonance imaging or computed tomography angiography, according to the committee. The determination as to the level of sports participation permitted requires a complex assessment of these various test results and can range from full participation in the case of the least affected to restrictions to low-intensity sports in those more severely affected.

Cyanotic CHD, including tetralogy of Fallot

Full clinical assessment, including laboratory and exercise testing, should be considered before any physical activity because this population is at very high risk of sudden death, according to the committee. Recommendations are complex and depend on the level of repair and its success, but, in general, significant restrictions are recommended for all but the most effectively treated patients.

Transposition of the great arteries after atrial switch (Mustard or Senning operation)

This is a population highly at risk, according to the committee. They appear to have a unique response to exercise with reports that a high proportion of sudden death events occur during exertion. In addition, evidence of exercise-induced arrhythmias on routine clinical testing has not been shown to reliably predict exercise-induced SCD events. Although recommendations vary, including strong restrictions for many, at best the most successful of these patients should only be considered for low- to moderate-intensity competitive sports, according to the committee.

Other conditions assessed and evaluated by the committee included congenitally corrected TGA, TGA after the arterial switch, Fontan procedure, elevated pulmonary vascular resistence in CHD, ventricular dysfunction after CHD surgery, and Ebstein anomaly of the tricuspid valve.

In all cases, complete physical assessment of these patients is recommended, especially due to the often highly individualized nature of the patient’s presentation of these conditions and the variety and variability of interventions that may have been performed. Such differentials make recommendations regarding sports participation a complex calculus, which the committee attempts to provide, listing whatever evidence is available.

The majority of these patients, however, will not be considered for the highest levels of competitive sports participation. Although, in almost all cases, the need for physical activity as a contributor to patient health and well-being is stressed at whatever level of performance is possible.

The report ”Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology,” was prepared by Dr. Van Hare and his colleagues on behalf of the American Heart Association Electrocardiography and Arrhythmias Committee of the Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular and Stroke Nursing, Council on Functional Genomics and Translational Biology, and the American College of Cardiology (doi: 10.1016/j.jacc.2015.09.032).

This report is one of the assessments and recommendations of 15 task forces on eligibility and disqualification recommendations for young athletes, nine of which are disease or multidisease related. The other six task forces focus on a variety of relevant topics and issues regarding the risks of young athletes on the field, including screening, the use of automated external defibrillators on the field, the use of dietary supplements and performance-enhancing drugs, sudden death, and the medical-legal perspectives involved.

All 15 task force reports were simultaneously published online in the Journal of the American College of Cardiology and the journal Circulation.

Dr. Van Hare and all but one member of the writing group had no disclosures. One member disclosed consultant/advisory committee associations with a variety of medical device companies.

[email protected]

Most children and young adult patients with congenital heart disease can and should engage in some form of physical activity and should avoid a sedentary lifestyle, according to a task force scientific statement from the American Heart Association and the American College of Cardiology (AHA/ACC).

This recommendation comes despite the fears of sudden cardiac death (SCD) in young athletes, which formed the initial impetus of the entire series of task force reports.

The recommended level of sports participation for patients with treated or untreated congenital heart defect, however, should consider the training and the competitive aspects of the sport itself and must be individualized to the patient. This means taking into account the patient’s current functional status, history of surgery, and the presence of implanted cardiac devices, according to the report by Dr. George F. Van Hare of Washington University, St. Louis, and his colleagues, which was published online in the Journal of the American College of Cardiology.

© Jody Dingle/Fotolia.com

The report breaks down its specific recommendations based upon the various types of congenital heart defect (CHD). Full details and nuances of the recommendations and their specific levels of evidence for each individual condition and the many variants can be found in the online publication. Below is a brief and selected summary for some of the most common defects and some of those most pertinent to sudden cardiac death in young athletes.

Simple shunting lesions (atrial septal defect, ventricular septal defect, patent ductus arteriosus): Treated and untreated

In addressing the three most common subtypes of CHD – ventricular septal defect (VSD, 34%), atrial septal defect (ASD, 13%), and patent ductus arteriosus (PDA, 10%) – the committee found no data that children with these lesions are related to acknowledged episodes of sudden cardiac death (SCD). This applied whether the defects were closed or remained open. “With rare exceptions, patients with hemodynamically insignificant CHD such as VSD, ASD, and PDA may participate competitively in all sports,” it concluded. These recommendations fall under class I; level of evidence C for almost all of these patients, according to the writing committee.

Congenital coronary anomalies: Treated and untreated

Anomalies of coronary arteries are the second-most commonly identified structural causes of SCD in competitive athletes, accounting for about 17% of such deaths in the United States, according to the report. The vast majority of sudden deaths associated with coronary anomalies occur during or shortly after exercise. Despite being less commonly represented in patients, among athletes who have died suddenly, anomalous origin of the left main or left anterior descending coronary artery from the right sinus of Valsalva is far more prevalent. In addition, SCDs are most strongly associated with the pattern in which the anomalous left coronary artery passes between the aorta and main pulmonary artery. Recommended return to intense athletic activities is only to be permitted at least 3 months after surgery, and with a demonstration of the absence of ischemia on postoperative stress testing, with evidence levels depending on the type of anomaly. Of note, in contrast, the committee indicated that athletes with an anomalous origin of a right coronary artery from the left sinus of Valsalva should simply be evaluated by an exercise stress test, and for those without symptoms or a positive exercise stress test, permission to compete can be considered after adequate counseling (class IIa; level of evidence C).

Pulmonary valve stenosis: Treated and untreated

The committee determined that athletes with mild pulmonary stenosis (PS) and normal right ventricular (RV) function can participate in all competitive sports, although annual reevaluation also is recommended (class I; level of evidence B). In addition, athletes treated by operation or balloon valvuloplasty who have achieved adequate relief of PS (gradient less than 40 mm Hg by Doppler) can participate in all competitive sports (class I; level of evidence B). Other patients should be restricted to low-intensity sports, according to the committee.

Aortic valve stenosis: Treated and untreated

Children and adolescents with aortic stenosis (AS) are differentiated between those with mild, moderate, and severe AS by physical examination, ECG, and Doppler echocardiography. In all cases, regardless of the degree of stenosis, patients with a history of fatigue, light-headedness, dizziness, syncope, chest pain, or pallor on exercise deserve a full evaluation. Annual re-evaluation is required for all patients with AS because the disease can progress. Patients with severe AS are at risk of sudden death, particularly with exercise. The committee determined that athletes with mild AS can participate in all competitive sports (class I; level of evidence B), but that athletes with severe AS should be restricted from all competitive sports, with the possible exception of low-intensity sports (class III; level of evidence B).

Coarctation of the aorta: Treated and untreated

Before a decision is made regarding exercise participation, a detailed evaluation should be conducted, including a physical examination, ECG, chest radiograph, exercise testing, transthoracic echocardiographic evaluation of the aortic valve and aorta, and either magnetic resonance imaging or computed tomography angiography, according to the committee. The determination as to the level of sports participation permitted requires a complex assessment of these various test results and can range from full participation in the case of the least affected to restrictions to low-intensity sports in those more severely affected.

Cyanotic CHD, including tetralogy of Fallot

Full clinical assessment, including laboratory and exercise testing, should be considered before any physical activity because this population is at very high risk of sudden death, according to the committee. Recommendations are complex and depend on the level of repair and its success, but, in general, significant restrictions are recommended for all but the most effectively treated patients.

Transposition of the great arteries after atrial switch (Mustard or Senning operation)

This is a population highly at risk, according to the committee. They appear to have a unique response to exercise with reports that a high proportion of sudden death events occur during exertion. In addition, evidence of exercise-induced arrhythmias on routine clinical testing has not been shown to reliably predict exercise-induced SCD events. Although recommendations vary, including strong restrictions for many, at best the most successful of these patients should only be considered for low- to moderate-intensity competitive sports, according to the committee.

Other conditions assessed and evaluated by the committee included congenitally corrected TGA, TGA after the arterial switch, Fontan procedure, elevated pulmonary vascular resistence in CHD, ventricular dysfunction after CHD surgery, and Ebstein anomaly of the tricuspid valve.

In all cases, complete physical assessment of these patients is recommended, especially due to the often highly individualized nature of the patient’s presentation of these conditions and the variety and variability of interventions that may have been performed. Such differentials make recommendations regarding sports participation a complex calculus, which the committee attempts to provide, listing whatever evidence is available.

The majority of these patients, however, will not be considered for the highest levels of competitive sports participation. Although, in almost all cases, the need for physical activity as a contributor to patient health and well-being is stressed at whatever level of performance is possible.

The report ”Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology,” was prepared by Dr. Van Hare and his colleagues on behalf of the American Heart Association Electrocardiography and Arrhythmias Committee of the Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular and Stroke Nursing, Council on Functional Genomics and Translational Biology, and the American College of Cardiology (doi: 10.1016/j.jacc.2015.09.032).

This report is one of the assessments and recommendations of 15 task forces on eligibility and disqualification recommendations for young athletes, nine of which are disease or multidisease related. The other six task forces focus on a variety of relevant topics and issues regarding the risks of young athletes on the field, including screening, the use of automated external defibrillators on the field, the use of dietary supplements and performance-enhancing drugs, sudden death, and the medical-legal perspectives involved.

All 15 task force reports were simultaneously published online in the Journal of the American College of Cardiology and the journal Circulation.

Dr. Van Hare and all but one member of the writing group had no disclosures. One member disclosed consultant/advisory committee associations with a variety of medical device companies.

[email protected]

A 25-year study of heart transplants in children with congenital heart disease (CHD) at one institution has found that results haven’t improved over time despite advances in technology and techniques. To improve outcomes, transplant surgeons may need to do a better job of selecting patients and matching patients and donors, according to study in the December issue of the Journal of Thoracic and Cardiovascular Surgery (J Thorac Cardiovasc Surg. 2015;150:1455-62).

“Strategies to improve outcomes in CHD patients might need to address selection criteria, transplantation timing, pretransplant and posttransplant care,” noted Dr. Bahaaldin Alsoufi, of the division of cardiothoracic surgery, Children’s Healthcare of Atlanta, Emory University. “The effect of donor/recipient race mismatch warrants further investigation and might impact organ allocation algorithms or immunosuppression management,” wrote Dr. Alsoufi and his colleagues.

The researchers analyzed results of 124 children with CHD who had heart transplants from 1988 to 2013 at Emory University and Children’s Healthcare of Atlanta. Median age was 3.8 years; 61% were boys. Ten years after heart transplantation, 44% (54) of patients were alive without a second transplant, 13% (17) had a second transplant and 43% (53) died without a second transplant. After the second transplant, 9 of the 17 patients were alive, but 3 of them had gone onto a third transplant. Overall 15-year survival following the first transplant was 41% (51).

The study cited data from the Registry of the International Society for Heart and Lung Transplantation that reported more than 11,000 pediatric heart transplants worldwide in 2013, and CHD represents about 54% of all heart transplants in infants.

A multivariate analysis identified the following risk factors for early mortality after transplant: age younger than 12 months (hazard ration [HR] 7.2) and prolonged cardiopulmonary bypass (HR 5). Late-phase mortality risk factors were age younger than 12 months (HR 3) and donor/recipient race mismatch (HR 2.2).

“Survival was not affected by era, underlying anomaly, prior Fontan, sensitization or pulmonary artery augmentation,” wrote Dr. Alsoufi and his colleagues.

Among the risk factors, longer bypass times may be a surrogate for a more complicated operation, the authors said. But where prior sternotomy is a risk factor following a heart transplant in adults, the study found no such risk in children. Another risk factor previous reports identified is pulmonary artery augmentation, but, again, this study found no risk in the pediatric group.

The researchers looked at days on the waiting list, with a median wait of 39 days in the study group. In all, 175 children were listed for transplants, but 51 did not go through for various reasons. Most of the children with CHD who had a heart transplant had previous surgery; only 13% had a primary heart transplant, mostly in the earlier phase of the study.

Dr. Alsoufi and coauthors also identified African American race as a risk factor for lower survival, which is consistent with other reports. But this study agreed with a previous report that donor/recipient race mismatch was a significant risk factor in white and African American patients (Ann Thorac Surg. 2009;87:204-9). “While our finding might be anecdotal and specific to our geographic population, this warrants some investigation and might have some impact on future organ allocation algorithms and immunosuppression management,” the researchers wrote.

The authors had no relevant disclosures. Emory University School of Medicine, Children’s Healthcare of Atlanta provided study funding.

A 25-year study of heart transplants in children with congenital heart disease (CHD) at one institution has found that results haven’t improved over time despite advances in technology and techniques. To improve outcomes, transplant surgeons may need to do a better job of selecting patients and matching patients and donors, according to study in the December issue of the Journal of Thoracic and Cardiovascular Surgery (J Thorac Cardiovasc Surg. 2015;150:1455-62).

“Strategies to improve outcomes in CHD patients might need to address selection criteria, transplantation timing, pretransplant and posttransplant care,” noted Dr. Bahaaldin Alsoufi, of the division of cardiothoracic surgery, Children’s Healthcare of Atlanta, Emory University. “The effect of donor/recipient race mismatch warrants further investigation and might impact organ allocation algorithms or immunosuppression management,” wrote Dr. Alsoufi and his colleagues.

The researchers analyzed results of 124 children with CHD who had heart transplants from 1988 to 2013 at Emory University and Children’s Healthcare of Atlanta. Median age was 3.8 years; 61% were boys. Ten years after heart transplantation, 44% (54) of patients were alive without a second transplant, 13% (17) had a second transplant and 43% (53) died without a second transplant. After the second transplant, 9 of the 17 patients were alive, but 3 of them had gone onto a third transplant. Overall 15-year survival following the first transplant was 41% (51).

The study cited data from the Registry of the International Society for Heart and Lung Transplantation that reported more than 11,000 pediatric heart transplants worldwide in 2013, and CHD represents about 54% of all heart transplants in infants.

A multivariate analysis identified the following risk factors for early mortality after transplant: age younger than 12 months (hazard ration [HR] 7.2) and prolonged cardiopulmonary bypass (HR 5). Late-phase mortality risk factors were age younger than 12 months (HR 3) and donor/recipient race mismatch (HR 2.2).

“Survival was not affected by era, underlying anomaly, prior Fontan, sensitization or pulmonary artery augmentation,” wrote Dr. Alsoufi and his colleagues.

Among the risk factors, longer bypass times may be a surrogate for a more complicated operation, the authors said. But where prior sternotomy is a risk factor following a heart transplant in adults, the study found no such risk in children. Another risk factor previous reports identified is pulmonary artery augmentation, but, again, this study found no risk in the pediatric group.

The researchers looked at days on the waiting list, with a median wait of 39 days in the study group. In all, 175 children were listed for transplants, but 51 did not go through for various reasons. Most of the children with CHD who had a heart transplant had previous surgery; only 13% had a primary heart transplant, mostly in the earlier phase of the study.

Dr. Alsoufi and coauthors also identified African American race as a risk factor for lower survival, which is consistent with other reports. But this study agreed with a previous report that donor/recipient race mismatch was a significant risk factor in white and African American patients (Ann Thorac Surg. 2009;87:204-9). “While our finding might be anecdotal and specific to our geographic population, this warrants some investigation and might have some impact on future organ allocation algorithms and immunosuppression management,” the researchers wrote.

The authors had no relevant disclosures. Emory University School of Medicine, Children’s Healthcare of Atlanta provided study funding.

A 25-year study of heart transplants in children with congenital heart disease (CHD) at one institution has found that results haven’t improved over time despite advances in technology and techniques. To improve outcomes, transplant surgeons may need to do a better job of selecting patients and matching patients and donors, according to study in the December issue of the Journal of Thoracic and Cardiovascular Surgery (J Thorac Cardiovasc Surg. 2015;150:1455-62).

“Strategies to improve outcomes in CHD patients might need to address selection criteria, transplantation timing, pretransplant and posttransplant care,” noted Dr. Bahaaldin Alsoufi, of the division of cardiothoracic surgery, Children’s Healthcare of Atlanta, Emory University. “The effect of donor/recipient race mismatch warrants further investigation and might impact organ allocation algorithms or immunosuppression management,” wrote Dr. Alsoufi and his colleagues.

The researchers analyzed results of 124 children with CHD who had heart transplants from 1988 to 2013 at Emory University and Children’s Healthcare of Atlanta. Median age was 3.8 years; 61% were boys. Ten years after heart transplantation, 44% (54) of patients were alive without a second transplant, 13% (17) had a second transplant and 43% (53) died without a second transplant. After the second transplant, 9 of the 17 patients were alive, but 3 of them had gone onto a third transplant. Overall 15-year survival following the first transplant was 41% (51).

The study cited data from the Registry of the International Society for Heart and Lung Transplantation that reported more than 11,000 pediatric heart transplants worldwide in 2013, and CHD represents about 54% of all heart transplants in infants.

A multivariate analysis identified the following risk factors for early mortality after transplant: age younger than 12 months (hazard ration [HR] 7.2) and prolonged cardiopulmonary bypass (HR 5). Late-phase mortality risk factors were age younger than 12 months (HR 3) and donor/recipient race mismatch (HR 2.2).

“Survival was not affected by era, underlying anomaly, prior Fontan, sensitization or pulmonary artery augmentation,” wrote Dr. Alsoufi and his colleagues.

Among the risk factors, longer bypass times may be a surrogate for a more complicated operation, the authors said. But where prior sternotomy is a risk factor following a heart transplant in adults, the study found no such risk in children. Another risk factor previous reports identified is pulmonary artery augmentation, but, again, this study found no risk in the pediatric group.

The researchers looked at days on the waiting list, with a median wait of 39 days in the study group. In all, 175 children were listed for transplants, but 51 did not go through for various reasons. Most of the children with CHD who had a heart transplant had previous surgery; only 13% had a primary heart transplant, mostly in the earlier phase of the study.

Dr. Alsoufi and coauthors also identified African American race as a risk factor for lower survival, which is consistent with other reports. But this study agreed with a previous report that donor/recipient race mismatch was a significant risk factor in white and African American patients (Ann Thorac Surg. 2009;87:204-9). “While our finding might be anecdotal and specific to our geographic population, this warrants some investigation and might have some impact on future organ allocation algorithms and immunosuppression management,” the researchers wrote.

The authors had no relevant disclosures. Emory University School of Medicine, Children’s Healthcare of Atlanta provided study funding.

Adult congenital heart disease significantly increases the risk of both hemorrhagic and ischemic stroke, particularly in individuals under 55 years of age, new data suggests.

A retrospective study of 29,638 adults aged 18-64 years with adult congenital heart disease (ACHD) showed that women aged 15-54 years with the disease were more than 12 times as likely to experience an ischemic stroke compared to the general population, while men had a nine-fold increase in risk.

Women aged over 55 years with ACHD had a four-fold higher risk of ischemic stroke, and men had a two-fold increase in risk, compared to the general population, according to a study published Nov. 23 in Circulation.

In the case of hemorrhagic stroke, women aged under 55 had a five-fold greater risk and men had a more than six-fold greater risk of ischemic stroke, while the risk for those older than 55 years was 2-3 times higher (Circulation 2015, November 23 [doi: 10.1161/CIRCULATIONAHA.115.011241]).

The risk of ischemic stroke increased significantly with heart failure, diabetes, or a recent myocardial infarction, and overall, 8.9% of men and 6.8% of women with ACHD who reached the age of 18 years had at least one stroke before age 65.

“Whether subgroups of patients with heart failure and sinus rhythm could benefit from an antithrombotic treatment is a matter of ongoing research in the general population and based on our findings may warrant further investigation in ACHD-patients,” wrote Dr. Jonas Lanz, from the McGill Adult Unit for Congenital Heart Disease Excellence, and co-authors.

The study was funded by the Heart and Stroke Foundation of Québec, the Fonds de Recherche en Santé Québec and the Canadian Institute of Health Research. There were no conflicts of interest declared.

Adult congenital heart disease significantly increases the risk of both hemorrhagic and ischemic stroke, particularly in individuals under 55 years of age, new data suggests.

A retrospective study of 29,638 adults aged 18-64 years with adult congenital heart disease (ACHD) showed that women aged 15-54 years with the disease were more than 12 times as likely to experience an ischemic stroke compared to the general population, while men had a nine-fold increase in risk.

Women aged over 55 years with ACHD had a four-fold higher risk of ischemic stroke, and men had a two-fold increase in risk, compared to the general population, according to a study published Nov. 23 in Circulation.

In the case of hemorrhagic stroke, women aged under 55 had a five-fold greater risk and men had a more than six-fold greater risk of ischemic stroke, while the risk for those older than 55 years was 2-3 times higher (Circulation 2015, November 23 [doi: 10.1161/CIRCULATIONAHA.115.011241]).

The risk of ischemic stroke increased significantly with heart failure, diabetes, or a recent myocardial infarction, and overall, 8.9% of men and 6.8% of women with ACHD who reached the age of 18 years had at least one stroke before age 65.

“Whether subgroups of patients with heart failure and sinus rhythm could benefit from an antithrombotic treatment is a matter of ongoing research in the general population and based on our findings may warrant further investigation in ACHD-patients,” wrote Dr. Jonas Lanz, from the McGill Adult Unit for Congenital Heart Disease Excellence, and co-authors.

The study was funded by the Heart and Stroke Foundation of Québec, the Fonds de Recherche en Santé Québec and the Canadian Institute of Health Research. There were no conflicts of interest declared.

Adult congenital heart disease significantly increases the risk of both hemorrhagic and ischemic stroke, particularly in individuals under 55 years of age, new data suggests.

A retrospective study of 29,638 adults aged 18-64 years with adult congenital heart disease (ACHD) showed that women aged 15-54 years with the disease were more than 12 times as likely to experience an ischemic stroke compared to the general population, while men had a nine-fold increase in risk.

Women aged over 55 years with ACHD had a four-fold higher risk of ischemic stroke, and men had a two-fold increase in risk, compared to the general population, according to a study published Nov. 23 in Circulation.

In the case of hemorrhagic stroke, women aged under 55 had a five-fold greater risk and men had a more than six-fold greater risk of ischemic stroke, while the risk for those older than 55 years was 2-3 times higher (Circulation 2015, November 23 [doi: 10.1161/CIRCULATIONAHA.115.011241]).

The risk of ischemic stroke increased significantly with heart failure, diabetes, or a recent myocardial infarction, and overall, 8.9% of men and 6.8% of women with ACHD who reached the age of 18 years had at least one stroke before age 65.

“Whether subgroups of patients with heart failure and sinus rhythm could benefit from an antithrombotic treatment is a matter of ongoing research in the general population and based on our findings may warrant further investigation in ACHD-patients,” wrote Dr. Jonas Lanz, from the McGill Adult Unit for Congenital Heart Disease Excellence, and co-authors.

The study was funded by the Heart and Stroke Foundation of Québec, the Fonds de Recherche en Santé Québec and the Canadian Institute of Health Research. There were no conflicts of interest declared.

Infants with congenital heart disease, specifically left heart syndrome, who had cardiac stem-cell therapy after surgery showed improved cardiac and brain function after 3 years when compared with infants who had standard therapy, according to latest results from a clinical trial of progenitor cell infusion in infants.

The prospective, controlled study, published in the November issue of the Journal of Thoracic and Cardiovascular Surgery (2015;150[5]:1198-1208), involved 14 infants with hypoplastic left heart syndrome (HLHS). Dr. Suguru Tarui and colleagues at Okayama University Hospital in Japan infused seven infants with intracoronary cardiosphere-derived cells (CDCs) 1 month after they had two- or three-stage palliative surgery. Seven controls had standard care alone. The trial is known TICAP (Three-year follow-up of the Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single-Ventricle Physiology) (ClinicalTrials.gov ID: NCT01273857).

Infants born with HLHS are known to have poor prognoses. HLHS has been associated with the highest mortality of all congenital heart lesions (Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015;18[1]:2-6).

The Okayama investigators conducted TICAP in 2011 and demonstrated the feasibility and safety of the intracoronary delivery of CDCs in infants with HLHS after staged procedures. The latest report looks at the secondary outcome of cardiac function through 36 months of follow-up, and is the first clinical trial to report on the mid-term results of cardiac progenitor therapy in congenital heart disease.

“CDC infusion could improve right ventricular function from 3 through 36 months of observation,” Dr. Tarui and colleagues said. “Patients treated by CDCs showed an increase in somatic growth and reduced heart failure status in mid-term follow-up.”

Upon entry into the study, all subjects were of similar age, body weight, and risk profiles. After 36 months, the CDC group showed no complications and significantly improved right ventricular injection fraction compared with controls. As a result, the CDC group had reduced brain natriuretic peptide levels, lower rates of unplanned catheterizations, and higher weight for age. No adverse events occurred in the CDC group, while two patients in the control group had complications (one developed heart failure, another developed enteropathy).

The investigators looked at predictors of cardiac functional efficacy in the CDC group and determined that younger age was associated with greater improvement of right ventricular ejection fraction, as measured on echocardiography. The lower weight-for-age Z score and reduced ejection fraction at the time of infusion may be predictors of cardiac function improvements at 3 years.

“This therapeutic strategy may merit somatic growth enhancement and reduce the incidence of heart failure,” Dr. Tarui and coauthors said.

They noted a number of limitations of their small clinical trial. The study was nonrandomized, and the cardiac interventions were not blinded, among others. “Larger phase II studies focusing on changes in cardiac function, myocardial fibrosis, and quality of life and clinical event rates are warranted to confirm these effects of CDC administration in patients with single ventricular physiology,” they said.

The government of Japan through its ministries of Health, Labor and Welfare, and Education, Culture Sports, Science and Technology provided funding for the study, as did the Research Foundation of Okayama University Hospital. The authors had no disclosures.

Infants with congenital heart disease, specifically left heart syndrome, who had cardiac stem-cell therapy after surgery showed improved cardiac and brain function after 3 years when compared with infants who had standard therapy, according to latest results from a clinical trial of progenitor cell infusion in infants.

The prospective, controlled study, published in the November issue of the Journal of Thoracic and Cardiovascular Surgery (2015;150[5]:1198-1208), involved 14 infants with hypoplastic left heart syndrome (HLHS). Dr. Suguru Tarui and colleagues at Okayama University Hospital in Japan infused seven infants with intracoronary cardiosphere-derived cells (CDCs) 1 month after they had two- or three-stage palliative surgery. Seven controls had standard care alone. The trial is known TICAP (Three-year follow-up of the Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single-Ventricle Physiology) (ClinicalTrials.gov ID: NCT01273857).

Infants born with HLHS are known to have poor prognoses. HLHS has been associated with the highest mortality of all congenital heart lesions (Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015;18[1]:2-6).

The Okayama investigators conducted TICAP in 2011 and demonstrated the feasibility and safety of the intracoronary delivery of CDCs in infants with HLHS after staged procedures. The latest report looks at the secondary outcome of cardiac function through 36 months of follow-up, and is the first clinical trial to report on the mid-term results of cardiac progenitor therapy in congenital heart disease.

“CDC infusion could improve right ventricular function from 3 through 36 months of observation,” Dr. Tarui and colleagues said. “Patients treated by CDCs showed an increase in somatic growth and reduced heart failure status in mid-term follow-up.”

Upon entry into the study, all subjects were of similar age, body weight, and risk profiles. After 36 months, the CDC group showed no complications and significantly improved right ventricular injection fraction compared with controls. As a result, the CDC group had reduced brain natriuretic peptide levels, lower rates of unplanned catheterizations, and higher weight for age. No adverse events occurred in the CDC group, while two patients in the control group had complications (one developed heart failure, another developed enteropathy).

The investigators looked at predictors of cardiac functional efficacy in the CDC group and determined that younger age was associated with greater improvement of right ventricular ejection fraction, as measured on echocardiography. The lower weight-for-age Z score and reduced ejection fraction at the time of infusion may be predictors of cardiac function improvements at 3 years.

“This therapeutic strategy may merit somatic growth enhancement and reduce the incidence of heart failure,” Dr. Tarui and coauthors said.

They noted a number of limitations of their small clinical trial. The study was nonrandomized, and the cardiac interventions were not blinded, among others. “Larger phase II studies focusing on changes in cardiac function, myocardial fibrosis, and quality of life and clinical event rates are warranted to confirm these effects of CDC administration in patients with single ventricular physiology,” they said.

The government of Japan through its ministries of Health, Labor and Welfare, and Education, Culture Sports, Science and Technology provided funding for the study, as did the Research Foundation of Okayama University Hospital. The authors had no disclosures.

Infants with congenital heart disease, specifically left heart syndrome, who had cardiac stem-cell therapy after surgery showed improved cardiac and brain function after 3 years when compared with infants who had standard therapy, according to latest results from a clinical trial of progenitor cell infusion in infants.

The prospective, controlled study, published in the November issue of the Journal of Thoracic and Cardiovascular Surgery (2015;150[5]:1198-1208), involved 14 infants with hypoplastic left heart syndrome (HLHS). Dr. Suguru Tarui and colleagues at Okayama University Hospital in Japan infused seven infants with intracoronary cardiosphere-derived cells (CDCs) 1 month after they had two- or three-stage palliative surgery. Seven controls had standard care alone. The trial is known TICAP (Three-year follow-up of the Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single-Ventricle Physiology) (ClinicalTrials.gov ID: NCT01273857).

Infants born with HLHS are known to have poor prognoses. HLHS has been associated with the highest mortality of all congenital heart lesions (Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015;18[1]:2-6).

The Okayama investigators conducted TICAP in 2011 and demonstrated the feasibility and safety of the intracoronary delivery of CDCs in infants with HLHS after staged procedures. The latest report looks at the secondary outcome of cardiac function through 36 months of follow-up, and is the first clinical trial to report on the mid-term results of cardiac progenitor therapy in congenital heart disease.

“CDC infusion could improve right ventricular function from 3 through 36 months of observation,” Dr. Tarui and colleagues said. “Patients treated by CDCs showed an increase in somatic growth and reduced heart failure status in mid-term follow-up.”

Upon entry into the study, all subjects were of similar age, body weight, and risk profiles. After 36 months, the CDC group showed no complications and significantly improved right ventricular injection fraction compared with controls. As a result, the CDC group had reduced brain natriuretic peptide levels, lower rates of unplanned catheterizations, and higher weight for age. No adverse events occurred in the CDC group, while two patients in the control group had complications (one developed heart failure, another developed enteropathy).

The investigators looked at predictors of cardiac functional efficacy in the CDC group and determined that younger age was associated with greater improvement of right ventricular ejection fraction, as measured on echocardiography. The lower weight-for-age Z score and reduced ejection fraction at the time of infusion may be predictors of cardiac function improvements at 3 years.

“This therapeutic strategy may merit somatic growth enhancement and reduce the incidence of heart failure,” Dr. Tarui and coauthors said.

They noted a number of limitations of their small clinical trial. The study was nonrandomized, and the cardiac interventions were not blinded, among others. “Larger phase II studies focusing on changes in cardiac function, myocardial fibrosis, and quality of life and clinical event rates are warranted to confirm these effects of CDC administration in patients with single ventricular physiology,” they said.

The government of Japan through its ministries of Health, Labor and Welfare, and Education, Culture Sports, Science and Technology provided funding for the study, as did the Research Foundation of Okayama University Hospital. The authors had no disclosures.

In the first report evaluating the impact of a clinical guideline that calls for the use of postoperative continuous electroencephalography (CEEG) on infants after they’ve had cardiopulmonary bypass surgery, investigators at Children’s Hospital of Philadelphia and the University of Pennsylvania validated the clinical utility of routine CEEG monitoring and found that clinical assessment for seizures without CEEG is not a reliable marker for diagnosis and treatment.

In a report online in the Journal of Thoracic and Cardiovascular Surgery (J. Thorac. Cardiovasc. Surg. 2015 [doi:10.1016/j.jtcvs.2015.03.045]), Dr. Maryam Naim and colleagues said that CEEG identified electroencephalographic seizures in 8% of newborns after cardiopulmonary bypass surgery. The study, conducted over 18 months, evaluated 172 newborns, none older than 1 month, with 161 (94%) having undergone postoperative CEEG. They had CEEG within 6 hours of their return to the cardiac intensive care unit.

Courtesy of JTCVS/AATS

The study classified electroencephalographic seizures as EEG-only (also termed nonconvulsive seizures, with no observable clinical signs either at bedside or via video) or electroclinical seizures. Dr. Naim and colleagues said the majority of seizures they identified with CEEG would not have been noticed otherwise as they had no clinically obvious signs or symptoms.

The American Clinical Neurophysiology Society (ACNS) recommends that cardiac surgeons consider continuous CEEG monitoring in high-risk neonates with congenital heart disease (CHD) after bypass surgery, but Dr. Naim and coauthors raised the question of whether seizure incidence would justify routine CEEG for all neonates with CHD who’ve had bypass surgery, especially as health systems place greater emphasis on quality improvement programs and cost-effective strategies. The authors said that neonates with all types of congenital heart disease had seizures.

“In adult populations, CEEG has not been shown to significantly increase hospital costs, but cost-effectiveness analyses have not been performed in neonates with CHD,” the authors said.

So they attempted to identify at-risk populations of newborns who would benefit most from routine CEEG monitoring. In a multivariable model that the investigators used, both delayed sternal closure and longer deep hypothermic circulatory arrest (DHCA) during surgery seemed predictive of seizures, but the odds ratios for both were low, “suggesting the statistically significant findings may not be very useful in focusing CEEG implementation on a high-risk group.”

Previous studies have reported that identifying and treating seizures in newborns who have had bypass surgery may reduce secondary brain injury and improve outcomes (Pediatrics 2008;121:e759-67), and the Boston Circulatory Arrest Study showed an association between postoperative seizures and lower reading and math scores and lower cognitive and functional skills later in life (Circulation 2011;124:1361-1369). The authors cited other studies that showed older, critically ill children with “high seizure burdens” have had worse outcomes. (Critical Care Medicine 2013;31:215-23; Neurology 2014;82:396-404; Brain 2014;137:1429-38). They also pointed out increased risk if the seizure is not treated. “While occurrence of a seizure is a marker of brain injury, there may also be secondary injury if the seizure activity is not terminated,” Dr. Naim and coauthors said.

The investigators concluded that postoperative CEEG to identify seizures “is warranted,” and while they found some newborns may be at greater risk of postbypass seizures than others, they advocated for “widespread” monitoring strategies.

Their work also questioned the effectiveness of non-CEEG assessment. In the study, clinicians identified bedside events indicative of seizures – what the study termed “push-button events” – in 32 newborns, or about 18% of patients, but none of the events had an EEG correlate, so they were considered nonepileptic. When the authors looked more closely at those “push-button” events, they found they ranged from abnormal body movement in 14 and hypertension in 7 to tachycardia and abnormal face movements, among other characterizations, in lesser numbers.

“Furthermore, push-button events by bedside clinicians, including abnormal movements and hypertensive episodes concerning for possible seizures, did not have any EEG correlate, indicating that bedside clinical assessment for seizures without CEEG monitoring is unreliable,” Dr. Naim and colleagues said.

As to whether identifying and treating postbypass seizures in young newborns with CHD will improve long-term neurodevelopment in these children, the authors acknowledged that further study is needed.

They reported having no financial disclosures.

In the first report evaluating the impact of a clinical guideline that calls for the use of postoperative continuous electroencephalography (CEEG) on infants after they’ve had cardiopulmonary bypass surgery, investigators at Children’s Hospital of Philadelphia and the University of Pennsylvania validated the clinical utility of routine CEEG monitoring and found that clinical assessment for seizures without CEEG is not a reliable marker for diagnosis and treatment.

In a report online in the Journal of Thoracic and Cardiovascular Surgery (J. Thorac. Cardiovasc. Surg. 2015 [doi:10.1016/j.jtcvs.2015.03.045]), Dr. Maryam Naim and colleagues said that CEEG identified electroencephalographic seizures in 8% of newborns after cardiopulmonary bypass surgery. The study, conducted over 18 months, evaluated 172 newborns, none older than 1 month, with 161 (94%) having undergone postoperative CEEG. They had CEEG within 6 hours of their return to the cardiac intensive care unit.

Courtesy of JTCVS/AATS

The study classified electroencephalographic seizures as EEG-only (also termed nonconvulsive seizures, with no observable clinical signs either at bedside or via video) or electroclinical seizures. Dr. Naim and colleagues said the majority of seizures they identified with CEEG would not have been noticed otherwise as they had no clinically obvious signs or symptoms.

The American Clinical Neurophysiology Society (ACNS) recommends that cardiac surgeons consider continuous CEEG monitoring in high-risk neonates with congenital heart disease (CHD) after bypass surgery, but Dr. Naim and coauthors raised the question of whether seizure incidence would justify routine CEEG for all neonates with CHD who’ve had bypass surgery, especially as health systems place greater emphasis on quality improvement programs and cost-effective strategies. The authors said that neonates with all types of congenital heart disease had seizures.

“In adult populations, CEEG has not been shown to significantly increase hospital costs, but cost-effectiveness analyses have not been performed in neonates with CHD,” the authors said.

So they attempted to identify at-risk populations of newborns who would benefit most from routine CEEG monitoring. In a multivariable model that the investigators used, both delayed sternal closure and longer deep hypothermic circulatory arrest (DHCA) during surgery seemed predictive of seizures, but the odds ratios for both were low, “suggesting the statistically significant findings may not be very useful in focusing CEEG implementation on a high-risk group.”

Previous studies have reported that identifying and treating seizures in newborns who have had bypass surgery may reduce secondary brain injury and improve outcomes (Pediatrics 2008;121:e759-67), and the Boston Circulatory Arrest Study showed an association between postoperative seizures and lower reading and math scores and lower cognitive and functional skills later in life (Circulation 2011;124:1361-1369). The authors cited other studies that showed older, critically ill children with “high seizure burdens” have had worse outcomes. (Critical Care Medicine 2013;31:215-23; Neurology 2014;82:396-404; Brain 2014;137:1429-38). They also pointed out increased risk if the seizure is not treated. “While occurrence of a seizure is a marker of brain injury, there may also be secondary injury if the seizure activity is not terminated,” Dr. Naim and coauthors said.

The investigators concluded that postoperative CEEG to identify seizures “is warranted,” and while they found some newborns may be at greater risk of postbypass seizures than others, they advocated for “widespread” monitoring strategies.

Their work also questioned the effectiveness of non-CEEG assessment. In the study, clinicians identified bedside events indicative of seizures – what the study termed “push-button events” – in 32 newborns, or about 18% of patients, but none of the events had an EEG correlate, so they were considered nonepileptic. When the authors looked more closely at those “push-button” events, they found they ranged from abnormal body movement in 14 and hypertension in 7 to tachycardia and abnormal face movements, among other characterizations, in lesser numbers.

“Furthermore, push-button events by bedside clinicians, including abnormal movements and hypertensive episodes concerning for possible seizures, did not have any EEG correlate, indicating that bedside clinical assessment for seizures without CEEG monitoring is unreliable,” Dr. Naim and colleagues said.

As to whether identifying and treating postbypass seizures in young newborns with CHD will improve long-term neurodevelopment in these children, the authors acknowledged that further study is needed.

They reported having no financial disclosures.

In the first report evaluating the impact of a clinical guideline that calls for the use of postoperative continuous electroencephalography (CEEG) on infants after they’ve had cardiopulmonary bypass surgery, investigators at Children’s Hospital of Philadelphia and the University of Pennsylvania validated the clinical utility of routine CEEG monitoring and found that clinical assessment for seizures without CEEG is not a reliable marker for diagnosis and treatment.

In a report online in the Journal of Thoracic and Cardiovascular Surgery (J. Thorac. Cardiovasc. Surg. 2015 [doi:10.1016/j.jtcvs.2015.03.045]), Dr. Maryam Naim and colleagues said that CEEG identified electroencephalographic seizures in 8% of newborns after cardiopulmonary bypass surgery. The study, conducted over 18 months, evaluated 172 newborns, none older than 1 month, with 161 (94%) having undergone postoperative CEEG. They had CEEG within 6 hours of their return to the cardiac intensive care unit.

Courtesy of JTCVS/AATS

The study classified electroencephalographic seizures as EEG-only (also termed nonconvulsive seizures, with no observable clinical signs either at bedside or via video) or electroclinical seizures. Dr. Naim and colleagues said the majority of seizures they identified with CEEG would not have been noticed otherwise as they had no clinically obvious signs or symptoms.

The American Clinical Neurophysiology Society (ACNS) recommends that cardiac surgeons consider continuous CEEG monitoring in high-risk neonates with congenital heart disease (CHD) after bypass surgery, but Dr. Naim and coauthors raised the question of whether seizure incidence would justify routine CEEG for all neonates with CHD who’ve had bypass surgery, especially as health systems place greater emphasis on quality improvement programs and cost-effective strategies. The authors said that neonates with all types of congenital heart disease had seizures.

“In adult populations, CEEG has not been shown to significantly increase hospital costs, but cost-effectiveness analyses have not been performed in neonates with CHD,” the authors said.

So they attempted to identify at-risk populations of newborns who would benefit most from routine CEEG monitoring. In a multivariable model that the investigators used, both delayed sternal closure and longer deep hypothermic circulatory arrest (DHCA) during surgery seemed predictive of seizures, but the odds ratios for both were low, “suggesting the statistically significant findings may not be very useful in focusing CEEG implementation on a high-risk group.”

Previous studies have reported that identifying and treating seizures in newborns who have had bypass surgery may reduce secondary brain injury and improve outcomes (Pediatrics 2008;121:e759-67), and the Boston Circulatory Arrest Study showed an association between postoperative seizures and lower reading and math scores and lower cognitive and functional skills later in life (Circulation 2011;124:1361-1369). The authors cited other studies that showed older, critically ill children with “high seizure burdens” have had worse outcomes. (Critical Care Medicine 2013;31:215-23; Neurology 2014;82:396-404; Brain 2014;137:1429-38). They also pointed out increased risk if the seizure is not treated. “While occurrence of a seizure is a marker of brain injury, there may also be secondary injury if the seizure activity is not terminated,” Dr. Naim and coauthors said.

The investigators concluded that postoperative CEEG to identify seizures “is warranted,” and while they found some newborns may be at greater risk of postbypass seizures than others, they advocated for “widespread” monitoring strategies.

Their work also questioned the effectiveness of non-CEEG assessment. In the study, clinicians identified bedside events indicative of seizures – what the study termed “push-button events” – in 32 newborns, or about 18% of patients, but none of the events had an EEG correlate, so they were considered nonepileptic. When the authors looked more closely at those “push-button” events, they found they ranged from abnormal body movement in 14 and hypertension in 7 to tachycardia and abnormal face movements, among other characterizations, in lesser numbers.

“Furthermore, push-button events by bedside clinicians, including abnormal movements and hypertensive episodes concerning for possible seizures, did not have any EEG correlate, indicating that bedside clinical assessment for seizures without CEEG monitoring is unreliable,” Dr. Naim and colleagues said.

As to whether identifying and treating postbypass seizures in young newborns with CHD will improve long-term neurodevelopment in these children, the authors acknowledged that further study is needed.

They reported having no financial disclosures.

SEATTLE – Autologous stem cell therapy improves surgery outcomes in children with hypoplastic left heart syndrome, according to the results of a small prospective trial from Okayama University in Japan.

The investigators cultured cardiosphere-derived cells (CDCs) – cardiac progenitor cells – from right atrium samples taken during stage 2 or 3 surgical palliations in seven HLHS children. A month later, they injected 300,000 CDCs/kg into the children’s coronary arteries by catheter, with each child getting cells cultured from their own tissue.

The cells seemed to jump-start the intrinsic regenerative properties of very young hearts. At 30 months follow-up, right ventricular mass and ejection fractions were about 10% greater in CDC treated patients compared to seven controls. Treated children also had better growth.

It’s possible the technique could help older children, too, and even adults, said lead investigator Dr. Shunji Sano, professor and chairman of the department of cardiovascular surgery at Okayama. He explained the work, its implications, and the next phase of research in an interview at the annual meeting of the American Association for Thoracic Surgery.

[email protected]

SEATTLE – Autologous stem cell therapy improves surgery outcomes in children with hypoplastic left heart syndrome, according to the results of a small prospective trial from Okayama University in Japan.

The investigators cultured cardiosphere-derived cells (CDCs) – cardiac progenitor cells – from right atrium samples taken during stage 2 or 3 surgical palliations in seven HLHS children. A month later, they injected 300,000 CDCs/kg into the children’s coronary arteries by catheter, with each child getting cells cultured from their own tissue.

The cells seemed to jump-start the intrinsic regenerative properties of very young hearts. At 30 months follow-up, right ventricular mass and ejection fractions were about 10% greater in CDC treated patients compared to seven controls. Treated children also had better growth.

It’s possible the technique could help older children, too, and even adults, said lead investigator Dr. Shunji Sano, professor and chairman of the department of cardiovascular surgery at Okayama. He explained the work, its implications, and the next phase of research in an interview at the annual meeting of the American Association for Thoracic Surgery.

[email protected]

SEATTLE – Autologous stem cell therapy improves surgery outcomes in children with hypoplastic left heart syndrome, according to the results of a small prospective trial from Okayama University in Japan.

The investigators cultured cardiosphere-derived cells (CDCs) – cardiac progenitor cells – from right atrium samples taken during stage 2 or 3 surgical palliations in seven HLHS children. A month later, they injected 300,000 CDCs/kg into the children’s coronary arteries by catheter, with each child getting cells cultured from their own tissue.

The cells seemed to jump-start the intrinsic regenerative properties of very young hearts. At 30 months follow-up, right ventricular mass and ejection fractions were about 10% greater in CDC treated patients compared to seven controls. Treated children also had better growth.

It’s possible the technique could help older children, too, and even adults, said lead investigator Dr. Shunji Sano, professor and chairman of the department of cardiovascular surgery at Okayama. He explained the work, its implications, and the next phase of research in an interview at the annual meeting of the American Association for Thoracic Surgery.

[email protected]

SEATTLE – Sano shunts outperform Blalock-Taussig shunts for Norwood procedures in neonates with hypoplastic left heart syndrome, according to a research registry study of 322 patients at the Cleveland Clinic and elsewhere.

The 166 newborns who had Sano shunts were matched to 166 who had Blalock-Taussig (BT) shunts.

“For comparable neonates with HLHS [hypoplastic left heart syndrome] undergoing Norwood operations, Sano offers better late survival [more than 3 years], less late tricuspid regurgitation, and perhaps less late right ventricular dysfunction than BT,” the investigators concluded.

Even so, Dr. Richard Ohye, professor of cardiac surgery at the University of Michigan, Ann Arbor, said the jury is still out on which shunt is best. He explained why in an interview at the American Association for Thoracic Surgery annual meeting. He also shared tips on shunt selection and explained a novel technique he has developed for doing a Sano shunt with a smaller hole in the right ventricle.

[email protected]

SEATTLE – Sano shunts outperform Blalock-Taussig shunts for Norwood procedures in neonates with hypoplastic left heart syndrome, according to a research registry study of 322 patients at the Cleveland Clinic and elsewhere.

The 166 newborns who had Sano shunts were matched to 166 who had Blalock-Taussig (BT) shunts.

“For comparable neonates with HLHS [hypoplastic left heart syndrome] undergoing Norwood operations, Sano offers better late survival [more than 3 years], less late tricuspid regurgitation, and perhaps less late right ventricular dysfunction than BT,” the investigators concluded.

Even so, Dr. Richard Ohye, professor of cardiac surgery at the University of Michigan, Ann Arbor, said the jury is still out on which shunt is best. He explained why in an interview at the American Association for Thoracic Surgery annual meeting. He also shared tips on shunt selection and explained a novel technique he has developed for doing a Sano shunt with a smaller hole in the right ventricle.

[email protected]

SEATTLE – Sano shunts outperform Blalock-Taussig shunts for Norwood procedures in neonates with hypoplastic left heart syndrome, according to a research registry study of 322 patients at the Cleveland Clinic and elsewhere.

The 166 newborns who had Sano shunts were matched to 166 who had Blalock-Taussig (BT) shunts.

“For comparable neonates with HLHS [hypoplastic left heart syndrome] undergoing Norwood operations, Sano offers better late survival [more than 3 years], less late tricuspid regurgitation, and perhaps less late right ventricular dysfunction than BT,” the investigators concluded.

Even so, Dr. Richard Ohye, professor of cardiac surgery at the University of Michigan, Ann Arbor, said the jury is still out on which shunt is best. He explained why in an interview at the American Association for Thoracic Surgery annual meeting. He also shared tips on shunt selection and explained a novel technique he has developed for doing a Sano shunt with a smaller hole in the right ventricle.

[email protected]