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Results of transvenous occlusion of secundum atrial septal defects with the fourth generation buttoned device: comparison with first, second, and third generation devices
Congenital Heart Disease
Cognitive Development After the Fontan Operation
Abstracts
Wernovsky G, Tiles KM, Gauvreau K et al. Circulation 2000;102: 883-889.
rotation of the gut. The authors of this study wished to characterize long-term outcomes and factors associated with mortality in patients with LAI. Methods: Single-center, retrospective review of 163 patients with LAI born between 1970 and 1998 with Kaplan-Meier survival estimates and multivariate analysis of independent factors associated with time-related mortality. Results: One hundred sixty-three patients were identified (0.4% of new diagnoses of congenital heart disease). Significant cardiac anomalies occurred in 87% with extracardiac conditions diagnosed in 36% of patients. 18% patients (4/22) with otherwise normal hearts died of complications of extracardiac anomalies. Of 71 patients with cardiac anomalies suitable for biventricular repair, 62 (87%) underwent operation with survival of 80% at one year, 71% at 5 years, 66% at 10 years, and 63% after 15 years. Of 70 patients with cardiac defects suitable for single ventricle palliation, 47 (67%) had surgery with survival of 73% at one year, 61% at 4 years, 53% at 10 years and 48% after 15 years (p ⬍ 0.001 vs. biventricular repair). Factors associated with mortality were congenital AV block (80% mortality), unbalanced single ventricle defects, coarctation of the aorta, biliary atresia, and other gastrointestinal malformations. Conclusions: Both cardiac (single-ventricle anatomy, AV block and coarctation of the aorta) and extracardiac (biliary atresia and gastrointestinal malformations) are associated with lower survival in patients with LAI. Perspective: This is the largest report to date of patients with the complex spectrum of anomalies associated with LAI in the modern operative era and documents fairly good long-term outcomes. Not surprisingly, patients with single ventricle palliation fared less well than those with biventricular repair. JK
Study Question: Congenital heart disease patients with single ventricle physiology are at highest risk for cognitive impairment presumably due to profound cyanosis in the neonatal period and the need for multiple surgical procedures requiring cardiopulmonary bypass or deep hypothermic circulatory arrest. In this study, the investigators quantitatively assessed cognitive development and achievement in these patients. Methods: 133 patients, mean age 11.1 years, underwent age-appropriate standardized IQ and achievement tests at a mean of 6.0 yrs after Fontan procedures performed from 1973-1991. Results: Full-scale, verbal, and performance IQ scores were 95.7 ⫾ 17.4, 96.6⫾ 18.1, and 95.6 ⫾ 16.4, respectively. All scores were significantly lower than the normal mean score of 100. Ten patients (7.8%) had full-scale IQ scores ⬍70, ⬎2 SD below the normal mean. Patients also scored significantly lower on achievement tests with a mean composite score of 91.6 ⫾ 15.4 vs. a normal mean of 100 (p ⬍ 0.001). 10% of patients scored ⬎2 SD below the mean. After adjustment for socioeconomic status, predictors of low IQ and/or achievement scores were diagnoses of “complex” congenital lesions including hypoplastic left heart syndrome, prior placement of a pulmonary artery “band,” and prior use of circulatory arrest before the Fontan procedure. Conclusions: Most patients with single ventricle palliated by the Fontan procedure have IQ and achievement scores within the normal range. However, overall performance is significantly lower than the general population. Perspective: This study provides significant hope for children with complex cyanotic congenital heart disease and their families. Although overall IQ scores were approximately 4 points lower than for the general population, they were well within the normal range. Surgical and anesthesia techniques and patient selection for the Fontan procedure have changed significantly since the patients in this study were operated on, lending further hope for normal cognitive development for these children. JK
Results of Transvenous Occlusion of Secundum Atrial Septal Defects With the Fourth Generation Buttoned Device: Comparison With First, Second, and Third Generation Devices Rao PS, Berger F, Rey C, et al. J Am Coll Cardiol 2000;36:58392.
Outcomes of Left Atrial Isomerism Over a 28-Year Period at a Single Institution
Study Question: 1) Does the addition of a second spring “button” to the current ASD single button occluder design prevent detachment of the occluder component from the counter-occluder (“unbuttoning”)? 2) What is the effectiveness of the ASD button occluder with this design modification as compared with prior generation button devices? Methods: Retrospective observational multicenter study of 423 patients who underwent percutaneous closure of simple secundum ASD with stretched ASD diameter ⬍30 mm and Qp/Qs ⬎ 1.5 with a fourth generation button occluder. Results were compared to 180 similar patients who under-
Gilljam T, McCrindle BW, Smallhorn JF, et al. J Am Coll Cardiol 2000;36:908 –916. Study Question: Left atrial isomerism (LAI), previously labeled polysplenia syndrome, constitutes a complex spectrum of cardiac and extracardiac anomalies. The most common identifying features are isomeric left atrial appendages, polysplenia, bilateral hyparterial bronchi, bilateral bilobed lungs, interrupted IVC, extrahepatic biliary atresia and mal-
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went ASD closure with first, second or third generation button devices. Results: The fourth generation device was successfully deployed in 417/423 patients vs. 166/180 patients with earlier design devices (p ⫽ NS). “Unbuttoning” occurred in 4/423 patients (0.9%) compared with 13/180 patients (7.2%) who received earlier generation devices (p ⬍ 0.001). 90% of the patients with the double button occluder had no or trivial residual interatrial shunt at 24 hours by echocardiography as compared to 92% of patients with the single button device (p ⫽ NS). Reintervention to close residual ASD was required in 5% of patients with fourth generation devices at 23 ⫾ 15 months of follow-up vs. 14/166 (8%) patients with older devices at 46 ⫾ 20 months (p ⬍0.02). The actuarial reintervention-free rates at 1, 2 and 5 years were not different for the two designs of ASD occluder. Conclusions: Significantly less unbuttoning occurred with fourth generation (double button) ASD occluding devices than with earlier button occluders. Overall efficacy in closure of ASD’s with this device was comparable to earlier button-type ASD closure devices. Perspective: At this time, the FDA has not approved any of the available devices for percutaneous closure of ASDs. This fourth generation button device is added to a growing number of such devices that have been demonstrated to safely and effectively close simple secundum ASDs percutaneously. In this study, only simple secundum ASDs with Qp/Qs ⬎ 1.5 were closed. Whether these devices will be as effective in larger and/or more complicated ASDs has yet to be determined. JK
ischemia. Of these 12 children, seven died and five underwent heart transplantation. In this small group, there were no statistically significant predictors of sudden death. Conclusions: Myocardial ischemia is common in children with RCM. Although not a predictor of mode of death in this series, ischemia is associated with a very poor prognosis. Syncope may be an ominous sign in pediatric patients with RCM. Perspective: RCM is a rare but lethal disease in the pediatric population with a very brief time between diagnosis and death. Symptoms or signs of myocardial ischemia or syncope warrant rapid evaluation and consideration for cardiac transplantation. The role of medical therapy in these children is unknown. JK
A Comparison of Ibuprofen and Indomethacin for Closure of Patent Ductus Arteriosus Van Overmeire B, Smets K, Lecoutere D, et al. N Eng J Med 2000;343:674 – 681. Study Question: In animals, parenteral ibuprofen has been shown to have equivalent efficacy to indomethacin for closure of patent ductus arteriosus (PDA) without compromise of cerebral, gastrointestinal, or renal perfusion. The investigators asked: What is the efficacy and safety of ibuprofen compared to indomethacin for PDA closure in premature infants with respiratory distress syndrome (RDS)? Methods: Non-blinded, randomized, multicenter trial of ibuprofen vs. indomethacin for PDA closure in 148 premature infants (24 to 32 weeks gestational age) with RDS (and no major congenital abnormalities) in five neonatal ICUs. Ductal patency was assessed using echocardiography by readers blinded to treatment assignment. Indomethacin was given as rescue therapy to infants who failed the first randomized treatment. Results: The rate of PDA closure was similar in both groups (66% with indomethacin vs. 70% with ibuprofen, p ⫽ NS). Survival at one month was not different between the treatment groups. Oliguria occurring during treatment was more frequent (18.9 % vs. 6.8%, p ⫽ 0.03) and increase in serum creatinine greater in the indomethacin-treated infants. The development of necrotizing enterocolitis and intraventricular hemorrhage were not different between treatment groups, but the numbers of these complications were small. Conclusions: Parenteral ibuprofen has similar efficacy to indomethacin for closure of PDA in premature infants with a lower risk of developing renal dysfunction during treatment. Long-term effects of ibuprofen on the gastrointestinal and central nervous systems are unknown. Perspective: 70% of infants born at ⬍28 weeks gestational age have PDA that require closure. Therefore, this pilot study demonstrates potential benefit for a large number of infants. Unfortunately, as pointed out in the accompanying editorial, parenteral ibuprofen is currently not available in the United States for clinical trials or treatment. JK
Sudden Death and Cardiovascular Collapse in Children With Restrictive Cardiomyopathy Rivenes SM, Kearney DL, Smith EO, et al. Circulation 2000;102: 876 – 882. Study Question: The purpose of this study was to evaluate potential risk factors for sudden cardiac death (SCD) in children with restrictive cardiomyopathy (RCM). Methods: Retrospective review of all pediatric patients diagnosed with restrictive cardiomyopathy at Texas Children’s Hospital over a 31-year period. Holter monitors, electrocardiograms and pathologic specimens were evaluated for evidence of acute or chronic myocardial ischemia. Results: Seventeen children with RCM, ranging from 0.7 to 12.2 years old, had records available for review. Five died suddenly on average 1.0 ⫾ 0.6 years after initial diagnosis. Four of 5 deaths occurred within 6 months of diagnosis. Three of five patients presented with syncope, one with chest pain and one with a heart murmur. All five patients had evidence of acute ischemia with marked (5.4 to 12.7 mm) ST depression on both initial ECG and Holter monitors. None had congestive heart failure. Nine of 12 patients without SCD had congestive heart failure. None had syncope. Evidence of ischemia was also common in this group with 8/13 ECGs with definite ischemia and two with bundle-branch block precluding a diagnosis of
ACC CURRENT JOURNAL REVIEW Jan/Feb 2001
83
Cognitive Development After the Fontan Operation
Abstracts
Wernovsky G, Tiles KM, Gauvreau K et al. Circulation 2000;102: 883-889.
rotation of the gut. The authors of this study wished to characterize long-term outcomes and factors associated with mortality in patients with LAI. Methods: Single-center, retrospective review of 163 patients with LAI born between 1970 and 1998 with Kaplan-Meier survival estimates and multivariate analysis of independent factors associated with time-related mortality. Results: One hundred sixty-three patients were identified (0.4% of new diagnoses of congenital heart disease). Significant cardiac anomalies occurred in 87% with extracardiac conditions diagnosed in 36% of patients. 18% patients (4/22) with otherwise normal hearts died of complications of extracardiac anomalies. Of 71 patients with cardiac anomalies suitable for biventricular repair, 62 (87%) underwent operation with survival of 80% at one year, 71% at 5 years, 66% at 10 years, and 63% after 15 years. Of 70 patients with cardiac defects suitable for single ventricle palliation, 47 (67%) had surgery with survival of 73% at one year, 61% at 4 years, 53% at 10 years and 48% after 15 years (p ⬍ 0.001 vs. biventricular repair). Factors associated with mortality were congenital AV block (80% mortality), unbalanced single ventricle defects, coarctation of the aorta, biliary atresia, and other gastrointestinal malformations. Conclusions: Both cardiac (single-ventricle anatomy, AV block and coarctation of the aorta) and extracardiac (biliary atresia and gastrointestinal malformations) are associated with lower survival in patients with LAI. Perspective: This is the largest report to date of patients with the complex spectrum of anomalies associated with LAI in the modern operative era and documents fairly good long-term outcomes. Not surprisingly, patients with single ventricle palliation fared less well than those with biventricular repair. JK
Study Question: Congenital heart disease patients with single ventricle physiology are at highest risk for cognitive impairment presumably due to profound cyanosis in the neonatal period and the need for multiple surgical procedures requiring cardiopulmonary bypass or deep hypothermic circulatory arrest. In this study, the investigators quantitatively assessed cognitive development and achievement in these patients. Methods: 133 patients, mean age 11.1 years, underwent age-appropriate standardized IQ and achievement tests at a mean of 6.0 yrs after Fontan procedures performed from 1973-1991. Results: Full-scale, verbal, and performance IQ scores were 95.7 ⫾ 17.4, 96.6⫾ 18.1, and 95.6 ⫾ 16.4, respectively. All scores were significantly lower than the normal mean score of 100. Ten patients (7.8%) had full-scale IQ scores ⬍70, ⬎2 SD below the normal mean. Patients also scored significantly lower on achievement tests with a mean composite score of 91.6 ⫾ 15.4 vs. a normal mean of 100 (p ⬍ 0.001). 10% of patients scored ⬎2 SD below the mean. After adjustment for socioeconomic status, predictors of low IQ and/or achievement scores were diagnoses of “complex” congenital lesions including hypoplastic left heart syndrome, prior placement of a pulmonary artery “band,” and prior use of circulatory arrest before the Fontan procedure. Conclusions: Most patients with single ventricle palliated by the Fontan procedure have IQ and achievement scores within the normal range. However, overall performance is significantly lower than the general population. Perspective: This study provides significant hope for children with complex cyanotic congenital heart disease and their families. Although overall IQ scores were approximately 4 points lower than for the general population, they were well within the normal range. Surgical and anesthesia techniques and patient selection for the Fontan procedure have changed significantly since the patients in this study were operated on, lending further hope for normal cognitive development for these children. JK
Results of Transvenous Occlusion of Secundum Atrial Septal Defects With the Fourth Generation Buttoned Device: Comparison With First, Second, and Third Generation Devices Rao PS, Berger F, Rey C, et al. J Am Coll Cardiol 2000;36:58392.
Outcomes of Left Atrial Isomerism Over a 28-Year Period at a Single Institution
Study Question: 1) Does the addition of a second spring “button” to the current ASD single button occluder design prevent detachment of the occluder component from the counter-occluder (“unbuttoning”)? 2) What is the effectiveness of the ASD button occluder with this design modification as compared with prior generation button devices? Methods: Retrospective observational multicenter study of 423 patients who underwent percutaneous closure of simple secundum ASD with stretched ASD diameter ⬍30 mm and Qp/Qs ⬎ 1.5 with a fourth generation button occluder. Results were compared to 180 similar patients who under-
Gilljam T, McCrindle BW, Smallhorn JF, et al. J Am Coll Cardiol 2000;36:908 –916. Study Question: Left atrial isomerism (LAI), previously labeled polysplenia syndrome, constitutes a complex spectrum of cardiac and extracardiac anomalies. The most common identifying features are isomeric left atrial appendages, polysplenia, bilateral hyparterial bronchi, bilateral bilobed lungs, interrupted IVC, extrahepatic biliary atresia and mal-
ACC CURRENT JOURNAL REVIEW Jan/Feb 2001
82
went ASD closure with first, second or third generation button devices. Results: The fourth generation device was successfully deployed in 417/423 patients vs. 166/180 patients with earlier design devices (p ⫽ NS). “Unbuttoning” occurred in 4/423 patients (0.9%) compared with 13/180 patients (7.2%) who received earlier generation devices (p ⬍ 0.001). 90% of the patients with the double button occluder had no or trivial residual interatrial shunt at 24 hours by echocardiography as compared to 92% of patients with the single button device (p ⫽ NS). Reintervention to close residual ASD was required in 5% of patients with fourth generation devices at 23 ⫾ 15 months of follow-up vs. 14/166 (8%) patients with older devices at 46 ⫾ 20 months (p ⬍0.02). The actuarial reintervention-free rates at 1, 2 and 5 years were not different for the two designs of ASD occluder. Conclusions: Significantly less unbuttoning occurred with fourth generation (double button) ASD occluding devices than with earlier button occluders. Overall efficacy in closure of ASD’s with this device was comparable to earlier button-type ASD closure devices. Perspective: At this time, the FDA has not approved any of the available devices for percutaneous closure of ASDs. This fourth generation button device is added to a growing number of such devices that have been demonstrated to safely and effectively close simple secundum ASDs percutaneously. In this study, only simple secundum ASDs with Qp/Qs ⬎ 1.5 were closed. Whether these devices will be as effective in larger and/or more complicated ASDs has yet to be determined. JK
ischemia. Of these 12 children, seven died and five underwent heart transplantation. In this small group, there were no statistically significant predictors of sudden death. Conclusions: Myocardial ischemia is common in children with RCM. Although not a predictor of mode of death in this series, ischemia is associated with a very poor prognosis. Syncope may be an ominous sign in pediatric patients with RCM. Perspective: RCM is a rare but lethal disease in the pediatric population with a very brief time between diagnosis and death. Symptoms or signs of myocardial ischemia or syncope warrant rapid evaluation and consideration for cardiac transplantation. The role of medical therapy in these children is unknown. JK
A Comparison of Ibuprofen and Indomethacin for Closure of Patent Ductus Arteriosus Van Overmeire B, Smets K, Lecoutere D, et al. N Eng J Med 2000;343:674 – 681. Study Question: In animals, parenteral ibuprofen has been shown to have equivalent efficacy to indomethacin for closure of patent ductus arteriosus (PDA) without compromise of cerebral, gastrointestinal, or renal perfusion. The investigators asked: What is the efficacy and safety of ibuprofen compared to indomethacin for PDA closure in premature infants with respiratory distress syndrome (RDS)? Methods: Non-blinded, randomized, multicenter trial of ibuprofen vs. indomethacin for PDA closure in 148 premature infants (24 to 32 weeks gestational age) with RDS (and no major congenital abnormalities) in five neonatal ICUs. Ductal patency was assessed using echocardiography by readers blinded to treatment assignment. Indomethacin was given as rescue therapy to infants who failed the first randomized treatment. Results: The rate of PDA closure was similar in both groups (66% with indomethacin vs. 70% with ibuprofen, p ⫽ NS). Survival at one month was not different between the treatment groups. Oliguria occurring during treatment was more frequent (18.9 % vs. 6.8%, p ⫽ 0.03) and increase in serum creatinine greater in the indomethacin-treated infants. The development of necrotizing enterocolitis and intraventricular hemorrhage were not different between treatment groups, but the numbers of these complications were small. Conclusions: Parenteral ibuprofen has similar efficacy to indomethacin for closure of PDA in premature infants with a lower risk of developing renal dysfunction during treatment. Long-term effects of ibuprofen on the gastrointestinal and central nervous systems are unknown. Perspective: 70% of infants born at ⬍28 weeks gestational age have PDA that require closure. Therefore, this pilot study demonstrates potential benefit for a large number of infants. Unfortunately, as pointed out in the accompanying editorial, parenteral ibuprofen is currently not available in the United States for clinical trials or treatment. JK
Sudden Death and Cardiovascular Collapse in Children With Restrictive Cardiomyopathy Rivenes SM, Kearney DL, Smith EO, et al. Circulation 2000;102: 876 – 882. Study Question: The purpose of this study was to evaluate potential risk factors for sudden cardiac death (SCD) in children with restrictive cardiomyopathy (RCM). Methods: Retrospective review of all pediatric patients diagnosed with restrictive cardiomyopathy at Texas Children’s Hospital over a 31-year period. Holter monitors, electrocardiograms and pathologic specimens were evaluated for evidence of acute or chronic myocardial ischemia. Results: Seventeen children with RCM, ranging from 0.7 to 12.2 years old, had records available for review. Five died suddenly on average 1.0 ⫾ 0.6 years after initial diagnosis. Four of 5 deaths occurred within 6 months of diagnosis. Three of five patients presented with syncope, one with chest pain and one with a heart murmur. All five patients had evidence of acute ischemia with marked (5.4 to 12.7 mm) ST depression on both initial ECG and Holter monitors. None had congestive heart failure. Nine of 12 patients without SCD had congestive heart failure. None had syncope. Evidence of ischemia was also common in this group with 8/13 ECGs with definite ischemia and two with bundle-branch block precluding a diagnosis of
ACC CURRENT JOURNAL REVIEW Jan/Feb 2001
83