Faculty Bibliography

OBJECTIVE: The management of patients with multiple ventricular septal defects remains controversial. Primary closure, interventional catheter techniques, and palliative surgery all may have a role, and specific management guidelines remain undefined. METHODS: We reviewed the records of all 33 patients with multiple ventricular septal defects undergoing repair between January 1988 and October 1996. Pulmonary artery hypertension was present in 21 patients (group 1), and pulmonary stenosis was present in the remaining 12 (group 2). Closure was accomplished from a right atriotomy alone in most patients, although an apical left ventriculotomy was used for apical defects. Among group 1 patients, the mean age at repair was 5.9 +/- 0.9 months. Major associated anomalies included coarctation (n = 6), straddling tricuspid valve (n = 1), and critical aortic stenosis (n = 1). Reoperation was performed in two patients for residual ventricular septal defects. Among group 2 patients, the mean age at repair was 6.6 +/- 3.2 years. Major associated anomalies included tetralogy of Fallot (n = 2), pulmonary stenosis (n = 4), double-outlet right ventricle with hypoplastic left ventricle (n = 1), and isolated left ventricular hypoplasia (n = 1). Three required reoperation for residual ventricular septal defect. RESULTS: There were no early or late deaths, no episodes of heart block, and no significant residual ventricular septal defects among group 1 patients. All group 1 patients remain free of significant residual cardiovascular conditions at a mean of 23.4 +/- 5.1 months. Among group 2 patients, there was one early death in a patient with double-outlet right ventricle and left ventricular hypoplasia. Complete heart block occurred in two patients and one required late mitral valve replacement. There were no late deaths, seven remain alive without significant residual defects at a mean of 36.2 +/- 8.0 months, and two required transplantation for left ventricular failure. CONCLUSIONS: Primary repair for infants with multiple ventricular septal defects is associated with good late outcomes. The right atrial approach is satisfactory for most muscular defects, although limited apical left ventriculotomy was used for apical defects. Pulmonary artery banding should be limited to patients with complex associated defects

OBJECTIVE: Coronary physiology in infants with congenital heart disease remains unclear. Our objective was to better understand coronary physiology in infants with congenital heart disease. METHODS: We used positron emission tomography with nitrogen 13-labeled ammonia to measure myocardial perfusion at rest and with adenosine (142 micrograms/kg/min x 6 minutes) in five infants after anatomic repair of a congenital heart lesion (group I), and in five infants after Norwood palliation for hypoplastic left heart syndrome (group II). The groups were matched for age, weight, and time from the operation. RESULTS: Resting coronary flow in the left ventricle in group I was 1.8 +/- 0.2 ml/min/gm; resting flow in the right ventricle in group II was 1.0 +/- 0.3 ml/min/gm (p = 0.003). Coronary flow with adenosine was 2.6 +/- 0.5 ml/min/gm in group I and 1.5 +/- 0.7 ml/min/gm in group II (p = 0.02). Absolute coronary flow reserve was the same in both groups (1.5 +/- 0.2 in group I vs 1.6 +/- 0.3 in group II, p = 0.45). Oxygen delivery was reduced in group II compared with group I at rest (16.1 +/- 4.2 ml/min/100 gm vs 28.9 +/- 4.42 ml/min/100 gm, p = 0.02) and with adenosine (25.5 +/- 8.1 ml/min/100 gm vs 42.3 +/- 5.8 ml/min/100 gm, p = 0.02). CONCLUSIONS: Infants with repaired heart disease have higher resting flow and less coronary flow reserve than previously reported for adults. After Norwood palliation, infants have less perfusion and oxygen delivery to the systemic ventricle than do infants with a repaired lesion. This may in part explain why the outcome for patients with Norwood palliation is less favorable than for others

Background. Many centers have adopted balloon valvuloplasty for treatment of infants with critical aortic stenosis because of historically poor early results and a lack of long-term results with surgical valvotomy. We evaluated our results with open aortic valvotomy over the past decade, specifically examining factors influencing survival and reintervention in the current era. Methods. From 1986 to 1996, 37 infants in the first 3 months of life underwent open aortic valvotomy for critical aortic stenosis. All patients underwent cardiopulmonary bypass, valvotomy, and valve debridement under direct vision with standard techniques. Results. Early mortality was 11% (4 of 37, 70% confidence limit 7% to 20%) and all early deaths were in neonates less than 2 weeks of age. Late death occurred in 6 patients a mean of 10 +/- 12 months (range, 2 to 36 months) after valvotomy. Actuarial survival, including operative deaths was 92% +/- 6% at 1 month, 78% +/- 9% at 1 year, and 73.4% +/- 10% at 10 years. In a multifactorial regression analysis, the best predictors of death were the presence of endocardial fibroelastosis and small body surface area and the best predictor of the need for late reintervention was preoperative aortic annular size. Thirteen patients required reintervention: repeat operation in 7 patients, balloon valvuloplasty in 3 patients, and both balloon valvuloplasty and reoperation in 3 patients. Actuarial freedom from reintervention postoperatively is 97% +/- 3% at 1 month, 73% +/- 9% at 1 year, and 55% +/- 11% at 10 years. Reintervention was for recurrent left ventricular outflow obstruction in 9 patients and mixed aortic stenosis and aortic insufficiency in 4. Echocardiography 4.3 +/- 2.5 years after aortic valvotomy in survivors who have not required reintervention (n = 20) revealed a Doppler peak instantaneous systolic gradient of 37 +/- 14 mm Hg and mild or less aortic regurgitation in 16 patients and moderate aortic regurgitation in 4 patients. Conclusions. Current surgical results with critical aortic stenosis in the neonate and young infant are acceptable in terms of both late survival, reintervention, and functional results in the majority of patients. Newer interventions, such as balloon valvuloplasty, should be carefully evaluated for long-term results and should be compared more appropriately to current surgical results to determine the best treatment modality for the neonate and infant with critical aortic stenosis

BACKGROUND: The purpose of this study was to determine the incidence of moderate to severe tricuspid valve regurgitation in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction, to assess the mechanisms responsible, and to evaluate the efficacy of surgical repair. METHODS AND RESULTS: We retrospectively reviewed clinical and echocardiographic data of 59 consecutive patients with HLHS operated on at our institution. Patients with a moderate or severe degree of tricuspid regurgitation (> or =2+) demonstrated by color flow Doppler echocardiography before the hemi-Fontan or Fontan operation who underwent tricuspid valve repair were included. Patients with HLHS and coexisting atrioventricular septal defect were excluded from the study. Severity of insufficiency was graded on a scale from 1 to 4+ by Doppler color flow mapping. Tricuspid valve morphology, degree of tricuspid valve regurgitation, and right ventricular function were assessed before and after tricuspid valvuloplasty. Eight patients (8/59; 14%) were found to have a minimum of 2+ tricuspid valve regurgitation before hemi-Fontan or Fontan operations. In five of eight patients (62%; Group A), the valve was found to be myxomatous, thickened, and redundant. There were multiple regurgitant jets in three of five (60%) patients in this group. In three of eight patients (37%; Group B), the valve had a normal echocardiographic appearance and regurgitation was the result of a lack of complete leaflet coaptation. All eight patients underwent tricuspid valvuloplasty, and all experienced a decrease in regurgitation. In five of eight (62%) patients, there was a reduction in insufficiency of two grades of severity and in three of eight (37%) patients, there was a one-grade improvement. Tricuspid valve stenosis was not documented in any of the patients after tricuspid valvuloplasty. In seven of eight (87%) patients, right ventricular function was assessed as fair before tricuspid valvuloplasty repair and improved to good in five of seven (71%) patients after the procedure. The remaining patient had good function both at baseline and after tricuspid valvuloplasty. CONCLUSIONS: Moderate to severe tricuspid valve regurgitation is a common finding in patients with HLHS undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation. Tricuspid valvuloplasty during either the hemi-Fontan or Fontan stages of reconstruction carries a high success rate and is associated with improved right ventricular function

BACKGROUND: Patients with univentricular hearts and ventriculoarterial discordance with potentially obstructed systemic blood flow continue to pose difficult management problems. The goals of neonatal palliative operations are to control pulmonary blood flow while avoiding pulmonary artery distortion, to relieve systemic outflow tract obstruction, and to avoid heart block. METHODS: Between January 1987 and December 1996, 38 patients with either tricuspid atresia or a double-inlet left ventricle and ventriculoarterial discordance underwent a modified Norwood procedure. Their mean age was 15 days, and their mean weight was 3.4 kg. Aortic arch anomalies were present in 92% of the patients. Morbidity and mortality statistics, intraoperative data, and postoperative echocardiograms were reviewed. RESULTS: There were 3 early deaths (7.8%) and 5 late deaths (13.1%). The actuarial survival rates at 1 month, 1 year, and 5 years were 89%, 82%, and 71%, respectively. Follow-up was complete in all children at a mean interval of 30 +/- 9 months. None of the patients had significant neoaortic valve insufficiency, and 1 patient required therapy for residual aortic arch obstruction. Nine patients (30% of the survivors) have undergone the hemi-Fontan procedure, and 18 patients (60%) successfully have undergone the Fontan procedure. CONCLUSIONS: In this patient population, we recommend the modified Norwood procedure as the neonatal palliative treatment of choice. It can be performed with acceptable early morbidity and mortality, and it improves suitability for the Fontan procedure. It reliably relieves all levels of systemic outflow tract obstruction, controls pulmonary blood flow, and avoids heart block

BACKGROUND: Aortic valve replacement with a pulmonary autograft (Ross procedure) is being applied more commonly in children. Although indications for this procedure have been expanded, the presence of a dilated aortic annulus has remained a relative contraindication. In this condition, the use of an undersized autograft in an enlarged aortic annulus may result in significant aortic regurgitation. METHODS: Among 68 children and young adults undergoing the Ross procedure, 15 (age range, 8 to 24 years) with severe aortic regurgitation or stenosis and an aortic annulus diameter that was at least 2 mm larger than the pulmonary annulus had aortic root tailoring. In this group, the diameter of the aortic annulus measured 26.6 +/- 1.3 mm (mean +/- standard error of the mean), whereas that of the pulmonary annulus was 22 +/- 0.9 mm. The mean annular difference was 4.6 +/- 0.7 mm (range, 2 to 12 mm). The aortic annulus was reduced by excising a triangular wedge of tissue posteriorly from the aortic valve annulus at the level of the commissure between the left and noncoronary cusps extending into the anterior leaflet of the mitral valve. The edges were reapproximated over a calibrated dilator to adjust the final size of the aortic annulus to 2 mm smaller than that of the pulmonary autograft. Circumferential felt strips were not used in any patient. RESULTS: All patients survived and morbidity was limited to one reoperation for bleeding. Doppler echocardiographic examination performed at discharge demonstrated that no patient had more than trace to 1+ aortic regurgitation and none had evidence of aortic stenosis. Over a mean follow-up period of 6.3 +/- 1.5 months (range, 1 to 16 months) there has been no late morbidity or mortality and no progression of aortic regurgitation. CONCLUSIONS: Aortic root tailoring further extends the use of the Ross procedure to patients with excessive aortic annular dilation while maintaining the potential for growth, which is particularly important in the pediatric population

INTRODUCTION: Transcatheter radiofrequency ablation is an effective, safe treatment of arrhythmias in children. However, despite technical advances, patients with obstructed venous access to the heart have not been candidates for this treatment. METHODS AND RESULTS: Two children (8.2 and 10.9 kg) with complex congenital heart disease, supraventricular tachycardia, and obstructed venous access underwent successful radiofrequency ablation of either a right (one patient) or left (one patient) accessory pathway using the transhepatic route to the heart. CONCLUSION: This experience underscores the usefulness of the transhepatic approach in small patients, in patients with limited venous access, and in patients with complex congenital heart disease

A retrospective review of hospital charges was performed in children > 1 year old with native coarctation of the aorta who underwent balloon angioplasty, primary surgical repair, or elective surgical repair after unsuccessful balloon angioplasty. Hospital charges were less overall in the balloon angioplasty group, although the failure rate was higher

We performed a prospective, randomized, placebo-controlled, double-blind trial to assess the efficacy of aprotinin in 61 children (median age 3.7 yr) undergoing reoperative open heart surgery (OHS). Three demographically similar groups were studied: large-dose aprotinin (ALD), small-dose aprotinin (ASD), and placebo (P). Over the first 24 postoperative hours fewer patients in the aprotinin groups received packed red cells (ALD, 53%; ASD, 89%; and P, 95%; P = 0.001), platelets (ALD, 32%; ASD, 50%; and P, 65%; P = 0.04), and fresh frozen plasma (ALD, 16%; ASD, 17%; and P, 60%; P = 0.003) than placebo patients. Most importantly, aprotinin patients had fewer exposures to banked blood components (ALD, median 1 U; and ASD, median 2 U) than P (median 6 U; P = 0.001), with no difference in overall complication rate. Use of aprotinin was associated with a savings in the patient charges for blood components, operating room time, and duration of hospitalization. In conclusion, aprotinin decreased the number of units of banked blood components used during the first 24 postoperative hours in reoperative pediatric OHS. Aprotinin thus decreases the risks associated with exposure to banked blood components and reduces hospital charges

BACKGROUND: The use of the extracorporeal membrane oxygenator (ECMO) for postoperative cardiac patients has not resulted in the same high success rate as when ECMO is used for neonates with pulmonary hypertension or pulmonary failure. The reason for this is poorly understood. METHODS AND RESULTS: We analyzed retrospectively all pediatric patients placed on ECMO after surgery for a congenital heart lesion between 1981 and 1995 (n = 64). Patients had a two-ventricular repair (A) or pulmonary blood flow supplied by an aortopulmonary shunt (B) or by a cavopulmonary connection (C). Indication for ECMO was unsatisfactory hemodynamics due to (1) ventricular dysfunction, (2) pulmonary failure, (3) pulmonary hypertension, or (4) a combination or (5) for unclear reasons. Hospital survival was related to these and other factors. Overall hospital survival was 33%; 42% of group A patients survived to discharge, whereas only 25% and 17% survived in groups B and C, respectively. Survival was unrelated to the indication for ECMO but appeared to be lower when ECMO was initiated in the operating room or > 50 hours after surgery. Except for one patient with pneumonia, no patient survived who was on ECMO for > 208 hours. ECMO discontinuation in nonsurvivors was due to neurological (30%) or multiple complications (39%), the lack of return of cardiac function (12%), or other reasons (15%). CONCLUSIONS: This review suggests that the diagnosis of single ventricle, initiation of ECMO in the operating room or > 50 hours after surgery, and ECMO for > 208 hours are associated with patient nonsurvival. Noncardiac complications more frequently led to discontinuation of ECMO than did failure of the return of cardiac function