Pressure-regulated volume control vs volume control ventilation in infants after surgery for congenital heart disease.

The objective of this investigation was to compare how two modes of positive pressure ventilation affect cardiac output, airway pressures, oxygenation, and carbon dioxide removal in children with congenital heart disease in the immediate postoperative period. The investigation used a one group pretest-post-test study design and was performed in the pediatric cardiac intensive care unit in a university-affiliated children's hospital. Nine infants were enrolled immediately after repair of tetralogy of Fallot (2) or atrioventricular septal defects (7) with mean weight = 5.5 kg (4.2-7.3 kg). Children were admitted to the pediatric cardiothoracic intensive care unit after complete surgical repair of their cardiac defect and stabilized on a Siemen's Servo 300 ventilator in volume control mode (VCV1) (volume-targeted ventilation with a square flow wave pattern). Tidal volume was set at 15 cc/kg (total). Hemodynamic parameters, airway pressures and ventilator settings, and an arterial blood gas were measured. Patients were then changed to pressure-regulated volume control mode (PRVC) (volume-targeted ventilation with decelerating flow wave pattern) with the tidal volume set as before. Measurements were repeated after 30 minutes. Patients were then returned to volume control mode (VCV2) and final measurements made after 30 minutes. The measurements and results are as follows: After correction of congenital heart defects in infants, mechanical ventilation using a decelerating flow wave pattern resulted in a 19% decrease in peak inspiratory pressure without affecting hemodynamics, arterial oxygenation, or carbon dioxide removal.

Evaluation of a noninvasive index of global ventricular function to predict rejection after pediatric cardiac transplantation.

A Doppler myocardial performance index, defined as the sum of the isovolumetric contraction and relaxation time divided by the ejection time (ICT + IRT/ET), reflects global cardiac function, and when applied to the left ventricle, may serve as a predictor of moderate rejection in pediatric cardiac transplant patients.

Early results of the fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome.

OBJECTIVES: The purpose of this study was to review a large, single institutional experience with the Fontan procedure for patients with hypoplastic left heart syndrome. METHODS: One hundred consecutive patients with "classic" hypoplastic left heart syndrome underwent Fontan palliation between February 1992 and April 1998. Patient demographic, morphologic, and procedural variables were examined and analyzed. In particular, two different surgical techniques were used: technique I (February 1992 to December 1995) employed cardiopulmonary bypass and moderate systemic hypothermia, and technique II (December 1995 to April 1998), profound hypothermia and circulatory arrest. A retrospective review of medical records was performed and variables were examined and analyzed. RESULTS: Hospital survival for the entire cohort was 89% (95% CI 83%-95%). The technique of operation, cardiopulmonary bypass time, and aortic crossclamp time were each strongly associated with survival. Survival for patients treated by technique I was 79% (95% CI 68-91%; n = 48) and for those treated by technique II, 98% (95% CI 94%-100%; n = 52). Cardiopulmonary bypass and crossclamp times were also highly correlated with time to extubation and length of intensive care unit stay. Preoperative pulmonary artery pressure was correlated with survival; preoperative oxygen saturation, right atrial pressure, pulmonary vascular resistance, pulmonary artery size, extent of aortopulmonary artery collaterals, and echocardiographic estimates of ventricular function and tricuspid regurgitation were not correlated with survival. CONCLUSIONS: Our recent experience with Fontan palliation for patients with hypoplastic left heart syndrome suggests that it is attended by low perioperative mortality. The precise operative technique used appears to be an important determinant of outcome, with the duration of cardiopulmonary bypass and crossclamping being particularly significant.

Utility of cardiac catheterization in pediatric cardiac patients on ECMO.

Cardiac catheterization has been utilized rarely in children on extracorporeal membrane oxygenation (ECMO). We performed a retrospective review of 15 children with congenital heart disease who had undergone catheterization while on ECMO from December 1990-December 1995. The procedures, including four interventions, were successful in all patients with adequate evaluation of clinical questions. Unexpected diagnostic information of clinical importance was obtained in 40%, and clinical management of patients was significantly altered in 73%. All patients tolerated the procedure and transport well. The only significant complication was a retroperitoneal hemorrhage in one patient after approximately 12 hr. Although no patients died at catheterization, overall survival was poor, with 50% weaning from ECMO, 29% surviving to discharge, and 14% surviving at follow-up. We conclude that diagnostic and interventional catheterization may be performed in patients on ECMO with acceptable morbidity and mortality; however, long-term survival in this population is poor.

Neonatal cardiac catheterization: A 10-year transition from diagnosis to therapy.

To assess the changing role of cardiac catheterization in the care of the neonate, a retrospective review of all catheterizations between January 1984 to December 1985 (group I) and January 1994 to December 1995 (group II) at C.S. Mott Children's Hospital was performed. Neonatal cardiac catheterization was performed more frequently (p = 0.02) in group I, comprising 14% (110 of 772) of all catheterizations versus 11% (93 of 880) in group II. Access was performed by cutdown in 15 patients (13 venous and 2 arterial), all in group I. In group I, 20 of 110 patients (18%) had balloon atrial septostomies; no other catheter interventions were performed. Interventions were more frequent (p = 0.003) and varied in group II, including 15 septostomies, 17 balloon valvuloplasties (13 pulmonary and 4 aortic), 2 coil embolizations of collaterals, and 1 cardiac biopsy. Despite the higher prevalence and complexity of interventions in group II, fluoroscopy times (median; range: 16 min; 2-55 vs 16 min; 1-107) were similar in both groups (p = not significant) as well as the prevalence of complications. Neonatal cardiac catheterizations are performed less frequently than they were a decade ago at our institution, and therapeutic interventions have become more common. Despite these changes, fluoroscopy time and the rate of complications have not increased.

Modified Norwood operation for single left ventricle and ventriculoarterial discordance: an improved surgical technique.

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.

Bidirectional superior cavopulmonary connection in young infants.

BACKGROUND: Bidirectional superior cavopulmonary connection (BSCC) has become widely used in patients with univentricular AV connections. However, concerns remain about perioperative morbidity and mortality and about the adequacy of oxygenation after cavopulmonary connection in very young patients. This report examines our experience with BSCC in young infants to evaluate whether young age affects operative outcome, to examine the effect of young age on postoperative oxygenation, and to define the lower age limit for successful use of the procedure. METHODS AND RESULTS: The records of the 85 consecutive patients < 6.5 months old who underwent BSCC from December 1990 through February 1995 were reviewed. The average patient age was 4.8 +/- 1.4 months (range, 5 weeks to 6.5 months), with 13 patients being < 3 months old. There were 5 hospital deaths (6%; 70% confidence limits, 3% to 10%). Pulmonary artery thrombosis occurred in 3 patients (4%; 70% confidence limits, 2% to 7%). Younger age was significantly associated with pulmonary artery thrombosis but not with operative death. Oxygenation (arterial PO2, and oxygen saturation) improved significantly and spontaneously over the first 48 hours after BSCC. Younger age had a significant adverse effect on oxygenation in the early postoperative period (first 48 hours). CONCLUSIONS: BSCC can be performed successfully in infants < 6 months old and as young as 5 weeks old. Within this patient population, younger age is not associated with perioperative death but is associated with pulmonary artery thrombosis and postoperative hypoxemia. We suggest that BSCC may be performed any time beyond the neonatal period in symptomatic patients and may be delayed until 4 to 6 months of age if completely elective.

Hemodynamic characteristics of neonates following first stage palliation for hypoplastic left heart syndrome.

BACKGROUND: It is widely held that the postoperative course of patients with hypoplastic left heart syndrome (HLHS) after stage 1 palliation is characterized by hemodynamic instability, which in part may be due to excessive pulmonary blood flow. Hence, avoidance of alkalosis and the use of minimally oxygen-enriched inspiratory gas are thought by many to be important, although there is little pertinent published data. This study was undertaken to characterize the postoperative course and to determine whether the FIO2 and blood pH are related to indices of hemodynamic stability in these infants. METHODS AND RESULTS: The postoperative course of 25 consecutive infants undergoing first stage palliation for HLHS were retrospectively reviewed and the following data were obtained: arterial pressure, arterial blood gas measurements, the inotropic agents used, and multiple respiratory parameters. There was one operative death, and 2 patients died within 2 days, but 22 were extubated (mean, 5.2 +/- 4.1 days after surgery). Hospital mortality was 24%. Mean pH was > or = 7.51 for the first 9 hours after surgery and was > or = 7.45 for the entire period. The mean FIO2 was > or = 50% for the first 18 hours. The PaO2 was appropriate (37 +/- 6 mm Hg at 1 hour after surgery, increasing to 45 +/- 5 mm Hg by hour 73). Only modest inotropic support was needed to maintain appropriate blood pressure. CONCLUSIONS: These data suggest that neither alkalosis nor relatively high inspired oxygen necessarily cause hemodynamic instability in these patients. To what extent these results are generalizable is unclear, but they suggest that there is nothing inherent with HLHS that mandates postoperative hemodynamic instability or unacceptable mortality.

Effectiveness of balloon valvuloplasty in the young adult with congenital aortic stenosis.

The objective of this study was to assess the effectiveness of balloon valvuloplasty in the young adult with congenital aortic stenosis and to compare its effectiveness with children. Percutaneous balloon valvuloplasty is effective in children with congenital aortic stenosis, but not in adults with acquired calcific aortic stenosis. Because effectiveness of balloon valvuloplasty in young adults with congenital aortic stenosis is not well defined, we evaluated the outcome in 15 patients aged 16-24 years (18 +/- 0.6; mean +/- SEM) who underwent balloon valvuloplasty from 1985 to 1993. The aortic valve annulus diameter ranged from 18.5 to 30 mm (24 +/- 0.9). The aortic valve was bicuspid in 12 and tricuspid in 3 patients, and calcification was present in one patient. Balloon valvuloplasty was performed using a double balloon technique in 12 patients and a single balloon technique in three patients. Three patients had inadequate relief of gradient with a residual peak systolic gradient > or = 70 mm Hg. Three patients required valve replacement-two patients for a residual gradient > or = 70 mg Hg, and one patient 4 years later for severe aortic valve regurgitation. Eight of the remaining 12 have undergone elective follow-up catheterization 1.2-2.5 years (1.5 +/- 0.1) later. The peak systolic aortic valve gradient decreased by 55% from 73 +/- 5.8 mm Hg to 35 +/- 5.4 mm Hg immediately postvalvuloplasty, and was 30 +/- 4.4 mm Hg at follow-up (P < 0.001). The left ventricular systolic pressure decreased from 179 +/- 7.5 to 147 +/- 6.5 mm Hg immediately postvalvuloplasty and was 147 +/- 4 mm Hg at follow-up. Aortic insufficiency was unchanged after valvuloplasty in 9, increased by 1+ in 4, and by 2+ in 2 patients. Balloon valvuloplasty was as effective in these young adults as in 70 children (age 6 +/- 0.7 years) with congenital aortic stenosis (peak systolic gradient pre- 79 +/- 3 mm Hg versus post- 34 +/- 2 mg Hg; at 1-2 years follow-up 34 +/- 4 mm Hg). Balloon valvuloplasty provides effective treatment in most young adults with congenital aortic stenosis, without early restenosis. Balloon valvuloplasty is as effective in young adults as in children, where it is currently the treatment of choice.

Intermediate results after complete repair of tetralogy of Fallot in neonates.

From July 1988 through September 1993, 30 neonates with symptomatic tetralogy of Fallot underwent complete repair. Sixteen patients had tetralogy and pulmonary stenosis, 9 had pulmonary atresia, 3 had nonconfluent pulmonary arteries, and 2 had both pulmonary atresia and nonconfluent pulmonary arteries. The median age at operation was 11 days (mean +/- standard error of the mean, 12.6 +/- 2.9 days), with a mean weight of 3.1 +/- 0.1 kg (range 1.5 to 4.4 kg). Preoperatively, 14 patients were receiving an infusion of prostaglandin, 13 were mechanically ventilated, and 6 required inotropic support. Right ventricular outflow tract obstruction was managed by a limited transannular patch in 25 patients, infundibular muscle division with limited resection in 15, and insertion of a right ventricle-pulmonary artery valved aortic homograft conduit in 5 patients. Follow-up was complete at a median interval of 24 months (range 1 to 62 months). There were no hospital deaths and two late deaths, for 1-month, 1-year, and 5-year actuarial survivals of 100%, 93%, and 93%, respectively. The hazard function for death had a rapidly declining single phase that approached zero by 6 months after the operation. Both late deaths occurred in patients with tetralogy of Fallot and pulmonary atresia who had undergone aortic homograft conduit reconstruction, so that the only independent risk factor for death was the use of a valved homograft conduit (p < or = 0.005). Eight patients required reoperation, resulting in 1-month, 1-year, and 5-year freedom from reoperation rates of 100%, 93%, and 66%, respectively. Indications for reoperation were branch left pulmonary artery stenosis in 5 patients, residual right ventricular outflow tract obstruction in 2 patients, and severe pulmonary insufficiency in 1 patient. Independent risk factors for reoperation included an intraoperative pressure ratio between the right and left ventricles of 0.75 or greater (p = 0.01), Doppler residual left pulmonary artery stenosis of 15 mm Hg or more, or Doppler right ventricular outflow tract obstruction gradient of 40 mm Hg or more at hospital discharge (p = 0.002 and 0.02, respectively). This series demonstrates the safety of early hemodynamic repair of symptomatic tetralogy of Fallot in neonates. It also emphasizes the importance of relieving all sources of right ventricular outflow tract obstruction at the initial operation, particularly that located at the site of insertion of the ductus arteriosus, which may be difficult to diagnose in the neonate before ductal closure occurs. The safety and efficacy of valved aortic homograft conduits in neonates requires further investigation.

Late follow-up of balloon angioplasty in children with a native coarctation of the aorta.

Between May 1984 and April 1993, 59 children underwent balloon angioplasty of a native coarctation at our institution. The follow-up protocol included a cardiac catheterization 1 to 2 years after angioplasty, which was performed in 90% of patients with > or = 2 years follow-up. Angioplasty caused an acute decrease in peak systolic gradient from 46 +/- 2 to 15 +/- 2 mm Hg, without early aneurysm or emergent surgical intervention in any patient. Based on follow-up data, a satisfactory result was obtained in 38 patients (64%; 70% confidence limit: 58% to 71%), defined as a residual systolic gradient < 20 mm Hg and no aneurysm. In these patients the gradient decreased acutely from 43 +/- 2 to 9 +/- 1 mm Hg, was 6 +/- 1 mm Hg at follow-up catheterization, and 9 +/- 2 mm Hg by clinical evaluation 4.4 +/- 0.3 years after angioplasty. Twenty-one patients (36%; 70% confidence limit: 29% to 42%) had an unsatisfactory result due to a residual gradient > or = 20 mm Hg (n = 19) or aneurysm formation (n = 3), or both. Restenosis occurred in 6 patients, and occurred more in infants than in children > or = 12 months of age (3 of 5 infants vs 3 of 41 children, p = 0.01). Thus, balloon angioplasty provides an effective initial treatment strategy for native coarctation in most children aged > 12 months.

Selective lung or heart-lung transplantation for pulmonary hypertension associated with congenital cardiac anomalies.

Fixed pulmonary hypertension has been a contraindication to correction of congenital heart defects. Beginning in February 1991, we pursued a policy of performing single-lung transplantation with intracardiac repair for selected patients with this physiology, reserving heart-lung transplantation for those with unreconstructable heart disease. Of 7 patients treated under this protocol, 5 underwent single-lung transplantation and intracardiac repair. The cardiac anomalies included complete atrioventricular canal (1), aortopulmonary window (1), atrial septal defect (1), and ventricular septal defect (2). One patient died perioperatively. All 4 patients surviving operation remained alive through the first postoperative year, but 3 died 13, 17, and 22 months after operation. Two other patients with pulmonary hypertension (1 with tricuspid atresia, 1 after failed Mustard procedure) received a heart-lung transplant and are well 15 and 18 months after operation. This experience demonstrates that selected patients with major intracardiac defects and pulmonary hypertension may have good early results after cardiac repair and single-lung transplantation, but that long-term results are considerably less favorable.

Central pulmonary artery growth patterns after the bidirectional Glenn procedure.

The changes in pulmonary artery size and hemodynamics in 30 patients with univentricular cardiac anatomy were examined before and after bidirectional Glenn procedures done between October 1989 and February 1992. Serial angiographic and hemodynamic examinations before and 17.6 +/- 1.6 months after bidirectional Glenn procedures were compared. At the follow-up study there was no significant change in diameter of the pulmonary artery ipsilateral to the bidirectional Glenn shunt, however, a significant decrease was noted in the diameter of the pulmonary artery contralateral to the bidirectional Glenn shunt (p = 0.04). There was also a 32% decrease in the Nakata index of total cross-sectional pulmonary artery area after the bidirectional Glenn procedure (p = 0.004). Total pulmonary blood flow and mean pulmonary artery pressure had decreased, and arterial oxygen saturation had increased at follow-up. These changes, however, did not correlate with the observed changes in pulmonary artery size. By linear regression analysis, a significant relationship was identified between the Nakata index before the bidirectional Glenn procedure and the absolute change in Nakata index (r = 0.83). A significant decrease in Nakata index occurred only in patients with a bidirectional Glenn shunt in place more than 15 months. Sixteen of the 30 patients subsequently underwent total cavo-pulmonary anastomosis with 7 requiring concurrent surgical pulmonary artery reconstruction. Changes in pulmonary artery size observed more than 15 months after the bidirectional Glenn procedure may have implications for subsequent Fontan repair in children with univentricular anatomy.

Improving results with first-stage palliation for hypoplastic left heart syndrome.

Between January 1990 and February 1993, 73 patients underwent first-stage reconstruction for hypoplastic left heart syndrome at the University of Michigan Medical Center. During this period, surgical reconstruction remained essentially constant and consisted of a pulmonary artery-to-aorta anastomosis with allograft augmentation of the ascending, transverse, and proximal descending aorta, restriction of pulmonary blood flow with a polytetrafluoroethylene shunt from the innominate artery to the central pulmonary artery confluence, and atrial septectomy. Hospital survival was 62 of 73 patients, 85% (70% confidence limits: 80% to 89%). These results stand in marked contrast to those obtained during the earlier years of our experience from 1986 to 1989 when only 21 of 50 patients (42%, 70% confidence limits: 35% to 49%) survived (p = 0.001). Among the most recent group of patients, only 2 of 7 patients older than 1 month of age at operation survived, whereas 60 of 66 (91%, 70% confidence limits: 87% to 94%) patients younger than 1 month of age survived (p = 0.0001). Anatomic subtype and ascending aortic diameter were not predictive of survival. Actuarial survivals for those patients younger than 1 month of age at the first-stage operation, including hospital deaths and subsequent operative procedures, were 81%, 74%, and 74% at 6 months, 1 year, and 2 years, respectively. These results indicate that survival for patients after first-stage reconstruction for hypoplastic left heart syndrome has significantly improved in recent years. Older age was a strong risk factor, with a hospital survival of 91% for those patients undergoing first-stage palliation within the first month of life. These data have important implications for the type of operative intervention and its timing.

Correction of total anomalous pulmonary venous connection in infancy.

From January 1985 through January 1993, 41 patients less than 1 year of age underwent operative correction of isolated total anomalous pulmonary venous connection. There were 24 boys and 17 girls. The median age at operation was 13 days (range 1 to 282 days) and weight was 3.6 kg (2.5 to 5.2 kg). Locations of the connections were supracardiac in 19, cardiac in 9, infracardiac in 11, and mixed supracardiac and cardiac in 2. Obstruction of the pulmonary veins was severe in 24, mild in 3, and absent in 14. Preoperative stabilization included mechanical ventilation for 15 patients for a mean duration of 2 1/2 days and extracorporeal membrane oxygenation for 1 patient for 1 day. All operations were performed with deep hypothermia and circulatory arrest (mean arrest time 34 minutes). Supracardiac connections were repaired by performing a side-to-side anastomosis between the pulmonary venous confluence and the dome of the left atrium through a superior approach between the superior vena cava and the aorta. Coronary sinus connections were repaired by enlarging the atrial septal defect and the coronary sinus communication with the left atrium and closing the atrial defect with a large patch. Infracardiac repairs included elevation and rotation of the heart to the right and an elongated side-to-side anastomosis between the common venous confluence and the left atrium. One patient died 1 week postoperatively of persistent pulmonary hypertension. Another patient, who was supported by extracorporeal membrane oxygenation before the operation, died 3 months after the operation as a consequence of pulmonary lymphangiectasia. All other patients are alive and well with a mean follow-up of 26 months (range 3 to 77 months). One patient required two subsequent reoperations for persistent pulmonary venous obstruction, and another patient had superior vena cava obstruction necessitating reoperation. Operative treatment of total anomalous pulmonary venous connection in infants can be performed with low mortality and an infrequent need for reoperations.

Intraoperative and percutaneous stenting of congenital pulmonary artery and vein stenosis.

BACKGROUND: Conventional surgical or balloon dilation therapy for pulmonary artery or vein stenosis has been unsatisfactory in many patients. Balloon-expandable stents offer a new form of treatment for these vascular stenoses and can be implanted percutaneously or intraoperatively. METHODS AND RESULTS: Between July 1991 and October 1992, 20 balloon-expandable Palmaz stents (Johnson & Johnson) were implanted in 16 children at median age and weight of 3.0 years and 12.8 kg, respectively. Stent implantation was performed intraoperatively (n = 15) if the patient was less than 1 year of age or less than 10 kg in weight, in cases where limited vascular access precluded percutaneous implantation, or as an adjunct to other intracardiac surgery. Otherwise, percutaneous stenting was performed (n = 5). Vessels were tested for distensibility by dilation with balloon catheters or vascular sounds. Stents were implanted using angioplasty catheter balloons chosen to achieve desired vessel diameter and inflated to 4 to 17 atm. Acute hemodynamic and cineangiographic studies were performed in all patients immediately after the procedure to 2 months after stenting. After pulmonary artery stent implantation, mean pulmonary artery diameter increased from 5.6 to 11.5 mm (P = .001), with a decrease in mean systolic pressure gradients from 43 to 8.0 mm Hg (P = .005). Follow-up cardiac catheterization (mean, 8.7 months) in 3 patients revealed no restenosis, thrombosis, or aneurysm formation. In patients in whom pulmonary vein stents were implanted, mean pressure gradients fell from 11 to 0.3 mm Hg (P = .03), and mean pulmonary capillary wedge pressure fell from 17 to 6.3 mm Hg (P = .03) immediately after stenting. At 2- to 6-month follow-up, cardiac catheterization documented restenosis within the stent in 2 of 3 patients. The third patient died 2 months after stenting from presumed vein reocclusion. CONCLUSIONS: When implanted intraoperatively or percutaneously, balloon-expandable endovascular stents have been efficacious in the treatment of pulmonary artery stenosis. Longer follow-up will be necessary to document the long-term effectiveness of pulmonary artery stenting. Preliminary data suggest that early restenosis is common after pulmonary vein stenting. The intraoperative approach extends stenting therapy to smaller children and to patients who have limited percutaneous access.

Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate.

The management of the neonate with absence of intrapericardial pulmonary arteries in association with complex intracardiac anomalies presents a challenging surgical problem. The more traditional approach of palliation with unilateral or bilateral systemic-pulmonary artery shunts may result in peripheral pulmonary artery stenoses and uneven distribution of pulmonary blood flow. In addition, this approach may lead to complicated reconstructive procedures necessitating reconstruction of the branch pulmonary artery with prosthetic material, which restricts pulmonary artery growth and often complicates reoperation. To avoid these potential limitations, we have performed primary unifocalization for absence of intrapericardial pulmonary arteries in eight consecutive neonates (median age 9 days) between May 1990 and December 1991. Absence of intrapericardial pulmonary arteries occurred in association with tetralogy of Fallot (n = 4), truncus arteriosus (n = 2), and transposition of the great arteries with pulmonary atresia (n = 2). Four patients had unilateral absence of the right (n = 1) or left (n = 3) intrapericardial pulmonary artery. In the remaining four patients, there was complete absence of both intrapericardial pulmonary arteries. Wide mobilization and excision of all ductal tissue before anastomosis was performed from a midline approach in seven patients. In one patient, a preliminary right thoracotomy was required. Primary unifocalization was performed simultaneously with complete repair in five patients. In the remaining three patients, unifocalization was part of a staged repair and included insertion of a systemic-pulmonary artery shunt to the reconstructed central pulmonary artery confluence. No operative or late cardiac deaths occurred, although one death occurred during subsequent repair of a tracheoesophageal fistula. Three patients underwent reoperation, and only one patient required revision of an anastomotic pulmonary artery stenosis. All survivors were growing normally at 2 to 22 months after operation (mean follow-up 10 months). Our experience suggests that primary reconstruction for the absence of intrapericardial pulmonary arteries can be successfully accomplished in the neonate. This approach provides uniform bilateral pulmonary blood flow, avoids prosthetic material in the branch pulmonary arteries, and may eliminate, or at least simplify, future reconstructive procedures.

The psychosocial impact of pediatric heart transplantation.

Children with terminal heart disease experience a dramatic improvement in functional status after heart transplantation but may be at increased risk for problems in psychosocial adaptation. Selected psychosocial outcomes were assessed in 49 pediatric heart transplant recipients and their families from five heart transplantation centers. Heart transplant recipients did not appear significantly different from their peers on self-report measures of self-concept and anxiety, but they showed significantly less social competence and more behavior problems than a normative population. Behavior problems observed were most frequently suggestive of depression and were significantly associated with greater family stress and diminished family resources for managing stress. The study findings further suggest that the heart transplant recipients' ability to verbalize or ventilate their feelings and concerns to others seems to facilitate psychosocial adaptation. Assessment of stress, resources, and coping is imperative to enable health professionals to promote the psychosocial adaptation of pediatric heart transplant recipients and their families.

Optimum treatment of discrete subaortic stenosis.

Discrete subaortic stenosis typically appears as a well-defined membrane beneath the aortic valve. To assess the merits of alternative approaches to this problem, we have reviewed the results of operations for discrete subaortic stenosis from 1978 through 1990. Excision of the subaortic membrane alone was performed in 16 patients (group I). Excision of the membrane with resection of septal muscle was performed in 24 patients (group II). The groups were similar in age at operation, duration of follow-up, and preoperative and postoperative transvalvar gradients. There were no operative or late deaths. Reoperations for recurrent subaortic stenosis were performed in 4 group I patients (25%; 70% confidence limits, 16% to 38%) and 1 group II patient (4%; 70% confidence limits, 2% to 11%). Pacemakers were inserted for postoperative complete heart block in 1 group I patient (6%; 70% confidence limits, 2% to 16%) and 2 group II patients (8%; 70% confidence limits, 4% to 16%). We conclude that muscle resection combined with membrane excision in patients with discrete subaortic stenosis does not increase the risk of death or heart block, and does lower the risk of reoperation for recurrent subaortic stenosis.