Orthotopic heart transplantation for complex congenital heart disease

Orthotopic heart transplantation has become standard therapy for end-stage cardiomyopathy in children and adults, but there has been much less experience with transplantation for complex congenital heart disease. In this report experience with orthotopic transplantation in seven children with various forms of complex congenital heart disease is reviewed. Diagnoses included hypoplastic left heart syndrome in two (after stage I palliation), left ventricular diverticulum in one, single ventricle in two (dextrocardia, atrial situs inversus, and total anomalous pulmonary venous return in one patient and post-Fontan repair in the second), D-transposition of the great arteries and ventricular septal defect (post-Senning repair and ventricular septal defect closure) in one, and Ebstein's anomaly with biventricular dysplasia in one. Six of the seven were hospital survivors and there has been one late death at 2 1/3 years. Modifications of the standard operative technique to fit the anatomic variations in these defects are reviewed.     

Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits

BACKGROUND: Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. METHODS: Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 +/- 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. RESULTS: Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% +/- 1.5% and 59.5% +/- 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time (p < or = 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio > or = 0.72 (p < or = 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% +/- 2.0% and 31.9% +/- 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size (p < or = 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. CONCLUSIONS: Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation.     

Venovenous extracorporeal membrane oxygenation for cyanotic congenital heart disease

Background

Severe, refractory hypoxemia complicating uncorrected cyanotic congenital heart disease is a potentially lethal condition, even when urgent surgical intervention is undertaken. When a viral pneumonia initiates hypoxemia, the likelihood of a satisfactory outcome is further reduced. We examined our policy of venovenous extracorporeal membrane oxygenation support through the hypoxic event and performing delayed surgery, if required, to separate from extracorporeal membrane oxygenation.

Methods

A single institution, retrospective review of an Institutional Review Board approved database was undertaken. Over a 6-year period, 18 instances were identified for 17 patients who became acutely hypoxemic from either inadequate pulmonary blood flow (8 instances) or a viral pneumonia (10 instances) complicating their cyanotic heart disease. Demographics, duration of venovenous extracorporeal membrane oxygenation and outcomes are reported.

Results

The length of venovenous extracorporeal membrane oxygenation ranged from 13.5 to 362.5 hours (mean 130 ± 121 hours). During 10 supports, operations were performed to facilitate weaning from support. In 7 patients, extracorporeal support was weaned during this surgery. Follow-up was obtained in all patients over a period ranging from 4 months to 7 years (mean 39.0 ± 23.0 months). There were two late deaths due to sepsis 1.4 and 2.5 months after extracorporeal support.

Conclusions

Venovenous extracorporeal membrane oxygenation allows time for the recovery of acute hypoxic insult and resolution of some viral pneumonia processes. Palliative surgical procedures may be safely undertaken during extracorporeal support. Viral pneumonia is a risk for prolonged support. Venovenous extracorporeal membrane oxygenation is useful in these high-risk patients.     

Pediatric heart transplantation for congenital heart disease and cardiomyopathy

Orthotopic heart transplantation has become an accepted therapy for adult patients with end-stage heart disease. In newborns and infants, this procedure is still controversial because of the unknown long-term results and the lack of donor organs. Since March 1988, we have performed orthotopic heart transplantation in 11 infants and children with hypoplastic left heart syndrome (n = 6), cardiomyopathy (n = 4), or congenital endocardial fibroelastosis (n = 1). The smallest infant was 3 days old and weighed 2,650 g. Four of 15 potential donors had to be refused for various medical reasons, and 4 were transferred to our hospital for organ retrieval. Seven hearts were procured remotely. We accepted weight mismatches up to 105% between donor and recipient. There were three perioperative deaths, two in patients 5 and 17 days old with hypoplastic left heart syndrome and 1 in a 2-year-old patient with a dilated cardiomyopathy. All 3 patients had drug-resistant right heart failure. A 2-year-old girl with a dilated cardiomyopathy died 2 months after transplantation owing to severe pulmonary embolism originating from the superior vena cava. The remaining 7 patients are alive and well between 1 month and 31 months after transplantation. Angiographic follow-up has not revealed signs of graft atherosclerosis at 2 years.     

Superior sternal cleft repair using autologous rib grafts in an infant with complex congenital heart disease

We report the case of an infant with superior cleft sternum and obstructed type III (infra-diaphragmatic) total anomalous pulmonary venous return. A previously undescribed approach is presented using autologous rib grafts without division of the intact inferior sternum. This technique provides protection of the anterior and superior mediastinum without the use of foreign material, destabilization of the sternum, or compromise of respiratory mechanics.     

Vascular endothelial growth factor in children with congenital heart disease

BACKGROUND: Children with cyanotic congenital heart disease may experience the development of abnormal vessels that become a source of significant morbidity. Abnormal vessel proliferation in these children may take several forms, including systemic-to-pulmonary collateral arteries, systemic-to-pulmonary venous collaterals, systemic venous collateral channels after bidirectional cavopulmonary anastomosis, and pulmonary arteriovenous malformations. However, no entity responsible for these abnormalities has been identified yet. This study determined whether children with cyanotic congenital heart disease have elevated serum levels of vascular endothelial growth factor (VEGF) and whether elevated VEGF correlated with these abnormal vessels. METHODS: Mean systemic room air oxygen saturation (SpO2), blood cell counts (RBC), and serum VEGF levels were measured preoperatively. Samples were obtained from 61 children with acyanotic heart disease (group N) and 102 children with cyanotic heart disease (group C) before cardiac surgery. Postoperative catheterization was performed 1-month after the operation to evaluate the abnormal vessels in group C. RESULTS: The VEGF level was significantly elevated in group C (355.0 +/- 287.1 pg/mL) compared with group N (203.0 +/- 221.6 pg/mL; p < 0.001). VEGF levels in patients with a single ventricle associated with asplenia syndrome (n = 7) in group C were significantly elevated (711.9 +/- 443.5 pg/mL) compared with other patients. There was no significant correlation between VEGF level and SpO2 or RBC. Abnormal vessels were diagnosed in 19.6% (20/102) patients in group C. There was no difference in VEGF levels between the patients with abnormal vessels (336.8 +/- 182.5 pg/mL) and the patients without abnormal vessels (359.1 +/- 306.8 pg/mL). CONCLUSIONS: Children with cyanotic heart disease have elevated systemic levels of VEGF, especially in those patients with a single ventricle associated with asplenia syndrome. There was no significant relationship in VEGF levels between the patients with abnormal vessels and without these vessels.