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Orthotopic transplantation for total anomalous pulmonary venous connection associated with complex congenital heart
A J Razzouk1, S R Gundry, R E Chinnock
1Loma Linda University Children's Hospital, Calif., USA.
Insights
Orthotopic heart transplantation offers a viable treatment for complex total anomalous pulmonary venous connection in children when biventricular repair is not possible. While improving outcomes, it carries risks of pulmonary venous obstruction post-transplant.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Transplantation Immunology
Background:
- Complex congenital heart defects, including total anomalous pulmonary venous connection (TAPVC), often lead to poor surgical outcomes.
- High morbidity and mortality rates are associated with TAPVC when combined with other severe cardiac malformations.
Purpose of the Study:
- To evaluate the efficacy and safety of orthotopic heart transplantation in pediatric patients with complex TAPVC.
- To assess the potential of transplantation to improve survival in cases where biventricular repair is not feasible.
Main Methods:
- Twelve pediatric patients (4 days to 6.8 years) with TAPVC and complex cardiac anomalies underwent orthotopic heart transplantation.
- Associated conditions included hypoplastic left heart syndrome, unbalanced atrioventricular canal, single ventricle with pulmonary atresia/stenosis, situs inversus, dextrocardia, asplenia, and polysplenia.
- Surgical techniques involved direct donor left atrium to pulmonary venous pool anastomosis or complex baffle reconstruction; palliative procedures were performed in some cases.
Main Results:
- One operative death occurred due to an oversized heart and vena caval thrombosis.
- Follow-up averaged 3 years; 18% of patients developed pulmonary venous obstruction post-transplant.
- One patient with obstruction required successful reoperation, while another died of sepsis after multiple reoperations.
Conclusions:
- Orthotopic heart transplantation is a feasible treatment option for pediatric patients with complex TAPVC unsuitable for biventricular repair.
- Transplantation can improve the prognosis for these high-risk patients but does not eliminate the risk of late pulmonary venous obstruction.
Background:
When total anomalous pulmonary venous connection is associated with other complex cardiac malformations, early and late postsurgical morbidity and mortality are excessive.
Methods:
In an attempt to modify this outcome, twelve children (4 days to 6.8 years of age) with total anomalous pulmonary venous connection and various congenital cardiac defects were treated with orthotopic heart transplantation. Associated cardiac diagnoses included the following: hypoplastic left heart syndrome (n = 2), unbalanced atrioventricular canal with pulmonary atresia (n = 2), and single ventricle with severe pulmonary stenosis (n = 3) or atresia (n = 5). Two patients had situs inversus, and two had dextrocardia with situs ambiguous. Eight patients had asplenia and one had polysplenia. Palliative pretransplantation procedures in five patients included the following: systemic to pulmonary artery shunt (n = 5), atrioventricular valve annuloplasty (n = 1) and classical Glenn shunt (n = 1). The donor left atrium was anastomosed directly to a common pulmonary venous pool in nine patients; whereas three children required complex reconstruction to baffle the pulmonary venous flow to the donor left atrium.
Results:
There was one operative death related to an oversized heart and vena caval thrombosis. Follow-up ranged from 16 months to 4.5 years (average 3 years). In two patients (18%) pulmonary venous obstruction developed 3 and 4 months after transplantation. Reoperation to relieve the obstruction was successful in one patient. The second patient underwent three such reoperations and died of sepsis 10 months after orthotopic heart transplantation.
Conclusion:
Orthotopic transplantation is a viable option for children with complex total anomalous pulmonary venous connection that precludes a biventricular repair. Transplantation may improve the dismal prognosis of those children, but it does not eliminate the potential for late pulmonary venous obstruction.