Proceedings: Use of right atrium to pulmonary artery valved conduit for 'correction' of single ventricle of hypoplastic right heart syndrome.
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Biomedical subjects
Publications and source records attributed to R Radley-Smith.
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The course and natural history of two children aged 1 yr and 5 yr with primitive ventricle with outlet chamber and normally related great arteries are described. They initially presented as ventricular septal defect with increased pulmonary blood flow and subsequently developed 'cyanotic attacks', chronic cyanosis and diminished pulmonary flow by acquiring subpulmonary obstruction of the bulboventricular foramen. Both had successful surgical treatment utilizing their own normal pulmonary valves by connecting the right atrium to the right ventricular outflow, with a Dacron conduit in one, and in the other by detaching the pulmonary artery and valve from the rudimentary outflow chamber and anastomosing it directly to the right atrial appendage. Changing morphology and its effect on function are discussed.
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Between September 1984 and October 1988, 27 patients underwent combined heart-lung transplantation for treatment of end-stage respiratory disease caused by cystic fibrosis. The actuarial patient survival was 78% at 1 year and 72% at 2 years. Bacterial respiratory infections were common in the early postoperative period and necessitated vigorous medical therapy. The dose of cyclosporine required in these patients was higher than in conventional transplant recipients, and this contributed to an increased cost of postoperative care. Lung function was greatly improved after transplantation, and long-term survivors achieved an excellent quality of life. Lymphoproliferative disorders developed in two patients; these disorders regressed after a reduction in immunosuppression. Two patients required retransplantation: one because of obliterative bronchiolitis and the other because of recurrent respiratory infections associated with a moderate tracheal stenosis and severe deterioration in lung function. A modification of the technique used for heart-lung transplantation allowed 20 hearts from cystic fibrosis patients to be used for subsequent heart transplantation. Immediate heart function was satisfactory in all cases. The actuarial survival of the recipients of these domino heart transplants was 75% at 1 year. No coronary artery disease was present in the 12 patients who have undergone coronary angiography at 1 year.
BACKGROUND: The presence of significant elevation of pulmonary vascular resistance is a major risk factor for death after orthotopic heart transplantation. The choice of procedure for the pediatric patient remains contentious. METHODS: We report three pediatric patients with pulmonary hypertension and raised pulmonary resistance, including two infants, in whom heterotopic transplantation was performed with smaller donor hearts. The hearts were anastomosed to provide left ventricular support alone because predominant left ventricular failure was present, the pulmonary resistance was expected to fall gradually after surgery, and we wished to avoid the potential problems of a pulmonary conduit associated with growth and possible reoperation. RESULTS: The clinical results have been satisfactory in all three patients, who were well and without symptoms. No pulmonary compromise was observed in the smaller thoracic cavity of the two infants. The transpulmonary gradient fell in all three patients, although this reduction was not immediate. CONCLUSIONS: This technique in carefully selected recipients can allow safer transplantation in pediatric patients with elevated pulmonary resistance, can increase the donor pool by allowing use of smaller hearts and nonideal donors, and may reduce the mortality on the transplant waiting list by providing earlier transplantation.