Seminars in thoracic and cardiovascular surgery
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Semin. Thorac. Cardiovasc. Surg. · Jul 1996
Lung transplantation in children with pulmonary hypertension and congenital heart disease.
Pulmonary vascular disease with or without associated congenital heart disease is the indication for lung transplantation in 30% of all children requiring this procedure. Although many of these children will have primary pulmonary hypertension, a substantial number are children whose pulmonary vascular disease is either associated with a congenital heart lesion or secondary to it. Heart-lung transplantation has been performed in these children in the past, but because of the scarcity of availability of donor heart-lung blocks, lung transplantation with repair of associated congenital cardiac lesions has of necessity been performed more frequently. ⋯ Although the overall experience with this complex group of patients is small, the results seem similar to the prior experience with heart-lung transplantation. Thus, we remain optimistic that this will continue to be a viable form of therapy for children with pulmonary vascular disease associated with congenital heart lesions. The quality of life of the surviving patients is excellent and limited only by those factors impacting upon lung transplantation in general, such as rejection, infection, and the development of bronchiolitis obliterans.