• T L Spray.
• Division of Cardiovascular Surgery, Children's Hospital of Philadelphia, PA 19104, USA.
• Semin. Thorac. Cardiovasc. Surg. 1996 Jul 1; 8 (3): 286-95.

AbstractPulmonary 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. As one might expect, the operative and postoperative management of these patients is a good deal more complex than pulmonary transplantation for primary lung diseases. The early mortality is correspondingly higher as well, although survival beyond that time is quite similar to that of lung transplantation for other diseases. Factors that increase the early mortality include previous thoracic procedures and the complexity of the underlying cardiac diagnosis. 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.

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