Lung transplantation and repair of complex congenital heart lesions in patients with pulmonary hypertension.

Abstract

Pulmonary vascular disease in conjunction with either a previously repaired or an unrepaired congenital heart defect is the third most common indication for lung transplantation in the pediatric age range. Because scarcity of donor organs remains a critical issue and heart-lung donor blocks are becoming diminishingly available, efforts must be directed towards other options such as combining lung transplantation with correction of the underlying congenital heart defect. Certain defects like congenital pulmonary vein stenosis are eradicated by removal of the diseased lungs, whereas others such as complete atrioventricular canal and pulmonary atresia with ventricular septal defect require cardioplegic arrest of the heart and intracardiac repair in conjunction with the lung transplantation. A breakdown of this patient population into subgroups may be helpful both in thinking about the pathophysiology and in determining appropriate indications and timing of transplantation. Earlier studies from our center showed the high-risk nature and formidable undertaking of caring for this complex group of patients. Through continued experience, there has been gradual improvement in early outcomes. As with all other groups of lung transplantation patients, obliterative bronchiolitis remains the major deterrent to long-term survival.