Heart transplantation to a physiologic single lung in patients with congenital heart disease

Background: Heart-lung transplantation has been recommended for patients with end-stage congenital heart disease (CHD) and single-lung physiology due to either discontinuous pulmonary arteries (PAs) and unilateral PA hypertension (HTN) or absence of 1 PA. Methods: Eleven patients with CHD and single-lung physiology underwent heart transplantation (HT). Diagnoses included: tetralogy of Fallot, absent left PA (n = 4); single-ventricle s/p classic Glenn (n 7), with absent left PA (n = 1); and severe left PA HTN (n = 6). Results: Mean time from last surgery was 13 +/- 8 years; mean number of operations (op) was 3.2 +/- 1.7. Mean age was 21 +/- 11 years (range 9.5 to 43). Complications and procedures before HT included hemoptysis (n = 2), plastic bronchitis (n = 1) and interventional catheterization (n = 6). Mean cardiopulmonary bypass and ischemic time was 275 +/- 72 and 268 +/- 75 minutes, respectively. Mean time to extubation was 4.6 +/- 3.2 days, and mean length of stay was 19 +/- 7 days. Post-operative morbidity included bleeding (n = 4), vocal cord paralysis (n = 1) and coil embolization of aortopulmonary collaterals (n = 3). Early post-operative survival was 82%. Cause of death was aortic rupture (n = 1) and bleeding (n = 1). Eight patients are alive 4 years (range 0.9 to 7.6) after HT. PA continuity was established in 6 patients; post-HT lung perfusion scan showed no increase in perfusion to. the left PA. One patient died from rejection 3 years post-HT. Conclusions: HT can be performed successfully in patients with single-lung physiology. HT is the procedure of choice in patients with end-stage CHD and a physiologic single lung.