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.
Lamour
Jacqueline M
JM
Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, NY, USA. jml14@columbia.edu
Hsu
Daphne T
DT
Quaegebeur
Jan M
JM
Pinney
Sean P
SP
Mital
Seema R
SR
Mosca
Ralph S
RS
Chen
Jonathan M
JM
Addonizio
Linda J
LJ
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000628:therapy*; D016027:Heart Transplantation; D006801:Humans; D008168:Lung / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D011182:Postoperative Care; D011183:Postoperative Complications; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology; D011666:Pulmonary Valve Stenosis / Q000150:complications; D011859:Radiography; D016019:Survival Analysis; D015996:Survival Rate