Pediatric lung transplant: do primary pulmonary hypertension patients have worse outcome?

Abstract

High mortality risk has been reported for primary pulmonary hypertension (PPH) pts undergoing lung transplantation (LTx). Do PPH children have worse outcomes after LTx than cystic fibrosis (CF) children? All pediatric PPH pts undergoing LTx at our institution were compared to CF pts undergoing LTx in the same period. Data collected: demographics, echocardiograms, ischemic times, cardiopulmonary bypass times (CPB), perioperative and 1-year survival. From 1994-2002, 10 PPH children (3M:7F; mean age at Tx 13.7 ± 3.4 years) underwent LTx. Seven were on IV prostacyclin and 3 took oral beraprost prior to Tx. There were 5 living donor double lobar lung (LDL) Txs and 5 cadaveric double LTxs. Prior to Tx, all PPH pts had severe right ventricular and right atrial dilatation with pancaking of the left ventricle. Mean tricuspid regurgitation gradient prior to Tx was 102.5 ± 18.4 mmHg (range 74-140 mm Hg). Six pts were NYHA Class III and 4 were NYHA Class IV. Mean PPH ischemic times were 148.1 ± 83.4 min (range 52.5- 294 min). Mean CPB times were 139.9 ± 40.6 min (range 85-214 min). 9 PPH pts survived the perioperative period (90%); 1 pt expired from non-specific graft failure. Of the 9 surviving pts, 1 pt is <6 months post-Tx and 8 pts have survived >1 year (100%). During the same period, 41 CF pts (22M:19F; mean age at Tx 14.9 ± 3.5 years) were transplanted. There were 34 LDL and 7 cadaveric LTxs. Mean CF ischemic times were 99.7 ± 52.6 min (range 44.5-249 min). Mean CPB times were 124.2 ± 36.2 min (range 75-260 min). Of 41 CF pts, 38 survived the perioperative period (93%). One-year CF Tx survival was 92%. There was no difference between groups in age (p = 0.33), gender distribution (p = 0.29), CPB time (p = 0.25), perioperative mortality (p = 1.0), or 1-year survival (p = 1.0). There was a significant difference in ischemic times (p = 0.02) and type of Tx surgery (p = 0.04). We conclude that pediatric PPH pts have similar perioperative and 1-year survival compared to CF pts undergoing LTx. Our findings support lung transplantation as a viable therapeutic option for pediatric PPH pts who fail to respond to medical therapy.