Congenital Heart Disease and a Single Lung: Is Heart Transplantation Alone an Option?

PURPOSE: Patients with end-stage heart failure and one functioning (Fx) lung are often referred for combined heart-lung transplantations (HLTx). However, this strategy carries significant waitlist & post-transplant mortality. Between 1987 and 2018, only 37% of the pediatric patients listed for HLTx were actually transplanted. This study aims to evaluate if heart transplant alone (HTx) remains a viable option for this patient population.

METHODS: Using both the Pediatric Health Information System (PHIS) and United Network for Organ Sharing (UNOS) databases, HTx recipients with one Fx lung and congenital heart disease between 2006 and 2015 (n=19) were identified. All other HTx recipients in a merged PHIS-UNOS database during the same period were classified as two Fx lungs (n=2,625). Post-transplantation survival rates for recipients with one and two Fx lungs were compared using Kaplan-Meier analysis.

RESULTS: HTx recipients with one Fx lung had lower post-transplantation survival rates than recipients with two Fx lungs (p=0.03) but comparable conditional 60-day survival rates (p=0.56). Recipients with one Fx lung had one- and five-year survival rates of 71% and 40% with 60-day conditional one- and five-year survival rates of 100% and 90%, respectively. Five out of the six deceased HTx recipients with one Fx lung died within 60 post-operative days and before being discharged. Renal dysfunction (GFR<60 mL/min/1.73 m²) at HTx was a significant predictor of mortality for patients with one Fx lung (p=0.03).

CONCLUSION: HTx recipients with one Fx lung can do well but seem to have a high early post-operative mortality that usually indicates high-risk recipients with multiple risk factors. Patient selection and timing of transplantation referral will be key to improving these outcomes. Keeping in mind the low percentage of waitlisted patients who actually make it to HLTx, HTx appears to be the best option for patients with end-stage heart failure and one Fx lung.