The impact of bronchiolitis obliterans on late morbidity and mortality after single and bilateral lung transplantation for pulmonary hypertension

Abstract

Primary pulmonary hypertension (PPH) is a rare cardiovascular disease with a variable course; however, in general, its prognosis is poor. Among the various treatment options available, transplantation (initially heart-lung transplantation, and later isolated single or bilateral lung transplantation) has become an accepted modality. Heart-lung transplantation is necessary only in a minority of patients because right ventricular recovery has been gratifying after isolated lung transplantation. Furthermore, the scarcity of suitable donor organs mandates the achievement of the maximal number of heart and lung transplants from the limited donor pool. Available published data show that both single and bilateral lung transplantation are suitable alternatives for the majority of patients with pulmonary hypertension. Bronchiolitis obliterans syndrome (BOS), the main cause of late mortality and morbidity in lung transplant recipients, affects pulmonary hypertensive patients as it does other recipient subgroups. The available data regarding the impact of BOS on single versus bilateral lung recipients with pulmonary hypertension are somewhat scanty. Although some have suggested that BOS is more prevalent among PPH recipients, this is not uniformly supported through the literature. Other reports have documented severe ventilation-perfusion imbalance associated with graft dysfunction secondary to BOS in single lung transplant recipients with PPH. Despite this, there are no available data to document a significant survival benefit for PPH patients receiving bilateral versus single lung transplantation. Our own transplantation experience at Washington University in St. Louis with pulmonary hypertension shows a trend toward better survival in bilateral lung recipients, although this difference is not significant. Ultimately, both single and bilateral lung replacement seem to be satisfactory transplant options in PPH. Both recipient groups are affected by BOS, and longer follow-up of larger numbers of patients may document superior survival and functional outcome with bilateral lung replacement.