Lung transplantation for pulmonary hypertension: management of pulmonary hypertension on the waiting list

Abstract

Despite the apparent improvement in outcomes for pulmonary arterial hypertension (PAH) with current medical therapy, for many patients, lung transplantation remains as an important therapeutic option. Such patients are at high risk for clinical deterioration while awaiting lung transplantation. Thus, the medical management is crucial to keep these tenuous patients acceptable on the waitlist. This includes optimization of PAH-targeted therapy and adjunctive therapies. For select patients, atrial septostomy may further expand the transplant window. Clinical deterioration of PAH, usually in the form of decompensated right-sided heart failure, often requires careful critical care management. Care of such patients is limited by high in-hospital mortality and a dearth of guiding evidence. Extracorporeal life support (ECLS) has been increasingly utilized to bridge select decompensated and otherwise moribund patients to lung transplantation. Overall, careful pre-transplant management may be contributing to the reduced waitlist mortality in recent years despite higher disease acuity.