TOPIC IMPORTANCE

Even though patients with pulmonary arterial hypertension have multiple therapeutic options, the disease can be refractory despite appropriate management. In patients with end-stage pulmonary arterial hypertension, lung transplantation has the potential both to extend survival and improve health-related quality of life. Pulmonary arterial hypertension is the only major diagnostic indication for transplantation that is not a parenchymal pulmonary process, and thus the care of these patients is unique.

REVIEW FINDINGS

This review focuses on the complexities of lung transplantation for patients with pulmonary arterial hypertension, presents the updated referral and listing criteria, and discusses the inequities in the organ allocation process that impact this disease group and the strategies to optimize outcomes for patients with pulmonary arterial hypertension who require lung transplantation.

SUMMARY

Lung transplantation is an effective and lifesaving therapy for patients with end-stage lung disease. Sadly, patients with pulmonary arterial hypertension face many challenges as it relates to transplantation including higher perioperative risks, inequities in the allocation system, and less favorable long-term outcomes. This review covers the complexities of transplantation in patients with pulmonary vascular disease.