Approach to Lung Transplantation in Pulmonary Arterial Hypertension: A Delphi Consensus on Behalf of the Transplant Task Force of the Pulmonary Vascular Research Institute

Abstract

Lung transplantation is indicated for selected patients with advanced pulmonary arterial hypertension (PAH). We used a modified Delphi process to develop recommendations on care of patients with PAH undergoing lung transplantation. This Delphi panel was recruited from the Pulmonary Vascular Research Institute's Innovative Drug Discovery Initiative - Lung Transplantation Workstream, consisting of clinical and research experts in PAH and lung transplantation. In this process, 29 panelists were given open-ended questions, querying topics related to lung transplantation in PAH. A steering group converted the responses into discrete statements. Panelists then rated agreement using a Likert scale in two further survey rounds: -5 (strongly disagree) to 5 (strongly agree). Consensus was defined as mean ≥ 2.5 or ≤ -2.5, with a standard deviation not crossing zero. Consensus was reached on 141 of 223 statements. Notable areas of consensus were for early discussions about transplantation, and agreement with previously published referral and listing criteria. There was agreement that lung transplantation could be offered in sick candidates, including those with concurrent renal or hepatic insufficiency. Bilateral lung transplantation was considered the procedure of choice for most patients, with rare indications for heart-lung transplantation. Consensus on bridging strategies included use of veno-arterial extracorporeal membrane oxygenation and preemptive awake cannulation in those with severe right ventricular dysfunction. Consensus was also achieved on intraoperative use of invasive hemodynamic monitoring, and prolonged postoperative circulatory support guided by hemodynamic response and echocardiography. Patients with PAH undergoing transplantation require specialized management, which differs somewhat from other candidates.

Figure 1

Overview of the Delphi consensus process to develop recommendations on lung transplantation for patients with pulmonary arterial hypertension (PAH). Developed by the Pulmonary Vascular Research Institute Innovative Drug Delivery Initiative (IDDI) Lung Transplantation Workstream.

Figure 2

When to start discussions about transplantation in patients with pulmonary arterial hypertension (PAH), when to refer patients with PAH for transplantation, and when to list patients with PAH for transplantation.

Figure 3

Type of organ transplant recommended for patients with pulmonary arterial hypertension.

Figure 4

Recommendations regarding bridging, intra‐operative management, and postoperative management for patients with pulmonary arterial hypertension requiring lung transplantation.