Lung transplantation for pulmonary hypertension

Abstract

From its identification as a distinct disease entity, understanding and management of pulmonary hypertension has continuously evolved. Diagnostic and therapeutic interventions have greatly improved the prognostic implications of this devastating disease, previously rapidly and uniformly fatal to one chronically managed by multi-disciplinary teams. Improved diagnostic algorithms and active research into biochemical signatures of pulmonary hypertension (PH) have led to earlier diagnosis of PH. Medical therapy has moved from upfront use of continuous intravenous prostaglandins to administration of combinations of oral medications targeting multiple pathways underlying this disease process. In addition to improved medical therapies, recently introduced interventions such as pulmonary endarterectomy and pulmonary artery balloon angioplasty for chronic thromboembolic pulmonary hypertension (CTEPH) give patients an increasing array of treatment options. Despite these many advances, lung transplantation remains the definitive treatment for patients with disease refractory to or progressing on best medical therapy. As our understanding of medical therapy has advanced, so to have best practices for lung transplantation. Recipient selection and approach to organ transplantation techniques have continuously evolved. Mechanical circulatory support has become increasingly employed to bridge patients through lung transplantation in the immediate post transplantation recovery. In this review, we give a history of lung transplantation for PH, an overview of PH, discuss current best practices and look to the future for insights into the care of these patients.

Pulmonary Hypertension in Interstitial Lung Disease: Management Options to Move Beyond Supportive Care

“…Specific indications for referral to PH expert centers include (1) severe disease (PVR > 5 WU) and (2) decompensated right-heart failure [ 1 ••, 53 , 54 ]. Additionally, due to the significant impact of PH-ILD on quality of life and exercise capacity, even patients with the non-severe disease (PVR < 5 WU) who are transplant candidates should also be considered for referral for treatment optimization and lung transplant evaluation [ 1 ].…”

Lung Transplantation for Pulmonary Arterial Hypertension: Optimized Referral and Listing Based on an Evolving Disease Concept

“…Organ transplantation, including heart–lung transplantation (HLTx) and LTx for PAH, is a young field, the main principle of which is that transplant programs should be positively considered when patients with PAH continue to deteriorate despite receiving optimized medical treatment [ 2 , 9 ]. In 1963, James Hardy and colleagues performed the first human LTx.…”

“…In 1981, Stanford University performed the first successful HLTx on a patient with “primary pulmonary hypertension” and incorporated the concept of HLTx/LTx into clinical practice for severe PAH [ 40 ]. Shortly thereafter, with the first long-term successful single LTx and significant advances in bilateral sequential LTx, the selection of LTx has become recognized as an effective treatment for PAH [ 9 , 11 , 41 , 42 ].…”

“…Although transplant referrals are too aggressive for moderate-risk patients, given that LTx presents unique challenges in patients with PAH, we should recognize the need for early referral in this population [ 38 ]. It is worth mentioning that patients such as PVOD that are known to respond poorly to medical treatment should be referred promptly [ 9 , 54 ]. Patients with POVD/PCH may have a higher risk of dying on the waiting list compared to patients with PAH in the era of lung allocation.…”

“…It has been reported that patients with PH tend to have the highest mortality rate on LTx waiting lists, ranging from 18% to 41%. In addition to the inherent severity of the disease, another noteworthy reason lay in that many patients deteriorated very rapidly after prolonged medical treatment, and thus patients with severe PH were prone to miss the optimal referral and transplantation window on account of delayed referral and listing, as well as a shortage of donor organs [ 2 , 9 , 10 ]. Therefore, the contradiction between adequate medical treatment and the appropriate timing of LTx referral and listing is currently a highly concerning and highly controversial topic in the PH field.…”