Is it time for a paradigm shift: Should double‐lung transplant be considered the treatment of choice for idiopathic pulmonary arterial hypertension and giant pulmonary aneurysm?

Abstract

Idiopathic pulmonary arterial hypertension is a rare condition, frequently complicated by pulmonary arteries’ aneurysm. Aggressive medical therapy is often unsatisfactory and lung transplantation remains the only option. We report a unique case of severe idiopathic pulmonary arterial hypertension complicated by a giant pulmonary aneurism, massive pulmonary valve regurgitation, and right ventricle dysfunction. The patient was, as our first choice, listed for heart–lung transplantation and remained in emergency list for more than 7 months. Unfortunately, due to further clinical deterioration and the unavailability of a heart–lung bloc, plan B was mandatory. The patient underwent a combined procedure including: double lung transplant, pulmonary artery plasty, and sutureless pulmonary valve prosthesis with open deployment (first‐in‐man use in such scenario). Postoperative outcome was uneventful. Our thought is that double lung transplantation and conventional combined pulmonary artery/valve surgery should be considered as the first option avoiding excessive waiting times and potential further clinical deterioration.

Bilateral lung transplantation for pulmonary artery aneurysm with severe pulmonary hypertension: An evolution or a revolution?

“…Similarly, should a morphologically normal valve that has regurgitation because of annular dilatation be repaired or replaced, and if replaced, with what valve type? The case described in this issue of the journal by Pelenghi et al, 13 and other published reports do not provide us with satisfactory answers, and we may not have these answers any time soon because of the rarity of the condition. But we are confident that as more cases are studied and followed longitudinally, we will be able to provide better recommendations for our patients.…”

“…28 As the lung transplantation field matures and because of the severe shortage of heart-lung donors, BLT has replaced HLT as the primary treatment option for patients with pulmonary hypertension and preserved right and left ventricular (LV) function. 29 In patients with high-pressure PAA with poor RV or LV function 13 and other published reports do not provide us with satisfactory answers, and we may not have these answers any time soon because of the rarity of the condition. But we are confident that as more cases are studied and followed longitudinally, we will be able to provide better recommendations for our patients.…”

“…27 In the case reported in the current issue of the journal, Pelenghi et al, 13 elected to replace the PV by the open deployment of an injectable porcine pulmonic valve (No-React® Injectable Bio-Pulmonic™ valves) that was designed for percutaneous deployment. 13 The problem with this valve is that it has very poor longterm outcomes when deployed percutaneously in pediatric patients due to either malposition or neointimal proliferation. 17 It remains to be seen how this valve performs in an adult patient on immunosuppression.…”

“…25,26 When the pulmonary valve (PV) is competent, a valvesparing technique should be performed, whereas the valve is repaired or replaced if there is significant deformity and regurgitation. 27 In the case reported in the current issue of the journal, Pelenghi et al, 13 elected to replace the PV by the open deployment of an injectable porcine pulmonic valve (No-React® Injectable Bio-Pulmonic™ valves) that was designed for percutaneous deployment. 13 The problem with this valve is that it has very poor longterm outcomes when deployed percutaneously in pediatric patients due to either malposition or neointimal proliferation.…”

“…In this issue of the Journal of Cardiac Surgery , Pelenghi et al 13 reported a successful operation that includes bilateral lung transplant (BLT), pulmonary artery aneurysmorrhaphy, and pulmonary valve replacement in a patient with severe idiopathic pulmonary arterial hypertension complicated by a giant pulmonary aneurysm, pulmonary valve regurgitation (PVR), and right ventricle dysfunction. The patient was initially listed for heart–lung transplant (HLT), but because of the unavailability of a heart–lung donor and clinical deterioration after 7 months on the waiting list, the patient's listing status was converted to BLT.…”