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.
- lung transplantation
- pulmonary arterial hypertension
- pulmonary artery aneurysm
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Cardiology and Cardiovascular Medicine