Lung Transplantation in Pulmonary Arterial Hypertension: The Portuguese Experience

Margarida V. Matias, Zenito Cruz, Catarina Figueiredo, Catarina Moita, Miguel Roxo, João E. Reis, Ana Rita Costa, João Santos Silva, João Maciel Barbosa, Paulo Calvinho, Luísa Semedo

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Abstract

Background: In patients with pulmonary arterial hypertension (PAH), refractory to medical therapy, lung transplantation emerges as an option. This study describes the outcomes of 8 PAH patients who underwent lung transplantation. Methods: A retrospective, single-center study was conducted among patients with PAH who underwent lung transplantation in our center. Results: Patients had a median age of 46 years, with female sex predominance (75%). Causes of HAP were pulmonary veno-occlusive disease (n = 5, 62.5%), idiopathic PAH (n = 2, 25%), and heritable PAH (n = 1, 12.5%). Pre-transplant hemodynamics revealed a median mean pulmonary artery pressure of 58.5 mm Hg (48-86). All patients received bilateral lung transplants with extracorporeal membrane oxygenation support, displaying immediate post-transplant hemodynamic improvement. Primary graft dysfunction grade 3 (PGD 3) was observed in 75% of patients. Five patients (62.5%) died, with a 72.9% survival at 12 months and 29.2% at 24 months post-transplantation. Conclusion: Our study reveals the complexity and challenges of lung transplants in patients with PAH. Despite notable immediate hemodynamic improvements, high rates of PGD 3 and the survival rate remain a concern. Further research to define optimal peri and post-transplant management to improve survival is required.

Original languageEnglish
Pages (from-to)1115 - 1120
JournalTransplantation Proceedings
Volume56
Issue number5
Early online date2024
DOIs
Publication statusPublished - Jun 2024

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