INSERM UMR_S 999 "Pulmonary Hypertension: Pathophysiology and Novel Therapies", Hôpital Marie Lannelongue, Le Plessis-Robinson, France.
Université Paris-Saclay, Faculté de Médecine, HPPIT, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Le Kremlin-Bicêtre, France.
Eur Respir J. 2024 Oct 31;64(4). doi: 10.1183/13993003.01193-2024. Print 2024 Oct.
Despite the progress made in medical therapies for treating pulmonary hypertension (PH), a subset of patients remain susceptible to developing a maladaptive right ventricular phenotype. The effective management of end-stage PH presents substantial challenges, necessitating a multidisciplinary approach and early identification of patients prone to acute decompensation. Identifying potential transplant candidates and assessing the feasibility of such a procedure are pivotal tasks that should be undertaken early in the treatment algorithm. Inclusion on the transplant list is contingent upon a comprehensive risk assessment, also considering the specific type of PH and various factors affecting waiting times, all of which should inform the decision-making process. While bilateral lung transplantation is the preferred option, it demands expert intra- and post-operative management to mitigate the heightened risks of pulmonary oedema and primary graft dysfunction in PH patients. Despite the availability of risk assessment tools, the occurrence of acute PH decompensation episodes can be unpredictable, potentially leading to refractory right ventricular failure even with optimal medical intervention, necessitating the use of rescue therapies. Advancements in right ventricular assist techniques and adjustments to graft allocation protocols for the most critically ill patients have significantly enhanced the survival in intensive care, affording the opportunity to endure while awaiting an urgent transplant. Given the breadth of therapeutic options available, specialised centres capable of delivering comprehensive care have become indispensable for optimising patient outcomes. These centres are instrumental in providing holistic support and management tailored to the complex needs of PH patients, ultimately enhancing their chances of a successful transplant and improved long-term prognosis.
尽管在治疗肺动脉高压 (PH) 的医学疗法方面取得了进展，但仍有一部分患者容易出现适应性不良的右心室表型。晚期 PH 的有效管理带来了巨大的挑战，需要多学科的方法和早期识别容易发生急性失代偿的患者。确定潜在的移植候选者并评估该手术的可行性是治疗算法早期应进行的关键任务。能否列入移植名单取决于全面的风险评估，同时还要考虑到特定类型的 PH 和影响等待时间的各种因素，所有这些因素都应告知决策过程。虽然双侧肺移植是首选，但需要专家进行术中及术后管理，以减轻 PH 患者肺水肿和原发性移植物功能障碍的风险。尽管有风险评估工具，但急性 PH 失代偿发作的发生可能是不可预测的，即使进行了最佳的药物干预，也可能导致难治性右心室衰竭，因此需要使用抢救治疗。右心室辅助技术的进步和对最危重患者移植供体分配协议的调整，极大地提高了重症监护的生存率，为等待紧急移植提供了机会。鉴于可供选择的治疗方法广泛，能够提供全面治疗的专门中心对于优化患者预后变得不可或缺。这些中心在为 PH 患者提供整体支持和管理方面发挥着重要作用，以满足他们的复杂需求，最终提高他们成功移植和改善长期预后的机会。
