Patsiou Vasiliki, Arvanitaki Alexandra, Farmakis Ioannis T, Anthi Anastasia, Demerouti Eftychia, Apostolopoulou Sotiria, Feloukidis Christos, Gourgiotis Panagiotis, Papadopoulos Georgios E, Chrysochoidis-Trantas Thomas, Mpatsouli Athina, Zimpounoumi Natalia, Mouratoglou Sophia-Anastasia, Brili Styliani, Leontsinis Ioannis, Stamatopoulou Vaia, Mitrouska Ioanna, Frogoudaki Alexandra, Frantzeskaki Frantzeska, Tsangaris Iraklis, Simitsis Panagiotis, Karyofyllis Panagiotis, Bechlioulis Aris, Naka Katerina K, Ziakas Antonios, Manginas Athanasios, Giannakoulas George
AHEPA University General Hospital, Pulmonary Hypertension and Congenital Heart Disease Unit, Thessaloniki, Greece.
Center for Thrombosis and Hemostasis, University Medical Center Mainz, Germany.
Int J Cardiol. 2025 Feb 15;421:132894. doi: 10.1016/j.ijcard.2024.132894. Epub 2024 Dec 9.
Pulmonary arterial hypertension (PAH) is a severe complication among adult patients with congenital heart disease (ACHD). This study presents real-world data on risk stratification, pharmacotherapy and survival rates in PAH-ACHD.
Data from PAH-ACHD patients were analyzed using The Hellenic Pulmonary Hypertension Registry (HOPE), spanning eight specialized centers between 2015 and 2023. Patients were categorized into low, intermediate, and high-risk groups using the ESC/ERS three-strata model to assess 1-year mortality risk.
A total of 93 PAH-ACHD patients were included (median age 38.5 years, 60.2 % women). Most patients had an atrial septal defect (37.6 %) or a ventricular septal defect (35.5 %), with 11.8 % presenting with complex ACHD. Eisenmenger syndrome was present in 35.5 % of patients. The proportion of low-risk patients nearly doubled from first to last assessment (24.7 % vs. 40.9 %, p = 0.001). Initially, 52.7 % of patients were on PAH monotherapy, with a subsequent shift towards combination therapy (73.1 %) during follow-up. Over a median follow-up of 5.9 years, 29 patients (31.1 %) died, with 1- and 5-year survival rates of 95.5 % and 81.9 %, respectively. Compared to the high-risk group, low- and intermediate-risk patients exhibited a 70 % and 50 % lower hazard of death, respectively. The lowest survival rates were observed in patients with Eisenmenger physiology.
Survival prospects were favorable for the non-high risk patients in this nationwide cohort of PAH-ACHD patients. The observed shift towards combination therapy use may have contributed to the improvement in 1-year ESC mortality risk, underscoring the importance of timely combination therapy with PAH drugs.
肺动脉高压（PAH）是成人先天性心脏病（ACHD）患者的一种严重并发症。本研究展示了PAH-ACHD患者风险分层、药物治疗及生存率的真实世界数据。
使用希腊肺动脉高压注册研究（HOPE）分析2015年至2023年期间八个专科中心的PAH-ACHD患者数据。采用ESC/ERS三层模型将患者分为低、中、高风险组，以评估1年死亡风险。
共纳入93例PAH-ACHD患者（中位年龄38.5岁，60.2%为女性）。大多数患者患有房间隔缺损（37.6%）或室间隔缺损（35.5%），11.8%为复杂ACHD。35.5%的患者存在艾森曼格综合征。从首次评估到末次评估，低风险患者比例几乎翻倍（24.7%对40.9%，p = 0.001）。最初，52.7%的患者接受PAH单药治疗，随访期间随后转向联合治疗（73.1%）。中位随访5.9年，29例患者（31.1%）死亡，1年和5年生存率分别为95.5%和81.9%。与高风险组相比，低风险和中风险患者的死亡风险分别降低了70%和50%。艾森曼格生理状态的患者生存率最低。
在这个全国性的PAH-ACHD患者队列中，非高风险患者的生存前景良好。观察到的联合治疗使用转变可能有助于改善1年ESC死亡风险，强调了PAH药物及时联合治疗的重要性。
