Department of Respirology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuou-ku, Chiba, Chiba, 260-8670, Japan.
Department of Respirology, Nissan Tamagawa Hospital, Tokyo, Japan.
BMC Pulm Med. 2021 Mar 16;21(1):89. doi: 10.1186/s12890-021-01452-3.
Portopulmonary hypertension (PoPH) refers to the simultaneous presentation of pulmonary arterial and portal hypertension. However, few reports have included the characteristics and treatments for patients with PoPH of Asian population; thus, we investigated the clinical characteristics, treatment, and survival of these patients in a Japanese cohort.
Pulmonary arterial hypertension (PAH) has been included in the National Research Project on Intractable Disease in Japan; therefore, we extracted data of patients with PoPH from the forms of newly registered cases of the project from 2012 to 2013 (for 2 years), and updated cases of the project in 2013 (Study 1, n = 36 newly registered forms, n = 46 updated forms). Additionally, for Study 2, we performed a retrospective, observational cohort study at Chiba University Hospital (n = 11). We compared the characteristics between patients with PoPH and those with idiopathic/heritable PAH (I/H-PAH).
Both studies showed higher cardiac outputs (COs) and cardiac indexes (CIs), lower pulmonary vascular resistance (PVR), and less treated with combination therapy in patients with PoPH than those with I/H-PAH. In Study 2, the overall and disease-specific survival between PoPH and I/H-PAH were similar. Conversely, many patients (45%) had to change their PAH-specific medicine because of adverse effects.
As seen in western countries, Japanese patients with PoPH showed higher COs and CIs, better exercise tolerance, and lower PVRs than patients with I/H-PAH. Further studies are needed to improve PoPH treatments.
门脉高压性肺高血压（PoPH）是指同时存在肺动脉高压和门脉高压。然而，很少有报告包括亚洲人群 PoPH 患者的特征和治疗方法；因此，我们在日本队列中研究了这些患者的临床特征、治疗和生存情况。
肺动脉高压（PAH）已被纳入日本难治性疾病国家研究项目；因此，我们从该项目 2012 年至 2013 年（2 年）新登记病例的表格中提取了 PoPH 患者的数据，并更新了该项目 2013 年的病例（研究 1，n=36 份新登记表格，n=46 份更新表格）。此外，在研究 2 中，我们在千叶大学医院进行了回顾性观察队列研究（n=11）。我们比较了 PoPH 患者和特发性/遗传性 PAH（I/H-PAH）患者的特征。
两项研究均显示 PoPH 患者的心输出量（CO）和心指数（CI）较高，肺动脉阻力（PVR）较低，联合治疗的比例较低。在研究 2 中，PoPH 和 I/H-PAH 之间的总生存率和疾病特异性生存率相似。相反，许多患者（45%）因不良反应而不得不改变其 PAH 特异性药物。
与西方国家相似，日本 PoPH 患者的 CO 和 CI 较高，运动耐量较好，PVR 较低。需要进一步研究以改善 PoPH 的治疗方法。
