使用肺血管疾病指数预测先天性心脏病相关肺动脉高压的长期预后。
Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.
作者信息
Chida-Nagai Ayako, Masaki Naoki, Maeda Kay, Sasaki Konosuke, Sato Hiroki, Muneuchi Jun, Ochiai Yoshie, Murayama Hiroomi, Tahara Masahiro, Shiono Atsuko, Shinozuka Atsushi, Kono Fumihiko, Machida Daisuke, Toyooka Shinichi, Sugimoto Seiichiro, Nakamura Kazufumi, Akagi Satoshi, Kondo Maiko, Kasahara Shingo, Kotani Yasuhiro, Koizumi Junichi, Oda Katsuhiko, Harada Masako, Nakajima Daisuke, Murata Akira, Nagata Hazumu, Yatsunami Koichi, Kobayashi Tomio, Matsunaga Yoshikiyo, Inoue Takahiro, Yamagishi Hiroyuki, Nakagawa Naomi, Ohtani Katsuki, Yamamoto Masaki, Ito Yushi, Hokosaki Tatsunori, Kuwahara Yuta, Masutani Satoshi, Nomura Koji, Wada Tsutomu, Sawada Hirofumi, Abiko Masayuki, Takahashi Tatsunori, Ishikawa Yuichi, Okada Seigo, Naitoh Atsushi, Toda Takako, Ando Tatsuya, Masuzawa Akihiro, Hoshino Shinsuke, Kawada Masaaki, Nomura Yuichi, Ueno Kentaro, Ohashi Naoki, Tachibana Tsuyoshi, Cao Yuchen, Ueda Hideaki, Yanagi Sadamitsu, Koide Masaaki, Mitsushita Norie, Higashi Kouji, Minosaki Yoshihiro, Hayashi Tomohiro, Okamoto Takashi, Kuraishi Kenji, Ehara Eiji, Ishida Hidekazu, Horigome Hitoshi, Murakami Takashi, Takei Kohta, Ishii Taku, Harada Gen, Hirata Yasutaka, Maeda Jun, Tatebe Shunsuke, Ota Chiharu, Hayabuchi Yasunobu, Sakazaki Hisanori, Sasaki Takashi, Hirono Keiichi, Suzuki Sayo, Yasuda Masahiro, Takeda Atsuhito, Sawai Madoka, Miyaji Kagami, Kitagawa Atsushi, Nakai Yosuke, Kakimoto Nobuyuki, Agematsu Kouta, Manabe Atsushi, Saiki Yoshikatsu
机构信息
Department of Pediatrics, Hokkaido University, Sapporo, Japan.
Division of Cardiovascular Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
出版信息
Front Cardiovasc Med. 2023 Sep 4;10:1212882. doi: 10.3389/fcvm.2023.1212882. eCollection 2023.
AIMS
Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH.
METHODS
This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death.
RESULTS
The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 ( = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; = .009).
CONCLUSIONS
The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.
目的
关于先天性心脏病相关肺动脉高压（CHD-PAH）长期预后的危险因素，现有数据有限。我们重点关注了肺血管疾病指数（IPVD），这是一种用于评估肺动脉病理标本的系统。IPVD根据严重程度将肺血管病变分为四类：（1）无内膜增厚，（2）内膜细胞增厚，（3）内膜纤维增厚，（4）中膜破坏，总体分级表示为这些分数的相加平均值。本研究旨在探讨IPVD与CHD-PAH长期预后之间的关系。
方法
这项回顾性研究检查了764例年龄小于20岁的CHD-PAH患者的肺病理图像，这些患者的肺标本在2001年至2020年间提交至日本肺血管研究所进行肺病理检查。各主治医生回顾性收集临床信息。主要终点是心血管死亡。
结果
所有患者的5年、10年、15年和20年无心血管死亡生存率分别为92.0%、90.4%、87.3%和86.1%。IPVD≥2.0的组生存率明显低于IPVD<2.0的组（P=0.037）。经与肺动脉高压相关的先天性异常综合征的存在以及肺活检时的年龄校正后的Cox比例风险模型显示了相似的结果（风险比4.46；95%置信区间：1.45-13.73；P=0.009）。
结论
IPVD评分系统有助于预测CHD-PAH的长期预后。对于IPVD≥2.0的患者，应更仔细地考虑治疗策略，包括选择如肺动脉环扎术等姑息性手术来限制肺血流量以及推迟心内修复。
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