Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease
Authors: 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 , Sawada Madoka , Miyaji Kagami , Kitagawa Atsushi , Nakai Yosuke , Kakimoto Nobuyuki , Agematsu Kouta , Manabe Atsushi , Saiki Yoshikatsu 
Published: September 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 &lt;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 &lt;2.0 (P = .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; P = .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.