Frontiers in Cardiovascular Medicine (Sep 2023)

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease

  • Ayako Chida-Nagai,
  • Ayako Chida-Nagai,
  • Ayako Chida-Nagai,
  • Naoki Masaki,
  • Kay Maeda,
  • Konosuke Sasaki,
  • Hiroki Sato,
  • Hiroki Sato,
  • Jun Muneuchi,
  • Yoshie Ochiai,
  • Hiroomi Murayama,
  • Masahiro Tahara,
  • Atsuko Shiono,
  • Atsushi Shinozuka,
  • Fumihiko Kono,
  • Daisuke Machida,
  • Shinichi Toyooka,
  • Seiichiro Sugimoto,
  • Kazufumi Nakamura,
  • Satoshi Akagi,
  • Maiko Kondo,
  • Shingo Kasahara,
  • Yasuhiro Kotani,
  • Junichi Koizumi,
  • Katsuhiko Oda,
  • Masako Harada,
  • Daisuke Nakajima,
  • Akira Murata,
  • Hazumu Nagata,
  • Koichi Yatsunami,
  • Tomio Kobayashi,
  • Yoshikiyo Matsunaga,
  • Takahiro Inoue,
  • Hiroyuki Yamagishi,
  • Naomi Nakagawa,
  • Katsuki Ohtani,
  • Masaki Yamamoto,
  • Yushi Ito,
  • Tatsunori Hokosaki,
  • Yuta Kuwahara,
  • Satoshi Masutani,
  • Satoshi Masutani,
  • Koji Nomura,
  • Tsutomu Wada,
  • Hirofumi Sawada,
  • Masayuki Abiko,
  • Tatsunori Takahashi,
  • Yuichi Ishikawa,
  • Seigo Okada,
  • Atsushi Naitoh,
  • Takako Toda,
  • Tatsuya Ando,
  • Akihiro Masuzawa,
  • Shinsuke Hoshino,
  • Masaaki Kawada,
  • Yuichi Nomura,
  • Kentaro Ueno,
  • Naoki Ohashi,
  • Tsuyoshi Tachibana,
  • Yuchen Cao,
  • Hideaki Ueda,
  • Sadamitsu Yanagi,
  • Masaaki Koide,
  • Norie Mitsushita,
  • Kouji Higashi,
  • Yoshihiro Minosaki,
  • Tomohiro Hayashi,
  • Takashi Okamoto,
  • Kenji Kuraishi,
  • Eiji Ehara,
  • Hidekazu Ishida,
  • Hitoshi Horigome,
  • Takashi Murakami,
  • Kohta Takei,
  • Taku Ishii,
  • Taku Ishii,
  • Gen Harada,
  • Yasutaka Hirata,
  • Jun Maeda,
  • Shunsuke Tatebe,
  • Chiharu Ota,
  • Yasunobu Hayabuchi,
  • Hisanori Sakazaki,
  • Takashi Sasaki,
  • Keiichi Hirono,
  • Sayo Suzuki,
  • Masahiro Yasuda,
  • Atsuhito Takeda,
  • Madoka Sawada,
  • Kagami Miyaji,
  • Atsushi Kitagawa,
  • Yosuke Nakai,
  • Nobuyuki Kakimoto,
  • Kouta Agematsu,
  • Atsushi Manabe,
  • Yoshikatsu Saiki

DOI
https://doi.org/10.3389/fcvm.2023.1212882
Journal volume & issue
Vol. 10

Abstract

Read online

AimsLimited 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.MethodsThis 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.ResultsThe 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 (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).ConclusionsThe 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.

Keywords