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; Naoki Masaki; Kay Maeda; Konosuke Sasaki; 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; 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; 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 Sawai; Kagami Miyaji; Atsushi Kitagawa; Yosuke Nakai; Nobuyuki Kakimoto; Kouta Agematsu; Atsushi Manabe; Yoshikatsu Saiki

doi:10.3389/fcvm.2023.1212882

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, Naoki Masaki, Kay Maeda, Konosuke Sasaki, 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 KotaniJunichi 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, 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, 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 Sawai, Kagami Miyaji, Atsushi Kitagawa, Yosuke Nakai, Nobuyuki Kakimoto, Kouta Agematsu, Atsushi Manabe, Yoshikatsu Saiki^*

^*Corresponding author for this work

Itoigawa Community Medical Support Unit

Research output: Contribution to journal › Article › peer-review

Abstract

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 (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.

Original language	English
Article number	1212882
Journal	Frontiers in Cardiovascular Medicine
Volume	10
DOIs	https://doi.org/10.3389/fcvm.2023.1212882
State	Published - 2023

Keywords

congenital heart disease
index of pulmonary vascular disease
outcome
pediatrics
pulmonary arterial hypertension

ASJC Scopus subject areas

Cardiology and Cardiovascular Medicine

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10.3389/fcvm.2023.1212882

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Chida-Nagai, A., Masaki, N., Maeda, K., Sasaki, K., Sato, H., Muneuchi, J., Ochiai, Y., Murayama, H., Tahara, M., Shiono, A., Shinozuka, A., Kono, F., Machida, D., Toyooka, S., Sugimoto, S., Nakamura, K., Akagi, S., Kondo, M., Kasahara, S., ... Saiki, Y. (2023). Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease. Frontiers in Cardiovascular Medicine, 10, Article 1212882. https://doi.org/10.3389/fcvm.2023.1212882

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

abstract = "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 (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.",

keywords = "congenital heart disease, index of pulmonary vascular disease, outcome, pediatrics, pulmonary arterial hypertension",

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

note = "Publisher Copyright: 2023 Chida-Nagai, Masaki, Maeda, Sasaki, Sato, Muneuchi, Ochiai, Murayama, Tahara, Shiono, Shinozuka, Kono, Machida, Toyooka, Sugimoto, Nakamura, Akagi, Kondo, Kasahara, Kotani, Koizumi, Oda, Harada, Nakajima, Murata, Nagata, Yatsunami, Kobayashi, Matsunaga, Inoue, Yamagishi, Nakagawa, Ohtani, Yamamoto, Ito, Hokosaki, Kuwahara, Masutani, Nomura, Wada, Sawada, Abiko, Takahashi, Ishikawa, Okada, Naitoh, Toda, Ando, Masuzawa, Hoshino, Kawada, Nomura, Ueno, Ohashi, Tachibana, Cao, Ueda, Yanagi, Koide, Mitsushita, Higashi, Minosaki, Hayashi, Okamoto, Kuraishi, Ehara, Ishida, Horigome, Murakami, Takei, Ishii, Harada, Hirata, Maeda, Tatebe, Ota, Hayabuchi, Sakazaki, Sasaki, Hirono, Suzuki, Yasuda, Takeda, Sawai, Miyaji, Kitagawa, Nakai, Kakimoto, Agematsu, Manabe and Saiki.",

year = "2023",

doi = "10.3389/fcvm.2023.1212882",

language = "英語",

volume = "10",

journal = "Frontiers in Cardiovascular Medicine",

issn = "2297-055X",

publisher = "Frontiers Media SA",

}

Chida-Nagai, A, Masaki, N, Maeda, K, Sasaki, K, Sato, H, Muneuchi, J, Ochiai, Y, Murayama, H, Tahara, M, Shiono, A, Shinozuka, A, Kono, F, Machida, D, Toyooka, S, Sugimoto, S, Nakamura, K, Akagi, S, Kondo, M, Kasahara, S, Kotani, Y, Koizumi, J, Oda, K, Harada, M, Nakajima, D, Murata, A, Nagata, H, Yatsunami, K, Kobayashi, T, Matsunaga, Y, Inoue, T, Yamagishi, H, Nakagawa, N, Ohtani, K, Yamamoto, M, Ito, Y, Hokosaki, T, Kuwahara, Y, Masutani, S, Nomura, K, Wada, T, Sawada, H, Abiko, M, Takahashi, T, Ishikawa, Y, Okada, S, Naitoh, A, Toda, T, Ando, T, Masuzawa, A, Hoshino, S, Kawada, M, Nomura, Y, Ueno, K, Ohashi, N, Tachibana, T, Cao, Y, Ueda, H, Yanagi, S, Koide, M, Mitsushita, N, Higashi, K, Minosaki, Y, Hayashi, T, Okamoto, T, Kuraishi, K, Ehara, E, Ishida, H, Horigome, H, Murakami, T, Takei, K, Ishii, T, Harada, G, Hirata, Y, Maeda, J, Tatebe, S, Ota, C, Hayabuchi, Y, Sakazaki, H, Sasaki, T, Hirono, K, Suzuki, S, Yasuda, M, Takeda, A, Sawai, M, Miyaji, K, Kitagawa, A, Nakai, Y, Kakimoto, N, Agematsu, K, Manabe, A & Saiki, Y 2023, 'Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease', Frontiers in Cardiovascular Medicine, vol. 10, 1212882. https://doi.org/10.3389/fcvm.2023.1212882

TY - JOUR

T1 - Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease

AU - Chida-Nagai, Ayako

AU - Masaki, Naoki

AU - Maeda, Kay

AU - Sasaki, Konosuke

AU - Sato, Hiroki

AU - Muneuchi, Jun

AU - Ochiai, Yoshie

AU - Murayama, Hiroomi

AU - Tahara, Masahiro

AU - Shiono, Atsuko

AU - Shinozuka, Atsushi

AU - Kono, Fumihiko

AU - Machida, Daisuke

AU - Toyooka, Shinichi

AU - Sugimoto, Seiichiro

AU - Nakamura, Kazufumi

AU - Akagi, Satoshi

AU - Kondo, Maiko

AU - Kasahara, Shingo

AU - Kotani, Yasuhiro

AU - Koizumi, Junichi

AU - Oda, Katsuhiko

AU - Harada, Masako

AU - Nakajima, Daisuke

AU - Murata, Akira

AU - Nagata, Hazumu

AU - Yatsunami, Koichi

AU - Kobayashi, Tomio

AU - Matsunaga, Yoshikiyo

AU - Inoue, Takahiro

AU - Yamagishi, Hiroyuki

AU - Nakagawa, Naomi

AU - Ohtani, Katsuki

AU - Yamamoto, Masaki

AU - Ito, Yushi

AU - Hokosaki, Tatsunori

AU - Kuwahara, Yuta

AU - Masutani, Satoshi

AU - Nomura, Koji

AU - Wada, Tsutomu

AU - Sawada, Hirofumi

AU - Abiko, Masayuki

AU - Takahashi, Tatsunori

AU - Ishikawa, Yuichi

AU - Okada, Seigo

AU - Naitoh, Atsushi

AU - Toda, Takako

AU - Ando, Tatsuya

AU - Masuzawa, Akihiro

AU - Hoshino, Shinsuke

AU - Kawada, Masaaki

AU - Nomura, Yuichi

AU - Ueno, Kentaro

AU - Ohashi, Naoki

AU - Tachibana, Tsuyoshi

AU - Cao, Yuchen

AU - Ueda, Hideaki

AU - Yanagi, Sadamitsu

AU - Koide, Masaaki

AU - Mitsushita, Norie

AU - Higashi, Kouji

AU - Minosaki, Yoshihiro

AU - Hayashi, Tomohiro

AU - Okamoto, Takashi

AU - Kuraishi, Kenji

AU - Ehara, Eiji

AU - Ishida, Hidekazu

AU - Horigome, Hitoshi

AU - Murakami, Takashi

AU - Takei, Kohta

AU - Ishii, Taku

AU - Harada, Gen

AU - Hirata, Yasutaka

AU - Maeda, Jun

AU - Tatebe, Shunsuke

AU - Ota, Chiharu

AU - Hayabuchi, Yasunobu

AU - Sakazaki, Hisanori

AU - Sasaki, Takashi

AU - Hirono, Keiichi

AU - Suzuki, Sayo

AU - Yasuda, Masahiro

AU - Takeda, Atsuhito

AU - Sawai, Madoka

AU - Miyaji, Kagami

AU - Kitagawa, Atsushi

AU - Nakai, Yosuke

AU - Kakimoto, Nobuyuki

AU - Agematsu, Kouta

AU - Manabe, Atsushi

AU - Saiki, Yoshikatsu

N1 - Publisher Copyright: 2023 Chida-Nagai, Masaki, Maeda, Sasaki, Sato, Muneuchi, Ochiai, Murayama, Tahara, Shiono, Shinozuka, Kono, Machida, Toyooka, Sugimoto, Nakamura, Akagi, Kondo, Kasahara, Kotani, Koizumi, Oda, Harada, Nakajima, Murata, Nagata, Yatsunami, Kobayashi, Matsunaga, Inoue, Yamagishi, Nakagawa, Ohtani, Yamamoto, Ito, Hokosaki, Kuwahara, Masutani, Nomura, Wada, Sawada, Abiko, Takahashi, Ishikawa, Okada, Naitoh, Toda, Ando, Masuzawa, Hoshino, Kawada, Nomura, Ueno, Ohashi, Tachibana, Cao, Ueda, Yanagi, Koide, Mitsushita, Higashi, Minosaki, Hayashi, Okamoto, Kuraishi, Ehara, Ishida, Horigome, Murakami, Takei, Ishii, Harada, Hirata, Maeda, Tatebe, Ota, Hayabuchi, Sakazaki, Sasaki, Hirono, Suzuki, Yasuda, Takeda, Sawai, Miyaji, Kitagawa, Nakai, Kakimoto, Agematsu, Manabe and Saiki.

PY - 2023

Y1 - 2023

N2 - 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 (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.

AB - 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 (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.

KW - congenital heart disease

KW - index of pulmonary vascular disease

KW - outcome

KW - pediatrics

KW - pulmonary arterial hypertension

UR - http://www.scopus.com/inward/record.url?scp=85171374138&partnerID=8YFLogxK

U2 - 10.3389/fcvm.2023.1212882

DO - 10.3389/fcvm.2023.1212882

M3 - 学術論文

C2 - 37731527

AN - SCOPUS:85171374138

SN - 2297-055X

VL - 10

JO - Frontiers in Cardiovascular Medicine

JF - Frontiers in Cardiovascular Medicine

M1 - 1212882

ER -

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

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