Abstract 4363851: Identification of ECG Abnormalities up to Two Years Prior to Symptomatic Manifestation of Pediatric Pulmonary Arterial Hypertension: A Japanese Nationwide School Screening Study

Background: Idiopathic/heritable pulmonary arterial hypertension (I/H-PAH), a disease with poor prognosis, often faces diagnostic delays due to nonspecific initial symptoms. In Japan, school ECG screening, performed in every 3 years for all students, identifies 41% of school-aged I/H-PAH patients; these typically show elevated pulmonary artery pressure (PAP) but have minimal symptoms (WHO-FC I/II) and preserved RV function. In this disease, the duration from the appearance of pulmonary vascular lesions to PH manifestation and the subsequent onset of RV failure are unknown. Objective: To address the precise pathological trajectory leading to PH manifestation, PH-related ECG findings at diagnosis (Dx) and pre-Dx in pediatric PAH patients were investigated utilizing Japan's school ECG screening system. Methods: Observational study of I/H-PAH patients (6-18 years), newly diagnosed 2005-2020, in Japanese pediatric cardiology teaching hospitals. School ECGs at/pre Dx were evaluated for PH-related findings (R≥1.5mV in V1, RV strain, Q in V1, IRBBB, inverted T waves) according to the criteria from the Japanese Society of Pediatric Cardiology and Cardiac Surgery. Results: In the survey, 118/143 institutions (82.5%) responded; 45 (38%) of those reported cases. 88 patients (51M, 37F; mean age 10.7y) were analyzed. Mode of detection of PAH were school ECG screening (n=36), symptoms (44), family history (5), and incidental findings (3). Compared to nonscreening group, the school screening group had higher proportion of WHO-FC I/II (91% vs 57%, p<.01) and lower plasma BNP (20 vs 102 pg/mL, p<.01), with comparable mean PAP (48 vs 55 mmHg, p=.65) and PVRI (12.1 vs 15.8 WU×m 2 , p=.38). PH-related ECG findings at Dx (n=87), which were present in 93% of all patients, were largely comparable between the groups (95% vs 91, p=.68), with the exception of higher prevalence of Q waves in V1 in the nonscreening (31% vs 15, p<.05) and R≥1.5mV in V1 in the screening group (59% vs 30, p<.05). Crucially, among 35 pre-Dx ECGs, PH-related abnormalities were observed in 77% (10/13) of those taken within 2 years of Dx, versus only 14% (3/22, p<.01) of those taken >2 years prior. Conclusion: Pre-Dx ECG analysis indicates PH-related findings can appear up to 2 years before symptom manifestation, suggesting a 1-2 year window of established PH with minimal symptoms in many pediatric patients. This highlights the potential for earlier diagnosis by performing ECG screening at even shorter intervals.

Authors

Institutions

Publication Details

Journal
Circulation
Published
2025-11-03
DOI
https://doi.org/10.1161/circ.152.suppl_3.4363851
Primary Topic
Pulmonary Hypertension Research and Treatments
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Abstract 4363851: Identification of ECG Abnormalities up to Two Years Prior to Symptomatic Manifestation of Pediatric Pulmonary Arterial Hypertension: A Japanese Nationwide School Screening Study

Kentaro Ueno, Kei Inai, Fujito Numano, Shinichi Takatsuki et al.
Circulation
Pulmonary Hypertension Research and Treatments
article

Abstract 4363851: Identification of ECG Abnormalities up to Two Years Prior to Symptomatic Manifestation of Pediatric Pulmonary Arterial Hypertension: A Japanese Nationwide School Screening Study

Kentaro Ueno, Kei Inai, Fujito Numano, Shinichi Takatsuki, H. Awano, Masako Harada, Hirofumi Sawada, Ryota Ebata, Takashi Kumamoto, Tatsunori Hokosaki, Hiroshi Ono, Hiromichi Hamada, Kenji Suda, Takahiro Mima, Kenji Waki, Norie Mitsushita, Jun Maeda, Hidekazu Ishida, Takashi Murakami, Tomoyuki Hasuwa, Gen Harada, Manatomo Toyono, Tetsu Takahashi, Shinsuke Hoshino, Hiroki Kuranobu, Yoshihide Mitani, M. Yokogawa, Keiko Uchida, Kentaro Ikeda, Masahiro Tahara, Hiroyuki Fukushima, Koichi Sagawa, Shigetoyo Kogaki, Shiro Baba, Takako Toda, Junko Shiono, Mitsuru Seki, Hiroyuki Yamagishi, Tatsuya Onishi, Jun Muneuchi, Kenichi Watanabe, Naoki Kuwabara, Hiroshi Suzuki, Masaki Yamamoto, Shin Takahashi, Tomotaka Nakayama, Kouji Higashi
article en

Abstract

Background: Idiopathic/heritable pulmonary arterial hypertension (I/H-PAH), a disease with poor prognosis, often faces diagnostic delays due to nonspecific initial symptoms. In Japan, school ECG screening, performed in every 3 years for all students, identifies 41% of school-aged I/H-PAH patients; these typically show elevated pulmonary artery pressure (PAP) but have minimal symptoms (WHO-FC I/II) and preserved RV function. In this disease, the duration from the appearance of pulmonary vascular lesions to PH manifestation and the subsequent onset of RV failure are unknown. Objective: To address the precise pathological trajectory leading to PH manifestation, PH-related ECG findings at diagnosis (Dx) and pre-Dx in pediatric PAH patients were investigated utilizing Japan's school ECG screening system. Methods: Observational study of I/H-PAH patients (6-18 years), newly diagnosed 2005-2020, in Japanese pediatric cardiology teaching hospitals. School ECGs at/pre Dx were evaluated for PH-related findings (R≥1.5mV in V1, RV strain, Q in V1, IRBBB, inverted T waves) according to the criteria from the Japanese Society of Pediatric Cardiology and Cardiac Surgery. Results: In the survey, 118/143 institutions (82.5%) responded; 45 (38%) of those reported cases. 88 patients (51M, 37F; mean age 10.7y) were analyzed. Mode of detection of PAH were school ECG screening (n=36), symptoms (44), family history (5), and incidental findings (3). Compared to nonscreening group, the school screening group had higher proportion of WHO-FC I/II (91% vs 57%, p<.01) and lower plasma BNP (20 vs 102 pg/mL, p<.01), with comparable mean PAP (48 vs 55 mmHg, p=.65) and PVRI (12.1 vs 15.8 WU×m 2 , p=.38). PH-related ECG findings at Dx (n=87), which were present in 93% of all patients, were largely comparable between the groups (95% vs 91, p=.68), with the exception of higher prevalence of Q waves in V1 in the nonscreening (31% vs 15, p<.05) and R≥1.5mV in V1 in the screening group (59% vs 30, p<.05). Crucially, among 35 pre-Dx ECGs, PH-related abnormalities were observed in 77% (10/13) of those taken within 2 years of Dx, versus only 14% (3/22, p<.01) of those taken >2 years prior. Conclusion: Pre-Dx ECG analysis indicates PH-related findings can appear up to 2 years before symptom manifestation, suggesting a 1-2 year window of established PH with minimal symptoms in many pediatric patients. This highlights the potential for earlier diagnosis by performing ECG screening at even shorter intervals.

CirculationVol. 152(Suppl_3)
Kurume University (JP), Kagoshima University (JP), University of Miyazaki (JP), Toho University (JP), University of Tsukuba (JP), Jichi Medical University (JP), Chiba University (JP), Shiga University of Medical Science (JP), Saga University (JP), Mie University (JP), Iwate Medical University (JP), Akita University (JP), Keio University (JP), Kyoto University (JP), Umma University (KE), Kurashiki Central Hospital (JP), Sasebo City General Hospital (JP), Toho University Omori Medical Center (JP), Chiba Kaihin Municipal Hospital (JP), Fukuoka Children's Hospital and Medical Center for Infectious Diseases (JP), Hiroshima General Hospital (JP), Kyoto City Hospital (JP), Kyushu Hospital (JP), Sapporo Minami Hospital (JP), Nagaoka Red Cross Hospital (JP), Tottori University Hospital (JP), Tottori Prefectural Central Hospital (JP), National Center For Child Health and Development (JP), Otsu Red Cross Hospital (JP), Gifu Prefectural General Medical Center (JP), Veterinary Medical Center (JP), Chiba Hospital (JP), Tokyo Women's Medical University (JP), Ibaraki Children's Hospital (JP), Shikoku Medical Center for Children and Adults (JP), Shizuoka Children's Hospital (JP), Yamagata University Hospital (JP), Nagasaki University (JP), University of Yamanashi (JP), Niigata University (JP), Kōchi University (JP), Yokohama City University (JP), The University of Osaka (JP)
Openalex Percentile: Top 54%
Pulmonary Hypertension Research and Treatments
DOISourceOpenAlex
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.