Detection of Pediatric Pulmonary Arterial Hypertension by School Electrocardiography Mass Screening

• Published in: American journal of respiratory and critical care medicine Vol. 199; no. 11; pp. 1397 - 1406
• Main Authors: Sawada, Hirofumi, Mitani, Yoshihide, Nakayama, Tomotaka, Fukushima, Hiroyuki, Kogaki, Shigetoyo, Igarashi, Takehiro, Ichida, Fukiko, Ono, Yasuo, Nakanishi, Toshio, Doi, Shozaburo, Ishikawa, Shiro, Matsushima, Masaki, Yamada, Osamu, Saji, Tsutomu
• Format: Journal Article
• Language: English
• Published: United States American Thoracic Society 01.06.2019
• Subjects: Achievement tests; Adolescent; Age; Cardiology; Cardiovascular disease; Child; Child, Preschool; Committees; Councils; Early Diagnosis; Electrocardiography; Electrocardiography - methods; Epidemiology; Familial Primary Pulmonary Hypertension - diagnosis; Female; Genomics; Heart surgery; Hemodynamics; Hospitals; Humans; Infant; Japan; Male; Mass Screening - methods; Mass Screening - statistics & numerical data; Medical prognosis; Medicine; Pediatrics; Population; Pulmonary arteries; Pulmonary hypertension; Retrospective Studies; School Health Services - statistics & numerical data; Schools; Society; Students; Veins & arteries; Japan; hypertension, pulmonary; mass screening; pediatrics; electrocardiography
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.201802-0375oc

To detect pulmonary arterial hypertension (PAH) at any early stage is a promising approach to optimize the outcome. To investigate the impact of school ECG-based screening on detecting idiopathic or heritable (I/H)-PAH in the general pediatric population. This was a nationwide survey of patients with I/H-PAH newly diagnosed at 3 months to 18 years of age in Japan during 2005-2012. Eighty-seven eligible patients (age range, 1-16 yr) were recruited. Among 68 (78%) patients diagnosed at greater than or equal to 6 years of age (the age of the first ECG-based screening), 28 (41%) were detected by the ECG-based screening (screening group) and 40 (59%) were recognized by their symptoms (  = 37) or coincidental occasions (  = 3; nonscreening group). In the screening group, the proportion of patients in World Health Organization functional class I/II at diagnosis was higher (96% vs. 60%;  < 0.001), plasma brain natriuretic peptide level was lower (149 ± 290 vs. 398 ± 559 pg/ml;  = 0.045), and 6-minute-walk distance was longer (420 ± 109 vs. 327 ± 104 m;  < 0.001) than the nonscreening group, despite similar values in mean pulmonary artery pressure (58 ± 17 vs. 61 ± 17 mm Hg;  = 0.42) and pulmonary vascular resistance index (18 ± 8 vs. 21 ± 11 Wood units ⋅ m ;  = 0.24) between groups. The proportion of patients on intravenous epoprostenol at the final visit was lower in the screening group than the nonscreening group (14% vs. 50;  = 0.004). These findings suggest that the ECG-based screening detects a unique subpopulation of pediatric patients with I/H-PAH that is associated with already established pulmonary hypertension but without obvious right heart failure and warrants investigating the prognostic significance of this system.