Detection of Pediatric Pulmonary Arterial Hypertension by School Electrocardiography Mass Screening

Abstract

<b>Rationale:</b> To detect pulmonary arterial hypertension (PAH) at any early stage is a promising approach to optimize the outcome. <b>Objectives:</b> To investigate the impact of school ECG-based screening on detecting idiopathic or heritable (I/H)-PAH in the general pediatric population. <b>Methods:</b> 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. <b>Measurements and Main Results:</b> 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 (<i>n</i> = 37) or coincidental occasions (<i>n</i> = 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%; <i>P</i> < 0.001), plasma brain natriuretic peptide level was lower (149 ± 290 vs. 398 ± 559 pg/ml; <i>P</i> = 0.045), and 6-minute-walk distance was longer (420 ± 109 vs. 327 ± 104 m; <i>P</i> < 0.001) than the nonscreening group, despite similar values in mean pulmonary artery pressure (58 ± 17 vs. 61 ± 17 mm Hg; <i>P</i> = 0.42) and pulmonary vascular resistance index (18 ± 8 vs. 21 ± 11 Wood units ⋅ m²; <i>P</i> = 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; <i>P</i> = 0.004). <b>Conclusions:</b> 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.