Abstract 12051: Early Detection of Pediatric Idiopathic Pulmonary Arterial Hypertension by School Electrocardiography Screening: A Nationwide Survey in Japan
Background: For the early detection of cardiovascular diseases, school electrocardiography (ECG) screening has been executed for all the first graders in elementary, middle, and high schools all over Japan since 1995. We tested the hypothesis that this system may have an impact on the early recognition of pediatric pulmonary arterial hypertension (PAH).
Methods: We conducted a nationwide survey of incident cases of idiopathic or heritable PAH at 0.3-18 years of age in 150 official pediatric cardiology education hospitals in Japan during 2005-12. Pulmonary hypertension (PH) was defined by catheterization as mean pulmonary artery pressure (PAP) ≥ 25mmHg, pulmonary capillary wedge pressure ≤12 mmHg and pulmonary vascular resisitance (PVR) index ≥ 3 wood units/m2. Clinical data at PAH diagnosis, at the 1st follow-up catheterization, and at the last visit were collected.
Results: Among a total of 88 eligible patients (median age, 9.0 years; male/female, 47/41; disease-targeting therapy (DTT) at the 1st follow-up catheterization, 86/88; the follow-up period, 2.8 years ± 2.0) recruited, 53 (60%) patients were initially recognized by their symptoms (symptom group), 28 (32%) were indicated by the school ECG screening, and 7 (8%) were found in the screening examination, including X-ray, for various reasons (the latter two groups, designated as the screening group). The proportion of patients in functional class I/II was higher (94 % vs 56, p≤.001), plasma brain natriuretic peptide level was lower (190 ± 73 pg/ml vs 551 ± 132, p=.019) and 6-minute walk distance was longer (412 ± 22 m vs 336 ± 20, p=.015) at PAH diagnosis in the screening group than the symptom group. Cardiac index (3.3 ± 0.18 L/min/M2 vs 3.0 ± 0.15, p=.26), mean PAP (57 ± 2.8 mmHg vs 61 ± 2.5, p=.33), and PVR index (17 ± 1.6 Um2 vs 20 ± 1.5, p=.16) at diagnosis were similar between the two groups. The proportion of deaths/lung transplants during the study period was higher in the symptom group than in the screening group (15% vs 3, p=.03), although DTT was similarly performed (97% vs 98, p=.55).
Conclusions: The present findings suggest that school ECG screening may be relevant to the early recognition and treatment of pediatric PAH patients, who are associated with severe PH and preserved right heart function.
- © 2013 by American Heart Association, Inc.