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Electrocardiographic changes and long-term prognosis of children diagnosed with hypertrophic cardiomyopathy by the school screening program for heart disease in Japan.

Journal of Cardiology 2019 December 11
BACKGROUND: In Japan, the school screening program for heart disease (SS) has been performed since 1973. However, little has been reported on the electrocardiogram (ECG) changes and long-term prognosis in patients with hypertrophic cardiomyopathy (HCM) detected by the SS.

METHODS: All 44 consecutive pediatric HCM patients (10.1 ± 3.0 years old), who had been originally consulted by the SS before the diagnosis of HCM from April 1981 to April 2017, were reviewed retrospectively.

RESULTS: At the SS, all patients showed mild or no symptoms. All patients showed ECG abnormalities, and 75 % had a high proposed ECG risk score (≧6). However, 30 % of them had no echocardiogram finding of myocardial hypertrophy. During the follow-up period (14.8 ± 10.0 years), life-threatening events (LTE) occurred in 11 (25 %) patients, and the first LTE occurred during exercise in 8 (18 %). The estimated LTE and heart failure death-free survival rate at 10 years was 64.9 %. The LTE-free survival rate was lower in patients without than in those with myocardial hypertrophy at the SS.

CONCLUSIONS: The SS was useful in detecting patients with HCM with mild or no symptoms at the early stage. However, our study indicated that early detection of HCM is not associated with improvement in the prognosis of the patients. Further studies are needed.

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