P KZN HCM

Clinical characteristics of hypertrophic cardiomyopathy in children; Eight‐year single center experience

Gulhan Tunca Sahin 

 

This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/ped.14393

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Abstract

Background

Hypertrophic cardiomyopathy (HCM) is the second most common pediatric cardiomyopathy. Although there are large body of literature about HCM in adults, it is limited in childhood. We evaluated various aspects of pediatric HCM patients treated at our center.

Patients and Methods

We identified 152 pediatric patients with HCM between October 2011 and October 2019. Besides clinical history, invasive and non‐invasive data were collected and evaluated.

Results

The mean age of patients was 8.9±5.7 years (1 month‐18 years) and 67.8% were male. The most frequent clinical symptoms were murmur and palpitations. Three cases (2%)had aborted sudden death as the first manifestation of HCM. Of these patients, 120(78.9%) were non‐syndromic HCM and 32(27.2%) were syndrome related . Asymmetric septal hypertrophy was common(48.3%) in non‐syndromic group, whereas concentric hypertrophy was common(56.2%) in syndromic group. Left ventricular outflow tract obstruction (LVOTO) in 39 (25.6%). Nine(5.9%) patients underwent electrophysiologic study and/or ablation and 16 patients had surgical intervention. ICD insertion was performed in 38 patients (26 transvenous, 12 epicardial). Three devices (7.9%) were inserted for secondary prevention; the remaining devices (92.1%) were inserted for primary prevention. Median follow up time was 27.1±22months. Five (3.3%) patients died during the follow up. No patients had heart transplantation or long‐term assist‐device.

Conclusion

The aetiology of HCM is heterogeneous and present at any age. It is important to determine timing of surgery and potential risks for sudden cardiac arrest. As most cases of HCM are familial, evaluation of family members at risk should be a routine component of clinical management.