Current therapeutic options in children with hypertrophic cardiomyopathy – own experience
Journal Title: Postępy Nauk Medycznych - Year 2014, Vol 27, Issue 9
Abstract
Introduction. The clinical course of hypertrophic cardiomyopathy (HCM) in children is very heterogeneous. In some children, the thickened heart muscle causes progressive heart failure and life-threatening arrhythmias. HCM is the most common cause of sudden cardiac death.Aim. The aim of study was retrospective analysis of clinical profile, current therapeutic options and sudden cardiac death risk assessment in children with HCM.Material and methods. We analyzed 108 children, mean age 9.7 ± 5.46 yrs with HCM diagnosed in the years 1991-2013. Mean follow-up was 6.4 ± 4.83 yrs. Patients demographics, clinical symptoms, treatment strategy as well as the results of echocardiography, ECG, 24 h Holter ECG, exercise test were analyzed.Results. Of the 108 patients studied 77 (71%) were treated only pharmacologically, in 4 (3.7) children RF catheter ablation was done, in 8 (7.4%) patients septal myectomy was performed. In 17 (15.7%) children with risk factors for sudden cardiac death ICD for primary (12/17) and secondary prevention (5/17) was implanted. The heart transplant was qualified in 6 (5.5%) patients who had symptoms of progressive heart failure. Among 108 patients 11 (10.6%) children died. The mean annual mortality rate was 1.34.Conclusions. In most children with HCM lifestyle modification and appropriate pharmacological therapy are sufficient, additional medical interventions are not necessary. In patients with symptomatic left ventricular outflow tract obstruction refractory to medical therapy a surgical myectomy should be considered. High-risk patients ought to be prospectively identified and ICD implantation should be considered, however further research is required to establish better criteria for primary prevention in children.
Authors and Affiliations
Lidia Ziółkowska, Monika Kowalczyk, Katarzyna Bieganowska, Wanda Kawalec
Keywords
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