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Varna Medical Forum

Therapeutic approach to idiopathic hypertrophic cardiomyopathy

K. Ganeva, P. Shivachev, L. Marinov, M. Bojadziev, M. Ivanova

Abstract

Idiopathic hypertrophic cardiomyopathy is a rare disease in childhood with a high potential of a lethal outcome. The hallmark of the disorder is myocardial hypertrophy that occurs in the absence of an obvious hemodynamic stimulus. Since the first case of an experimental treatment of an infant described in 1971, beta blockers have become one of the leading medication options with variations of the dose regimens. According to the current literature data and clinical trials the use of beta blockers in high doses is consistent with a high percentage of survival.

We`re presenting a clinical case of an 11-month old infant, who was admitted to our clinic with symptoms of congestive cardiac failure. The chest radiography and echocardiography results showed a severe symmetric hypertrophic cardiomyopathy without an obstruction in the left ventricular outflow tract. The treatment was started with Propranolol in a titrating dose until reaching the dose of 5mg/kg/24hours. Several weeks later, echocardiography examination showed a significant improvement in the left ventricle diastolic function with some degree of regression of myocardial hypertrophy.

The use of high doses of beta blockers together with the monitoring of the clinical state could improve the prognosis and survival in patients with idiopathic hypertrophic cardiomyopathy.


Keywords

hypertrophic cardiomyopathy, beta blockers, survival

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References

2011 ACCF/AHA Guideline for diagnosis and treatment of hypertrophic cariomyopathy.

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DOI: http://dx.doi.org/10.14748/vmf.v5i0.2914

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