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Rodriguez-Gonzalez M, Castellano-Martinez A, Grujic B, et al. Disopyramide as rescue treatment in a critically ill infant with obstructive hypertrophic cardiomyopathy refractory to beta blockers. J Cardiol Cases. 2017;15(6):209-213doi: 10.1016/j.jccase.2017.03.004.
Rodriguez-Gonzalez, M., Castellano-Martinez, A., Grujic, B., & Prieto-Heredia, M. A. (2017). Disopyramide as rescue treatment in a critically ill infant with obstructive hypertrophic cardiomyopathy refractory to beta blockers. Journal of cardiology cases, 15(6), 209-213. https://doi.org/10.1016/j.jccase.2017.03.004
Rodriguez-Gonzalez, Moises, et al. "Disopyramide as rescue treatment in a critically ill infant with obstructive hypertrophic cardiomyopathy refractory to beta blockers." Journal of cardiology cases vol. 15,6 (2017): 209-213. doi: https://doi.org/10.1016/j.jccase.2017.03.004
Rodriguez-Gonzalez M, Castellano-Martinez A, Grujic B, Prieto-Heredia MA. Disopyramide as rescue treatment in a critically ill infant with obstructive hypertrophic cardiomyopathy refractory to beta blockers. J Cardiol Cases. 2017 Apr 14;15(6):209-213. doi: 10.1016/j.jccase.2017.03.004. eCollection 2017 Jun. PMID: 30279782; PMCID: PMC6149641.
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J Cardiol Cases. 2017 Apr 14;15(6):209-213. doi: 10.1016/j.jccase.2017.03.004. eCollection 2017 Jun.

Disopyramide as rescue treatment in a critically ill infant with obstructive hypertrophic cardiomyopathy refractory to beta blockers.

Journal of cardiology cases

Moises Rodriguez-Gonzalez, Ana Castellano-Martinez, Branislava Grujic, María Azahara Prieto-Heredia

Affiliations

  1. Pediatric Cardiology Department, Hospital Universitario Puerta del Mar, Cadiz, Spain.

PMID: 30279782 PMCID: PMC6149641 DOI: 10.1016/j.jccase.2017.03.004

Abstract

Hypertrophic obstructive cardiomyopathy (HOCM) is the most common known cause of sudden death in children beyond infancy and in young athletes. Cases reported indicate that steroid-induced HOCM is usually a benign disorder. The normalization of cardiac morphological changes and clinical signs observed after the discontinuation of steroid therapy indicates that the effects on cardiac muscle are dose-dependent and reversible. However, the management of patients with symptomatic-HOCM presenting in infancy represents a major challenge because left ventricular outflow tract obstruction is a major risk factor associated with increased mortality in pediatric patients. We report a critically ill infant with steroid-induced HOCM resistant to beta-blockers who was successfully treated with disopyramide without relevant adverse events. Adult guidelines and pediatric experts suggest pharmacological therapy with beta-blockers or verapamil as the first- and second-line approach. However, these drugs are not always an option, especially in critical patients, hence, alternative therapeutic options are required. For these cases, disopyramide could be an alternative drug in spite of the little evidence on its safety and efficacy in pediatric patients. Our experience supports this cause, and the need for prospective studies on its use in the management of hypertrophic cardiomyopathy in children.

Keywords: Disopyramide; HCM, hypertrophic cardiomyopathy; HOCM, hypertrophic obstructive cardiomyopathy; Hypertrophic cardiomyopathy; LVOTO, left ventricular outflow tract obstruction; Left ventricular outflow tract obstruction; MV, mechanical ventilation; SCD, sudden cardiac death

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