Stepwise Management of Refractory Ventricular Arrhythmias in Desmoplakin Cardiomyopathy: A Case Report

桥粒蛋白心肌病难治性室性心律失常的分步治疗:病例报告

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Abstract

Desmoplakin cardiomyopathy is a rare, genetic condition that often leads to dangerous arrhythmias, ventricular scarring, and eventual heart failure. Here, the case of a 50-year-old woman suffering from both ventricular storm and biventricular heart failure is discussed. Her ventricular tachycardia proved resistant to several antiarrhythmic medications, which made it difficult to maintain proper cardiac output. To stabilize her condition temporarily, a stellate ganglion block was performed until she could undergo successful orthotopic heart transplantation. When antiarrhythmic drugs are unsuitable or fail to control electrical storms, stellate ganglion blockade presents a valid alternative. For patients with desmoplakin cardiomyopathy, especially when combined with mechanical circulatory support, this approach can serve as an effective bridge to cardiac transplant.

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