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Stepwise Management of Refractory Ventricular Arrhythmias in Desmoplakin Cardiomyopathy: A Case Report
Grace Ibitamuno1, Erica D Wittwer1, Ruben J Crespo-Diaz2
1Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, USA.
Insights
Desmoplakin cardiomyopathy can cause severe heart failure and arrhythmias. A stellate ganglion block offered a temporary solution, acting as a bridge to heart transplantation when medications failed.
Area of Science:
- Cardiology
- Genetics
- Neurology
Background:
- Desmoplakin cardiomyopathy is a rare genetic disorder.
- It frequently causes dangerous arrhythmias, ventricular scarring, and heart failure.
- Managing electrical storms in these patients presents significant clinical challenges.
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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