Management of Malignant Arrhythmia in Apical Hypertrophic Cardiomyopathy: A Case Report

Area of Science:

• Cardiology
• Genetics

Background:

• Apical hypertrophic cardiomyopathy (ApHCM) is a rare variant often lacking specific diagnostic criteria and risk stratification guidelines.
• It is less frequently associated with sarcomere mutations compared to other forms of hypertrophic cardiomyopathy.

Purpose of the Study:

• To present a case of ApHCM with complex comorbidities, focusing on clinical presentation and management.
• To emphasize the challenges in managing arrhythmogenic risks and refractory ventricular arrhythmias in ApHCM.

Main Methods:

• Case report of a 41-year-old male with type 1 diabetes and alcohol use disorder presenting with symptoms suggestive of ApHCM.
• Diagnostic evaluations included echocardiography, assessment of electrolyte imbalances, and monitoring for ventricular arrhythmias.
• Management involved intensive care, cardiopulmonary resuscitation (CPR), and implantation of an implantable cardioverter-defibrillator (ICD).

Main Results:

• The patient exhibited severe apical hypertrophy consistent with ApHCM, significant electrolyte imbalances, and non-sustained ventricular tachycardia (NSVT).
• He developed polymorphic ventricular tachycardia progressing to ventricular fibrillation (VF), requiring CPR and intensive care.
• An ICD was successfully implanted for secondary prevention of sudden cardiac death.

Conclusions:

• This case underscores the significant arrhythmogenic risks associated with ApHCM, including refractory ventricular arrhythmias.
• Tailored management strategies are crucial, particularly in patients with co-occurring substance use disorders where standard therapies may be contraindicated.
• Surgical options like apical myectomy may improve functional capacity in select ApHCM patients.