Noncompaction Cardiomyopathy

Main Article Content

Michael W Luong
Silvana Mettini
Diego Delgado

Keywords

Noncompaction, cardiomyopathy, heart failure

Abstract

Noncompaction cardiomyopathy (NC) is an uncommon type of genetic cardiomyopathy that is characterized by trabeculations and recesses within the ventricular myocardium, most commonly affecting the LV. Patients typically present with heart failure symptoms and also often have concurrent diastolic and systolic dysfunction. In addition to developing heart failure, these patients appear to also be at risk for thromboembolic events or arrhythmias including atrial fibrillation or ventricular tachycardia. It is important to recognize this rare cause of heart failure because early diagnosis may lead to a better prognosis. Moreover, the management of these patients may differ from that of patients with other causes of cardiomyopathy. Criteria used to diagnose NC is not widely available but cardiac MRI and Doppler studies are important to perform. Similarly, there are no specific guidelines used for managing patients with NC leaving clinicians to formulate treatment strategies based on personal experience. In this case report, we present two cases along the spectrum of NC and make suggestions as to what we propose to be the most effective management strategy. We suggest the best way of treating NC patients is directly related to their condition based on their signs and presenting symptoms.