Left Ventricular Noncompaction Cardiomyopathy and Recurrent Polymorphic Ventricular Tachycardia: A Case Report and Literature Review

Abstract

Introduction: Noncompaction cardiomyopathy is a rare phenotype of cardiomyopathy associated with severe cardiac arrhythmia and thromboembolic complications.

Case presentation: A 55-year-old woman presented with frank pulmonary edema and received a diagnosis of noncompaction cardiomyopathy.

Discussion: Left ventricular noncompaction cardiomyopathy is increasingly being diagnosed because of advances in imaging modalities. It is important to differentiate this new phenotype of cardiomyopathy from others because its diagnosis, management, and prognosis differ. We reviewed the literature and summarized the diagnostic criteria, associated complications, initial and long-term management, and the recommendation for family screening.

Figure 1

Parasternal short-axis view of transthoracic echocardiogram with measurement of wall thickness showing A) normal nonaffected wall, B) noncompacted subendocardial layer, and C) thinner and compacted subepicardial layer. Ratio of B:A is greater than 2.

Figure 2

Transesophageal echocardiogram with wall thickness measurement showing A) normal nonaffected wall, B) noncompacted subendocardial layer, and C) thinner and compacted subepicardial layer. Ratio of B:A is greater than 2.

Figure 3

Cardiac magnetic resonance image showing short-axis of left ventricular apical myocardium. Compacted myocardium was 7.1 mm (bottom dashed line), and the noncompacted myocardium was 21 mm (top dashed line), resulting in a noncompacted-to-compacted ratio of approximately 3.0.