Title : Into the Wall: Left Ventricular Noncompaction
Abstract:
Left ventricular noncompaction (LVNC) is a rare, congenital disorder affecting the lining of myocardial tissue. This distinct phenotype includes blood-filled trabeculations and deep recesses in the uncompressed endocardial wall with a thin, compact mesocardium. Clinical presentation ranges from asymptomatic to heart failure, lethal arrhythmias and thromboembolism. A validated and accepted criteria for radiographic diagnosis describes the ratio of noncompacted to compacted myocardium >2:1. LVNC can be sporadic or familial, though specific environmental triggers have not clearly been established.
Case Presentation:
A 22-year-old Hispanic male with history of end-stage renal disease secondary to glomerulonephritis, severe COVID-19 pneumonia and recent diffuse alveolar hemorrhage currently on prolonged glucocorticoid taper presented with acute heart failure. Echocardiogram demonstrated cardiomyopathy with left ventricular ejection fraction (LVEF) 35-40%, mild LV hypertrophy and moderate global hypokinesis. Lack of ischemic risk factors led to evaluation with cardiac MRI. Findings of mid-to-apical LV hypertrabeculation and noncompacted to compacted myocardial ratio of 3 were consistent with LV noncompaction. Arrhythmias or thromboembolic events were not observed. Guideline-directed medical therapy was initiated along with referral for genetic testing.
Discussion:
Left ventricular noncompaction is an uncommon etiology of cardiomyopathy. Recognition is valuable due to known complications, including sudden cardiac death, arrhythmia and stroke. The pathogenesis underlying this young adult’s phenotype is not clearly identified. His symptoms developed in the setting of COVID-19 pneumonia, diffuse alveolar hemorrhage with significant anemia and prolonged steroid exposure. These correlates have not previously been described in association with LV noncompaction and their significance is unknown.
