Non - Compaction Cardiomyopathy in an Apparently Healthy Patient: A Case Report

Non-compaction cardiomyopathy is a rare genetic cardiomyopathy caused by a disorder during endocardial morphogenesis and could be accompanied by life-threatening complications on presentation. The major clinical manifestations of Non-compaction cardiomyopathy are heart failure, arrhythmias, embolic events and sudden death. The left ventricle is the most commonly reported affected site, but a few cases of right ventricular involvement have also been reported. We describe the perioperative management of a young patient who was posted for total abdominal hysterectomy, with no co morbid conditions, unremarkable pre anesthetic evaluation and normal investigations, who developed congestive heart failure in the post-operative period. The diagnosis was confirmed after an emergency echocardiogram. Despite an increasing awareness and interest in this anomaly, however, there is still little knowledge regarding diagnostic criteria, symptoms and prognosis of this rare and unique congenital disorder categorized as unclassified cardiomyopathy. The rarity of this condition has limited case reports and perioperative guidelines in the literature. Diagnosing and differentiating this uncommon condition from other forms of cardiomyopathy are important as treatment and prognosis may differ significantly. Our current understanding of isolated left ventricular non-compaction, including diagnostic criteria, management and prognosis, is discussed. The prognosis of the disease is determined by its complications; the death rate and heart transplantation frequency can be as high as 50%.