Noncompaction cardiomyopathy: Manifestation as a surgical pitfall – rare but real

Abstract

Noncompaction cardiomyopathy is characterized by hyper-trabeculation of the myocardium. The patients present with heart failure and variable combination of arrhythmias and thromboembolism. Although several articles have shed light on the medical aspect of this disease, none have highlighted its surgical relevance. A death following ligation of a patent ductus arteriosus prompted us to evaluate the surgical aspects of this disease. Autopsy records from 2003 to 2012 were reviewed, and cases identified as noncompaction cardiomyopathy were retrieved and analyzed. Cases with obligatory hyper-trabeculation were excluded. Thirteen patients were found to have noncompaction cardiomyopathy in 9427 autopsies performed. Their ages ranged from 4 months to 55 years; 10 were children, and all 3 adults were over 45 years of age. Only one patient had an antemortem diagnosis of noncompaction cardiomyopathy on echocardiography. There were 7 postoperative deaths. Although noncompaction cardiomyopathy is rare, missing its diagnosis in a patient operated on for another indication can spell disaster postoperatively. As the clinical picture is nonspecific and the disease is not very well known, it needs vigilance on the part of the echocardiographer and surgeon.