A 36‐year‐old white man presented with a 4‐week history of palpitations, presyncope, and progressive dyspnea on exertion. He reported a significant decrease in his exercise tolerance over the last few months. Family history was significant for congestive heart failure in his father at age 56. Physical exam revealed a heart rate of 100 with a irregularly irregular rhythm. Blood pressure was 120/82. Serial cardiac biomarkers were within normal limits. Chest x‐ray was benign. An electrocardiogram revealed atrial fibrillation with rapid ventricular response. Transthoracic echocardiography showed an ejection fraction (EF) of 15% with severe global hypokinesis of the left ventricle with severe diastolic dysfunction. The left ventricle showed deep trabeculae and intertrabecular recesses in the inferolateral, anterior, middle, apical septum, and apex. Color Doppler showed blood flow within intertrabecular recesses. Findings were consistent with the diagnosis of noncompaction cardiomyopathy. Beta‐blockers were started for rate control, and anticoagulation with warfarin was initiated for prevention of thromboembolism.
Ventricular noncompaction is a rare form of a genetic car‐diomyopathy. It is an example of a pathological arrest in myocardial development characterized by a hypertrabeculated, thick myocardium with several intercommunicating recesses. Prevalence in the adult population is about 1 in 2000. Clinically patients present with heart failure symptoms including dyspnea, poor exercise tolerance in young people, lower‐extremity edema, and rarely with ventricular or atrial arrhythmias such as ventricular tachycardia, bundle branch block, Wolf‐Parkinson‐White syndrome, or atrial fibrillation. Transthoracic echocardiography is the modality of choice for diagnosis. Most commonly Stöllberger criteria are used to diagnose this condition: (1) more than 3 anatomically confirmed trabeculations within 1 imaging plane, apical to the insertion of the papillary muscles; (2) visualization of intertrabecular recesses perfused from the ventricular cavity on color Doppler imaging.
Atrial fibrillation in noncompaction cardiomyopathy is a rare complication. The clinical course can be worsened by clot formation in intertrabecular recesses. There are no randomized studies regarding the usage of anticoagulation in these patients. However, consensus among cardiovascular physicians suggests use of warfarin in these patients because of the increased risk of clot formation in the trabecular recesses in atrial fibrillation patients. Goal INR recommendations are between 2.0 to 3.0. The oral anticoagulation should also be considered in patients with a prior history of embolic phenomenon, confirmed left ventricular thrombi, or severely impaired left ventricular systolic function, but should not be routinely administered to all if asymptomatic.
S. Arora ‐ none; D. Bhatt ‐ none; A. Kumar ‐ none