Diffuse mural thrombus in a patient with thoracic aortic aneurysm

Abstract

window in assessment of the LV apex lack of knowledge about this uncommon disease. Case report: A 29-year-old male with history of atypical chest pain was referred to our clinic. During his evaluation, intracardiac mass was discovered on a routine echocardiogram. There were no other symptoms of heart failure or coronary artery disease. Patient had no family history of heart disease or sudden death. On admission his physical examination was unremarkable. The 12-lead electrocardiography (ECG) showed normal sinus rhythm. Transthoracic echocardiogram and cardiac magnetic resonance imaging (MRI) were performed. Echocardiogram revealed a prominent cystic mass in the apical interventricular septum with normal left ventricular function, and color-doppler showed no flow within the cystic mass (Figure 1). MRI showed a typical pattern of noncompacted myocardium predominantly of the left ventricle (LV). Ratio of noncompacted to compacted area was 2.7 (pathological >2) (Figure 2). The LV was mildly dilated with preserved systolic function (LVEF: 50%). The ambulatory ECG documented sinus rhythm. There were no atrial fibrillation and ventricular arrhythmias. Lacking indications for more aggressive therapy, chronic anticoagulation with warfarin was prescribed. The patient was considered for regular follow-up to include assessment of exercise tolerance, measurement of ventricular size and function, and use of continuous ambulatory ECG. First degree relatives were screened with echocardiography. Discussion: Noncompaction of the ventricular myocardium is a recently recognized genetic cardiomyopathy. The prevalence is 0.014% of patients referred to the echocardiography laboratory, but the true prevalence is unclear. Clinical manifestations may range from being asymptomatic to presenting with heart failure, malignant arrhythmias, sudden cardiac death, or systemic thromboembolism. Echocardiography is the method of choice in establishing the diagnosis of ILVN. However, echocardiography depends on the experience and knowledge of the investigator and has poor echo window in assessment of the LV apex, which is the most commonly noncompacted area. MRI is especially useful when the myocardial involvement is subtle, and better distinguishes the compacted and non-compacted myocardial layers than echocardiography. In our patient, echocardiography revealed a cystic mass in the interventricular septum, but the MRI showed a typical pattern of noncompacted myocardium. Although echocardiography is the diagnostic method of ILVN, this case highlights the diagnostic benefits of MRI over echocardiography.