A 31-year-old woman with a complaint of atypical chest pain but without dyspnea or palpitation was referred to our echocardiography laboratory for further evaluation. Her medical history was unremarkable, and her electrocardiography showed a normal sinus rhythm without any abnormal findings.
Transthoracic echocardiography revealed preserved systolic functions and normal sizes for both left and right ventricles as well as hypertrabeculation in the left ventricular apex (extending to the mid part of the inferior and lateral walls) and the right ventricular apex, with deep recess formation and flow within, suggestive of biventricular noncompaction cardiomyopathy [Video 1 and [Figure 1]]. The patient was referred for cardiac magnetic resonance imaging, which confirmed the transthoracic echocardiographic findings [Video 2 and [Figure 2]].
Figure 1: Transthoracic echocardiography in the four-chamber view, demonstrating hypertrabeculation with deep recesses. LV, left ventricle; RV, right ventricle
The left ventricular noncompaction is a rare cardiomyopathy characterized by noncompacted thickness-to-compacted thickness ratio in systole of more than 2, deep recesses, and blood flow in these recesses, and apical and mid-ventricular segments are the most commonly affected places. The left ventricular noncompaction can present with heart failure, arrhythmia, and thromboembolism, and even sudden cardiac death may occur in association with other congenital heart disease. Favorable remodeling and improvement in ventricular systolic function is documented with appropriate medical therapy in patients with reduced systolic function and dilated ventricle.
The right ventricular noncompaction cardiomyopathy is rare and biventricular involvement is rarer. The reliable criteria for definition and clinical diagnosis of the right ventricular noncompaction cardiomyopathy are not accessible, but significant trabeculation in two-third of the right ventricular surface can be used as helpful criteria. We should remember that right ventricular hypertrabeculation might be a result of right ventricular volume or pressure overload secondary to left heart side abnormalities, and overdiagnosis of the right ventricular noncompaction should be reduced by pathologic or imaging studies such as cardiac magnetic resonance imaging. Although biventricular cardiomyopathy is a rare condition, cardiologists should consider the possibility of its presence in their daily routine practice.