Multimodality imaging of mid-ventricular obstruction in left ventricular noncompaction

Noncompaction cardiomyopathy (NCM) is a myocardial disorder, which is thought to occur due to the failure of left ventricle (LV) compaction during embryogenesis, leading to distinct morphological characteristics in the ventricular chamber. The exact prevalence of NCM is still unknown. Several limitations for this assessment are the different diagnostic criteria, the heterogeneous populations, and the retrospective design of most studies. The reported prevalence of NCM in patients referred to echocardiography laboratories ranges between 0.014 and 1.26%. We report of a 42-year-old woman that underwent transthoracic echocardiography for the recent onset of a transient ischemic attack. Echo images revealed a rare case of apical left ventricular noncompaction (LVNC) (Panel A, Supplementary data online, Video S1) with mid-ventricular obstruction (white arrows). A mid-ventricular diastolic gradient of 20 mmHg was present after Valsalva maneuver at pulsed-wave Doppler analysis (Panel B). A diastolic gradient was also present during ventricular ectopic beats (Panel C) with mild increase in the systolic gradient (Panel D). Cardiac magnetic resonance (CMR) two-chamber views (Panel E: diastole; Panel F: systole) confirmed apical LVNC (Supplementary data online, Video S2). Late-gadolinium analysis (Panels G and H) showed a large area of apical myocardial fibrosis compatible with LV aneurysm. Usually, LVNC leads to systolic heart failure and sudden cardiac death. The presence of mid-ventricular obstruction with apical aneurysm is a rare condition with important prognostic and therapeutic implications in LVNC. Multimodality cardiac imaging with CMR integration could better identify patients with large extension of myocardial fibrosis, an important marker for systolic dysfunction progression and ventricular arrhythmias. Moreover, CMR could be more sensitive than echocardiography alone in detecting apical thrombi. Supplementary data are available at European Heart Journal – Cardiovascular Imaging online.