C83. Recurrent Syncope in Isolated Right Ventricular Non-Compaction Cardiomyopathy: A Case Report

Isolated right ventricular non-compaction cardiomyopathy (iRVNC) is a rare disease, and often under diagnosed. The patients might come with right heart failure, ventricular arrhythmias and systemic embolism. A 64 –yo female presented with episodes of recurrent syncope. There is thickened trabecular on apical right ventricular (RV), moderate tricuspid regurgitation and moderate probability of pulmonary hypertension on echocardiography. Furthermore, cardiac MRI on this patient confirned hypertrabeculated and dilatation of RV, spongiform appearance of the RV apical, non-compacted (NC) thickness of the RV was 23.5 mm at the apical level with compacted thickness (C) of 2 mm (NC to C ratio 11,7), hypertrophic moderator band, and dilatation RA and global hypokinetic and decreased function of RV. Hence, the diagnosis was an isolated right ventricular non-compaction cardiomyopathy, without LV involvement. The medication given including sildenafil, nebivolol, spironolactone, digoxin and edoxaban. Lesson learned from this case, syncope might be one of iRVNC symptoms. Multiple modalities could be needed for diagnostic confirmation for patients with syncope. Echocardiography as a diagnostic tool is useful to find prominent trabeculations and when NC/C ratio >2. If the echocardiographic findings were inconclusive, cardiac MRI was could be performed for better discrimination between compact and non-compact inner and outer layers of myocardium with NC/C ratio > 2,3.