부산대학교 도서관

72–year–old patient with paroxysmal atrial fibrillation and hypertension. Mother died suddenly at the age of 40 from an unknown cause. The patient worked at a high physical and emotional intensity (firefighter) and denies syncopal episodes. During an hospitalisation for pneumonia, an echocardiogram was performed that showed dilatation of the outflow tract and basal portion of the right ventricle (RV), apical bulging and right ventricular (RV) free wall dyskinesia. Absence of signs of pulmonary hypertension. The left ventricle was of normal size with preserved ejection fraction (EF 60%). the electrocardiogram showed inversion of the T wave from V1 to V4 and in the lower leads. He underwent a coronary CT scan, which was negative. In view of the clinical findings, cardiac MRI was requested, which showed severe dilatation of the free wall of RV, which was dyskinetic and hypertrabeculated and reduced RV EF (38%). At T1 sequences, diffuse and trasmural late gadolinium enahcement of the RV free wall. The Holter ECG recorded 773 monomorphic ventricular extrasystoles. The patient underwent genetic analysis, which is still in progress. This is an established case of arrhythmogenic right ventricular dysplasia that was accidentally diagnosed at an advanced age. This finding is unexpected as such a benign prognosis has rarely been described in the literature and in the natural history of this cardiomyopathy. In this particular situation, it is also very difficult to choose the best management strategy even in light of the recently published new guidelines.