CONGRESS ABSTRACT

A case of a 30-yo woman with bileaflets mitral valve prolapse (MVP) and minimal regurgitation is presented. She complained of palpitations and prelipotimic states. She experienced sporadic dizziness, forcing her to sit down to prevent her from falling to the ground. Clinical exam was normal; ECG showed frequent polymorphic premature ventricular complexes (PVC). Holter exam showed 17% complex ventricular arrhythmias (VA): polymorphic, bi- and trigeminate PVCs and bursts of unsustained polymorphic ventricular tachycardia. Exercise ECG test showed PVC accentuation and the presence of coupled PVCs. Echocardiogram (US) showed bileaflets prolapse, trivial regurgitation, MAD, curling and pickelhaube helmet sign (PKHS). Cardiac chambers were normal. The geometric analysis of MV showed the absence of the coaptation triangle (CPT  ⃤ ). CMR showed areas of myocardial fibrosis (MF) on inferior basal wall, near the MV hinge: Fig 1. Based on the presence of MVP, with MAD, curling and PKHS, severe VA, with MF at CMR, the diagnosis of malignant MV prolapse was done. The Heart Team elected to perform minimally invasive mitral repair surgery, even if MV regurgitation was minimal. A limited triangular resection of P2 scallop was done, a pair of Gore-Tex cords were implanted on P2 and also a prosthetic mitral ring. MV geometry was reconstructed by moving the coaptation plane into the LV inflow chamber. Curling, MAD and PKHS disappeared after surgery. Palpitations and prelipotimia vanished without therapy as well. On exercise ECG, isolated PVCs were found, and on ECG Holter PCV was reduced to 1.7%. CONCLUSIONS US identifies patients (pts) with MVP at risk of sudden death by grouping of MAD + Curling + PKHS.  Complex VA and the presence of MF on CMR are mandatory criteria for the diagnosis of malignant MVP. By analyzing MV geometry focused on the mechanic of posterior mitral leaflets, US detects also the mechanism of MF: the conversion of a grade 3 lever – from a normal MV, to a grade 1 lever in pts with malignant MVP. In both cases the fulcrum of the lever always remains on the MV ring. From a geometrical point of view, before surgery, the coaptation plane of the MV was abnormal, due to the loss of the CPT  ⃤ . Surgery restores the normal MV geometry, bringing the coaptation into the LV inflow chamber: Fig 2. In this case, surgery was chosen as the only solution to break the malignant vicious circle caused by the abnormal posterior leaflet mechanics and abnormal MV geometry.