P167 CARDIAC ARREST IN SUSPECTED LONG QT: APPROACH TO ARRHYTHMIC MITRAL VALVE PROLAPSE

Abstract

Abstract Mitral valve prolapse is a common condition. It affects 1–3% of the population. Clinical manifestations range from the absence of symptoms to sudden cardiac death. The arrhythmogenic mechanism is related to fibrotic changes in myocardial posterior caused by valve prolapse. ECG abnormalities indicative of high risk are inverted or biphasic T waves, ST depression, QT prolongation or dispersion, and BEV originating from the left outflow tract or papillary muscles. The echocardiogram allows the identification of mitral prolapse and disjunction of the mitral annulus (MAD), while MRI shows fibrosis. ICD implantation should be considered in high–risk patients with documented ventricular arrhythmias. Clinical case A 29–year–old woman with a family history of sudden cardiac death reported a state of absolute well–being until September 2022 when she presented with syncope with referred pulse failure for which she underwent cardiac massage, without defibrillation, by family members. At the arrival of 118 the patient presented bigeminal ventricular extrasystole with superior axis and BBsx type morphology (Fig.1). When she arrived at the hospital, ECG showed long QT (484 ms) associated with hypokalemia (2.8 mmol / l). During hospitalization, the coronary angiography was normal, while the cardiac MRI showed a globular and dilated left ventricle with preserved EF, thickening of the mitral leaflets, prolapse of both leaflets more evident at the level of P2/P3, MAD more evident in P3 with separation maximum end–systolic of 12 mm and absence of edema (Fig. 2). The patient underwent ICD implantation. In the presence of hypertelorism, prognathism, and flat feet, even in the absence of anomalies affecting the aorta and epiaortic vessels, scarring defects or ease to joint sprain, genetic screening was started for channelopathies and collagen diseases. Conclusions The approach to the patient with cardiac arrest and typical facies must be based on a systemic overview of the patient, whose phenotypic characteristics may suggest involvement of connective tissue diseases. In the future, the cardiologist must develop adequate knowledge to fully collect the information that the patient provides not only during instrumental examinations but also during an adequate clinical objective examination and facies, possibly supported by artificial intelligence.