Associazione Nazionale Medici Cardiologi Ospedalieri

CONGRESS ABSTRACT

CONGRESS ABSTRACT

BARLOW CARDIOMYOPATHY CAUSING CARDIAC ARREST: A CASE REPORT

Fabrizio Cola Padova (Padova) – Aopd | Barbara Bauce Padova (Padova) – Padova | Perazzolo Marra Martina Padova (Padova) – Padova | De Lazzari Manuel Padova (Padova) – Padova | Corrado Domenico Padova (Padova) – Padova | Nai Fovino Luca Padova (Padova) – Padova

A 31 years old male patient experienced witnessed cardiac arrest (CA), with ventricular fibrillation (VF) as first detected rhythm. Return of spontaneous circulation (ROSC) was achieved after CPR, administration of adrenaline, amiodarone and 3 direct-current defibrillation shocks. ECG after ROSC was negative for STEMI showing sinus tachycardia, first degree AVB, intraventricular conduction delay, flattened T wave in leads I and aVL. On the same day, the patient was transferred to our cardiology intensive care unit mechanically ventilated and supported with dobutamine, both suspended after the first day of hospital stay. Brain CT and CNGF were negative, while thorax CT showed extensive bilateral posterior consolidation areas plus air bronchogram. Hospitalization therapy consisted of bisoprolol and double antibiotic strategy for febrile episodes and temporary septic state due to urinary tract infection; telemetry monitoring detected no VA episodes. TEE showed LV ejection fraction on the lower limit (52%) with diffuse hypokinesia, moderate MR and a normal right ventricular function. For excluding acute myocarditis with arrhythmic presentation, endomyocardial biopsy was performed, yielding negative results. Cardiac magnetic resonance (CMR) demonstrated normal LV dimensions, mildly reduced LVEF (47%) with hypokinesia of anterior-lateral mid-apical portion, basal hypertrophy with associated adjacent mid-portion thinning, normal RV dimensions with mildly reduced EF (46%) due to diffuse hypokinesia, mild mitral valve prolapses of both not particularly thickened leaflets, MAD with a maximum tele-systolic extension of 11 mm, curling of the inferior-lateral basal region. Tissue characterization showed normal T1 and T2 mapping values and non ischemic, intramyocardial late gadolinium enhancement in the inferior-lateral basal wall. Such findings were contextualized as part of Barlow Cardiomyopathy. Bearing in mind the CA, the young age of the patient and the CMR result, SICD therapy for secondary prevention was adopted. Discharge therapy implied bisoprolol. During post-discharge follow-up visit, neither arrhythmias nor interventions were detected on SICD, TEE showed normal bi-ventricular systolic function with mild MR (F4). ECG showed sinus rhythm, HR 75 bpm, first degree AVB, intraventricular conduction delay, isolated flattened T wave in lead III. The cardiomyopathy genetic panel yielded negative results and drug therapy was not changed till next follow up visit.