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Calcific degeneration and rupture of the aortic valve and ascending aorta： from cardiac auscultation to multimodality imaging

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Journal of Geriatric Cardiology 2015年第5期12卷 580-583页

作者： Davide Stolfo Pasquale Gianfagna Enrico Fabris Davide Zanuttini gianfranco sinagra Alessandro Proclemer Gaetano Nucifora Department of Cardiology 'Ospedali Riuniti' and University of Trieste Trieste Italy Cardiothoracic Department University Hospital 'Santa Maria della Misericordia ' Udine Italy

A 76-year-old Caucasian woman was admitted to the emergency room and referred for cardiac evaluation for dyspnea and abrupt onset of cough three weeks ago. She had a history of well-controlled arterial hypertension and was on adequate oral anticoagulant therapy for permanent atrial fibrillation. Previous thoracic injuries, connective tissue disorders or recent infections were excluded. No chest pain or syncope was reported. Blood pressure was 150/50 mmHg in both arms, heart rate was 90 beats/min. Physical examination revealed bilateral pleural effusion and mild peripheral edema with slight jugular venous distension. A loud “wood-sawing” or “sea-gull” diastolic murmur over the right upper sternal border and mesocardium was present, raising the suspicion of severe aortic regurgitation due to possible calcific degeneration and laceration of the aortic valve. The ECG showed atrial fibrillation with aspecific repolarization abnormalities. The laboratory tests showed mild elevation of C-reactive protein (22 mg/L, normal value< 5 mg/L), with normal white blood cell count (8620/μL) and normal procalcitonin (0.05 ng/mL). Brain-natriuretic- peptide (BNP) was increased (233 pg/mL). Chest X-ray revealed bilateral pleural effusion and calcification of the aortic arch. Transthoracic echocardiography (Figure 1) showed a thickened and calcific aortic valve with severe regurgitation due to prolapse of the non-coronary cusp. A concomitant moderate aortic stenosis (aortic valve area: 1.4 cm~2) was present. Left ventricular size (end-diastolic diameter: 49 mm; end-diastolic volume: 78 mL) and ejection fraction (60%) were normal. A transesophageal echocardiography (TEE) was subsequently performed (Figure 2 and Movie 1 and 2), confirming the prolapse of the non-coronary cusp and showing its laceration with partial eversion of the medial portion in the outflow tract. Three-dimensional transverse section displayed the systolic motion of the flailing portion of the cusp. Moreover, TEE detec

关键词：主动脉心脏多模态破裂钙化成像听诊呼吸困难

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Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges

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World Journal of Cardiology 2014年第12期6卷 1234-1244页

作者： Bruno Pinamonti Francesca Brun Luisa Mestroni gianfranco sinagra Cardiovascular Department Ospedali Riuniti of Trieste34100Trieste Italy Cardiovascular Institute and Adult Medical Genetics Program University of Colorado Denver AMC Aurora CO 80045-2507 United States

Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement. Diagnosis of ARVC remains a clinical challenge mainly at its early stages and in patients with minimal echocardiographic right ventricular(RV) abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow ventricular tachycardia, Brugada syndrome, and myocarditis, due to arrhythmic expressivity and biventricular involvement. The identification of ARVC can be often challenging, because of the heterogeneous clinical presentation, highly variable intra- and inter-family expressivity and incomplete penetrance. This genotypephenotype "plasticity" is largely unexplained. A familial history of ARVC is present in 30% to 50% of cases, and the disease is considered a genetic cardiomyopathy, usually inherited in an autosomal dominant pattern with variable penetrance and expressivity; in addition, autosomal recessive forms have been reported(Naxos disease and Carvajal syndrome). Diagnosis of ARVC relays on a scoring system, with major or minorcriteria on the Revised Task Force Criteria. Implantable cardioverter defibrillators(ICDs) are increasingly utilized in patients with ARVC who have survived sudden death(SD)(secondary prevention). However, there are few data available to help identifying ARVC patients in whom the prophylactic implantation of an ICD is truly warranted. Prevention of SD is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are the mainstay of treatment of ARVC.

关键词： cardiomyopathy tachycardia Brugada diagnostic genetics familial unexplained outflow inherited challenging

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Treatment of Supraventricular Arrhythmias by Transcatheter Radiofrequency Ablation： The Experience of the Electrophysiology Unit of the University Hospital

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Journal of Pharmacy and Pharmacology 2017年第1期5卷 5-8页

作者： Zecchin Massimo Porto Andrea Bianco Elisabetta gianfranco sinagra Stroili Manuela Cardiovascular Department ASU1 of Trieste Trieste 34149 Italy Department of Technology Assessment ASU1 of Trieste Trieste 34125Italy

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