Arrhythmogenic right ventricular cardiomyopathy/dysplasia

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a heart muscle disease clinically characterized by life-threatening ventricular arrhythmias. Its prevalence has been estimated to vary from 1:2,500 to 1:5,000. ARVC/D is a major cause of sudden death in the young and athletes. The pathology consists of a genetically determined dystrophy of the right ventricular myocardium with fibro-fatty replacement to such an extent that it leads to right ventricular aneurysms. The clinical picture may include: a subclinical phase without symptoms and with ventricular fibrillation being the first presentation; an electrical disorder with palpitations and syncope, due to tachyarrhythmias of right ventricular origin; right ventricular or biventricular pump failure, so severe as to require transplantation. The causative genes encode proteins of mechanical cell junctions (plakoglobin, plakophilin, desmoglein, desmocollin, desmoplakin) and account for intercalated disk remodeling. Familiar occurrence with an autosomal dominant pattern of inheritance and variable penetrance has been proven. Recessive variants associated with palmoplantar keratoderma and woolly hair have been also reported. Clinical diagnosis may be achieved by demonstrating functional and structural alterations of the right ventricle, depolarization and repolarization abnormalities, arrhythmias with the left bundle branch block morphology and fibro-fatty replacement through endomyocardial biopsy. Two dimensional echo, angiography and magnetic resonance are the imaging tools for visualizing structural-functional abnormalities. Electroanatomic mapping is able to detect areas of low voltage corresponding to myocardial atrophy with fibro-fatty replacement. The main differential diagnoses are idiopathic right ventricular outflow tract tachycardia, myocarditis, dialted cardiomyopathy and sarcoidosis. Only palliative therapy is available and consists of antiarrhythmic drugs, catheter ablation and implantable cardioverter defibrillator. Young age, family history of juvenile sudden death, QRS dispersion ≥ 40 ms, T-wave inversion, left ventricular involvement, ventricular tachycardia, syncope and previous cardiac arrest are the major risk factors for adverse prognosis. Preparticipation screening for sport eligibility has been proven to be effective in detecting asymptomatic patients and sport disqualification has been life-saving, substantially declining sudden death in young athletes

Low-dose acetyl salicylic acid versus oral anticoagulation after bioprosthetic aortic valve replacement. Final report of the ACTION registry.

BACKGROUND: The administration of antiplatelet agents versus anticoagulation after bioprosthetic aortic valve replacement (AVR) remains controversial. This study examined the safety and efficacy of anticoagulation with a vitamin-K antagonist (VKA) versus low-dose acetyl salicylic acid (ASA), up to 6 months after bioprosthetic AVR. METHODS: The ACTION Registry prospectively collected data at 47 medical centers in Europe, Canada and India. The investigators were free to prescribe the postoperative antithrombotic regimen of their choice. Between January 2006 and June 2009, 1118 patients underwent AVR alone or combined with coronary artery bypass graft (CABG), of whom 500 received a VKA and 618 received ASA. RESULTS: Patients who received VKA had a higher prevalence of peripheral vascular disease, chronic renal insufficiency and coronary artery disease. At 180 days, 14 anticoagulated patients (2.8%) suffered a thromboembolism (TE) versus 9 patients (1.5%) treated with ASA (P=0.12) and 18 anticoagulated patients (3.6%) suffered major bleeding (MB) versus 8 patients (1.3%) in the ASA group (P=0.01). MB or TE occurred in 31 patients (6%) treated with VKA versus 17 patients (2.8%) treated with ASA (P=0.003). By multiple variable analysis, preoperative cerebrovascular accident and peripheral vascular disease were strong predictors of postoperative TE, MB or both, in patients treated with VKA, though not in patients treated with ASA. CONCLUSIONS: Compared with ASA, treatment with VKA was associated with higher morbidity within 6 months after bioprosthetic AVR, suggesting that, particularly after concomitant CABG surgery, recipients of bioprosthetic AVR should receive prophylactic ASA instead of VKA