Lack of current implantable cardioverter defibrillator guidelines application for primary prevention of sudden cardiac death in Latin American patients with heart failure: a cross-sectional study.

AIMS: This cross-sectional study evaluated the application of accepted international implantable cardioverter defibrillator (ICD) guidelines for primary prevention of sudden cardiac death in patients with heart failure. METHODS AND RESULTS: The PLASMA (Probabilidad de Sufrir Muerte Arritmica) study was designed to characterize management of cardiac patients in Latin America. Twelve centres included 1958 consecutively admitted patients in cardiology units in 2008 and 2009. Discharged patients were evaluated for primary prevention, ICD indication and prescription by general cardiologists. Of 1711 discharged patients, 1525 (89%) had data available for evaluating indication status. Class I indications for ICD therapy were met for 153 (10%) patients based on collected data. Only 20 (13%, 95% confidence interval: 7.7-18.4%) patients with indication were prescribed an ICD. Patients prescribed an ICD were younger than patients who were not prescribed an ICD (62 vs. 68 years, P < 0.01). The reasons given by cardiologists for not prescribing an ICD for 133 patients with an indication were: indication criteria not met (75%), life expectancy <1 year (9.7%), rejection by the patient (5.2%), no medical coverage paying for the device (3.7%), psychiatric patient (2.2%), and other reasons (4.2%). CONCLUSIONS: In Latin America, international guidelines for primary prevention ICD implantation are not well followed. The main reason is that cardiologists believe that patients do not meet indication criteria, even though study data confirm that criteria are met. This poses a significant challenge and underlines the importance of continuous and improved medical education.

New phenotype of familial dilated cardiomyopathy and conduction disorders.

BACKGROUND: Familial dilated cardiomyopathy (FDCM) is attributed to defects in cytoskeletal proteins, and different patterns of inheritance and phenotypic expressions according to assorted-protein modifications have been identified to date. We describe a clinical family study with 24 individuals in 3 generations affected by dilated cardiomyopathy (DCM) and cardiac conduction abnormalities. METHODS AND RESULTS: After a follow-up period of 25 +/- 14 months, DCM developed in 7 male adults, 6 with associated arterioventricular block (AVB); and 10 female and 7 male adults had several degrees of isolated AVB. This particular clinical expression, with a strong predominance of dilation of the heart developing in the male population and the vertical distribution of patients affected with AVB, is consistent with autosomal dominant inheritance involving both cardiac abnormalities. CONCLUSIONS: The presence of isolated AVB or that associated with DCM in a large number of individuals in the same family, in which members of the male sex seems to be predominantly affected by cardiac dilatation, differs from other FDCMs that have been described previously. This FDCM has an autosomal dominant pattern of inheritance with variable phenotypic expressivity, in which AVB may constitute in itself the only manifestation of this entity. To date, we have been unable to identify the mechanism of inheritance, and we advance some theoretical considerations about possible mechanisms.