In patients with HCM, myocardial fibrosis as measured by late gadolinium enhancement cardiovascular magnetic resonance is an independent predictor of adverse outcome. (The Prognostic Significance of Fibrosis Detection in Cardiomyopathy; NCT00930735).

IVNC is associated with a broad spectrum of clinical and pathophysiological findings, and the overall natural history and prognosis may be better than previously thought. Adult patients with incidental or familial discovery of IVNC have an encouraging outlook, whereas those who have symptoms of heart failure, a history of sustained ventricular tachycardia or an enlarged left atrium have an unstable course and more severe prognosis.

Background-Mitral valve (MV) repair is preferred over replacement in clinical guidelines and is an important determinant of the indication for surgery in degenerative mitral regurgitation (MR). Yet, the level of evidence supporting current recommendations is low, and recent data cast doubts on its validity in the current era. Accordingly, the aim of the present study was to analyze very long-term outcome after MV repair and replacement for degenerative MR with a flail leaflet. Methods-MIDA is a multicenter registry enrolling patients with degenerative MR with a flail leaflet in 6 tertiary European and US centers. We analyzed the outcome after MV repair (n=1,709) and replacement (n= 213), overall, by propensity score matching and by inverse probability-of-treatment weighting. Results-At baseline, patients undergoing MV repair were younger, had more comorbidities and were more likely to present with a posterior leaflet prolapse than those undergoing MV replacement. After propensity score matching as well as after inverse probability-of-treatment weighting, the 2 treatments groups were balanced and absolute standardized differences were usually below 10%, indicating adequate match. Operative mortality (defined as a death occurring within 30 days from surgery or during the same hospitalization) was lower after MV repair than after replacement, both in the entire (1.3 vs 4.7%; p<0.001) and in propensity-matched population (0.2% vs 4.4%; p<0.001). During a mean follow-up of 9.2 years, 552 deaths were observed, of which 207 were of cardiovascular origin. Twenty-year survival was better after MV repair than after MV replacement, both in the entire (46% vs 23%, p<0.001) and in matched population (41% vs 24%, p<0.001). Similar superiority of MV repair were obtained in patients' subsets based on age, sex or any stratification criteria (all p<0.001). MV repair was also associated with reduced incidence of reoperations and valve-related complications.Conclusions-Among patients with degenerative MR with a flail leaflet, referred to mitral surgery, MV repair was associated with lower operative mortality, better long-term survival and fewer valve-related complications compared to MV replacement.

The prevalence of AFD gene mutations in a large, consecutive cohort of European patients with unexplained LVH is 0.5%.

Background-Hypertrophic cardiomyopathy caused by mutations in the cardiac troponin T gene (TNNT2) has been associated with a high risk of sudden cardiac death (SCD) and mild left ventricular hypertrophy. However, previous studies are limited by sample size, cross-sectional design, and few data in relatives.