To determine factors predictive of mortality in patients undergoing emergency mitral valve surgery in the setting of severe post-infarction regurgitation. Patients admitted for an acute myocardial infarction who required urgent mitral valve surgery for severe regurgitation were studied. Factors predictive of outcome were analysed. Fifty-five consecutive patients (mean 65+/-10 years, 37 males) were included. The infarct was inferior in 31 patients, posterior in 10, anterior in 9 and lateral in 5. Thirty-four patients (62%) were in Killip class IV. Peroperative findings confirmed total papillary muscle rupture in 25 patients (posteromedial in 21, anterolateral in 4), and partial rupture in 12 patients (posteromedial in 10, anterolateral in 2). Papillary muscle dysfunction without rupture was responsible for regurgitation in 18 patients (posteromedial in 15, anterolateral in 3). The mitral valve was replaced by a prosthesis in all but 4 patients, who had valvuloplasty. Coronary angiography was done in 32 patients, of whom 18 underwent concomitant coronary artery bypass grafting and 2 balloon angioplasty. Surgery was performed on average 7 days after infarction. Thirteen patients (24%) died during the perioperative period. Absence of coronary revascularisation was significantly associated with increased perioperative mortality (34% vs. 9%, P = 0.02). Of the 42 surviving patients, there were 5 deaths during a mean follow-up of 4.0+/-3.7 years. In patients with acute post-infarction mitral regurgitation, perioperative mortality is high, but can be improved with concomitant CABG in addition to valve surgery. Long-term outcome of survivors is favourable.

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results: In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05–7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65–0.75)] and calibration slope of 1.01 (95% CI 0.99–1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion: Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC.

In the early nineties, few years before the birth of Europace, the clinical and scientific world of familial arrhythmogenic conditions was revolutionized by the identification of the first disease-causing genes. The explosion of genetic studies over a 15-year period led to the discovery of major disease-causing genes in practically all channelopathies and cardiomyopathies, bringing insight into the pathophysiological mechanisms of these conditions. The birth of next generation sequencing allowed a further step forward and other significant genes, as CALM1–3 in channelopathies and FLN C and TTN in cardiomyopathies were identified. Genotype–phenotype studies allowed the implementation of the genetic results in diagnosis, risk stratification, and therapeutic management with a different level of evidence in different arrhythmogenic conditions. The influence of common genetic variants, i.e. SNPs, on disease manifestation was proved in mid-twenties, and in the last 10 years with the advent of genome-wide association studies performed in familial arrhythmogenic diseases, the concept of polygenic risk score has been consolidated. Now, we are at the start of another amazing phase, i.e. the initiation of first gene therapy clinical trials.