Background—: Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular electric instability remains elusive. Methods and Results—: The cardiac pathology registry of 650 young adults (≤40 years of age) with SCD was reviewed, and cases with MVP as the only cause of SCD were re-examined. Forty-three patients with MVP (26 females; age range, 19–40 years; median, 32 years) were identified (7% of all SCD, 13% of women). Among 12 cases with available ECG, 10 (83%) had inverted T waves on inferior leads, and all had right bundle-branch block ventricular arrhythmias. A bileaflet involvement was found in 70%. Left ventricular fibrosis was detected at histology at the level of papillary muscles in all patients, and inferobasal wall in 88%. Living patients with MVP with (n=30) and without (control subjects; n=14) complex ventricular arrhythmias underwent a study protocol including contrast-enhanced cardiac magnetic resonance. Patients with either right bundle-branch block type or polymorphic complex ventricular arrhythmias (22 females; age range, 28–43 years; median, 41 years), showed a bileaflet involvement in 70% of cases. Left ventricular late enhancement was identified by contrast-enhanced cardiac magnetic resonance in 93% of patients versus 14% of control subjects ( P <0.001), with a regional distribution overlapping the histopathology findings in SCD cases. Conclusions—: MVP is an underestimated cause of arrhythmic SCD, mostly in young adult women. Fibrosis of the papillary muscles and inferobasal left ventricular wall, suggesting a myocardial stretch by the prolapsing leaflet, is the structural hallmark and correlates with ventricular arrhythmias origin. Contrast-enhanced cardiac magnetic resonance may help to identify in vivo this concealed substrate for risk stratification.

Aims We conducted a multi-centre, prospective, controlled, randomized trial to investigate the adjunctive role of ablation therapy to antiarrhythmic drug therapy in preventing atrial fibrillation (AF) relapses in patients with paroxysmal or persistent AF in whom antiarrhythmic drug therapy had already failed. Methods and results One hundred and thirty seven patients were randomized to ablation and antiarrhythmic drug therapy (ablation group) or antiarrhythmic drug therapy alone (control group). In the ablation group, patients underwent cavo-tricuspid and left inferior pulmonary vein (PV)-mitral isthmus ablation plus circumferential PV ablation. The primary end-point of the study was the absence of any recurrence of atrial arrhythmia lasting >30 s in the 1-year follow-up period, after 1-month blanking period. Three (4.4%) major complications were related to ablation: one patient had a stroke during left atrium ablation, another suffered transient phrenic paralysis, and the third had a pericardial effusion which required pericardiocentesis. After 12 months of follow-up, 63/69 (91.3%) control group patients had at least one AF recurrence, whereas 30/68 (44.1%) (P<0.001) ablation group patients had atrial arrhythmia recurrence (four patients had atrial flutter, 26 patients AF). Conclusion Ablation therapy combined with antiarrhythmic drug therapy is superior to antiarrhythmic drug therapy alone in preventing atrial arrhythmia recurrences in patients with paroxysmal or persistent AF in whom antiarrhythmic drug therapy has already failed.

Background—: Arrhythmic mitral valve prolapse (MVP) is characterized by myxomatous leaflets and left ventricular (LV) fibrosis of papillary muscles and inferobasal wall. We searched for morphofunctional abnormalities of the mitral valve that could explain a regional mechanical myocardial stretch. Methods and Results—: Thirty-six (27 female patients; median age: 44 years) arrhythmic MVP patients with LV late gadolinium enhancement on cardiac magnetic resonance and no or trivial mitral regurgitation, and 16 (6 female patients; median age: 40 years) MVP patients without LV late gadolinium enhancement were investigated by morphofunctional cardiac magnetic resonance. Mitral annulus disjunction (median: 4.8 versus 1.8 mm; P <0.001), end-systolic mitral annular diameters (median: 41.2 versus 31.5; P =0.004) and end-diastolic mitral annular diameters (median: 35.5 versus 31.5; P =0.042), prevalence of posterior systolic curling (34 [94%] versus 3 [19%]; P 1.5 (22 [61%] versus 4 [25%]; P =0.016) were higher in MVP patients with late gadolinium enhancement than in those without. A linear correlation was found between mitral annulus disjunction and curling ( R =0.85). A higher prevalence of auscultatory midsystolic click (26 [72%] versus 6 [38%]; P =0.018) was also noted. Histology of the mitral annulus showed a longer mitral annulus disjunction in 50 sudden death patients with MVP and LV fibrosis than in 20 patients without MVP (median: 3 versus 1.5 mm; P <0.001). Conclusions—: Mitral annulus disjunction is a constant feature of arrhythmic MVP with LV fibrosis. The excessive mobility of the leaflets caused by posterior systolic curling accounts for a mechanical stretch of the inferobasal wall and papillary muscles, eventually leading to myocardial hypertrophy and scarring. These mitral annulus abnormalities, together with auscultatory midsystolic click, may identify MVP patients who would need arrhythmic risk stratification.

Background—: Catheter ablation of atrial fibrillation (AFCA) is an established therapeutic option for rhythm control in symptomatic patients. Its efficacy and safety among patients with left ventricular systolic dysfunction is based on small populations, and data concerning long-term outcome are limited. We performed this meta-analysis to assess safety and long-term outcome of AFCA in patients with left ventricular systolic dysfunction, to evaluate predictors of recurrence and impact on left ventricular function. Methods and Results—: A systematic review was conducted in MEDLINE/PubMed and Cochrane Library. Randomized controlled trials, clinical trials, and observational studies including patients with left ventricular systolic dysfunction undergoing AFCA were included. Twenty-six studies were selected, including 1838 patients. Mean follow-up was 23 (95% confidence interval, 18–40) months. Overall complication rate was 4.2% (3.6%–4.8%). Efficacy in maintaining sinus rhythm at follow-up end was 60% (54%–67%). Meta-regression analysis revealed that time since first atrial fibrillation ( P =0.030) and heart failure ( P =0.045) diagnosis related to higher, whereas absence of known structural heart disease ( P =0.003) to lower incidence of atrial fibrillation recurrences. Left ventricular ejection fraction improved significantly during follow-up by 13% ( P <0.001), with a significant reduction of patients presenting an ejection fraction <35% ( P <0.001). N-terminal pro-brain natriuretic peptide blood levels decreased by 620 pg/mL ( P <0.001). Conclusions—: AFCA efficacy in patients with impaired left ventricular systolic function improves when performed early in the natural history of atrial fibrillation and heart failure. AFCA provides long-term benefits on left ventricular function, significantly reducing the number of patients with severely impaired systolic function.