Cardiac Outcomes in Adults With Mitochondrial Diseases.
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Savvatis Konstantinos, Vissing Christoffer Rasmus, Klouvi Lori, Florian Anca, Rahman Mehjabin, Béhin Anthony, Fayssoil Abdallah, Masingue Marion, Stojkovic Tanya, Bécane Henri Marc, Berber Nawal, Mochel Fanny, Duboc Denis, Fontaine Bertrand, Krett Bjørg, Stalens Caroline, Lejeune Julie, Pitceathly Robert D S, Lopes Luis, Saadi Malika, Gossios Thomas, Procaccio Vincent, Spinazzi Marco, Tard Céline, De Groote Pascal, Dhaenens Claire-Marie, Douillard Claire, Echaniz-Laguna Andoni, Quinlivan Ros, Hanna Michael G, Yilmaz Ali, Vissing John, Laforêt Pascal, Elliott Perry, Wahbi Karim
Аннотация
OBJECTIVES: We developed prediction models to estimate the risk of HF and arrhythmic MACE in this population.
METHODS: We determined the incidence and searched for predictors of HF and arrhythmic MACE using Cox regression in 600 adult patients from a multicenter registry with genetically confirmed mitochondrial diseases.
RESULTS: Over a median follow-up time of 6.67 years, 29 patients (4.9%) reached the HF endpoint, including 19 hospitalizations for nonterminal HF, 2 cardiac transplantations, and 8 deaths from HF. Thirty others (5.1%) reached the arrhythmic MACE, including 21 with third-degree or type II second-degree atrioventricular blocks, 4 with sinus node dysfunction, and 5 sudden cardiac deaths. Predictors of HF were the m.3243A>G variant (HR: 4.3; 95% CI: 1.8-10.1), conduction defects (HR: 3.0; 95% CI: 1.3-6.9), left ventricular (LV) hypertrophy (HR: 2.6; 95% CI: 1.1-5.8), LV ejection fraction <50% (HR: 10.2; 95% CI: 4.6-22.3), and premature ventricular beats (HR: 4.1; 95% CI: 1.7-9.9). Independent predictors for arrhythmia were single, large-scale mtDNA deletions (HR: 4.3; 95% CI: 1.7-10.4), conduction defects (HR: 6.8; 95% CI: 3.0-15.4), and LV ejection fraction <50% (HR: 2.7; 95% CI: 1.1-7.1). C-indexes of the Cox regression models were 0.91 (95% CI: 0.88-0.95) and 0.80 (95% CI: 0.70-0.90) for the HF and arrhythmic MACE, respectively.
CONCLUSIONS: We developed the first prediction models for HF and arrhythmic MACE in patients with mitochondrial diseases using genetic variant type and simple cardiac assessments.