Cardiac Outcomes in Adults With Mitochondrial Diseases.
J Am Coll Cardiol
; 80(15): 1421-1430, 2022 10 11.
Article
em En
| MEDLINE
| ID: mdl-36202532
ABSTRACT
BACKGROUND:
Patients with mitochondrial diseases are at risk of heart failure (HF) and arrhythmic major adverse cardiac events (MACE).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.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Doenças Mitocondriais
/
Insuficiência Cardíaca
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article