Assuntos
Implante de Prótese de Valva Cardíaca , Valva Pulmonar , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/fisiopatologia , Masculino , Valva Pulmonar/cirurgia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Taquicardia Ventricular/fisiopatologia , Taquicardia Ventricular/cirurgia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologia , Valor Preditivo dos Testes , Adolescente , Adulto Jovem , Adulto , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/diagnóstico , Imagem Cinética por Ressonância Magnética , Criança , Resultado do TratamentoRESUMO
BACKGROUND: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. METHODS AND RESULTS: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 [95% CI, 0.65-0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03-0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. CONCLUSIONS: We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.