Asunto(s)
Implantación de Prótesis de Válvulas Cardíacas , Válvula Pulmonar , Tetralogía de Fallot , Humanos , Tetralogía de Fallot/cirugía , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/fisiopatología , Masculino , Válvula Pulmonar/cirugía , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/fisiopatología , Femenino , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Valor Predictivo de las Pruebas , Adolescente , Adulto Joven , Adulto , Insuficiencia de la Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/fisiopatología , Insuficiencia de la Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/etiología , Insuficiencia de la Válvula Pulmonar/diagnóstico , Imagen por Resonancia Cinemagnética , Niño , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Operación de Switch Arterial , Insuficiencia Cardíaca , Transposición de los Grandes Vasos , Adolescente , Adulto , Femenino , Humanos , Masculino , Adulto Joven , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatología , Operación de Switch Arterial/efectos adversos , Insuficiencia Cardíaca/diagnóstico , Estudios Longitudinales , Valor Predictivo de las Pruebas , Reproducibilidad de los Resultados , Estudios Retrospectivos , Medición de Riesgo/métodos , Factores de Riesgo , Factores de Tiempo , Transposición de los Grandes Vasos/cirugíaRESUMEN
INTRODUCTION: Pacemaker implantation can be challenging in patients with congenital heart disease. METHODS AND RESULTS: In a patient with Ebstein disease and symptomatic sinus node dysfunction, despite multiple attempts, the Micra® pacemaker could not be implanted in the severely dilated right ventricle. In that context, and after iodine injection to confirm the appropriate location, the Micra® pacemaker was successfully implanted in the right appendage at the first attempt. CONCLUSION: Despite the recent development of dedicated dual-chamber leadless pacemaker, to the best of our knowledge, this case is the first off-label Micra® implantation in a right appendage.
Asunto(s)
Apéndice Atrial , Estimulación Cardíaca Artificial , Marcapaso Artificial , Humanos , Apéndice Atrial/fisiopatología , Apéndice Atrial/cirugía , Apéndice Atrial/diagnóstico por imagen , Síndrome del Seno Enfermo/terapia , Síndrome del Seno Enfermo/fisiopatología , Síndrome del Seno Enfermo/diagnóstico , Anomalía de Ebstein/cirugía , Anomalía de Ebstein/fisiopatología , Resultado del Tratamiento , Masculino , Diseño de EquipoRESUMEN
BACKGROUND: Functional abnormalities of the ascending aorta (AA) have been mainly reported in young patients who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA). OBJECTIVES: To compare systolic, diastolic brachial and central blood pressures (bSBP, bDBP, cSBP, cDBP), aortic biomechanical parameters, and left ventricular (LV) afterload criteria in adult ASO patients with healthy controls and to assess their relationships with LV remodeling and aortic size. MATERIALS AND METHODS: Forty-one prospectively enrolled patients (16.8 to 35.8 years) and 41 age- and sex-matched healthy volunteers underwent cardiac MRI to assess LV remodeling with simultaneous brachial BP estimation. After MRI, carotid-femoral tonometry was performed to measure pulse wave velocity (cfPWV), cSBP and cDBP for further calculation of pulse pressure (cPP), AA distensibility (AAD), and AA and LV elastance (AAE, LVE). RESULTS: bSBP, bDBP, cSBP,cDBP and cPP were all significantly higher in ASO group than in controls: cSBP (116.5 ± 13.8 vs 106.1 ± 12.0, p < 0.001), cDBP (72.5 ± 6.9 vs 67.1 ± 9.4, p = 0.002), cPP (44.0 ± 12.1 vs 39.1 ± 8.9, p = 0.003) and not related to aortic size. AAD were decreased in ASO patients vs controls (4.70 ± 2.72 vs 6.69 ± 2.16, p < 0.001). LV mass was correlated with bSBP, cSBP, cPP (ρ = 0.48; p < 0.001), while concentric LV remodeling was correlated with AAE (ρ = 0.60, p < 0.001) and LVE (ρ = 0.32, p = 0.04), but not with distensibility. CONCLUSION: Even without reaching arterial hypertension, aortic sBP and PP are increased in the adult TGA population after ASO, altering the pulsatile components of afterload and contributing to LV concentric remodeling.