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Background: Pulmonary autograft reinforcement to prevent dilatation and subsequent neo-aortic valve regurgitation has been reported; however, data on long-term function of the neo-aortic valve after this modified Ross procedure are lacking. Our objective here was to assess long-term outcomes of the modified Ross procedure with autograft reinforcement using the reimplantation technique. Patients: The outcomes of 61 consecutive patients managed using the Dacron-conduit reinforced Ross procedure between 2009 and 2021 were reviewed. Most patients had a unicuspid or bicuspid aortic valve (n = 52; 85%), predominant aortic valve regurgitation (n = 42; 77%), and >30 mm dilatation of the ascending aorta (n = 33; 54%). A prior aortic valve procedure was noted in 47 patients (77%) patients, including 38 (62%) with surgical repair and 9 (15%) with balloon dilatation. The pulmonary autograft was reimplanted within a Dacron conduit with a median diameter of 25.6 mm (range, 20-30 mm) using the David valve-sparing aortic root replacement technique. Results: All patients survived. The median age at surgery was 16.8 years (range, 6-38 years). Neo-aortic valve replacement was required in 3 patients (4.9%; 95% CI, 0.34%- 12.7%) because of infective endocarditis, left ventricular false aneurysm, and leaflet perforation, respectively; the repeat procedure was done early in 2 of these patients (2 of 61; 3%). Six patients required right ventricular outflow conduit replacement, 5 by surgery and 1 percutaneously. The median duration of follow-up was 90 months (range, 10-124 months). The 5- and 10-year rates of reintervention-free survival were 84.3% (95% CI, 74%-95%) and 81.6% (95% CI, 72%-93%), respectively, and 5-year survival without aortic reintervention was 94.5% (95% CI, 88%-100%), with little change at 10 years. No patients experienced deterioration of initial neo-aortic valve function (ie, regurgitation or stenosis). Conclusions: Autograft reinforcement using the reimplantation technique allowed expansion of Ross procedure indications to all patients requiring aortic valve replacement and prevented neo-aortic root dilatation. Failures were uncommon. Long-term follow-up data showed stable neo-aortic valve function.
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Introducción y objetivos: El diagnóstico y manejo terapéutico de pacientes con cardiopatías congénitas se basa en gran medida en los resultados de la ecocardiografía. Las diferentes técnicas de fusión de imagen han supuesto un reciente avance en la obtención de imágenes cardíacas. Nuestro objetivo ha sido el de evaluar la viabilidad de la fusión de la tomografía computarizada (TC) y la ecocardiografía transtorácica (ETT) 3D en niños y adultos con cardiopatías congénitas. Métodos: Se incluyeron de forma prospectiva 13 pacientes con cardiopatías congénitas y 1 paciente con corazón normal los cuales se sometieron a TC y ETT 3D como parte de su seguimiento habitual. Se describieron los pasos necesarios para completar el proceso de fusión (alineación, puntos de referencia y superposición), la navegación y la evaluación de las imágenes. Resultados: La edad media fue de 9,5 años [2,7-15,7], el 57% eran varones y la superficie corporal media fue de 0,9 m2 [0,6-1,7]. Se clasificaron las cardiopatías como simples (n=4, 29%), moderadas (n=4, 29%) o complejas (n=6, 42%). La fusión 3D ETT-TC fue exitosa en todos los pacientes. La mediana del tiempo total para completar el proceso de fusión fue de 735 segundos [628-1163], sin diferencias significativas según el grado de complejidad de la cardiopatía. Los puntos de referencia se modificaron significativamente en las cardiopatías congénitas complejas. Conclusiones: La técnica de fusión 3D ETT-TC en una población de niños y adultos con diversas cardiopatías congénitas es viable y precisa según demostramos. La visualización simultánea de diferentes estructuras cardíacas podría ayudar a comprender las características anatómicas de las cardiopatías congénitas sin limitaciones en cuanto a la edad, el peso o la complejidad.(AU)
Introduction and objectives: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. Methods: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. Results: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. Conclusions: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.(AU)
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Humanos , Masculino , Femenino , Cardiopatías Congénitas , Tomografía Computarizada por Rayos X , Ecocardiografía , Diagnóstico por Imagen , CardiologíaRESUMEN
INTRODUCTION AND OBJECTIVES: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. METHODS: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. RESULTS: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. CONCLUSIONS: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.
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Ecocardiografía Tridimensional , Cardiopatías Congénitas , Niño , Adulto , Humanos , Masculino , Femenino , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Ecocardiografía/métodos , Corazón/diagnóstico por imagen , Ecocardiografía Tridimensional/métodos , Tomografía Computarizada por Rayos XRESUMEN
The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (20182020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.