Cardio-TC en la coartación aórtica compleja. Interrupción del arco aórtico con ventrículo derecho de doble salida

Aortic arch interruption is defined by the lack of anatomical continuity between the ascending and descending aorta, considered an extreme form of coarctation. It´s usually associated with other cardiac anomalies, like double outlet right ventricle. The technological advance in Cardiac CT has allowed in the last years a great diagnostic precision with minimum risk for the patient. We present the case of a 4-day-old infant with a diagnosis of complex aortic coarctation studied through CT and a brief literature review of this topic subject.La interrupción del arco aórtico se define por la falta de continuidad anatómica entre la aorta ascendente y la descendente, considerándose una forma extrema de coartación. Generalmente se encuentra asociada con otras anomalías cardíacas, como puede ser el ventrículo derecho de doble salida. El avance tecnológico en cardio-TC ha permitido en los últimos años una gran precisión diagnóstica con mínimo riesgo para el paciente. Se presenta el caso de un neonato de 4 días de vida con diagnóstico de coartación aórtica compleja estudiado mediante cardio-TC junto con una breve revisión bibliográfica del tema

The CoDiNOS trial protocol: an international randomised controlled trial of intravenous sildenafil versus inhaled nitric oxide for the treatment of pulmonary hypertension in neonates with congenital diaphragmatic hernia

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that impairs normal lung development, causing pulmonary hypertension (PH). PH in CDH newborns is the main determinant for morbidity and mortality. Different therapies are still mainly based on 'trial and error'. Inhaled nitric oxide (iNO) is often the drug of first choice. However, iNO does not seem to improve mortality. Intravenous sildenafil has reduced mortality in newborns with PH without CDH, but prospective data in CDH patients are lacking. METHODS AND ANALYSIS: In an open label, multicentre, international randomised controlled trial in Europe, Canada and Australia, 330 newborns with CDH and PH are recruited over a 4-year period (2018-2022). Patients are randomised for intravenous sildenafil or iNO. Sildenafil is given in a loading dose of 0.4 mg/kg in 3 hours; followed by continuous infusion of 1.6 mg/kg/day, iNO is dosed at 20 ppm. Primary outcome is absence of PH on day 14 without pulmonary vasodilator therapy and/or absence of death within the first 28 days of life. Secondary outcome measures include clinical and echocardiographic markers of PH in the first year of life. We hypothesise that sildenafil gives a 25% reduction in the primary outcome from 68% to 48% on day 14, for which a sample size of 330 patients is needed. An intention-to-treat analysis will be performed. A p-value (two-sided) <0.05 is considered significant in all analyses. ETHICS AND DISSEMINATION: Ethics approval has been granted by the ethics committee in Rotterdam (MEC-2017-324) and the central Committee on Research Involving Human Subjects (NL60229.078.17) in the Netherlands. The principles of the Declaration of Helsinki, the Medical Research Involving Human Subjects Act and the national rules and regulations on personal data protection will be used. Parental informed consent will be obtained. TRIAL REGISTRATION NUMBER: NTR6982; Pre-results

Percutaneous interventions in Fontan circulation

Different percutaneous interventional procedures are needed to reach and maintain adequate anatomical and physiological conditions for the Fontan circulation. We aim to describe the experience gained at a childrens hospital in such interventions, and to analyze the clinical outcomes. Retrospective study of all patients with Fontan circulation completed between 1995 and 2013. We analyzed the clinical characteristics and the different types of percutaneous interventions performed, considering three different periods of time: before Glenn surgery, between Glenn and Fontan surgeries, and after Fontan was completed. Survival and time to indication of percutaneous interventions in each period were analyzed, as well as the clinical situation at last follow-up. Of the 91 patients analyzed, 46 (50.5%) required percutaneous interventions. The most frequent procedures were pulmonary artery angioplasty and angioplasty of the Fontan conduit. Estimated survival at 10, 20 and 30 years of age was 96.2%, 94.7% and 89.4%, respectively. There were no significant differences in survival of patients undergoing percutaneous interventions or not. Overall survival and time to indication of percutaneous interventions were significantly lower in the group of patients with right morphology systemic ventricle. Patients with fenestrated Fontan required interventions more frequently. At the end of follow-up, 66 patients (72.5%) were asymptomatic, without significant differences between patients who underwent or did not undergo percutaneous interventions. Interventional catheterization procedures are often necessary to reach and maintain the fragile Fontan circulation, mainly in patients with right morphology systemic ventricles and fenestrated Fontan conduits

Percutaneous interventions in Fontan circulation

Introduction and objectives: Different percutaneous interventional procedures are needed to reach and maintain adequate anatomical and physiological conditions for the Fontan circulation. We aim to describe the experience gained at a children's hospital in such interventions, and to analyze the clinical outcomes. Methods: Retrospective study of all patients with Fontan circulation completed between 1995 and 2013. We analyzed the clinical characteristics and the different types of percutaneous interventions performed, considering three different periods of time: before Glenn surgery, between Glenn and Fontan surgeries, and after Fontan was completed. Survival and time to indication of percutaneous interventions in each period were analyzed, as well as the clinical situation at last follow-up. Results: Of the 91 patients analyzed, 46 (50.5%) required percutaneous interventions. The most frequent procedures were pulmonary artery angioplasty and angioplasty of the Fontan conduit. Estimated survival at 10, 20 and 30 years of age was 96.2%, 94.7% and 89.4%, respectively. There were no significant differences in survival of patients undergoing percutaneous interventions or not. Overall survival and time to indication of percutaneous interventions were significantly lower in the group of patients with right morphology systemic ventricle. Patients with fenestrated Fontan required interventions more frequently. At the end of follow-up, 66 patients (72.5%) were asymptomatic, without significant differences between patients who underwent or did not undergo percutaneous interventions. Conclusions: Interventional catheterization procedures are often necessary to reach and maintain the fragile Fontan circulation, mainly in patients with right morphology systemic ventricles and fenestrated Fontan conduits

¿Funcionan igual las prótesis pulmonares porcinas en menores de edad que en adultos? Una llamada a la prudencia

Introducción: El implante de una prótesis pulmonar para recuperar la funcionalidad de la vía de salida derecha, operación frecuente en congénitos adultos, comienza a hacerse extensiva a la infancia para prevenir la disfunción ventricular. Objetivos: Comparar resultados de la bioprótesis pulmonar porcina en mayores/menores de 18 años, analizando factores de riesgo de mortalidad y disfunción protésica. Efectos de la válvula sobre el grado funcional y la funcionalidad del ventrículo derecho. Métodos: Todos los pacientes a los que se implantó una bioprótesis entre 1999-2015. Análisis estadístico SPSS20.0. Resultados: Ciento dos válvulas en 101 pacientes. Mortalidad hospitalaria 2,9% (3,7% adultos vs. 0% menores, p < 0,001). Procedimientos quirúrgicos asociados en el 72,5%. Mortalidad 0% si la bioprótesis era único procedimiento. Factores de riesgo para mortalidad (p < 0,05): tiempo de circulación extracorpórea, implante de bioprótesis en adulto. Seguimiento completo, medio 4 ± 3,7 años (4,4 adultos vs. 2,4 menores, p = 0,017). Un fallecido tardío. Disfunción protésica: 6,1% (1,3% adultos vs. 23,8% menores, p < 0,001). Factores de riesgo de disfunción protésica en análisis univariante (p < 0,05): conducto/prótesis previo en vía de salida, indicación implante estenosis pulmonar, implante bioprótesis en menores, peso bajo y menor superficie corporal. En multivariante permanecieron indicación implante estenosis y superficie corporal baja. Tras la cirugía, mejoraron el grado funcional y los volúmenes del ventrículo (p < 0,05), no la fracción de eyección. Conclusiones: El implante de una bioprótesis pulmonar porcina conlleva baja mortalidad y recupera el volumen del ventrículo, pero no su función. En los menores debería reservarse el procedimiento para cuando la función ventricular esté en riesgo, ya que degeneran más, y más precozmente

The CoDiNOS trial protocol: an international randomised controlled trial of intravenous sildenafil versus inhaled nitric oxide for the treatment of pulmonary hypertension in neonates with congenital diaphragmatic hernia

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that impairs normal lung development, causing pulmonary hypertension (PH). PH in CDH newborns is the main determinant for morbidity and mortality. Different therapies are still mainly based on 'trial and error'. Inhaled nitric oxide (iNO) is often the drug of first choice. However, iNO does not seem to improve mortality. Intravenous sildenafil has reduced mortality in newborns with PH without CDH, but prospective data in CDH patients are lacking. METHODS AND ANALYSIS: In an open label, multicentre, international randomised controlled trial in Europe, Canada and Australia, 330 newborns with CDH and PH are recruited over a 4-year period (2018-2022). Patients are randomised for intravenous sildenafil or iNO. Sildenafil is given in a loading dose of 0.4 mg/kg in 3 hours; followed by continuous infusion of 1.6 mg/kg/day, iNO is dosed at 20 ppm. Primary outcome is absence of PH on day 14 without pulmonary vasodilator therapy and/or absence of death within the first 28 days of life. Secondary outcome measures include clinical and echocardiographic markers of PH in the first year of life. We hypothesise that sildenafil gives a 25% reduction in the primary outcome from 68% to 48% on day 14, for which a sample size of 330 patients is needed. An intention-to-treat analysis will be performed. A p-value (two-sided) <0.05 is considered significant in all analyses. ETHICS AND DISSEMINATION: Ethics approval has been granted by the ethics committee in Rotterdam (MEC-2017-324) and the central Committee on Research Involving Human Subjects (NL60229.078.17) in the Netherlands. The principles of the Declaration of Helsinki, the Medical Research Involving Human Subjects Act and the national rules and regulations on personal data protection will be used. Parental informed consent will be obtained. TRIAL REGISTRATION NUMBER: NTR6982; Pre-results.status: publishe