Bridge to transplant in single-ventricle anatomy: subpulmonary support with EXCOR ventricular assist device associated with pulmonary artery reconstruction.

Patients with a single ventricle circulation continue to be a challenge for a heart transplant. The patients in this cohort, often in poor clinical condition with frequent hepatic and renal impairment having undergone previous multiple surgical procedures, are often allosensitive and consequently have an increased risk of post-transplant complications. Although the most recently published series results are improved, this group of patients, with preserved ventricular function, continues to have a higher mortality rate. Moreover, the operation can be complicated by anatomical differences among the pulmonary arteries. This case report presents a child with tricuspid atresia and pulmonary vascular resistance contraindicating Fontan surgery, unfavourable anatomy of the central pulmonary arteries and in poor clinical condition. An EXCOR ventricular assist device (Berlin Heart) was used for sub- pulmonary mechanical circulatory support and pulmonary bifurcation reconstruction, as a bridge to a transplant.

Primary cardiac fibroma in infants: A case report and review of cases of cardiac fibroma managed through orthotopic heart transplant.

Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually show a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.

Masa pericárdica ¿Qué hay detrás? / Pericardial mass: What lies behind?

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Tolvaptán en un caso de síndrome cardiorrenal pediátrico: ¿Cuál es su papel? / Tolvaptan in a pediatric cardiorenal syndrome: What is its role?

El síndrome cardiorrenal se define como la asociación de insuficiencia renal y cardíaca de forma aguda o crónica. Se establece una resistencia a los diuréticos convencionales que hace difícil el manejo de estos niños. El tolvaptán, un antagonista de los receptores de vasopresina, ha sido empleado con éxito en adultos, aunque la experiencia en niños es muy limitada. Se presenta el caso de una paciente de 5 años en lista de trasplante cardíaco que padecía síndrome cardiorrenal. Había tenido una hospitalización prolongada con buena respuesta a dosis mínimas de tolvaptán (0,1 mg/kg/día). Se analizaron las curvas de urea, creatinina, sodio y volumen urinario, y se evidenció una mejoría llamativa en la función renal. Al cuarto día de haber iniciado el tratamiento, se le pudo dar el alta con seguimiento ambulatorio y buena evolución hasta el trasplante. El tolvaptán podría considerarse una opción de tratamiento en niños con resistencia a diuréticos convencionales e insuficiencia cardíaca, especialmente, cuando presentan insuficiencia renal.

The cardiorenal syndrome has been defined as a situation in which therapy to relieve congestive symptoms of heart failure is limited by a decreased renal function. The resistance to conventional diuretic treatment makes difficult managing these patients. Tolvaptan, a selective vasopressin-2 receptor antagonist, has been used successfully in adults with this pathology but the experience with children is very limited. A five-year-old girl with renal failure waiting for a heart transplant is presented. Tolvaptan (0.1 mg/kg/day) was started at very low dosage, resulting in an excellent response. We analyzed the creatinine, urea, urine volume and sodium evolution. Renal function also improved. She could be discharged after four days of treatment (156 days of hospitalization) with ambulatory favourable follow-up until heart transplant. Tolvaptan should be considered in pediatric cases of conventional diuretic-resistant congestive heart failure, especially when complicated by kidney disease.

[Tolvaptan in a pediatric cardiorenal syndrome: What is its role?] / Tolvaptán en un caso de síndrome cardiorrenal pediátrico: ¿Cuál es su papel?

The cardiorenal syndrome has been defined as a situation in which therapy to relieve congestive symptoms of heart failure is limited by a decreased renal function. The resistance to conventional diuretic treatment makes difficult managing these patients. Tolvaptan, a selective vasopressin-2 receptor antagonist, has been used successfully in adults with this pathology but the experience with children is very limited. A five-year-old girl with renal failure waiting for a heart transplant is presented. Tolvaptan (0.1 mg/kg/day) was started at very low dosage, resulting in an excellent response. We analyzed the creatinine, urea, urine volume and sodium evolution. Renal function also improved. She could be discharged after four days of treatment (156 days of hospitalization) with ambulatory favourable follow-up until heart transplant. Tolvaptan should be considered in pediatric cases of conventional diuretic-resistant congestive heart failure, especially when complicated by kidney disease.

El síndrome cardiorrenal se define como la asociación de insuficiencia renal y cardíaca de forma aguda o crónica. Se establece una resistencia a los diuréticos convencionales que hace difícil el manejo de estos niños. El tolvaptán, un antagonista de los receptores de vasopresina, ha sido empleado con éxito en adultos, aunque la experiencia en niños es muy limitada. Se presenta el caso de una paciente de 5 años en lista de trasplante cardíaco que padecía síndrome cardiorrenal. Había tenido una hospitalización prolongada con buena respuesta a dosis mínimas de tolvaptán (0,1 mg/kg/día). Se analizaron las curvas de urea, creatinina, sodio y volumen urinario, y se evidenció una mejoría llamativa en la función renal. Al cuarto día de haber iniciado el tratamiento, se le pudo dar el alta con seguimiento ambulatorio y buena evolución hasta el trasplante. El tolvaptán podría considerarse una opción de tratamiento en niños con resistencia a diuréticos convencionales e insuficiencia cardíaca, especialmente, cuando presentan insuficiencia renal.

Biomarcadores cardiacos en el síndrome de bajo gasto cardiaco en el posoperatorio de cirugía de cardiopatías congénitas en niños / Cardiac biomarkers of low cardiac output syndrome in the postoperative period after congenital heart disease surgery in children

Introducción y objetivos: Determinar el valor del péptido natriurético auricular, el péptido natriurético cerebral, la copeptina, la región medial de la proadrenomedulina (MR-proADM) y la troponina I cardiaca (cTn-I) como indicadores de síndrome de bajo gasto cardiaco posoperatorio en niños con cardiopatía congénita intervenidos en circulación extracorpórea (CEC).Métodos: Estudio piloto prospectivo observacional, realizado durante 2 años, que incluyó a 117 niños (edad, 10 días-180 meses) intervenidos de cardiopatías congénitas en CEC, clasificados según presentaran o no síndrome de bajo gasto cardiaco. Los biomarcadores se determinaron tras 2, 12, 24 y 48 h del posoperatorio. Se utilizó un modelo de regresión logística multivariable para evaluar los factores asociados al bajo gasto cardiaco. Resultados: Tenían síndrome de bajo gasto cardiaco 33 pacientes (29%). Tras el ajuste por las demás variables, los valores plasmáticos de cTn-I > 14 ng/ml a las 2 h de CEC (odds ratio = 4,05; intervalo de confianza del 95%, 1,29-12,64; p = 0,016) y de MR-proADM > 1,5 nmol/l a las 24 h (odds ratio = 15,54; intervalo de confianza del 95%, 4,41-54,71; p < 0,001) fueron los únicos predictores independientes de bajo gasto cardiaco.Conclusiones: Los resultados indican que las concentraciones de cTn-I elevadas 2 h después de la CEC son, por sí solas, un predictor independiente de síndrome de bajo gasto cardiaco. Este valor predictivo se incrementa cuando se asocia con cifras de MR-proADM elevadas 24 h tras CEC. Estos 2 biomarcadores cardiacos podrían ayudar en la toma de decisiones terapéuticas en cuidados intensivos pediátricos, incluidas modificaciones en el tipo de soporte circulatorio (AU)

Introduction and objectives: To assess the predictive value of atrial natriuretic peptide, β-type natriuretic peptide, copeptin, mid-regional pro-adrenomedullin (MR-proADM) and cardiac troponin I (cTn-I) as indicators of low cardiac output syndrome in children with congenital heart disease undergoing cardiopulmonary bypass (CPB). Methods: After corrective surgery for congenital heart disease under CPB, 117 children (aged 10 days to 180 months) were enrolled in a prospective observational pilot study during a 2-year period. The patients were classified according to whether they developed low cardiac output syndrome. Biomarker levels were measured at 2, 12, 24, and 48 hours post-CPB. The clinical data and outcome variables were analyzed by a multiple logistic regression model. Results: Thirty-three (29%) patients developed low cardiac output syndrome (group 1) and the remaining 84 (71%) patients were included in group 2. cTn-I levels > 14 ng/mL at 2 hours after CPB (OR, 4.05; 95%CI, 1.29-12.64; P = .016) and MR-proADM levels > 1.5 nmol/L at 24 hours following CPB (OR, 15.54; 95%CI, 4.41-54.71; P < .001) were independent predictors of low cardiac output syndrome. Conclusions: Our results suggest that cTn-I at 2 hours post-CPB is, by itself, an evident independent early predictor of low cardiac output syndrome. This predictive capacity is, moreover, reinforced when cTn-I is combined with MR-proADM levels at 24 hours following CPB. These 2 cardiac biomarkers would aid in therapeutic decision-making in clinical practice and would also enable clinicians to modify the type of support to be used in the pediatric intensive care unit (AU)

Cardiac Biomarkers of Low Cardiac Output Syndrome in the Postoperative Period After Congenital Heart Disease Surgery in Children.

INTRODUCTION AND OBJECTIVES: To assess the predictive value of atrial natriuretic peptide, ß-type natriuretic peptide, copeptin, mid-regional pro-adrenomedullin (MR-proADM) and cardiac troponin I (cTn-I) as indicators of low cardiac output syndrome in children with congenital heart disease undergoing cardiopulmonary bypass (CPB). METHODS: After corrective surgery for congenital heart disease under CPB, 117 children (aged 10 days to 180 months) were enrolled in a prospective observational pilot study during a 2-year period. The patients were classified according to whether they developed low cardiac output syndrome. Biomarker levels were measured at 2, 12, 24, and 48 hours post-CPB. The clinical data and outcome variables were analyzed by a multiple logistic regression model. RESULTS: Thirty-three (29%) patients developed low cardiac output syndrome (group 1) and the remaining 84 (71%) patients were included in group 2. cTn-I levels >14 ng/mL at 2hours after CPB (OR, 4.05; 95%CI, 1.29-12.64; P=.016) and MR-proADM levels>1.5 nmol/L at 24hours following CPB (OR, 15.54; 95%CI, 4.41-54.71; P<.001) were independent predictors of low cardiac output syndrome. CONCLUSIONS: Our results suggest that cTn-I at 2hours post-CPB is, by itself, an evident independent early predictor of low cardiac output syndrome. This predictive capacity is, moreover, reinforced when cTn-I is combined with MR-proADM levels at 24hours following CPB. These 2 cardiac biomarkers would aid in therapeutic decision-making in clinical practice and would also enable clinicians to modify the type of support to be used in the pediatric intensive care unit.