Intravenous prostaglandin E1 (alprostadil) bolus in ductus arteriosus-dependent CHD: valid or absolutely contraindicated?

The use of prostaglandin E1 is well documented in ductus arteriosus-dependent CHD or in neonatal pulmonary pathologies that cause severe pulmonary hypertension. The intravenous infusion is well established in loading infusion and maintenance with an onset of action of 30 minutes until 2 hours or even more. Our aim is to report three patients with pulmonary atresia that presented hypercyanotic spell due to a ductal spasm during cardiac catheterisation in whom the administration of a bolus of alprostadil reversed the spasm and increased pulmonary flow, immediately stabilising the condition of the patients allowing subsequent successful stent placement with no serious complications or sequelae after the administration of the bolus. More studies are needed to make a recommendation regarding the use of alprostadil in bolus in cases where the ductal spasm might jeopardise the life of the patient.

Validación del instrumento Infant Malnutrition and Feeding Checklist for Congenital Heart Disease, una herramienta para identificar riesgo de desnutrición y dificultades de alimentación en lactantes con cardiopatías congénitas / Validation of the instrument “Infant Malnutrition and Feeding Checklist for Congenital Heart Disease”, a tool to identify risk of malnutrition and feeding difficulties in infants with congenital heart disease

Introducción: actualmente, se han diseñado diversas herramientas para detectar oportunamente el riesgo de desnutrición en niños hospitalizados. En aquellos con diagnóstico de cardiopatías congénitas (CC), solo existe una herramienta desarrollada en Canadá, llamada Infant Malnutrition and Feeding Checklist for Congenital Heart Disease (IMFC:CHD), la cual fue diseñada en idioma inglés. Objetivo: evaluar la validez y confiabilidad de la adaptación en español de la herramienta IMFC:CHD en lactantes con CC. Métodos: estudio transversal de validación realizado en dos etapas: la primera, de traducción y adaptación transcultural de la herramienta; y la segunda, de validación de la nueva herramienta traducida, donde se obtuvieron las evidencias de confiabilidad y validez. Resultados: en la primera etapa se obtuvo la herramienta traducida y adaptada al idioma español; para la segunda etapa se incluyeron 24 lactantes con diagnóstico de CC. Se evaluó la validez de criterio concurrente entre la herramienta de tamizaje y la evaluación antropométrica, obteniéndose un acuerdo sustancial (κ = 0,660, IC 95 %: 0,36-0,95). Para la validez de criterio predictiva, la cual fue comparada con los días de estancia hospitalaria, se obtuvo un acuerdo moderado (κ = 0,489, IC 95 %: 0,1-0,8). La confiabilidad de la herramienta se evaluó mediante consistencia externa, midiendo la concordancia interobservador, y se obtuvo un acuerdo sustancial (κ = 0,789, IC 95 %: 0,5-0,9); la reproducibilidad de la herramienta mostró un acuerdo casi perfecto (κ = 1, IC 95 %: 0,9-1,0). Conclusiones: la herramienta IMFC:CHD mostró una adecuada validez y confiabilidad, por lo que podría considerarse un recurso útil para la identificación de desnutrición grave. (AU)

Introduction: currently, various tools have been designed to timely detect the risk of malnutrition in hospitalized children. In those with a diagnosis of congenital heart disease (CHD), there is only one tool developed in Canada: Infant Malnutrition and Feeding Checklist for Congenital Heart Disease (IMFC:CHD), which was designed in English. Objective: to evaluate the validity and reliability of the Spanish adaptation of the IMFC:CHD tool in infants with CHD. Methods: cross-sectional validation study carried out in two stages. The first, of translation and cross-cultural adaptation of the tool, and the second, of validation of the new translated tool, where evidence of reliability and validity were obtained. Results: in the first stage, the tool was translated and adapted to the Spanish language; for the second stage, 24 infants diagnosed with CHD were included. The concurrent criterion validity between the screening tool and the anthropometric evaluation was evaluated, obtaining a substantial agreement (κ = 0.660, 95 % CI: 0.36-0.95) and for the predictive criterion validity, which was compared with the days of hospital stay, moderate agreement was obtained (κ = 0.489, 95 % CI: 0.1-0.8). The reliability of the tool was evaluated through external consistency, measuring theinter-observer agreement, obtaining a substantial agreement (κ = 0.789, 95 % CI: 0.5-0.9), and the reproducibility of the tool showed an almost perfect agreement (κ = 1, CI 95 %: 0.9-1.0). Conclusions: the IMFC:CHD tool showed adequate validity and reliability, and could be considered as a useful resource for the identification of severe malnutrition. (AU)

[Validation of the instrument "Infant Malnutrition and Feeding Checklist for Congenital Heart Disease", a tool to identify risk of malnutrition and feeding difficulties in infants with congenital heart disease]. / Validación del instrumento Infant Malnutrition and Feeding Checklist for Congenital Heart Disease, una herramienta para identificar riesgo de desnutrición y dificultades de alimentación en lactantes con cardiopatías congénitas.

Introduction: Introduction: currently, various tools have been designed to timely detect the risk of malnutrition in hospitalized children. In those with a diagnosis of congenital heart disease (CHD), there is only one tool developed in Canada: Infant Malnutrition and Feeding Checklist for Congenital Heart Disease (IMFC:CHD), which was designed in English. Objective: to evaluate the validity and reliability of the Spanish adaptation of the IMFC:CHD tool in infants with CHD. Methods: cross-sectional validation study carried out in two stages. The first, of translation and cross-cultural adaptation of the tool, and the second, of validation of the new translated tool, where evidence of reliability and validity were obtained. Results: in the first stage, the tool was translated and adapted to the Spanish language; for the second stage, 24 infants diagnosed with CHD were included. The concurrent criterion validity between the screening tool and the anthropometric evaluation was evaluated, obtaining a substantial agreement (κ = 0.660, 95 % CI: 0.36-0.95) and for the predictive criterion validity, which was compared with the days of hospital stay, moderate agreement was obtained (κ = 0.489, 95 % CI: 0.1-0.8). The reliability of the tool was evaluated through external consistency, measuring the inter-observer agreement, obtaining a substantial agreement (κ = 0.789, 95 % CI: 0.5-0.9), and the reproducibility of the tool showed an almost perfect agreement (κ = 1, CI 95 %: 0.9-1.0). Conclusions: the IMFC:CHD tool showed adequate validity and reliability, and could be considered as a useful resource for the identification of severe malnutrition.

Introducción: Introducción: actualmente, se han diseñado diversas herramientas para detectar oportunamente el riesgo de desnutrición en niños hospitalizados. En aquellos con diagnóstico de cardiopatías congénitas (CC), solo existe una herramienta desarrollada en Canadá, llamada Infant Malnutrition and Feeding Checklist for Congenital Heart Disease (IMFC:CHD), la cual fue diseñada en idioma inglés. Objetivo: evaluar la validez y confiabilidad de la adaptación en español de la herramienta IMFC:CHD en lactantes con CC. Métodos: estudio transversal de validación realizado en dos etapas: la primera, de traducción y adaptación transcultural de la herramienta; y la segunda, de validación de la nueva herramienta traducida, donde se obtuvieron las evidencias de confiabilidad y validez. Resultados: en la primera etapa se obtuvo la herramienta traducida y adaptada al idioma español; para la segunda etapa se incluyeron 24 lactantes con diagnóstico de CC. Se evaluó la validez de criterio concurrente entre la herramienta de tamizaje y la evaluación antropométrica, obteniéndose un acuerdo sustancial (κ = 0,660, IC 95 %: 0,36-0,95). Para la validez de criterio predictiva, la cual fue comparada con los días de estancia hospitalaria, se obtuvo un acuerdo moderado (κ = 0,489, IC 95 %: 0,1-0,8). La confiabilidad de la herramienta se evaluó mediante consistencia externa, midiendo la concordancia interobservador, y se obtuvo un acuerdo sustancial (κ = 0,789, IC 95 %: 0,5-0,9); la reproducibilidad de la herramienta mostró un acuerdo casi perfecto (κ = 1, IC 95 %: 0,9-1,0). Conclusiones: la herramienta IMFC:CHD mostró una adecuada validez y confiabilidad, por lo que podría considerarse un recurso útil para la identificación de desnutrición grave.

Presentación y manejo de las cardiopatías congénitas en el primer año de edad / Presentation and management of congenital heart disease in the first year of age

Resumen Las cardiopatías congénitas (CC) se definen como una anomalía estructural del corazón o de los grandes vasos intratorácicos. Constituyen la malformación congénita más frecuente al nacimiento. Al menos un tercio de los pacientes requieren algún tipo de intervención antes del año de edad. Las manifestaciones clínicas de las cardiopatías en la etapa neonatal se presentan con un amplio contexto clínico y se pueden confundir con problemas a nivel pulmonar o infeccioso, lo que dificulta su diagnóstico y con ello contribuyendo de forma importante a la mortalidad y morbilidad de estos pacientes, ya que se retrasa el diagnóstico y manejo oportuno. El monitoreo por oximetría de pulso en el periodo neonatal se utiliza actualmente como método diagnóstico para la detección de cardiopatías congénitas críticas; a pesar de que las detecta en forma temprana, en muchos países aún no se lleva a cabo. El objetivo de este artículo es ofrecer un panorama general de la presentación clínica, aspectos diagnósticos y manejo inicial de las CC en el primer año de edad que pueda ser de utilidad a los médicos de primer contacto para mejorar la atención en este grupo de pacientes.

Abstract Congenital heart disease (CHD) is defined as a structural abnormality of the heart or large intrathoracic vessels. They constitute the most frequent congenital malformation at birth. At least one third of patients require some type of intervention before the year of age. The clinical manifestations of heart disease in the neonatal stage are presented with a wide clinical context and can be confused with problems at the pulmonary or infectious level making difficult to diagnose them and thereby contributing significantly to the mortality and morbility of these patients since the diagnosis is delayed and timely handling. Pulse oximetry monitoring in the neonatal period is currently used as a diagnostic method for the detection of critical congenital heart disease. Although it detects them early, in many countries it is not yet carried out. The objective of this article is to offer an overview of the clinical presentation, diagnostic aspect and initial management of CHD in the first year of age that may be useful to first contact physicians to improve the management of this group of patients.

Pilot phase experience of the International Quality Improvement Collaborative catheterization registry.

OBJECTIVES: To describe the development of a quality collaborative for congenital cardiac catheterization centers in low and middle-income countries (LMICs) including pilot study data and a novel procedural efficacy measure. BACKGROUND: Absence of congenital cardiac catheterization registries in LMICs led to the development of the International Quality Improvement Collaborative Congenital Heart Disease Catheterization Registry (IQIC-CHDCR). As a foundation for this initiative, the IQIC is a collaboration of pediatric cardiac surgical programs from LMICs. Participation in IQIC has been associated with improved patient outcomes. METHODS: A web-based registry was designed through a collaborative process. A pilot study was conducted from October through December 2017 at seven existing IQIC sites. Demographic, hemodynamic, and adverse event data were obtained and a novel tool to assess procedural efficacy was applied to five specific procedures. Procedural efficacy was categorized using ideal, adequate, and inadequate. RESULTS: A total of 429 cases were entered. Twenty-five adverse events were reported. The five procedures for which procedural efficacy was measured represented 48% of cases (n = 208) and 71% had complete data for analysis (n = 146). Procedure efficacy was ideal most frequently in patent ductus arteriosus (95%) and atrial septal defect (90%) device closure, and inadequate most frequently in coarctation procedures (100%), and aortic and pulmonary valvuloplasties (50%). CONCLUSIONS: The IQIC-CHDCR has designed a feasible collaborative to capture catheterization data in LMICs. The novel tool for procedural efficacy will provide valuable means to identify areas for quality improvement. This pilot study and lessons learned culminated in the full launch of the IQIC-CHDCR.

Presentation and management of congenital heart disease in the first year of age / Presentación y manejo de las cardiopatías congénitas en el primer año de edad

Congenital heart disease (CHD) is defined as a structural abnormality of the heart or large intrathoracic vessels. They constitute the most frequent congenital malformation at birth. At least one third of patients require some type of intervention before the year of age. The clinical manifestations of heart disease in the neonatal stage are presented with a wide clinical context and can be confused with problems at the pulmonary or infectious level making difficult to diagnose them and thereby contributing significantly to the mortality and morbility of these patients since the diagnosis is delayed and timely handling. Pulse oximetry monitoring in the neonatal period is currently used as a diagnostic method for the detection of critical congenital heart disease. Although it detects them early, in many countries it is not yet carried out. The objective of this article is to offer an overview of the clinical presentation, diagnostic aspect and initial management of CHD in the first year of age that may be useful to first contact physicians to improve the management of this group of patients.

Las cardiopatías congénitas (CC) se definen como una anomalía estructural del corazón o de los grandes vasos intratorácicos. Constituyen la malformación congénita más frecuente al nacimiento. Al menos un tercio de los pacientes requieren algún tipo de intervención antes del año de edad. Las manifestaciones clínicas de las cardiopatías en la etapa neonatal se presentan con un amplio contexto clínico y se pueden confundir con problemas a nivel pulmonar o infeccioso, lo que dificulta su diagnóstico y con ello contribuyendo de forma importante a la mortalidad y morbilidad de estos pacientes, ya que se retrasa el diagnóstico y manejo oportuno. El monitoreo por oximetría de pulso en el periodo neonatal se utiliza actualmente como método diagnóstico para la detección de cardiopatías congénitas críticas; a pesar de que las detecta en forma temprana, en muchos países aún no se lleva a cabo. El objetivo de este artículo es ofrecer un panorama general de la presentación clínica, aspectos diagnósticos y manejo inicial de las CC en el primer año de edad que pueda ser de utilidad a los médicos de primer contacto para mejorar la atención en este grupo de pacientes.

Del soplo inocente a la hipertensión arterial pulmonar / From an innocent heart murmur to pulmonary arterial hypertension

Resumen Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Abstract Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

From an innocent heart murmur to pulmonary arterial hypertension.

Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Outcomes of the Non-fenestrated Fontan Procedure at High Altitude.

BACKGROUND: Although high altitude has been considered a risk factor for the Fontan operation, and an indication for fenestration, there is a paucity of data to support its routine use. Fenestration, with its necessary right to left induced shunt, together with the lower partial pressure of oxygen found with progressive altitude, can significantly decrease hemoglobin oxygen saturation, and therefore, it would be desirable to avoid it. OBJECTIVE: To analyze immediate and medium-term results of the non-fenestrated, extracardiac, Fontan procedure at high altitude. METHODS: Retrospective analysis of data from consecutive patients who underwent non-fenestrated, extracardiac, Fontan procedure at two institutions located in Mexico City at 2,312 m (7,585 ft) and 2,691 m (8,828 ft) above sea level. High altitude was not considered a risk factor. RESULTS: Thirty-nine patients were included, with a mean age of 6.7 years. Mean preoperative indexed pulmonary vascular resistance was 1.7 Wood units. Seventy-nine percent of the patients extubated in the operating room. There was one in-hospital death (2.56%) and one at follow-up. Median chest tube drainage time was 6.5 and 6 days for the right and left pleural spaces. Median oxygen saturation at discharge was 90%. At a median follow-up of six months, all survivors, except one, had good tolerance to daily life activities. CONCLUSIONS: The present study shows good short- and medium-term results for the non-fenestrated, extracardiac, Fontan operation at altitudes between 2,300 and 2,700 m and might favor this strategy over fenestration to improve postoperative oxygen saturation. Further studies to examine the long-term outcomes of this approach need to be considered.