[Diagnosis and treatment in a series of patients with cardiac corrected transposition of the great arteries (double discordance)]. / Diagnóstico y tratamiento en una serie de pacientes con transposición corregida de las grandes arterias (doble discordancia cardiaca).

Objective: The atrio-ventricular and ventricle-arterial double discordance (DD) or corrected transposition of the great arteries is a rare heart disease, it occurs in 0.02-0.07 of every 1,000 live newborns. The objective of the study is to describe the diagnosis, treatment and evolution of a series of patients with DD. Method: A retrospective and descriptive study was carried out, reviewing the records of patients diagnosed with DD in the last 22 years. Descriptive statistics were performed. Numerical variables were obtained using means and standard deviation and categorical variables using frequencies and percentages. Results: Thirty patients were studied in 22 years with a ratio of 1.5:1 for men, with a mean age of 20 months. The situs was solitus in 24/29 patients (82.7%). Ventricular septal defect was the most frequent lesion in 25/29 (86.2%) Tricuspid insufficiency in 70%. Four patients diagnosed with pulmonary atrial hypertension. With atrio-ventricular block 20%. One with Wolff-Parkinson-White syndrome. Surgical treatment was carried out in 70% of patients. Eight with Glenn procedure (26.6%) and 4 with Fontan surgery (13.3%). Follow-up ranged from 1 month to 17 years. Five died (16.6%). Of the 25 patients in follow-up, 18 patients (72%) had normal ventricular function, 5 with Grade II Ross classification (20%) and 2 in Grade III (8%). Conclusions: The quality of life of these patients is improving and there is still controversy in the literature about the ideal time to perform the most appropriate surgical procedure.

Objetivo: La doble discordancia auriculo-ventricular y ventrículo-arterial (DD) o transposición corregida de las grandes arterias, se presenta en 0.02-0.07 de cada 1,000 recién nacidos vivos. El objetivo del estudio es describir el diagnóstico, tratamiento y evolución de pacientes con DD. Método: Se realizó un estudio retrospectivo y descriptivo, revisando los registros de pacientes con DD en los últimos 22 años. Se realizó estadística descriptiva. Las variables numéricas se obtuvieron mediante medias y desviación estándar y las categóricas mediante frecuencias y porcentajes. Resultados: Se estudiaron 30 pacientes con una relación de 1.5:1 para el varón, con una edad media de 20 meses. El situs fue solitus en 24/29 pacientes (82.7%). La comunicación interventricular fue la lesión más frecuente en 25/29 pacientes (86.2%), insuficiencia tricuspídea en el 70%. Cuatro pacientes con diagnóstico de hipertensión arterial pulmonar. Con bloqueo atrio-ventricular un 20%. Uno con síndrome de Wolff-Parkinson-White. El tratamiento quirúrgico se realizó en el 70% de los pacientes. Con procedimiento de Glenn 8 (26.6%) y 4 cirugías de Fontan (13.3%). El seguimiento fue de 1 mes a 17 años. Cinco fallecieron (16.6%). De los 25 restantes, 18 pacientes (72%) con función ventricular normal, 5 con clasificación de Ross grado II (20%) y 2 en G III (8%). Conclusiones: La calidad de vida de estos pacientes está mejorando, aún existe controversia sobre el momento ideal para realizar el procedimiento más adecuado quirúrgico.

COVID-19 international experience in paediatric patients with congenital heart disease.

OBJECTIVE: As COVID-19 continues to affect the global population, it is crucial to study the impact of the disease in vulnerable populations. This study of a diverse, international cohort aims to provide timely, experiential data on the course of disease in paediatric patients with congenital heart disease (CHD). METHODS: Data were collected by capitalising on two pre-existing CHD registries, the International Quality Improvement Collaborative for Congenital Heart Disease: Improving Care in Low- and Middle-Income Countries and the Congenital Cardiac Catheterization Project on Outcomes. 35 participating sites reported data for all patients under 18 years of age with diagnosed CHD and known COVID-19 illness during 2020 identified at their institution. Patients were classified as low, moderate or high risk for moderate or severe COVID-19 illness based on patient anatomy, physiology and genetic syndrome using current published guidelines. Association of risk factors with hospitalisation and intensive care unit (ICU) level care were assessed. RESULTS: The study included 339 COVID-19 cases in paediatric patients with CHD from 35 sites worldwide. Of these cases, 84 patients (25%) required hospitalisation, and 40 (12%) required ICU care. Age <1 year, recent cardiac intervention, anatomical complexity, clinical cardiac status and overall risk were all significantly associated with need for hospitalisation and ICU admission. A multivariable model for ICU admission including clinical cardiac status and recent cardiac intervention produced a c-statistic of 0.86. CONCLUSIONS: These observational data suggest risk factors for hospitalisation related to COVID-19 in paediatric CHD include age, lower functional cardiac status and recent cardiac interventions. There is a need for further data to identify factors relevant to the care of patients with CHD who contract COVID-19 illness.

Early Bidirectional Glenn Procedure as Initial Surgical Palliation for Functionally Univentricular Heart with Common Arterial Trunk.

We report an unusual case of a 2-month-old baby with a diagnosis of common arterial trunk and double outlet right ventricle with a remote type ventricular septal defect. Taking into consideration the physiologic moment and anatomic findings of the patient, we planned and successfully performed a bidirectional Glenn procedure as its first palliative procedure.

Análisis de costoefectividad de la oximetría de pulso como prueba de detección de las cardiopatías congénitas críticas en México.

OBJETIVO: Realizar un análisis de costoefectividad de la oximetría de pulso como prueba de detección neonatal de las cardiopatías congénitas críticas (CCC) en México. Material y métodos. Se realizó un estudio de evaluación económica desde la perspectiva de los proveedores de servicios de salud. A través de un árbol de decisiones se comparó la examinación física versus la examinación física junto con la prueba de oxi-metría de pulso (POP). RESULTADOS: Por cada 10 000 recién nacidos, la alternativa con la POP detectó 32 casos adicionales de CCC, con una razón de costoefectividad incremental de 1 219 USD y una probabilidad de costoefectividad mayor a 90% con una disposición a pagar de 25 000 USD por cada detección temprana. Conclusión. La costoefectividad de la POP, como tamiz neonatal cardiaco, es mayor en México que en países de altos ingresos, y representa una inversión costoefectiva para ganar años de vida en la población infantil de México.

Management of Hypoplastic Left Heart Syndrome in Low-Resource Settings and the Ethics of Decision-Making.

Hypoplastic left heart syndrome (HLHS) is possibly the most challenging congenital heart defect to confront in any setting. The highly specialized infrastructure and resources needed to treat HLHS is not available in many low-resource settings. However, low-resource settings must not be assumed to be synonymous with low- and middle-income countries as national income is not necessarily indicative of a country's prioritization of healthcare resources. Besides, a low-resource setting may be institution-specific as well as country-specific. We have stratified institutional capabilities for addressing the requirements of treatment for HLHS into five levels based on the capacity for diagnosis, intervention, and post-discharge monitoring. Depending on institutional capabilities, children born with HLHS in low-resource settings experience a spectrum of outcomes ranging from death without diagnosis to the hybrid or Norwood stage 1 palliation. The decision-making is ethically challenging when resources are scarce and economic efficiency must be considered in the context of distributive justice. Even in settings that would be classified as resource-rich where survival after surgery and quality of life afterward keep improving, not every parent would choose surgical intervention for their hypothetical child with HLHS.

Surgical Management for Late Presentation of Interrupted Aortic Arch with Aortopulmonary Window.

We report a case of advanced age at presentation of interrupted aortic arch with aortopulmonary window. Although the association between these two defects is well established, their overall prevalence is extremely low. This case presents us with an excellent opportunity to discuss decision making regarding aortic arch repair techniques according to age at which primary surgical correction is intended.

Early Extubation in a Pediatric Cardiac Surgery Program Located at High Altitude.

BACKGROUND: Early extubation is performed either in the operating room or in the cardiovascular intensive care unit during the first 24 postoperative hours; however, altitude might possibly affect the process. The aim of this study is the evaluation of early extubation feasibility of patients undergoing congenital heart surgery in a center located at 2,691 m (8,828 ft.) above sea level. MATERIAL AND METHODS: Patients undergoing congenital heart surgery, from August 2012 through December 2018, were considered for early extubation. The following variables were recorded: weight, serum lactate, presence or not of Down syndrome, optimal oxygenation and acid-base status according to individual physiological condition (biventricular or univentricular), age, bypass time, and ventricular function. Standardized anesthetic management with dexmedetomidine-fentanyl-rocuronium and sevoflurane was used. If extubation in the operating room was considered, 0.08 mL/kg of 0.5% ropivacaine was injected into the parasternal intercostal spaces bilaterally before closing the sternum. RESULTS: Four hundred seventy-eight patients were operated and 81% were early extubated. Mean pre- and postoperative SaO2 was 92% and 98%; postoperative SaO2 for Glenn and Fontan procedures patients was 82% and 91%, respectively. Seventy-three percent of patients who underwent Glenn procedure, 89% of those who underwent Fontan procedure (all nonfenestrated), and 85% with Down syndrome were extubated in the operating room. Reintubation rate in early extubated patients was 3.6%. CONCLUSION: Early extubation is feasible, with low reintubation rates, at 2,691 m (8,828 ft.) above sea level, even in patients with single ventricle physiology.

Norwood Procedure in a Patient With Hypoplastic Left Heart Syndrome, Right Aortic Arch, and Right Descending Aorta.

We describe the surgical treatment of a patient with hypoplastic left heart syndrome and right aortic arch.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica.

Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease. Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery. Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Objetivo: Siguiendo el notable trabajo realizado por la Asociación Mexicana de Especialistas en Cardiopatías Congénitas (Asociación Mexicana de Especialistas en Cardiopatías Congénitas: AMECC) con el desarrollo de un registro nacional para la cirugía cardíaca congénita, la Sociedad Mundial de Pediatría y Cirugía Cardíaca Congénita ha implementado una plataforma internacional para recopilar datos y analizar los resultados de los niños con cardiopatía congénita. Metodología: Este manuscrito propone una posible colaboración entre la base nacional de datos cardiacos congénitos de México (RENACCAPE) y la Base de Datos Mundial para la Cirugía Cardíaca Pediátrica y Congénita (WDPCHS). Conclusión: Esta asociación promovería el deseo de los países de seguir desarrollando procesos de mejora de la calidad y mejorar el tratamiento general de los niños con cardiopatía congénita.

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.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica.

Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease. Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery. Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Objetivo: Siguiendo el notable trabajo realizado por la Asociación Mexicana de Especialistas en Cardiopatías Congénitas (Asociación Mexicana de Especialistas en Cardiopatías Congénitas: AMECC) con el desarrollo de un registro nacional para la cirugía cardíaca congénita, la Sociedad Mundial de Pediatría y Cirugía Cardíaca Congénita ha implementado una plataforma internacional para recopilar datos y analizar los resultados de los niños con cardiopatía congénita. Metodología: Este manuscrito propone una posible colaboración entre la base nacional de datos cardiacos congénitos de México (RENACCAPE) y la Base de Datos Mundial para la Cirugía Cardíaca Pediátrica y Congénita (WDPCHS). Conclusión: Esta asociación promovería el deseo de los países de seguir desarrollando procesos de mejora de la calidad y mejorar el tratamiento general de los niños con cardiopatía congénita.

The world database for pediatric and congenital heart surgery: A collaboration with the Registro Nacional de Cirugía Cardiaca Pediátrica / La base de datos mundial de cirugía cardíaca pediátrica y congénita: una colaboración con el Registro Nacional de Cirugía Cardiaca Pediátrica (RENACCAPE)

Abstract Objective: Following the notable work accomplished by the Mexican Association of Specialists in Congenital Heart Disease (Asociación Mexicana de Especialistas en Cardiopatías Congénitas) with the development of a national registry for congenital cardiac surgery, the World Society for Pediatric and Congenital Heart Surgery has implemented an international platform to collect data and analyze outcomes of children with congenital heart disease. Methodology: This manuscript proposes a possible collaboration between Mexico's national congenital cardiac database (Registro Nacional de Cirugía Cardíaca Pediátrica) and the World Database for Pediatric and Congenital Heart Surgery. Conclusion: Such a partnership would advance the countries' desire for the ongoing development of quality improvement processes and improve the overall treatment of children with congenital heart disease.

Resumen Objetivo: Siguiendo el notable trabajo realizado por la Asociación Mexicana de Especialistas en Cardiopatías Congénitas (Asociación Mexicana de Especialistas en Cardiopatías Congénitas: AMECC) con el desarrollo de un registro nacional para la cirugía cardíaca congénita, la Sociedad Mundial de Pediatría y Cirugía Cardíaca Congénita ha implementado una plataforma internacional para recopilar datos y analizar los resultados de los niños con cardiopatía congénita. Metodología: Este manuscrito propone una posible colaboración entre la base nacional de datos cardiacos congénitos de México (RENACCAPE) y la Base de Datos Mundial para la Cirugía Cardíaca Pediátrica y Congénita (WDPCHS). Conclusión: Esta asociación promovería el deseo de los países de seguir desarrollando procesos de mejora de la calidad y mejorar el tratamiento general de los niños con cardiopatía congénita.

Outcomes of hypoplastic left heart syndrome and fetal aortic valvuloplasty in a country with suboptimal postnatal management.

BACKGROUND: Fetal aortic stenosis (AoS) may progress to hypoplastic left heart syndrome (HLHS) in utero. There are currently no data, prenatal or postnatal, describing survival of fetuses or neonates with AoS or HLHS in a country with suboptimal postnatal management. STUDY DESIGN: Prospective cohort study performed in Mexico, including cases diagnosed with AoS and HLHS within a 6-year period. AoS patients fulfilling previously published criteria for evolving HLHS (eHLHS) were offered fetal aortic valvuloplasty. Outcome variables were perinatal mortality, postnatal management, type of postnatal circulation, and overall survival. RESULTS: Fifty-four patients were included: 16 AoS and 38 HLHS. Eighteen patients had associated anomalies and/or an abnormal karyotype. Seventy-four percent of HLHS received comfort measures, with only three cases reporting an attempt at surgical palliation, and one survivor of the first stage. Fetal aortic valvuloplasty was performed successfully in nine cases of eHLHS. Overall postnatal survival was 44% in AoS with fetal aortic valvuloplasty, and one case (ongoing) in the HLHS group. CONCLUSIONS: HLHS in Mexico carries more than a 95% risk of postnatal death, with little or no experience at surgical palliation in most centers. Fetal aortic valvuloplasty in AoS may prevent progression to HLHS and in this small cohort was associated with ≈50% survival.

A Novel Private-Public Hybrid Model for Treatment of Congenital Heart Disease in Mexico.

Mortality after surgery for congenital heart disease (CHD) in Mexico is significantly higher than in high-income countries due to structural, medical, and financial factors. In Mexico, public hospitals have a large volume of patients but inadequate quality control systems, whereas private hospitals, although having higher quality control systems, have an insufficient number of patients to build programs of excellence. We describe the creation of a novel hybrid private-public program in Mexico that leverages the advantages of both sectors while establishing an integrated multidisciplinary unit that has allowed us to improve the quality of care for patients with CHD.

Partnership models for the establishment of sustainable paediatric cardiac surgical and cardiac intensive care programmes in low- and middle-income countries.

The care of patients with CHD remains a challenge in low- and middle-income countries. Their health systems have not been able to achieve consistently high performance in this field. The large volume of patients, manpower constraints, inconsistencies in the level and type of background training of the teams caring for this patient population, and the inadequate quality control systems are some of the barriers to achieving excellence of care. We describe three different international projects supporting the paediatric cardiac surgical and paediatric cardiac intensive care programmes in Latin America, Asia, and the Caribbean.

Seguridad de la re-esternotomía media en el tratamiento paliativo de pacientes con corazón univentricular / Safety of repeat median sternotomy in the palliative treatment of patients with a univentricular heart

Resumen: Objetivo: Describir la morbimortalidad operatoria relacionada a re-esternotomía media en pacientes con corazón univentricular en el Instituto Nacional de Pediatría. Método: Estudio de serie de casos retrospectiva, descriptiva y analítica del año 2001 al 2016. Resultados: Se encontraron 65 pacientes que requirieron 76 procedimientos. Hubo 59 primeras re-esternotomías, con promedio de edad de 36 meses (4-176 meses) y peso 12.2 kg (3.2-21.5 kg); se realizaron 40 procedimientos de Glenn y 19 procedimientos de Fontan. Se practicaron 17 segundas re-esternotomías, con promedio de edad de 89 meses (48-156 meses) y 22.7 kg de peso (14.4-41 kg), en quienes se realizaron 17 procedimientos de Fontan. Hubo dos lesiones en primeras re-esternotomías, una lesión a la coronaria derecha con cambios electrocardiográficos, y una apertura incidental de la aurícula derecha que causó hipotensión. Reportamos una muerte secundaria a lesión de la aorta con sangrado masivo durante una segunda re-esternotomía, lo que representa una morbimortalidad del 3.9%. Conclusiones: Concluimos que la re-esternotomía es un procedimiento seguro en nuestro centro.

Abstract: Objective: To establish the morbidity and mortality of patients with univentricular hearts who underwent a repeat median sternotomy at the Instituto Nacional de Pediatría. Method: A retrospective review was performed on the clinical charts of all patients who under-went a repeat median sternotomy from 2001 to 2016. Results: Sixty-five patients underwent 76 surgeries by repeat median sternotomy. Fifty-nine patients had a first repeat median sternotomy, with a mean age of 36 months (range: 4-176 months) and a mean weight of 12.2 kg (range: 3.2-21.5 kg). Forty patients had a Glenn procedure, and 19 patients had a Fontan procedure. There were 17 patients with a second repeat median sternotomy, with a mean age of 89 months (range 48-156 months), and a mean weight of 22.7 kg (14.4-41 kg). A Fontan procedure was performed on all these 17 patients. A section of the right coronary artery with electrocardiographic changes and a right atrium tear that caused hypotension occurred during first repeat sternotomy. An aortic tear occurred during a second repeat sternotomy with massive bleeding and subsequent death. This represents 3.9% of re-entry injuries. Conclusion: It is concluded that repeat median sternotomy is a safe procedure.

[Safety of repeat median sternotomy in the palliative treatment of patients with a univentricular heart]. / Seguridad de la re-esternotomía media en el tratamiento paliativo de pacientes con corazón univentricular.

OBJECTIVE: To establish the morbidity and mortality of patients with univentricular hearts who underwent a repeat median sternotomy at the Instituto Nacional de Pediatría. METHOD: A retrospective review was performed on the clinical charts of all patients who underwent a repeat median sternotomy from 2001 to 2016. RESULTS: Sixty-five patients underwent 76 surgeries by repeat median sternotomy. Fifty-nine patients had a first repeat median sternotomy, with a mean age of 36 months (range: 4-176 months) and a mean weight of 12.2 kg (range: 3.2-21.5 kg). Forty patients had a Glenn procedure, and 19 patients had a Fontan procedure. There were 17 patients with a second repeat median sternotomy, with a mean age of 89 months (range 48-156 months), and a mean weight of 22.7 kg (14.4-41 kg). A Fontan procedure was performed on all these 17 patients. A section of the right coronary artery with electrocardiographic changes and a right atrium tear that caused hypotension occurred during first repeat sternotomy. An aortic tear occurred during a second repeat sternotomy with massive bleeding and subsequent death. This represents 3.9% of re-entry injuries. CONCLUSION: It is concluded that repeat median sternotomy is a safe procedure.

Supravalvular aortic stenosis associated to infectious endocarditis and cerebral vascular disease in a patient with Williams-Beuren Syndrome.

The Williams-Beuren syndrome is a rare genetic disease characterized by: (a) typical facial features; (b) psychomotor retardation with a specific neurocognitive profile; (c) cardiovascular condition and (d) likely transient hypocalcemia in infancy. The objective of this study was to describe the clinic evolution and diagnosis of patient with this syndrome that was associated with endocarditis caused by Streptococcus parasanguis in the ascending aorta and an aneurism located in the fronto-temporal area, which produced a parenchymal hematoma in the left lobe, and subarachnoid hemorrhage. He was treated with ceftriaxone and dicloxacillin. Then we proceeded to correct the aneurysm and perform vegetation resection in aortic arteries with supravalvular aortic stenosis correction. The evolution after one year has been favorable and is currently without neurologic sequelae. A 5-year-old male patient presented a diagnosis of supravalvular aortic stenosis. After cardiac catheterization was performed, he presented a fever and right side paresis. The echocardiogram showed multiple vegetations in the ascendant aortic arch and the supraortic arteries. The blood cultures reported S. parasanguis. The magnetic resonance showed a subarachnoid hemorrhage with an aneurysm and a hematoma.