Anomalía de Ebstein en recién nacido / Ebstein's anomaly in newborn

Introducción: La anomalía de Ebstein es una rara malformación con presentación sintomática respiratoria leve a grave a causa de defectos cardiacos congénitos manifestados durante la niñez. Objetivo: Exponer el caso en el cual el diagnóstico oportuno de esta enfermedad permitió el tratamiento integral del paciente. Presentación del caso: Recién nacido a término, quien a las siete horas de su nacimiento presentó saturación de oxígeno disminuida, examen físico sin cianosis, soplo pansistólico en la auscultación y cardiomegalia detectada por rayos X de tórax. Se realizó ecocardiograma que permitió diagnosticar al bebé como portador de la enfermedad de Ebstein con manifestaciones leves. Una vez que tiene mejoría clínica, se le proporcionó el alta médica con indicación de valoración por cardiología pediátrica. Conclusiones: La anomalía de Ebstein puede comenzar en el período neonatal con sintomatología de severidad variable. La sospecha clínica permite la confirmación diagnóstica y el seguimiento estrecho, sobre todo en casos severos(AU)

Introduction: Ebstein's anomaly is a rare malformation with mild to severe respiratory symptomatic presentation due to congenital heart defects manifested during childhood. Objective: To present the case in which the timely diagnosis of this disease allowed the comprehensive treatment of the patient. Case presentation: Full-term newborn, who at seven hours of birth presented decreased oxygen saturation, physical examination without cyanosis, pansystolic murmur in auscultation and cardiomegaly detected by chest X-rays. An echocardiogram was performed to diagnose the baby as a carrier of Ebstein's disease with mild manifestations. Once he had clinical improvement, he was discharged with an indication for assessment by pediatric cardiology. Conclusions: Ebstein's anomaly may begin in the neonatal period with symptoms of variable severity. Clinical suspicion allows for diagnostic confirmation and close follow-up, especially in severe cases(AU)

Persistencia de conducto arterioso en adulto: a propósito de un caso / Persistence of the duct arteriosus in adults: a propos of a case

Congenital heart disease includes a wide range of heart defects that appear at birth, corresponding to the most frequent group of genetic alterations. They represent the most frequent birth defects in the world, affecting millions of newborns annually. Chile is not exempt from this public health problem, estimating a prevalence of 8-10 per 1,000 live births, similar to international figures. Some of these defects are not diagnosed in a timely manner due to various causes, including causes such as poor clinical translation and limited accessibility to the Public Health system. Thanks to the improvement of technological resources, more cases of congenital heart disease are diagnosed every day and the time of diagnosis is getting earlier. The case presented below refers to a 47-year-old male patient with several comorbidities, who underwent a Doppler echocardiogram during his hospitalization due to acute respiratory failure, where a systodiastolic flow was detected in one of the compatible pulmonary arteries, with a patent ductus arteriosus.

Qual Deve Ser o Tratamento de Primeira Linha para o Fechamento de Persistência de Canal Arterial Hemodinamicamente Significativo em Bebês Prematuros? / What Should be the First-line Treatment for the Closure of Hemodynamically Significant Patent Ductus Arteriosus in Premature Infants?

Resumo Fundamento É importante saber qual medicamento usar como tratamento de primeira linha para fechar o duto. Objetivos O objetivo deste estudo é comparar a eficácia e os efeitos colaterais das formas intravenosas (IV) de ibuprofeno e paracetamol e contribuir para a literatura investigando o primeiro medicamento selecionado no tratamento clínico da persistência do canal arterial (PCA). Métodos Nosso estudo foi realizado entre janeiro de 2017 e dezembro de 2019. Foram incluídos no estudo bebês prematuros com peso ao nascer (PN) ≤1500 g e idade gestacional (IG) ≤32 semanas. No período do estudo, todos os bebês com persistência do canal arterial hemodinamicamente significativa (hsPCA) receberam ibuprofeno intravenoso (IV) como resgate como tratamento clínico primário ou tratamento com paracetamol IV se houvesse contraindicações para o ibuprofeno. Os pacientes foram divididos em dois grupos: pacientes que receberam ibuprofeno IV e pacientes que receberam paracetamol IV. Resultados Desses pacientes, 101 receberam paracetamol IV e 169 receberam ibuprofeno IV. A taxa de sucesso do fechamento da PCA com o primeiro curso do tratamento foi de 74,3% no grupo de paracetamol IV e 72,8% no grupo de ibuprofeno IV (p=0,212). Conclusões Nossos resultados mostram que o paracetamol IV é tão eficaz quanto o ibuprofeno IV no tratamento de primeira linha de hsPCA, podendo se tornar o tratamento preferencial para o controle de hsPCA.

Abstract Background It is important which medicine to use as a first-line treatment to close the duct. Objectives The aim of this study is to compare the effectiveness and side effects of intravenous (IV) forms of ibuprofen and paracetamol and to contribute to the literature investigating the first drug selected in the medical treatment of patent ductus arteriosus (PDA). Methods Our study was conducted between January 2017 and December 2019. Premature infants with birth weight (BW) ≤1500 g and gestational age (GA) ≤32 weeks were included in the study. In the study period, all infants with hemodynamically significant patent ductus arteriosus (hsPDA) were given rescue intravenous (IV) ibuprofen as a primary medical treatment or IV paracetamol treatment if there were contraindications for ibuprofen. The patients were divided into two groups: patients receiving IV ibuprofen and patients receiving IV paracetamol. Results Of these patients, 101 were given IV paracetamol and 169 were given IV ibuprofen. The success rate of PDA closure with first-course treatment was 74.3% in the IV paracetamol group and 72.8% in the IV ibuprofen group (p=0.212). Conclusions Our results show that IV paracetamol is as effective as IV ibuprofen in the first-line treatment of hsPDA, and can become the preferred treatment for the management of hsPDA.

Caso Clínico: Resolución quirúrgica de coartación de aorta en un paciente pediátrico / Case Report: Surgical resolution of aortic coarctation in pediatric patient

INTRODUCCIÓN: La coartación de aorta es una cardiopatía congénita con una incidencia de 4 por cada 10 000 nacidos vivos y puede asociarse o no a ductus arterioso persistente u otras malformaciones. Suele ser asintomático, normalmente se diagnostica mediante los signos clásicos; con gradiente de presión arterial entre extremidades, pulsos reducidos en extremidades inferiores, hipertensión arterial en miembros superiores o, en casos graves, fallo cardiaco izquierdo. Su resolución puede ser percutánea o quirúrgica, dependiendo de la edad y sus características. CASO CLÍNICO: Paciente masculino de 6 años, asintomático, con sospecha de coartación aórtica, por presentar una diferencia entre presiones arteriales de miembros superiores e inferiores, extremidades inferiores con pulsos reducidos y soplo sistólico en foco aórtico. Se solicitó ecocardiograma que informó válvula aórtica bicúspide con rafe, insuficiencia ligera y coartación de aorta; angiotomografía evidenció coartación de aorta yuxtaductal; cateterismo cardiaco objetivó casi nulo paso de contraste a través del defecto, por lo que se programó tratamiento quirúrgico. EVOLUCIÓN: La corrección quirúrgica se realizó mediante coartectomía con anastomosis termino-terminal más cierre del conducto arterioso. Posterior a la intervención se logró objetivar una mejora relevante en el gradiente de presión entre las extremidades. En el postquirúrgico el paciente presentó hipertensión que logró ser controlada, evolucionó favorablemente y recibió el alta a los 4 días sin tratamiento antihipertensivo ambulatorio. CONCLUSIÓN: La expectativa de vida en pacientes intervenidos para corregir su cardiopatía congénita es superior en relación a aquellos que no son intervenidos, por lo que el diagnóstico oportuno supone una herramienta importante para mejorar la calidad y esperanza de vida.

BACKGROUND: Coarctation of the aorta is a congenital heart disease with an incidence of 4 per 10 000 live births, it may or may not be associated with patent ductus arteriosus as well as other malformations. It is usually asymptomatic and diagnosed by its classic signs such as; arterial pressure gradient between extremities, reduced pulses in the lower extremities, arterial hypertension in the upper extremities or, in severe cases, left heart failure. Its resolution can be percutaneous or surgical, depending on the patient's age and the characteristics of the defect. CASE REPORTS: A 6-year-old male patient, asymptomatic, with suspected aortic coarctation, due to a difference between arterial pressures in the upper and lower limbs, lower limbs with reduced pulses, and a systolic murmur in the aortic focus. An echocardiogram was requested, which reported a bicuspid aortic valve with raphe, mild regurgitation, and coarctation of the aorta; CT angiography showed coarctation of the juxtaductal aorta; cardiac catheterization showed almost no passage of contrast through the defect, so surgical treatment was scheduled. EVOLUTION: Surgical correction was performed by coartectomy with end-to-end anastomosis and closure of the ductus arteriosus. After the intervention, a relevant improvement in the pressure gradient between the extremities was observed. In the postoperative period the patient presented hypertension, that we managed to control, the patient progressed favorably and was discharged after 4 days without antihypertensive treatment. CONCLUSION: Life expectancy in patients who underwent surgery to correct a congenital heart disease is higher than in those who don't, so timely diagnosis is an important tool to improve life quality and life expectancy.

Péptido natriurético cerebral tipo B como marcador de sobrecarga hemodinámica del conducto arterioso permeable en el recién nacido prematuro / B-type brain natriuretic peptide as marker of hemodynamic overload of the patent ductus arteriosus in the preterm infant

Resumen Introducción: El ecocardiograma es el método de referencia para el diagnóstico del conducto arterioso permeable (CAP) hemodinámicamente significativo (CAP-hs) del recién nacido prematuro (RNP). El péptido natriurético tipo B (BNP) puede ser útil en el diagnóstico y el manejo del CAP-hs. Objetivo: Evaluar la utilidad del BNP como marcador de sobrecarga hemodinámica del conducto arterioso permeable en el RNP con edad gestacional < 32 semanas o peso < 1500 gramos, e identificar el mejor punto de corte para los valores de BNP que mejor prediga un CAP con repercusión hemodinámica que requiera tratamiento farmacológico o quirúrgico. Método: Estudio retrospectivo, observacional y descriptivo de RNP < 32 semanas de gestación o peso < 1500 gramos en los que se realizó ecocardiograma y determinación del BNP. Análisis de muestra global y por subgrupos, en función del CAP-hs. Resultados: Se analizaron 29 pacientes. Se encontró una correlación significativa entre la relación CAP/peso y los valores del BNP (prueba de Spearman: 0.71; intervalo de confianza del 95%: 0.45-0.87; p < 0.001). El mejor punto de corte del BNP para predecir CAP-hs fue 486.5 pg/ml, con una sensibilidad del 81% y una especificidad del 92% (p < 0.001). Conclusión: El punto de corte del BNP identificado en el presente estudio se correlacionó con la presencia de CAP-hs.

Abstract Introduction: The echocardiogram is the gold standard, in the diagnosis of the hemodynamically significant patent ductus arteriosus (hs-PDA) of the premature newborn (PNB). Type B natriuretic peptide (BNP) may be useful in the diagnosis and management of CAP-hs. Objective: To assess the utility of BNP as a marker of hemodynamic overload of the patent ductus arteriosus in newborns with gestational age < 32 weeks or weight < 1500 grams, and to identify the best cut-off point for BNP levels that would best predict a PDA with hemodynamic impact requiring pharmacological and/or surgical treatment. Methods: Retrospective, observational and descriptive study of PNB < 32 weeks gestation or weight < 1500 grams, in which echocardiogram and BNP determination was performed. Analysis of the global sample and by subgroups, depending on the hs-PDA status was performed. Results: A total of 29 patients were analyzed. A significant correlation was found between the PDA/weight ratio and BNP levels (Spearman: 0.71; 95% confidence interval: 0.45-0.87; p < 0.001). The best BNP cut-off point to predict CAP-hs was 486.5 pg/ml with a sensitivity of 81% and specificity of 92% (p < 0.001). Conclusion: The BNP cut-off point identified in the present study was correlated with the presence of CAP-hs.

Eficacia y seguridad del cierre quirúrgico del conducto arterioso permeable por el cirujano pediatra general: ensayo clínico / Effectiveness and safety of the surgical closure of permeable arteriosus conduct by the general pediatric surgeon: clinical trial

Resumen Antecedentes: El conducto arterioso permeable (CAP) es un defecto cardiaco congénito y se considera un problema de salud pública. Se presenta en un alto porcentaje de recién nacidos y en algunos mayores de 1 mes. El cierre farmacológico es el tratamiento inicial preferido, ya que ha tenido excelentes resultados; sin embargo, en aquellos casos en los que no es posible, está indicado el cierre quirúrgico. Objetivo: Evaluar la eficacia y la seguridad del cierre quirúrgico del CAP por cirujanos pediatras sin especialidad en cirugía cardiovascular. Método: Ensayo clínico realizado en pacientes del Hospital General de Occidente, centro hospitalario público de segundo nivel, con diagnóstico de CAP, que requirieron corrección quirúrgica. Se revisaron en forma retrospectiva los expedientes de enero de 2001 a diciembre de 2018. Resultados: Se incluyeron 224 pacientes divididos en dos grupos: grupo I, con 184 (82%) recién nacidos, y grupo II, con 40 (18%) niños grandes de 2 meses a 8 años de edad. A todos se les realizó cierre quirúrgico: 3 por toracoscopía y 221 por toracotomía posterolateral izquierda. Presentaron complicaciones 36 pacientes, lo que representa el 16% del total; solo el 5.3% fueron complicaciones mayores. Fallecieron 24 pacientes en el posoperatorio, lo que representa una mortalidad del 10.7%; ninguno falleció por complicaciones transquirúrgicas. El CAP es un defecto cardíaco congénito que se presenta en alto porcentaje en pacientes prematuros. El cierre farmacológico es el principal tratamiento por tener excelentes resultados en recién nacidos; sin embargo, en aquellos casos en los que no sea posible está indicado el cierre quirúrgico. Todos los pacientes fueron operados por cirujanos pediatras generales, con una sobrevida global del 92%. Conclusiones: En los hospitales donde no hay cirujano cardiovascular pediátrico ni cardiólogo intervencionista, la corrección quirúrgica del CAP puede ser llevada a cabo por un cirujano pediatra. La técnica es reproducible, fácil de realizar y con mínimas complicaciones.

Abstract Background: The Patent Ductus Arteriosus (PDA) is congenital heart defect and is considered a public health problem. It occurs in a high percentage of newborns and in some older than 1 month. Pharmacological closure is the preferred initial treatment, as it has had excellent results; however, in those cases where it is not possible, surgical closure is indicated. Objective: The objective is to evaluate the efficacy and safety of the surgical closure of the patent PDA when it is carried out by pediatric surgeons without specialization in cardiovascular surgery. Methods: This study was conducted at the West General Hospital, a 2nd level public hospital, with the diagnosis of patent ductus arteriosus that required surgical correction. For the collection of the information, the files from January 2001 to December 2018 were retrospectively reviewed. Results: 224 patients were included; divided into two groups: Group I: 184 (82%) "newborns" and Group II: 40 (18%) "big children" with ages from 2 months to 8 years. All had a surgical closure; 3 by thoracoscopy and 221 by left posterolateral thoracotomy. 36 patients presented complications representing 16% of the total of patients, only 5.3% were major complications. 24 patients died in the postoperative period, representing a mortality of 10.7%, none died due to trans-surgical complications. PDA is a congenital heart defect that occurs in a high percentage of premature patients. The pharmacological closure is the principal treatment because it has had excellent results in newborns; however, in those cases where it is not possible, surgical closure it´s indicated. All patients were operated by general pediatric surgeons, with a global survival of 92%. Conclusions: We conclude that in hospitals where there is no pediatric cardiovascular surgeon or interventional cardiologist, the surgical correction of the PDA can be carried out by a general pediatric surgeon. The technique is reproducible, easy to perform and with minimal complications.

Bloqueo caudal más anestesia general balanceada en un paciente con pentalogía de Cantrell, presentación de un caso clínico / Caudal block plus inhaled general anesthesia in a patient with pentalogy of Cantrell, presentation of a clinical case

The pentalogy of Cantrell is a disorder characterized by congenital abnormalities in the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and cardiac anomalies. It is a rare disease with 250 cases registered around the world. The anesthetic implications will require a specialized management given the ventilatory mechanics and cardiac function which are compromised by the disease in the newborn. We present the case of a female patient with pentalogy of Cantrell without prenatal diagnosis, who had an operative procedure to correct patent ductus arteriosus and abdominal mesh placement under balanced general anesthesia with sevoflurane and fentanyl plus caudal block. This case is reported to provide our experience in the anesthetic management of this type of patients.

La pentalogía de Cantrell es una enfermedad caracterizada por anormalidades congénitas de la pared abdominal supraumbilical, esternón inferior, diafragma, pericardio diafragmático y anomalías cardiacas. Se trata de una enfermedad rara con 250 casos registrados alrededor del mundo. Las implicaciones anestésicas requieren de un manejo especializado debido a la mecánica ventilatoria y función cardíaca que se encuentran comprometidas en el recién nacido. Se presenta el caso de una recién nacida portadora de pentalogía de Cantrell, no diagnosticada prenatalmente, quien fue sometida a corrección de ductus arterioso persistente y colocación de malla abdominal bajo anestesia general balanceada con sevofluorano y fentanilo más bloqueo caudal. Se reporta el presente caso para brindar nuestra experiencia en el manejo anestésico de este tipo de pacientes.

Patent ductus arteriosus closure: experience from a tertiary referral center / Experiência de uma maternidade terciária no fechamento do canal arterial patente

ABSTRACT Objective: To characterize the number and methods of closure of Persistent Ductus Arteriosus (PDA) over a span of 16 years in a third level maternity hospital. Methods: Retrospective study of neonates born between January 2003 and Deccember 2018, who underwent ductus arteriosus closure by pharmacological, surgical and/or transcatheter methods. Gestational age, birth weight, number and methods of closures per year were evaluated. The success rate of the pharmacologic method was calculated, as well as the mortality rate. The association between mortality and birthweight, treatment used and treatment failure was explored. Results: There were 47,198 births, 5,156 were preterm, 325 presented PDA and 106 were eligible for closure (median gestational age - 27 weeks, birthweight <1000 g - 61%). Frequency of PDA closure decreased during the study period, especially starting in 2010. Success rate with pharmacologic treatment was 62% after the first cycle and 74% after the second. After drug failure, 12 underwent surgical ligation and two underwent transcatheter closure. Exclusive surgical ligation was indicated in four infants. Ibuprofen replaced indomethacin in 2010, and acetaminophen was used in three infants. Among the 106 infants, hospital mortality was 12% and it was associated with birthweight <1000 g (13/65 <1000 vs. 0/41 >1000 g; p=0.002) and with failure in the first pharmacologic treatment cycle (13/27 with failure, vs. 0/75 without failure; p<0.001). Conclusions: The national consensus published in 2010 for the diagnosis and treatment of PDA in preterm infants led to a decrease in the indication for closure. Pharmacological closure was the method of choice, followed by surgical ligation. Birthweight <1000 g and first cycle of pharmacologic treatment failure were associated with higher mortality.

RESUMO Objetivo: Caraterizar o número e métodos de fechamento de canal arterial durante 16 anos numa maternidade de nível terciário. Métodos: Estudo retrospetivo de nascidos entre 01 de janeiro de 2003 a 31 de dezembro de 2018 submetidos a fechamento do canal arterial por métodos farmacológico, cirúrgico e/ou percutâneo. Avaliaram-se idade gestacional, sexo, peso ao nascimento, número de fechamentos por ano e método utilizado. Aferiram-se as taxas de sucesso de método farmacológico e de mortalidade e sua associação com peso ao nascer, fármaco utilizado e insucesso do fechamento. Resultados: Verificaram-se 47.198 recém-nascidos, 5.156 prematuros, dos quais 325 com canal arterial patente, sendo 106 com indicação para fechamento (idade gestacional mediana 27 semanas, peso <1000 g em 61%). Verificou-se diminuição do número de fechamentos ao longo dos anos, sobretudo a partir de 2010. O fechamento ocorreu em 62% após primeiro ciclo de tratamento farmacológico e em 74% após segundo. Após insucesso farmacológico, 12 realizaram ligadura cirúrgica e dois, fechamento percutâneo. Houve indicação de ligadura cirúrgica exclusiva em quatro. O ibuprofeno substituiu a indometacina em 2010. O acetaminofen foi usado em três doentes. A mortalidade nos 106 pacientes foi de 12%, associando-se ao peso ao nascer (13/65 <1000 vs. 0/41 >1000 g; p=0,002) e à falha do primeiro ciclo de tratamento farmacológico (13/27 com falha vs. 0/75 com sucesso; p<0,001). Conclusões: Consenso nacional de 2010 para diagnóstico e tratamento do canal arterial nos prematuros levou à diminuição do número de fechamentos desse canal. O fechamento farmacológico foi o método mais utilizado, seguido da ligadura cirúrgica. Peso <1000 g e falha no primeiro ciclo de fechamento farmacológico se associaram à maior mortalidade.

Alternative treatment for patent ductus arteriosus: a therapeutic challenge / Tratamento alternativo do canal arterial pervio: um desafio terapêutico

Abstract The ductus arteriosus is a fetal structure that spontaneously closes in 90% of newborns. Patency 3 months after birth is considered a congenital heart disease that, if untreated, can progress to serious cardiovascular complications. This report aims to review an alternative treatment with an aortic endoprosthesis in a 49-year-old man who presented with dyspnea on moderate exertion associated with a heart murmur. He was diagnosed with persistent ductus arteriosus (PDA) with cardiac complications. Clinical management was unsuccessful and surgical treatment was indicated. Endovascular treatment with a thoracic endoprosthesis was indicated and performed successfully. Percutaneous closure is the preferred method in adult patients. Endovascular intervention using an endoprosthesis is a safe and effective option, in addition to being applicable regardless of the anatomy of the PDA. This case demonstrates the natural history of the pathology and presents a safe and effective alternative for its management.

Resumo O canal arterial é uma estrutura fetal que se fecha espontaneamente em 90% dos recém-nascidos. A permeabilidade por mais de 3 meses é considerada uma cardiopatia congênita que, se não tratada, pode evoluir para complicações cardíacas graves. Este trabalho relata um tratamento alternativo com endoprótese aórtica. Trata-se de um homem, 49 anos, que apresentou dispneia aos moderados esforços, associada com um sopro cardíaco. Foi diagnosticado com canal arterial persistente (PCA) já com complicações cardíacas. Tentou-se manejo clínico sem sucesso, sendo indicado tratamento cirúrgico. Optou-se pelo tratamento endovascular com uma endoprótese torácica, o qual foi realizado com sucesso. O fechamento percutâneo é o método preferido em pacientes adultos. A intervenção endovascular com o uso da endoprótese é uma possibilidade segura e eficaz, além de ser aplicável independentemente da anatomia do PCA. Este caso, além de demonstrar a história natural desta patologia, apresenta uma alternativa segura e eficaz empregada no seu manejo.

Pseudocoartación aórtica en niños con ductus arterioso persistente: dos casos de una patología extremadamente infrecuente en menos de 48 horas / Aortic pseudocoarctation in children with persistent ductus arteriosus: two cases of an extremely uncommon pathology in less than 48 hours

Introducción: mientras la persistencia del ductus arterioso (PDA) es la cardiopatía congénita más frecuente, encontrar una pseudocoartación aórtica es muy poco frecuente y lo es más hallarlo en niños y solo hay unos cuantos casos reportados de este hallazgo en niños asociado a PDA. Material y Métodos: se describen los casos de 2 pacientes (1 con diagnóstico preoperatorio y otro sin él) con esta asociación: tanto los datos preoperatorios, los hallazgos transoperatorios, y su manejo trans y postoperatorio que operamos en menos de 48 horas. (AU)

Introduction: while the persistence of ductus arteriosus (PDA) is the most frequent congenital heart disease, finding an aortic pseudocoarctation is very rare and more find it in children and there are only a few reported cases of this finding in children associated with PDA. Material and Methods: we describe the cases of 2 patients (1 with preoperative diagnosis and another without it) with this association: the preoperative data, the transoperative findings, and their trans and postoperative management that we operated on in less than 48 hours. (AU)

Functional echocardiography training in the neonatal intensive care unit: comparing measurements and results with the pediatric cardiologist / Treinamento em ecocardiografia funcional na unidade de terapia intensiva neonatal: comparando as medidas e resultados com o cardiologista pediátrico

Abstract Objectives: Functional echocardiography is a valuable tool in the neonatal intensive care unit, but training programs are not standardized. The aim was to report an functional echocardiography training program for neonatologists and to describe the agreement of their measurements with the pediatric cardiologist. Methods: Functional echocardiography training lasted 32 h. After training program, the neonatologists performed functional echocardiography in the neonatal intensive care unit and were required to measure left cardiac chambers dimensions, left ventricle systolic function, right and left ventricular output, ductus arteriosus diameter, and flow pattern. Images were recorded by the equipment and reviewed offline by the pediatric cardiologist. The Bland-Altman test was used for quantitative variables and the kappa test, for qualitative variables. Results: Twenty-two trained neonatologists performed 100 functional echocardiography exams. Ductus arteriosus identification and flow pattern had substantial agreement (kappa = 0.91 and 0.88, respectively), as well as its diameter (mean difference = 0.04 mm). The mean difference for the aortic root was −1.2 mm; left atrium, 0.60 mm; left ventricle diastolic diameter, −0.90 mm; left ventricle systolic diameter, −0.30 mm. Shortening fraction and ejection fraction correlated well with broad limits of agreement, −2.96% (14.88; −20.82%) and −-3.43% (15.54; −22.40%), respectively. Right and left ventricular output had broad limits of agreement, 16.69 mL/kg/min (222.76; −189.37) and 23.57 mL/kg/min (157.88; −110), respectively. There was good agreement between interpretations of normal or low cardiac output (76.7% for right ventricular output; 75.7% for left ventricular output). Conclusion: This functional echocardiography training program enabled neonatologists to obtain adequate skills in performing the images, obtaining good agreement with the cardiologist in simple hemodynamic measurements and ductus arteriosus evaluation.

Resumo Objetivos: A ecocardiografia funcional é uma ferramenta valiosa na unidade de terapia intensiva neonatal, mas os programas de treinamento não são padronizados. Nosso objetivo foi relatar um programa de treinamento em ecocardiografia funcional para neonatologistas e descrever a concordância de suas medidas com o cardiologista pediátrico. Métodos: O treinamento em ecocardiografia funcional durou 32 horas. Após o programa de treinamento, os neonatologistas faziam ecocardiografia funcional na unidade de terapia intensiva neonatal e mediam as dimensões das câmaras cardíacas esquerdas, função sistólica do ventrículo esquerdo, débito cardíaco do ventrículo direito e débito cardíaco do ventrículo esquerdo, diâmetro do canal arterial e o padrão de fluxo. As imagens foram registradas no equipamento e revisadas offline pelo cardiologista pediátrico. O teste de Bland-Altman foi usado para variáveis quantitativas e o teste Kappa para variáveis qualitativas. Resultados: Foram feitas por 22 neonatologistas treinados 100 ecocardiografias funcionais. A identificação do canal arterial e o padrão de fluxo apresentaram concordância substancial (Kappa = 0,91 e 0,88, respectivamente), bem como seu diâmetro (diferença média = 0,04 mm). A diferença média foi de -1,2 mm para a raiz da aorta, 0,60 mm para o átrio esquerdo, -0,90 mm para o diâmetro diastólico do ventrículo esquerdo e de -0,30 mm para o diâmetro sistólico do ventrículo esquerdo. A fração de encurtamento e a fração de ejeção apresentaram boas correlações, com amplos limites de concordância, respectivamente -2,96% (14,88; -20,82%) e -3,43% (15,54; -22,40%). Os débitos cardíacos do ventrículo direito e do ventrículo esquerdo apresentaram amplos limites de concordância, 16,69 mL/kg/min (222,76; -189,37) e 23,57 mL/kg/min (157,88; -110), respectivamente. Houve boa concordância entre a interpretação de débito cardíaco normal ou baixo (76,7% de débito ventricular direito; 75,7% de débito ventricular esquerdo). Conclusão: Esse programa de treinamento em ecocardiografia funcional permitiu aos neonatologistas obter habilidades adequadas na realização das imagens, com boa concordância com o cardiologista em medidas hemodinâmicas simples e avaliação do canal arterial.

Unusual presentation of patent ductus arteriosus in elderly patient

Abstract We presented a case of a 56-year-old man with giant pulmonary artery aneurysm caused by a misdiagnosed patent ductus arteriosus, severe multivalvular disease and active aortic valve endocarditis successfully treated by surgery. The correct diagnosis was missed despite preoperative diagnostics because the small patent ductus arteriosus was located at the distal part of common pulmonary trunk and a huge regurgitant signal overlapped its Doppler signal. Thorough evaluation of every patient, regardless of age, is necessary to recognize and treat this congenital anomaly.

Extrapleural closure of patent Ductus Arteriosus: how we do it

Abstract Patent ductus arteriosus (PDA) is a clinical condition mostly found in premature newborns. Among several medical, surgical and interventional treatment options, extrapleural ligation through a left minithoracotomy is recognized as a safe, efficient and less expensive technique. In fact, it requires short surgical times, grants good exposure of the duct and nearby structures (e.g., thoracic duct, left recurrent laryngeal nerve), and avoids pleural space opening and subsequent pulmonary complications in preterm patients. This approach seems ideal due to its lower costs, especially in developing countries with a high birth rate and limited resources.

Análisis de los resultados del programa de ecocardiografía funcional neonatal en un hospital pediátrico de tercer nivel / Analysis of the results of the neonatal functional echocardiography program in a third-level pediatric hospital

Resumen Introducción: Se ha demostrado la utilidad del estudio ecocardiográfico en las enfermedades que afectan a los neonatos en estado crítico, por lo que se debe capacitar al neonatólogo para la toma y la interpretación de variables hemodinámicas que apoyen la toma de decisiones. El objetivo de este estudio fue describir los resultados del programa de ecocardiografía funcional neonatal y comparar las variables clínicas y hemodinámicas entre sobrevivientes y no sobrevivientes. Métodos: Durante un periodo de 2 años se realizó un estudio observacional, transversal y comparativo en neonatos que fueron evaluados con ecocardiografía funcional neonatal por alteraciones hemodinámicas del conducto arterioso persistente, hipertensión pulmonar aguda y crónica, estado de choque y búsqueda de trombos y vegetaciones. Se evaluaron parámetros de funcionalidad de los ventrículos derecho e izquierdo, presión pulmonar y subrogados de sobrecarga pulmonar. Se realizó un análisis comparativo (U de Mann Whitney y Χ2). De acuerdo con los resultados, se emitieron recomendaciones para el inicio, el ajuste o el retiro de fármacos vasoactivos. Resultados: Se realizaron 269 estudios en 119 neonatos (64.7% prematuros, mortalidad del 15%) atendidos por hipertensión pulmonar aguda (38%), conducto arterioso persistente (27%), choque (19%), hipertensión pulmonar crónica (14%) o búsqueda de trombos (2%). Se recomendó cambiar el manejo en el 45% de los estudios. El grupo de no sobrevivientes presentó diferencias significativas en los parámetros de función sistólica del ventrículo derecho y aumento de su poscarga. Conclusiones: La causa principal de la evaluación con ecocardiografía funcional neonatal fue la hipertensión pulmonar. De forma global, se recomendó un ajuste farmacológico en el 45% de los casos.

Abstract Background: Echocardiography is useful in the group of comorbidities of critically ill newborns. The targeted neonatal echocardiography program trains neonatologists for acquiring and interpreting hemodynamic variables to support decision making. This study aimed to describe the results of the functional echocardiography program (fNE) and compare clinical and hemodynamic variables between survivors and non-survivors. Methods: Observational, cross-sectional, and comparative study of neonates that received a fNE evaluation for hemodynamic disturbances related to patent ductus arteriosus (PDA), acute and chronic pulmonary hypertension (aPH, cPH), state of shock and thrombus/vegetations surveillance for two years. Functional parameters of the right and left ventricle, pulmonary pressure, and surrogates of pulmonary over circulation were assessed. Comparative analysis with U Mann Whitney test and Χ2 was performed. Based on the results, recommendations to start, adjust, or withdraw vasoactive medications were issued. Results: Of 269 studies on 119 neonates (65% premature, 15% mortality), the reasons for consultation were aPH (38%), PDA (27%), shock (19%), cPH (14%), and thrombus surveillance (2%). A change in management was recommended on 45% of studies. Non-survivors presented significant differences in the right ventricular (RV) systolic performance and an increased right ventricular afterload. Conclusions: The main indication for fNE was pulmonary hypertension. A pharmacological adjustment was recommended on 45% of the cases.

Caracterización de cardiopatías congénitas en Manizales 2010-2016 / Characterization of Congenital Heart Defects in Manizales 2010-2016 / Caracterização de cardiopatias congênitas em Manizales, Colômbia, 2010-2016

Resumen: Las cardiopatías congénitas son la tercera causa de malformaciones congénitas y una de las principales responsables de la mortalidad en periodo neonatal en el mundo; se originan a partir de alteraciones en el desarrollo embrionario. La incidencia es variable; en Colombia, se estima que afectan del 2 al 3 % de los recién nacidos vivos. En 2015, 20,8 % de los niños menores de cinco años afectados con cardiopatías congénitas fallecieron a causa de estas alteraciones. El presente es un estudio descriptivo y retrospectivo. Se incluyeron los datos de 252 historias clínicas de pacientes con diagnóstico de cardiopatía congénita entre los años 2010 y 2016, el 56 % de la población encontrada fue de sexo femenino. Predominaron las cardiopatías congénitas no cianosantes (87 %). La mayor prevalencia fue el ductus arterioso permeable (35,3 %). La ecocardiografía Doppler se utilizó en 98,4 % de los casos; con respecto al manejo, el 47,2 % de los pacientes estuvieron en seguimiento, siendo este más común que las alternativas médicas o quirúrgicas. En conclusión, el ductus arterioso permeable, la comunicación interventricular y la comunicación interatrial son las cardiopatías no ciano-santes más frecuentes en la población estudiada y se asocian a hipertensión pulmonar que ameritan mayor seguimiento. La tetralogía de Fallot fue la cardiopatía congénita cianosante más común.

Abstract: Congenital heart defects are the third cause of congenital malformations and one of the main causes of mortality in the neonatal period in the world; they originate from alterations in embryonic development. The incidence is variable; in Colombia, it is estimated that they affect 2 to 3% of live newborns. In 2015, 20.8% of children under the age of five affected with congenital heart defects died from these disorders. This is a descriptive and retrospective study. Data of 252 clinical records of patients diagnosed with congenital heart defect between 2010 and 2016 were included, showing that 56% of this population were female. Acyanotic Congenital Heart Diseases predominated (87%). The highest prevalence was patent ductus arteriosus (35.3%). Doppler echocardiography was used in 98.4% of cases; regarding management, 47.2% of patients were in follow-up, this being more common than medical or surgical alternatives. In conclusion patent ductus arteriosus, ventricular septal defect and interatrial septal defect are the most frequent acyanotic heart diseases in the population under study and they are associated with pulmonary hypertension that require higher follow-up. Tetralogy of Fallot was the most common cyanotic congenital heart disease.

Resumo: As cardiopatias congênitas são a terceira causa de malformações congênitas e uma das principais responsáveis pela mortalidade no período neonatal no mundo. São originadas a partir de alterações no desenvolvimento embrionário. A incidência é variável; na Colômbia, é estimado que afetam de 2 a 3 % dos recém-nascidos vivos. Em 2015, 20,8 % das crianças menores de cinco anos afetadas com cardiopatias congênitas faleceram por causa dessas alterações. Este estudo é descritivo e retrospectivo. Foram incluídos dados de 252 prontuários de pacientes com diagnóstico de cardiopatia congênita entre 2010 e 2016, 56 % da população encontrada foi do sexo feminino. Predominaram as cardiopatias congênitas não cianóticas (87 %). A maior persistência foi o dueto arterioso permeável (35,3 %). A ecocardiografìa Doppler foi utilizada em 98,4 % dos casos; quanto ao manejo, 47,2 % dos pacientes estiveram em seguimento, o que foi mais comum do que as alternativas médicas ou cirúrgicas. Em conclusão, o ducto arterioso permeável, a comunicação interventricular e a comunicação interatrial são as cardiopatias não cianóticas mais frequentes na população estudada e são associadas com a hipertensão pulmonar que merecem maior seguimento. A tetralogia de Fallot foi a cardiopatia congènita cianòtica mais comum.

General anesthesia combined with erector spinae plane block for ductus arteriosus closure: two case reports / Anestesia geral associada a bloqueio do plano do músculo eretor da espinha para encerramento de persistência de canal arterial: dois relatos de caso

Abstract Background: Failure of ductus arteriosus closure in preterm neonates results in a left-to-right shunt that leads to variable severities of hemodynamic and respiratory distress. When medical therapy fails, surgical ligation via left lateral thoracotomy remains an alternative approach and can be performed in the operating room or at the bedside with a low mortality rate. Opioid-based anesthesia is a frequent choice among anesthesiologists who manage patent ductus arteriosus cases based on the suppression of the stress response and maintenance of hemodynamic stability. This rationale suggests that regional anesthesia may also be an advantageous technique and may benefit earlier weaning from ventilation. Blocking afferent signals before incision may also modulate the long-term consequences of altered sensory perception and pain responses. Case report: We present two cases of general anesthesia combined with erector spinae plane block as part of multimodal anesthesia in premature twins undergoing patent ductus arteriosus closure. Discussion: In these cases, the use of erector spine plane block combined with general anesthesia was efficient to minimize the negative impact of surgery and allowed a reduction in the amount of intraoperative opioid use for patent ductus arteriosus closure.

Resumo Justificativa: A persistência do canal arterial em neonatos prematuros resulta em shunt esquerdo-direito com alterações hemodinâmicas e desconforto respiratório de gravidade variável. Quando o tratamento clínico não é bem sucedido, o fechamento cirúrgico via toracotomia lateral esquerda continua sendo a abordagem alternativa, e pode ser realizado no centro cirúrgico ou à beira leito com baixa taxa de mortalidade. A anestesia baseada em opioides é frequentemente escolhida pelos anestesiologistas nos casos de fechamento de canal arterial devido à supressão de resposta ao estresse e manutenção da estabilidade hemodinâmica. Essa justificativa sugere que a anestesia regional também pode ser uma técnica vantajosa e que promove desmame mais precoce do ventilador. O bloqueio dos estímulos aferentes antes da incisão também pode modular os efeitos no longo-prazo, tanto da percepção sensorial quanto das respostas à dor. Relato de caso: Apresentamos dois casos de anestesia geral associada a bloqueio do plano do músculo eretor da espinha como parte de anestesia multimodal em gêmeos prematuros submetidos a fechamento de canal arterial persistente. Discussão: Nos dois casos descritos, o bloqueio do plano do músculo eretor da espinha associado à anestesia geral foi eficiente para minimizar o impacto negativo da cirurgia, e possibilitou a redução na quantidade de opioide usado durante cirurgia para fechamento de persistência canal arterial.

Impresión 3D de rara patología congénita de aorta y vasos supraaórticos / 3-Dimensional impression of a rare congenital disease of aortic and supra-aortic vessels

Resumen El divertículo de Kommerell es una anomalía de muy baja incidencia y prevalencia en la edad pediátrica. Se reporta el caso de un paciente de 11 años de edad, con diagnóstico de divertículo de Kommerell con arco aórtico derecho y origen anómalo de arteria subclavia izquierda desde la rama pulmonar izquierda a través de conducto arterioso persistente. Dado que es una anomalía cardiovascular compleja se decidió realizar un modelo impreso en 3 D, el cual proporcionó una mejor comprensión de su distribución espacial, tamaño y forma real, como si fuera una pieza de anatomía patológica. Este modelo ayudó en la toma de decisiones, planificación y seguridad de la ejecución de una posible cirugía cardiaca. Este es el primer reporte de caso de este tipo de anomalía, así como el primer prototipo cardíaco impreso en modo tridimensional realizado en Perú para el tratamiento de la misma.

Abstract Kommerell's diverticulum is an anomaly of very low incidence and prevalence in paediatrics. A case is presented of an 11 year-old patient with a diagnosis of Kommerell's diverticulum with a right aortic arch and a left subclavian artery of anomalous origin from the pulmonary branch through a patent ductus arteriosus. Given that this was a complex cardiovascular anomaly, it was decided to make a print model in 3-D. This provided a better understanding of its spatial distribution, size, and real shape, as it was a histopathology piece. This model helped in taking planning and safety decisions on any possible cardiac surgery. This is the first report of a case of this type of anomaly, as well as the first prototype of a cardiac print in 3-dimensional mode, performed in Peru.

Clinical outcomes after more conservative management of patent ductus arteriosus in preterm infants / Desfechos clínicos após tratamento mais conservador da persistência do canal arterial em prematuros

Abstract Objective: Management of patent ductus arteriosus is still controversial. This study aimed to describe the impact of a more conservative approach on treatment rates and on main outcomes of prematurity, especially in preterm infants with <26 weeks of gestation. Method: Clinical charts review of infants ≤30 weeks with patent ductus arteriosus between 2009 and 2016 at two centers. In 2011, the authors changed patent ductus arteriosus management: in first period (2009-2011), patients who failed medical treatment underwent surgical closure; in second period (2012-2016), only those with cardiopulmonary compromise underwent surgical ligation. Medical treatment, surgical closure, mortality, and survival-without-morbidity were compared. Results: This study included 188 patients (27 ± 2 weeks, 973 ± 272 grams); 63 in P1 and 125 in P2. In P2, significantly lower rates of medical treatment (85.7% P1 versus 56% P2, p < 0.001) and surgical closure (34.5% P1 versus 16.1% P2, p < 0.001) were observed. No differences were found in chronic lung disease (28.8% versus 13.9%, p = 0.056), severe retinopathy of prematurity (7.5% versus 11.8%, p = 0.403), necrotizing enterocolitis (15.5% versus 6.9%, p = 0.071), severe intraventricular hemorrhage (25.4% versus 18.4%, p = 0.264), mortality (17.5% versus 15.2%, p = 0.690) or survival-without-morbidity adjusted OR = 1.10 (95% CI: 0.55-2.22); p = 0.783. In P2, 24.5% patients were discharged with patent ductus arteriosus. The subgroup born between 23 and 26 weeks (n = 82) showed significant differences: lower incidence of chronic lung disease (50% versus 19.6%, p = 0.019) and more survival-without-morbidity (20% versus 45.6%, p = 0.028) were found. Conclusion: A conservative approach in preterm infants with patent ductus arteriosus can avoid medical and surgical treatments, without a significant impact in survival-without-morbidity. However, two-thirds of preterm infants under 26 weeks are still treated.

Resumo Objetivo O tratamento da persistência do canal arterial ainda é controverso. Nosso objetivo foi descrever o impacto de uma abordagem mais conservadora em nossas taxas de tratamento e nos principais desfechos da prematuridade, especialmente em prematuros com < 26 semanas de gestação. Método Revisão de prontuários de lactentes com ≤ 30 semanas e persistência do canal arterial entre 2009-2016 em dois centros. Em 2011, mudamos o manejo da persistência do canal arterial: no primeiro período (2009-2011), os pacientes que não apresentaram sucesso com o tratamento clínico foram submetidos a fechamento cirúrgico; no segundo período (2012-2016), apenas aqueles com comprometimento cardiopulmonar foram submetidos ao fechamento cirúrgico. Comparamos o tratamento clínico, fechamento cirúrgico, mortalidade e sobrevida sem morbidade. Resultados Foram incluídos 188 pacientes (27 ± 2 semanas, 973 ± 272 gramas); 63 em P1 e 125 em P2. Em P2, foram observadas taxas significativamente mais baixas de tratamento clínico (85,7% no P1 versus 56% no P2, p < 0,001) e fechamento cirúrgico (34,5% no P1 versus 16,1% no P2, p < 0,001). Não foram encontradas diferenças em relação à doença pulmonar crônica (28,8% versus 13,9%, p = 0,056), retinopatia grave da prematuridade (7,5% versus 11,8%, p = 0,403), enterocolite necrosante (15,5% versus 6,9%, p = 0,071), hemorragia intraventricular grave (25,4% versus 18,4%, p = 0,264), mortalidade (17,5% versus 15,2%, p = 0,690) ou OR ajustado pela sobrevida sem morbidade = 1,10 (IC95%: 0,55-2,22); p = 0,783. Em P2, 24,5% dos pacientes receberam alta com persistência do canal arterial. O subgrupo nascido entre 23 a 26 semanas (n = 82) apresentou diferenças significativas, foram encontradas menor incidência de doença pulmonar crônica (50% versus 19,6%, p = 0,019) e maior sobrevida sem morbidade (20% versus 45,6%, p = 0,028). Conclusão Uma abordagem conservadora em prematuros com persistência do canal arterial pode evitar tratamentos clínicos e cirúrgicos, sem um impacto significativo na sobrevida sem morbidade. No entanto, dois terços dos prematuros com menos de 26 semanas ainda são tratados.