Hospitalizaciones por cardiopatías congénitas en la Unidad de Cardiología Pediátrica del Centro Hospitalario Pereira Rossell / Hospitalization due to congenital heart disease at the Pereira Rossell Pediatric Hospital's Cardiology Unit / Internações por cardiopatia cardíaca congênita na Unidade de Cardiologia Pediátrica do Centro Hospitalar Pereira Rossell

Introducción: las cardiopatías congénitas (CC) son patologías frecuentes en pediatría. Son causa de importante morbimortalidad en la infancia. Son pacientes complejos, que requieren abordaje integral, en equipo interdisciplinario. Objetivos: describir las características epidemiológicas, clínicas y evolutivas de niños portadores de CC asistidos en la Unidad de Cardiología del HP-CHPR durante el año 2015, así como su estado nutricional y los factores que pudieran influir en este. Material y método: estudio descriptivo, retrospectivo, a través de la revisión de historias clínicas de pacientes portadores de CC, hospitalizados en la Unidad de Cardiología del HP-CHPR en 2015. Se describieron características epidemiológicas y clínicas de los pacientes. Resultados: en el período evaluado egresaron 63 pacientes, que representaron una tasa de 6,58/1.000 egresos hospitalarios. Tenían un promedio de edad de 23,8 meses. Un total de 8 pacientes fueron diagnosticados en el período prenatal, 54 presentaban CC no cianóticas. Habían sido sometidos a tratamiento quirúrgico 24 niños, 13 con cirugía correctiva. Se diagnosticó desnutrición en 43%. Esta fue más prevalente en portadores de CC cianótica, de defectos complejos, y de cromosomopatías u otras malformaciones. Conclusión: los niños con CC representaron un pequeño porcentaje de quienes requirieron hospitalización en el HP-CHPR. El porcentaje de desnutrición fue elevado. Este estudio confirma la importante morbimortalidad de los niños pequeños portadores de CC. Es importante reconocer el mayor riesgo de estos pacientes y actuar en forma individualizada y oportuna.

Introduction: congenital heart disease (CHD) is a frequent pathology among children. It may cause significant morbidity and mortality during childhood. These are complex patients, who require a comprehensive approach and an interdisciplinary team. Objective: to describe the epidemiological, clinical and evolutionary characteristics of children with CHD assisted in the Cardiology Department of the Children Hospital-CHPR in 2015. Material and Methods: descriptive, retrospective study, review of clinical records of patients with CHD, hospitalized in the Cardiology Department of the Children's Hospital PRHC in 2015. Epidemiological and clinical characteristics of patients were described. Results: 63 patients were hospitalized during the evaluated period, a total rate of 6.58/1.000 discharges. They had an average age of 23.8 months. Eight patients were diagnosed in the prenatal period, 54 presented non-cyanotic CHD. Twenty-four children had undergone surgical treatment, 13 had had corrective surgery. Malnutrition was diagnosed in 43% and it was more prevalent in cyanotic CHD carriers, complex defects, and chromosomopathies or other malformations. Conclusion: CHD represented a small percentage of the children who required hospitalization at the PH-PRHC. The percentage of malnutrition was high. The study confirmed the significant morbidity and mortality of these patients. It is important to recognize the highest risk of these patients and act in a customized and timely fashion.

Introdução: as cardiopatias congênitas (CC) são patologias comuns em pediatria. Elas são uma causa significativa de morbidade e mortalidade na infância. Os pacientes são complexos e requerem uma abordagem integral e uma equipe interdisciplinar. Objetivos: descrever as características epidemiológicas, clínicas e evolutivas de crianças com CC atendidas na Unidade de Cardiologia do Hospital Pediátrico PHPR durante o ano de 2015, descrever seu estado nutricional e os fatores que poderiam influenciá-lo. Material e métodos: estudo descritivo, retrospectivo, feito por meio da revisão de prontuários de pacientes com CC, internados na Unidade de Cardiologia do HP-CHPR em 2015. Foram descritas as características epidemiológicas e clínicas dos pacientes. Resultados: no período avaliado, 63 pacientes receberam alta hospitalar, representando uma taxa de 6,5/1.000 altas hospitalares. Eles tinham uma idade média de 23,8 meses. Oito pacientes foram diagnosticados no período pré-natal, 54 apresentavam CC não cianótica. 24 crianças tinham sido submetidas a tratamento cirúrgico, 13 tinham recebido cirurgia corretiva. 43% foram diagnosticadas com desnutrição, a qual foi mais prevalente em portadores de CC cianótica, defeitos complexos e malformações cromossômicas ou outras. Conclusão: os CCs representaram um pequeno percentual de crianças que necessitaram de internação no HP-CHPR. O percentual de desnutrição foi alto. Este estudo confirma a morbidade e mortalidade significativas de crianças pequenas com CC. É importante reconhecer o risco aumentado desses pacientes e agir de forma individualizada e oportuna.

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge / Gravidez em Portadoras de Cardiopatias Congênitas Complexas: Um Constante Desafio

Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

Resumo Fundamento: A contínua habilidade na conduta das cardiopatias congênitas complexas (CCC) tem permitido o alcance da idade fértil. Contudo, a heterogeneidade das lesões cardíacas na idade adulta limita a estimativa do prognóstico da gravidez. Objetivo: Estudar a evolução materno-fetal das gestantes portadoras de CCC e analisar as variáveis presumíveis de prognóstico. Método: No período de 10 anos, 435 gestantes portadoras de cardiopatias congênitas foram consecutivamente incluídas no Registro do Instituto do Coração (Registro-InCor). Dentre elas, foram selecionadas 42 gestações em 40 mulheres com CCC (24,5 ± 3,4 anos) que haviam sido desaconselhadas a engravidar. As cardiopatias de base distribuíram-se em: transposição das grandes artérias, atresia pulmonar, atresia tricúspide, ventrículo único, dupla via de saída de ventrículo direito, dupla via de entrada de ventrículo esquerdo e outras lesões estruturais. As cirurgias realizadas foram Rastelli, Fontan, Jatene, Senning, Mustard e outros procedimentos combinados, como tunelização, Blalock Taussing e Glenn. Oito pacientes (20%) não haviam sido operadas, e 19 (47,5%) apresentavam hipoxemia. O protocolo de atendimento incluiu: registro da saturação de oxigênio, hemoglobina sérica, hematócrito, ajuste das medicações, anticoagulação individualizada e hospitalização a partir de 28 semanas de gestação, em face da gravidade do quadro clínico e obstétrico. Na análise estatística, o nível de significância adotado foi de 0,05. Resultado: Somente 17 gestações (40,5%) não tiveram complicações maternas nem fetais. Houve 13 problemas maternos (30,9%) e 2 mortes (4,7%) causadas por hemorragia pós-parto e pré-eclâmpsia grave, ambas em pacientes que apresentavam hipoxemia. Houve 7 perdas fetais (16,6%), 17 bebês prematuros (40,5%) e 2 recém-nascidos (4,7%) com cardiopatia congênita. As complicações materno-fetais foram significativamente maiores em pacientes que apresentavam hipoxemia (p < 0,05). Conclusão: O alcance da idade reprodutiva em pacientes com CCC é crescente; contudo, a má evolução materno-fetal desaconselha a gravidez, particularmente nas pacientes que apresentam hipoxemia.

Caracterización de las cardiopatías congénitas en la provincia de Matanzas. Estudio de trece años / Characterization of congenital heart diseases in Matanzas province. A thirteen years study

RESUMEN Introducción: el incremento de las cardiopatías congénitas en los últimos años y la relevancia que ha alcanzado en la provincia de Matanzas como una de las primeras causas de muerte infantil, es un tema que preocupa a los especialistas; sin embargo, la gestión en la búsqueda de nuevos conocimientos en esta área de interés aún no es suficiente. Objetivo: caracterizar la morbimortalidad por cardiopatías congénitas en la provincia de Matanzas en el período comprendido de enero del 2002 a diciembre del 2014. Materiales y métodos: se realizó un estudio descriptivo, transversal cuyo universo de trabajo estuvo conformado por todos los pacientes de edad pediátrica a los cuales se les diagnosticó cardiopatía congénita y fueron atendidos por el servicio de Cardiología del Hospital Pediátrico Docente "Eliseo Noel Caamaño". Se incluyeron todos los niños diagnosticados por ultrasonografía durante el período embriofetal. Resultados: la comunicación interventricular resultó ser la más frecuente. Hubo predominio de cardiopatías en pacientes masculinos y procedencia urbana; siendo diagnosticadas la mayoría en la etapa neonatal. La tasa de prevalencia por año presenta una tendencia creciente, mientras que la de mortalidad resultó decreciente. El Síndrome de Down fue la entidad genética más frecuente asociada con las cardiopatías congénitas al igual que las malformaciones craneofaciales. Conclusiones: estos resultados muestran el verdadero comportamiento de esta entidad en esta provincia (AU).

ABSTRACT Introduction: the increase of congenital heart diseases in recent years and the relevance it has achieved in the province of Matanzas as one of the main causes of infantile death is a matter of concern for specialists; however, management of search for new knowledge in this area of interest is still not enough. Objective: to characterize morbidity and mortality due to congenital heart disease in the province of Matanzas in the period from January 2002 until December 2014. Materials and methods: A descriptive, cross-sectional study was carried out in a universe formed by all the patients of pediatric age, diagnosed with congenital heart disease and treated by the Cardiology service of the Teaching Pediatric Hospital "Eliseo Noel Caamaño". All the children diagnosed by ultrasonography during the embryofetal period were included. Results: intraventricular communication was the most frequent. There it was a general predominance of heart disease in male patients, and from urban origins, most of them diagnosed at the neonatal stage. The prevalence rate per year shows an increasing tendency, while mortality decreased. Down syndrome was the most common genetic entity associated to congenital heart disease as well as craniofacial malformations. Conclusions: these results show the true behavior of this entity in our province (AU).

Quadricuspid aortic valve: a comprehensive review

Abstract Quadricuspid aortic valve (QAV) is a rare congenital heart disease. The functional status of QAV is predominantly a pure aortic regurgitation. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated disorders. Significant valvular regurgitation and (or) stenosis is often present with subsequent operation performed at the fifth to sixth decade of life. The functional status of QAV is predominantly regurgitant; whereas pure stenotic QAV can be as few as in only 0.7% of the patients. QAV is usually an isolated anomaly, but other congenital heart defects can be present in 18-32% of the patients. About one-fifth of them require a surgical operation. Tricuspidalization is a preferred technique for QAV repair. As not all the patients with a QAV necessarily warrant a surgical operation, decision-making in patient selection and surgical procedure of choice are crucial. Antibiotic prophylaxis against infective endocarditis is necessary in the QAV patients with unequal-sized cusps.

Aleitamento materno em crianças com cardiopatia congênita: prevalência e fatores associados / Breastfeeding in children with congenital heart disease: prevalence and associated factors

This study evaluated the prevalence of breastfeeding in children with congenital heart disease and its association with characteristics of mothers, children and treatment. This is a cross-sectional study that included 94 children from 0 to 10 years old with congenital heart disease hospitalized in tertiary public hospital specialized in heart disease, between August and December 2011. Data were obtained from the medical records and from a structured questionnaire applied to the mothers or guardians. Data were analyzed in Stata version 11.0 and described using frequency and measures of central tendency and dispersion. The associations were verified by the Pearson chi-square test. The prevalence of children who never received exclusive breastfeeding was 27%. Of those who received it, 44.8% were breastfed until 4 months of age and 26.9% until 6 months old. The prevalence of children who received non exclusive breastfeeding until 6 months of age was 38.1%, and of those who received it until one year of age, it was 21.4%..Exclusive breastfeeding was associated only with the completion of surgery (p=0.002). The total duration of breastfeeding was associated with the age of introduction of other milks (p<0.001), mother age (p=0.011) and maternal marital status (p=0.037). The guidance and support to breastfeeding in children with congenital heart disease becomes important, especially from health professionals and family members, in order to reintroduce it as soon as possible after surgery, avoiding the early introduction of other milks, respecting children?s nutritional and clinical needs.

Este estudio evaluó la prevalencia de la lactancia materna en niños con cardiopatía congé-nita y su asociación con características de las madres, de los niños y del tratamiento de esa enfermedad. Se trata de un estudio transversal que incluyó a 94 niños de 0 a 10 años con cardiopatía congénita, internados en un hospital público terciario especializado en enfermedades del corazón, entre agosto y diciembre de 2011. Los datos se obtuvieron de las historias clínicas y de la aplicación de un cuestionario estructurado a las madres o tutores. Los datos fueron analizados en Stata versión 11.0 y descritos utilizando frecuencias y medidas de tendencia central y de dispersión. Las asociaciones fueron verificadas mediante la prueba de chi-cuadrado. La prevalencia de niños que nunca recibieron lactancia materna exclusiva fue de 27%. De los que sí la recibieron, el 44,8% lo hizo hasta los 4 meses y el 26,9% hasta los 6 meses. La prevalencia de niños que recibieron lactancia materna no exclusiva hasta los 6 meses fue del 38,1% y hasta 1 año del 21,4%. La lactancia materna exclusiva se asoció sólo con la realización de cirugía (p=0,002). La duración total de la lactancia materna se asoció con la edad de introducción de otras leches (p<0,001), la edad materna (p=0,011) y el estado civil materno (p=0,037). Se concluye que la orientación y el apoyo a la lactancia materna en niños con cardiopatías congénitas es importante, sobre todo por parte de los profesionales de la salud y miembros de la familia, con el fin de restablecerla lo antes posible después de la cirugía, evitando así la introducción temprana de otros tipos de leche, respetando las necesidades nutricionales y clínicas del niño.

Este estudo avaliou a prevalência do aleitamento materno em crianças com cardiopatia congênita e sua associação com características maternas, da criança e do tratamento. Trata-se de estudo transversal, que incluiu 94 crianças de zero a dez anos, portadoras de cardiopatia congênita, internadas em hospital público terciário especializado em cardiopatias, entre agosto e dezembro de 2011. Os dados foram obtidos por meio do prontuário médico e de um questionário estruturado aplicado às mães ou aos responsáveis. Os dados foram analisados no software Stata versão 11.0 e descritos por meio de frequência e medidas de tendência central e de dispersão. As associações foram verificadas pelo teste do quiquadrado de Pearson. A prevalência de crianças que nunca receberam aleitamento materno exclusivo foi de 27%. Das que receberam, 44,8% assim permaneceram até quatro meses e 26,9%, até seis meses. A prevalência de crianças que receberam aleitamento materno não exclusivo até os seis meses foi de 38,1% e, até um ano, de 21,4%. O aleitamento materno exclusivo mostrou associação apenas com a realização de tratamento cirúrgico (p=0,002). O tempo total de aleitamento materno mostrou associação com a idade de introdução de outros leites (p<0,001), a idade materna (p=0,011) e o estado civil materno (p=0,037). Torna-se importante a orientação e o apoio ao aleitamento materno (AM) em crianças com cardiopatia congênita, especialmente por parte dos profissionais da saúde e familiares, a fim de reintroduzi-lo o mais rápido possível após a cirurgia, evitando a introdução precoce de outros leites e respeitando as necessidades nutricionais e clínicas da criança.

Tratamento das cardiopatias congênitas em Sergipe: proposta de racionalização dos recursos para melhorar a assistência / Heart defects treatment in Sergipe: propose of resources' rationalization to improve care

OBJETIVO: Avaliar o tratamento das cardiopatias congênitas realizadas de 2000 a 2009. MÉTODOS: A amostra constituiu-se de todos os pacientes submetidos a correção cirúrgica para cardiopatias congênitas por dez anos em Sergipe, Brasil. Os pacientes foram operados em três hospitais localizados na cidade de Aracaju (SE, Brasil), capital do estado de Sergipe. O estudo foi dividido em dois períodos, definidos pela data do início da centralização das cirurgias. As variáveis coletadas foram: faixa etária, gênero, diagnóstico pós-operatório, destino, tipo de cirurgia e hospital em que foi realizado o procedimento e a classificação RACHS -1. RESULTADOS: No período I, a estimativa do déficit de cirurgia foi de 69%, ocorrendo decréscimo no período II para 55,3%. O diagnóstico pós-operatório mais frequente foi de fechamento de comunicação interventricular (20,5%), fechamento de canal arterial (20,2%) e da comunicação interatrial (19%). Houve correlação estatisticamente significativa entre mortalidade esperada pelo RACHS-1 e a observada na amostra. A avaliação do RACHS-1 como fator preditor da mortalidade hospitalar por meio da curva ROC demonstrou área de 0,860 IC 95% 0,818 a 0,902, com P < 0,0001. CONCLUSÃO: Os resultados deste estudo indicam que a centralização e a organização dos recursos existentes são necessárias para melhora no desempenho das correções cirúrgicas das cardiopatias congênitas.

OBJECTIVE: This study aims evaluate the treatment of congenital heart disease conducted from 2000 to 2009. METHODS: The sample consisted of all patients undergoing surgical correction for congenital heart disease for ten years in Sergipe, Brazil. The patients were operated in three hospitals located in the city of Aracaju, capital of the state of Sergipe (Brazil). The study was divided into two periods defined by the start date of centralization of surgery. The variables collected were: age, sex, postoperative diagnosis, destination, type of surgery and hospital where the procedure was performed and the classification RACHS -1. RESULTS: In the period I, the estimate deficit of surgery was 69% decrease occurring in the period II to 55.3%. The postoperative diagnosis was more frequent closure of the interventricular communication (20.5%), closure of patent ductus arteriosus (20.2%) and atrial septal defect (19%). There was a statistically significant correlation between the expected mortality RACHS-1 and observed in the sample. The evaluation of RACHS-1 as a predictor of hospital mortality by ROC curve showed area of 0.860 95% CI 0.818 to 0.902 with P <0.0001. CONCLUSION: The results of this study indicate that the centralization and organization of existing resources are needed to improve the performance of surgical correction of congenital heart diseases.

Epidemiología y registro de las cardiopatías congénitas en Costa Rica / Epidemiology and registry of congenital heart disease in Costa Rica

OBJETIVO: Caracterizar la población de niños que nacen con cardiopatías congénitas (CC) en Costa Rica y evaluar sus procesos de registro. MÉTODOS: Estudio observacional exploratorio que incluyó a todos los niños con CC diagnosticadas en el Hospital Nacional de Niños entre el 1 de mayo de 2006 y el 1 de mayo de 2007. Tomando en cuenta los niños menores de 1 año y su respectiva cohorte de nacimientos, se estimaron prevalencias con intervalos de confianza de 95 por ciento (IC95 por ciento) según sexo, tipo de cardiopatía, edad al diagnóstico, edad materna, residencia habitual y malformaciones extracardiacas asociadas. Se compararon los datos con el Centro de Registro de Enfermedades Congénitas (CREC). RESULTADOS: Durante el período estudiado se diagnosticaron 534 casos con CC. Los casos en menores de 1 año fueron 473 dentro de una cohorte de nacimientos de 77 140 -prevalencia de 0,6 por ciento (IC95 por ciento: 0,5-0,7). Con base en datos del CREC, se demostró que al nacimiento no se detectan 71 por ciento de los casos. La edad promedio al diagnóstico en niños menores de 1 año fue de 46,6 días. No hubo diferencias por sexo. La prevalencia de CC en hijos de madres de 35 años o más fue significativamente mayor, aunque al excluir las cromosomopatías este riesgo perdió su significancia estadística. Las provincias del país con puertos marítimos fueron las de mayor riesgo en hijos de madres adolescentes. Las CC más frecuentes fueron los defectos del tabique interventricular e interauricular, persistencia del conducto arterioso, estenosis valvular pulmonar, defectos del tabique aurículo ventricular, coartación de aorta y tetralogía de Fallot. El 34 por ciento de las CC fueron múltiples, 11,2 por ciento se asociaron a cromosomopatías y 19 por ciento tenían malformaciones congénitas asociadas. CONCLUSIONES: La prevalencia de CC en Costa Rica está dentro del rango informado a nivel mundial. Se halló que en el CREC había un importante subregistro de CC debido principalmente a los criterios de edad aplicados. Los resultados sugieren que la edad materna (menores de 20 años y mayores de 34 años) es un factor asociado a la ocurrencia de CC.

OBJECTIVE: Characterize the population of children born with congenital heart disease (CHD) in Costa Rica and evaluate the country's registry processes. METHODS: Exploratory observational study that included all children with CHD diagnosed at the National Children's Hospital between 1 May 2006 and 1 May 2007. Considering children under 1 year of age and their respective birth cohort, prevalence was estimated by sex, type of heart disease, age at diagnosis, maternal age, habitual residence, and associated extracardiac malformations, with 95 percent confidence intervals (95 percent CI). The data was compared with those of the Congenital Disease Registry Center (CREC). RESULTS: During the period studied, 534 cases with CHD were diagnosed. There were 473 cases in children under 1 year of age in a birth cohort of 77 140 children. Prevalence was 0.6 percent (95 percent CI: 0.5-0.7). Based on CREC data, it was demonstrated that 71 percent of the cases were not detected at birth. The average age of diagnosis in infants under 1 year of age was 46.6 days. There were no differences by sex. Prevalence of CHD in children of mothers aged 35 years or over was significantly higher. However, when chromosomal abnormalities were excluded, the risk was no longer statistically significant. The provinces in the country with maritime ports were the areas with the highest risk in children of adolescent mothers. The most common CHDs were ventricular and atrial septal defects, patent ductus arteriosus, pulmonary valve stenosis, atrioventricular septal defects, coarctation of the aorta, and tetralogy of Fallot. Thirtyfour percent of the cases of CHD were multiple, 11.2 percent were associated with chromosomal abnormalities, and 19 percent had associated congenital malformations. CONCLUSIONS: CHD prevalence in Costa Rica is within the range reported globally. Significant underreporting of CHD was found in the CREC, primarily due to the age criteria applied. The results suggest that maternal age (under 20 and over 34) is a factor associated with CHD.

Cardiopatias congênitas em um serviço de referência: evolução clínica e doenças associadas / Congenital heart diseases in a reference service: clinical evolution and associated illnesses

FUNDAMENTO: Inúmeros fatores vêm contribuindo para a mudança do perfil do paciente com cardiopatia congênita (CC), incluindo o diagnóstico pré-natal e a disponibilidade de novos procedimentos terapêuticos. O conhecimento dessas mudanças é fundamental para um melhor atendimento. OBJETIVOS: Descrever o perfil dos pacientes com CC de um serviço de referência no Estado do Rio Grande do Sul, Brasil. MÉTODOS: Trata-se de um estudo transversal, com 684 pacientes portadores de CC, em um serviço de cardiologia pediátrica, de janeiro de 2007 a maio de 2008. Esses pacientes foram entrevistados (e/ou seus pais) e examinados (malformações congênitas, medidas antropométricas), além de terem seus prontuários revisados para mais detalhes sobre as cardiopatias, procedimentos e ecocardiografia. RESULTADOS: A idade dos pacientes variou de 16 dias a 66 anos, sendo 51,8 por cento do sexo feminino, com 93,7 por cento de brancos. A idade média determinada pelo diagnóstico foi de 15,8 ± 46,8 meses. As CC mais prevalentes foram a comunicação interventricular, a persistência do canal arterial e a Tetralogia de Fallot. Dos pacientes analisados, 59,1 por cento, com idade média de 44,3 ± 71,2 meses, realizaram algum procedimento terapêutico; 30,4 por cento tinham malformações congênitas extracardíacas; e 12 pacientes tinham síndrome genética comprovada. Quanto ao desenvolvimento, 46,6 por cento tiveram atraso ponderoestatural e 13,7 por cento atraso neuropsicomotor. Além disso, 18,4 por cento apresentaram história familiar de cardiopatia congênita. CONCLUSÕES: O atraso neuropsicomotor e o baixo ganho ponderoestatural podem estar associados às CC. Estabelecer um perfil dos pacientes com CC atendidos em uma instituição de referência pode servir como base para o planejamento adequado do atendimento desta população.

BACKGROUND: Several factors, which include prenatal diagnosis and availability of new therapeutic procedures, have contributed to change the profile of patients with congenital heart disease (CHD). Knowing these changes is important to a better health care. OBJECTIVES: Description of profile of patients with CHD in a reference service in the State of Rio Grande do Sul, Brazil. METHODS: It is a cross-sectional study including 684 patients with CHD in a service of pediatric cardiology from January 2007 to May 2008. We interviewed the patients (and/or their parents) and examined these patients (congenital malformations, anthropometric measures). Moreover, their charts were reviewed in order to detail heart diseases, procedures and echocardiography. RESULTS: Patients were from 16 days to 66 years old, 51.8 percent were female, and 93.7 percent were Caucasian. The mean age at diagnosis was 15.8 ± 46.8 months. Ventricular septal defect, patent ductus arteriosus and Tetralogy of Fallot were the most prevalent CHD. 59.1 percent of examined patients, whose average age was 44.3 ± 71.2 months, have been undergoing therapeutic procedures; 30.4 percent had congenital extracardiac malformations; and 12 patients had genetic syndrome. Regarding development, 46.6 percent had low weight and height gain, and 13.7 percent had neuropsychomotor delay. Furthermore, 18.4 percent had family history of congenital heart disease. CONCLUSIONS: Neuropsychomotor delay and low weight and height gain may be related to CHD. Establishing a profile of patients with CHD, who were treated at an institution of reference, may function as a basis in which health care of this population can be planed appropriately. (Arq Bras Cardiol 2010; 94(3):313-318)

Concordancia entre ecocardiografía prenatal y posnatal en pacientes con cardiopatías congénitas: Hospital de Niños Dorctor, Roberto Del Río / Concordance between prenatal and posnatal echocardiography in patients with congenital heart defect

Antecedentes: En Chile las malformaciones congénitas son la primera causa de mortalidad neonatal precoz, siendo las cardiopatías congénitas su principal factor. La incorporación de la ecocardiografía prenatal es de gran ayuda en su pesquisa precoz. Objetivos: Evaluar la ecocardiografía prenatal como prueba diagnóstica y su concordancia con la ecocardiografía posnatal. Métodos: Se analizaron embarazadas derivadas al Hospital de Niños Dr. Roberto del Río entre abril 2004 y abril 2008, por sospecha de cardiopatías congénitas con posterior control neonatal. Resultados: Se evaluaron 188 embarazadas tanto con diagnóstico de cardiopatía congénita como con ecocardiografía normal. La edad gestacional promedio de derivación fue de 32 semanas, siendo la principal causa la sospecha de cardiopatía congénita en el examen obstétrico de rutina. Como prueba diagnóstica se obtuvo una sensibilidad de 100 por ciento y especificidad de 40 por ciento con una moderada concordancia entre ésta y el examen postnatal. Como prueba diagnóstica la ecocardiografía prenatal, se obtuvo una sensibilidad de 100 por ciento y especificidad de 89 por ciento del examen, al ser evaluada como cardiopatías en general, con una muy buena concordancia entre ésta y el examen postnatal. Discusión: Existe una tardía derivación y diagnóstico prenatal de las cardiopatías congénitas a nivel nacional, siendo importante capacitar aún más a los obstetras que realizan este tamizaje. La ecocardiografía prenatal realizada en nuestro centro por cardiólogos es una confiable herramienta diagnóstica con una muy buena concordancia con la ecocardiografía posnatal.

Background: In Chile, congenital malformations are the first cause of early neonatal mortality, the congenital heart defects its the main factor. The incorporation of antenatal echocardiography is very helpful in your research early. Objectives: To evalúate prenatal echocardiography as a diagnosis test and their concordance with postnatal echocardiography. Methods: We analyzed pregnancy who were derived to the Hospital de Niños Dr. Roberto del Rio, between April 2004 and April 2008, where studied because suspicion of having congenital heart defects with subsequent neonatal control. Results: 188 pregnancy were evaluated either with a diagnosis of congenital heart defect or with normal echocardiography. The mean gestational age of derivation was 32 weeks, the main causes the suspicion of congenital heart defect on routine obstetric examination. When being studied as diagnosis test it was obtained a sensitivity of 100 percent and a specificity of 40 percent with a modérate concordance between both echocardiographies. When analyzing with the diagnosis test prenatal echocardiography, it was obtained a sensitivity of 100 percent and a specificity of 89 percent of the review, when evaluated as general heart defect, with a very good concordance between it and the postnatal examination. Discussion: There is a late referral and prenatal diagnosis of congenital heart defect at national level, being important to train obstetricians who perform this screening. Prenatal echocardiography performed by cardiologists at our center is a reliable diagnosis tool with a very good concordance with postnatal echocardiography.

Prevalência de cardiopatias congênitas em portadores da síndrome de Down na cidade de Pelotas (RS) / Prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil

OBJETIVO: Determinar a prevalência de cardiopatias congênitas em portadores da síndrome de Down na cidade de Pelotas (RS), descrevendo os tipos mais frequentes e avaliando os fatores associados. MÉTODOS: Estudo transversal que incluiu crianças portadoras de síndrome de Down nascidas e residentes em Pelotas no período de janeiro de 2000 a dezembro de 2005. Os dados foram obtidos através de entrevistas realizadas nos domicílios dos pacientes, com as mães ou seus familiares legais. Para o estudo dos fatores relacionados à presença de cardiopatia congênita, foram realizadas análises univariada e bivariada. RESULTADOS: Foram entrevistadas 47 mães de pacientes com síndrome de Down, sendo que 22 (46,8 por cento) pacientes apresentavam diagnóstico de cardiopatia congênita. Destes, 28 por cento receberam avaliação cardiológica precoce, antes dos 3 meses de vida. A cardiopatia mais frequente foi a comunicação interatrial (17 por cento); o defeito do septo atrioventricular ocorreu em cinco pacientes. A análise bivariada entre o desfecho presença de cardiopatia congênita e os fatores preditores idade materna, idade paterna, cor dos pais e da criança, presença de outras malformações e sexo da criança mostraram que as associações não foram estatisticamente significativas. CONCLUSÕES: As prevalências de síndrome de Down e de cardiopatia congênita em nossa região apresentam-se semelhantes às encontradas por outros autores, ressaltando-se a importância da suspeita diagnóstica e do encaminhamento precoce por parte dos pediatras para avaliação cardiológica. Destaca-se também o baixo número de pacientes que realizaram cariótipo. Além disso, o número de malformações associadas foi inferior ao encontrado por outros autores.

OBJECTIVE: To determine the prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil, describing the most frequent types and assessing the associated factors. METHODS: Cross-sectional study including children with Down syndrome who were born and lived in Pelotas from January 2000 to December 2005. Data were collected by means of home interviews with mothers or guardians. Univariate and bivariate analyses were carried out to analyze the factors related to congenital heart defect. RESULTS: Forty-seven mothers of patients with Down syndrome were interviewed. Twenty-two (46.8 percent) of the patients had a diagnosis of congenital heart defect. Of them, 28 percent had early cardiac evaluation before 3 months of life. The most frequent heart defect was interatrial communication (17 percent); atrioventricular septal defect affected five patients. Bivariate analysis between the outcome congenital heart defect and the predicting factors maternal age, paternal age, parents' and child's skin color, presence of other malformations and child's sex showed that the associations were not statistically significant. CONCLUSIONS: The prevalence of Down syndrome and congenital heart defects in our region is similar to the rates found by other authors; therefore, we highlight the importance of diagnostic suspicion and early referral by pediatricians to cardiac evaluation. Another relevant aspect is the small number of patients who underwent karyotype testing. In addition, the number of associated malformations was lower than that found by other authors.

Criss-cross heart: diagnóstico fetal / Criss-cross heart: fetal diagnose

Criss-cross é um tipo de cardiopatia congênita rara, com incidência inferior a 0,1 por cento e caracterizada pelo cruzamento do fluxo de entrada dos ventrículos. As associações com comunicação interventricular e estenose pulmonar são as mais comuns. O ecocardiograma fetal com mapeamento de fluxo a cores é o método de escolha para o diagnóstico pré-natal dessa cardiopatia.

Consulta cardiológica infantil Fundacor 2003-2006: Táchira-Venezuela: [revisión] / Pediatric cardiologic outpatient clinic Fundacor 2003-2006: Táchira-Venezuela: [review]

La Fundacion Andina del Corazón(Fundacor) se encarga del diagnóstico, tratamiento, prevención y rehabilitación en pacientes con enfermedades cardiovasculares. Conocer la casuística de la consulta cardiológica infantil de Fundacor entre enero 2003 y diciembre 2006, determinando: distribución por edad, sexo, procedencia, estado nutricional, presión arterial, tipo y motivo de referencia, estudios realizados, diagnóstico principal, diagnóstico asociado, tratamiento recibido y evolución. Estudio retrospectivo, descriptivo de datos registrados de 1292 pacientes en consulta de primera vez, entre 0 días y 16 años, de ambos sexos y con historia clínica completa. De acuerdo al grupo etario, la consulta fue mas frecuente en los pre-escolares (36,3 por ciento) y escolares (27,6 por ciento). Relación varones/hembras de 1,3:1. Procedieron principalmente del municipio San Cristobal (42,1 por ciento). Predominó la normalidad en los indicadores peso/edad, talla/edad, índice de masa corporal y en la presión arterial. Principales motivos de consulta: soplo (38,4 por ciento) y dolor toraxico (12,2 por ciento). Exámenes: 1179 electrocardiogramas, 353 ecocardiogramas. Pacientes referidos por médicos: 64,6 por ciento y sin referencia 35,4 por ciento. Evaluaciones normales: 79,8 por ciento y patológicas: 20,2 por ciento. Se evidenciaron defectos septales, tetralogía de Fallot, coartación aórtica, estenosis valvular principalmente. Pacientes con Síndrome de Down 33, 81, 9 por ciento con defectos cardiacos. Cardiopatías corregidas 18,7 de ellas en la institucion. No ameritaron control 87,7 por ciento. Ocurrieron cuatro muertes. Primera publicación sobre casuística relacionada con atención cardiológica infantil en el estado Táchira. Resultados similares a estadísticas nacionales e internacionales. Son necesarios la referencia oportuna por pediatras, obstetras y médicos generales y mejores recursos para aumentar el número de casos corregidos.

Fundacor takes charge of the diagnosis, treatment, prevention and rehabilitation in patients with cardiovascular diseases. Get knowledge of casuistic about pediatric cardiology’s consultation since january 2003 to december 2006, determining: distribution for age, sex, procedence, nutritional status, blood pressure, type and motive of reference, executed studies, principal diagnosis, diagnosis associated, received treatment and evolution. Retrospective and descriptive study of information registered of 1292 patients in first consultation, between 0 days and 16 years, of both sexes and with clinical complete history. According to age group: pre-school (36.3%) and school-age children (27.6%). Relation boys/girls 1.3/1. They came principally from the San Cristobal’s county (42.1%). Predominance of normal weight/age, height/age, body mass index and blood pressure. Principal motive of consultation: heart murmur (38.4%) and chest pain (12.2%). Examinations: 1179 electrocardiogram, 353 ecocardiogram. The 64.6% of the patients were refered by physicians; 35.4%t came without reference. There were 79.8% of normal evaluations and 20.2% of pathological ones. Main congenital heart defects: septal defects, tetralogia of Fallot, aortic coarctation, valvular stenosis. In 33 patients with Down’s syndrome, 81.9% cardiopathy. Cardiopathies were corrected in 18 cases, 7 of them in the institution. Patients who no need control: 87.7%. Four deaths happened. This is the first publication on casuistic related to cardiological infantile attention in Táchira state of Venezuela. Results were similar to national and international reports. the opportune reference for pediatricians, obstetricians and physician and to increase the number of corrected cases by provided better resources are necessary.

Total anomalous pulmonary veins connection: the perioperative risk factors

Operative reconstruction of total anomalous pulmonary veins connection [TAPVC] in the literature has high morbidity and mortality. The aim of the study was to investigate the perioperative risk factors to lower the mortality rate. We retrospectively studied the cases of 42 infants operated for Total Anomalous Pulmonary Veins Connection between August 2002 to August 2007 at Damascus University Cardiovascular Surgical Center. We evaluate the role of right ventricle pressure, Left Ventricle End Diastolic Volume [LVEDN], the anatomical type of anomalous, age and weight of patients, pulmonry infections and delay in doing surgery as perioperative risk factors. Our study demonstrates that neither Left Ventricle End Diastolic Volume nor RV pressure were risk factors. Pulmonary infections secondary to long-standing pulmonary congestion was the only important risk factor. It is highly recommended to operate in the first months of life before the occurring of any pulmonary infection

Fatores associados ao insucesso no desmame ventilatório de crianças submetidas a cirurgia cardíaca pediátrica / Factors associated with failure in ventilatory weaning of children undergone pediatric cardiac surgery

OBJETIVO: Verificar os fatores associados ao insucesso no desmame de crianças submetidas a correção cirúrgica de cardiopatias congênitas. MÉTODOS: Estudo de coorte. A amostra foi composta de 29 crianças. As cardiopatias foram divididas em cianóticas (nove crianças) e acianóticas (20 crianças). Foram estudadas crianças de 0 a 5 anos, submetidas a cirurgia cardíaca com permanência em ventilação mecânica invasiva. RESULTADOS: O grupo insucesso apresentou valor menor na SpO2 e valores maiores nos dias de permanência em VMI e na FiO2 quando comparados ao grupo sucesso (P<0,05). Observou-se que quatro (14 por cento) crianças tiveram insucesso no desmame, sendo necessário retornar a prótese ventilatória antes de 48 horas após a extubação. CONCLUSÃO: Os dias de VMI, a SpO2 reduzida e a FiO2 elevada foram os principais fatores associados à falha na extubação neste grupo de pacientes.

OBJECTIVE: To assess factors associated with failure in ventilatory weaning of children undergone surgical correction of congenital heart diseases. METHODS: Cohort study with 29 children. The heart diseases were divided into cyanotic (nine children) and acyanotic (20 children). We studied children from 0 to 5 years who underwent heart surgery with invasive mechanical ventilation. RESULTS: The failure group presented smaller value in SpO2 and higher values during days of stay in IMV and FiO2 when compared to the success group (P<0.05). It was observed that 4 children (14 percent) had failed in weaning, being necessary to return the ventilatory prosthesis before 48 hours after extubation. CONCLUSION: The days of IMV, SpO2 and FiO2 were the main factors associated with failed extubation in this group of patients.

Cateterismo terapéutico en cardiopatías congénitas del adulto / The role therapeutic catheterization for the management of adults with congenital heart defects

The aim of this report was to analyze the impact of the interventional catheterism for the management of congenital heart disease in adults, considering the most frequent procedures, their results and the future possibility of new indications

Consensus on timing of intervention for common congenital heart disease.

JUSTIFICATION: Separate guidelines are needed for determining the optimal timing of intervention in children with congenital heart diseases in India, because of their frequent late presentation, undernutrition and co-existing morbidities. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 26th August 2007 at the All India Institute of Medical Sciences, New Delhi, India, supported by Cardiological Society of India. OBJECTIVES: To frame evidence based guidelines for (i) appropriate timing of intervention in congenital heart diseases; (ii) assessment of operability in left to right shunt lesions; and (iii) prophylaxis of infective endocarditis in these children. RECOMMENDATIONS: Evidence based recommendations are provided for timing of intervention in common congenital heart diseases including left to right shunt lesions (atrial septal defect, ventricular septal defect, patent ductus arteriosus and others); obstructive lesions (coarctation of aorta, aortic stenosis, pulmonary stenosis); and cyanotic defects (tetralogy of Fallot, transposition of great arteries, total anomalous pulmonary venous connection, truncus arteriosus). Guidelines are also given for assessment of operability in left to right shunt lesions and for infective endocarditis prophylaxis.

Incidence of congenital heart disease in tertiary care hospital.

AIMS AND OBJECTIVES: The present study was under taken to determine the spectrum, clinical profile and outcome of patients with congenital heart disease (CHD) admitted to a tertiary care hospital. MATERIALS AND METHODS: This is a retrospective, observational hospital based study conducted during January 2006 to December 2006. Out of 14461 admissions, 84 were cases of CHD. Only patients with echocardiographic proof of CHD were included in the study. Detailed clinical and laboratory findings and outcome of all cases were noted in pre-structured formats. Data were entered in MS-excel. Data was analyzed by software SPSS version 10. RESULTS: The incidence of CHD was 5.8 per 1000 hospitalized patients. Out of 84 CHD cases, 51 were males and 33 females; with a male to female ratio of 1.5:1. CHD presented more frequently during infancy (46 %). Acyanotic heart disease was detected in 58 (69 %) cases while cyanotic heart disease was detected in 26 (31%) cases. Among acyanotic heart disease, ventricular septal defect (VSD) was found in 49 (58.3%), atrial septal defect (ASD) in 4 patients (4.8%), endocardial cushion defect (ECD) in 2 patients (2.4%) and dextrocardia was found in 3 patients (3.6%). Among cyanotic heart disease, Tetralogy of Fallot (TOF) accounted for 13.1%, total anomalous pulmonary venous connection (TAPVC) 3.6%, transposition of great arteries (TGA) with VSD 1.2% and unspecified cases of heart disease was found in 13.1%. VSD and TOF were the most common lesions while other CHD like ASD, dextrocardia, TAPVC, ECD, TGA with VSD were encountered less frequently. The most common clinical presentations were failure to thrive (FTT) and developmental delay (86.9%), breathlessness (69%), lower respiratory tract infection (LRTI) (52%), congestive cardiac failure (CCF) (46%), cyanosis (20.2%), cyanotic spell (9.5%) and infective endocarditis (9.5%). The mortality rate was 20 %. CONCLUSION: The incidence of CHD was 5.8 per thousand hospitalized children. VSD, TOF were the most common congenital cardiac lesion. VSD was observed either isolated or associated with other lesions like TGA. The mortality rate was 20 %. The mortality usually occurred in those patients complicated with congestive cardiac failure, lower respiratory tract infection and infective endocarditis.