Drenaje venoso anómalo. Vena cava superior izquierda persistente que drena en la aurícula derecha y vena cava superior derecha que drena en la aurícula izquierda: reporte de un caso / Anomalous Venous Drainage. Persistent Left Superior Vena Cava Draining into the Right Atrium and Right Superior Vena Cava Draining into the Left Atrium: Case Report

Introducción: Entre 0,3-0,5% de niños nacidos presentan una vena cava superior izquierda persistente, lo que lo hace una de las malformaciones congénitas más frecuentes del drenaje venoso. El drenaje de esta en la aurícula derecha, además del drenaje de la vena cava superior derecha en la aurícula izquierda, es extremadamente infrecuente. Caso Clínico: Se presenta el caso de un infante de 8 meses asintomático que es llevado a consulta por presentar desaturaciones. El examen físico es normal. El ecocardiograma de contraste con solución salina muestra una vena cava superior izquierda persistente que drena en la aurícula derecha y un retorno anómalo de la vena cava superior derecha. Se realiza una corrección quirúrgica y evoluciona de forma favorable. Conclusión: La presentación en simultáneo de una vena cava superior izquierda persistente que drena en la aurícula derecha y una vena cava derecha que drena en la aurícula izquierda es extremadamente rara. La mayoría de los casos registrados se diagnosticaron de manera incidental en personas adultas al no presentar síntomas.

Introduction: Between 0.3-0.5% of children born have a persistent left superior vena cava, which makes it one of the most frequent congenital malformations of venous drainage. Drainage of this vein into the right atrium, in addition to drainage of the right superior vena cava into the left atrium, is extremely rare. Clinical case: We present a case of an asymptomatic 8-month-old infant who was taken to the clinic for desaturations. Physical examination was normal. The contrast echocardiogram with saline solution showed a persistent left superior vena cava draining into the right atrium and an anomalous return of the right superior vena cava. Surgical correction was performed and the patient evolved favorably. Conclusion: The simultaneous presentation of a persistent left superior vena cava draining into the right atrium and a right superior vena cava draining into the left atrium is extremely rare. Most of the reported cases were diagnosed incidentally in adults in the absence of symptoms.

Transición y transferencia del adolescente con cardiopatía congénita / Transition and transfer of the adolescent with congenital heart disease

En las últimas décadas los avances médicos han permitido la sobrevida de los niños con cardiopatías congénitas hacia la adolescencia y adultez. Un número sustancial de pacientes tienen lesiones persistentes o residuales que requerirán asistencia durante toda la vida. El manejo exitoso durante el proceso de transición en pacientes con cardiopatía congénita (CC) requiere un enfoque integral y colaborativo. Es fundamental desarrollar un programa de transición planificado que incorpore educación y autocontrol. La educación continua y la participación activa de pacientes y familias son esenciales. La formación de especialistas y la creación de unidades de atención de Adolescentes y Adultos con cardiopatía congénita (AACC) garantizarán una mayor supervivencia y calidad de vida en esta creciente población de pacientes en Argentina. La investigación continua y la implementación de mejores prácticas, con el apoyo de políticas sanitarias, son clave para abordar los desafíos y controversias en la gestión de la transición y transferencia (AU)

In recent decades, medical advances have allowed children with congenital heart disease (CHD) to survive into adolescence and adulthood. A substantial number of these patients have persistent or residual lesions that require lifelong care. Successful management during the transition process for patients with CHD requires a comprehensive and collaborative approach. It is critical to develop a planned transition program that incorporates education and self-management. Continuing education and the active participation of patients and families are essential. The training of specialists and the creation of Adolescent and Adult Congenital Heart Disease (AACHD) care units will ensure greater survival and quality of life for this growing patient population in Argentina. Continued research and implementation of best practices, supported by health policies, are key to addressing the challenges and controversies in transition and transfer management (AU)

Principales características clínicas y epidemiológicas de pacientes con miocardiopatía periparto

Introducción: Las enfermedades cardiovasculares representan una causa importante de morbilidad y mortalidad durante la gestación, entre las que se destaca la miocardiopatía, que cursa como un síndrome de insuficiencia cardíaca. Objetivo: Caracterizar a pacientes con miocardiopatía periparto según variables clínicas y epidemiológicas de interés para la investigación. Métodos: Se realizó un estudio descriptivo y transversal de las 18 pacientes con diagnóstico de miocardiopatía periparto, asistidas en el Hospital General Docente Dr. Juan Bruno Zayas Alfonso de Santiago de Cuba en el período comprendido desde octubre de 2015 hasta diciembre de 2022. Resultados: En la serie predominaron las pacientes mayores de 35 años de edad (edad promedio de 32,6 años), además de la descendencia africana (50,0 %), la hipertensión arterial crónica (44,4 %) y la multiparidad (8,9 %) como factores de riesgo y la insuficiencia del ventrículo izquierdo como manifestación clínica. La fracción de eyección de dicho ventrículo estuvo regularmente disminuida y la respuesta al tratamiento farmacológico fue satisfactoria en el total de la muestra. Conclusiones: La miocardiopatía en el periparto es de baja incidencia en este centro; sin embargo, por la gravedad que representa, se impone el diagnóstico temprano y la intervención del personal especializado para evitar complicaciones.

Introduction: Cardiovascular diseases represent an important cause of morbidity and mortality during pregnancy, cardiomyopathy is notable as a syndrome of heart failure. Objective: To characterize patients with peripartum cardiomyopathy according to clinical and epidemiological variables of interest for the investigation. Methods: A descriptive and cross-sectional study of 18 patients with diagnosis of peripartum cardiomyopathy was carried out. They were assisted at Dr. Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba from October, 2015 to December, 2022. Results: In the series there was a prevalence of patients over 35 years (32.6 average age), besides African descendant (50.0 %), chronic hypertension (44.4 %) and multiparity (8.9 %) as risk factors and the left ventricle failure as clinical manifestation. The ejection fraction of this ventricle was regularly diminished and the pharmacological treatment response was satisfactory in all the sample. Conclusions: Peripartum cardiomyopathy is of low incidence in this center; however, due to its seriousness, the early diagnosis and the specialized staff intervention are necessary to avoid complications.

Odontopediatría en el paciente con enfermedad sistémica / Pediatric dentistry in patients with systematic disease

Esta revisión ofrece un enfoque sistemático para establecer una prestación de atención dental segura, integral, coordinada y orientada a la familia del niño con complejidades médicas. Sugerimos que adoptar un enfoque individualizado basado en la fortaleza para la evaluación de niños con afecciones médicas complejas ofrece la base más segura para la prestación de atención en pacientes con enfermedad cardiaca y asma. El objetivo de esta revisión es brindar una visión razonada de atención en el paciente comprometido sistémicamente, basados en protocolos internacionales, y una serie de pasos y modificaciones que deben ser consideradas al momento del manejo odontológico. Se realizó la búsqueda científica en bases digitales contemplando información en idiomas inglés y español, acerca del manejo del paciente dependiendo de su diagnóstico médico y sus complicaciones. Concluimos que el tratamiento dental de pacientes pediátricos con enfermedades sistémicas se puede llevar a cabo de manera eficiente en presencia de un equipo dental bien equipado y apoyo de los padres. Un dentista pediátrico juega un papel importante en el alivio de la ansiedad del niño, manteniendo una relación positiva y haciendo que los servicios de atención se conviertan en experiencias cómodas y libres de complicaciones para pacientes infantiles con enfermedades sistémicas de base, como cardiopatías y/o asma (AU)

This review provides a systematic approach to establishing safe, comprehensive, coordinated, and family-oriented dental care delivery for the child with medical complexities. We suggest that adopting an individualized, strength-based approach to evaluating children with complex medical conditions provides the surest basis for delivering care to heart disease and asthma patients. This review aims to provide a reasoned care approach for the patient with a systemic compromise based on international protocols and a series of steps and modifications that should be considered during dental management. A scientific search was conducted in digital databases, including information in English and Spanish, on managing patients according to their medical diagnosis and complications. We conclude that dental treatment of pediatric patients with systemic diseases can be carried out efficiently in the presence of a well-equipped dental team and parental support. A pediatric dentist plays a vital role in relieving the child's anxiety, maintaining a positive relationship, and making care services become comfortable and complication-free experiences for pediatric patients with underlying systemic diseases, such as heart disease and/or asthma (AU)

Epidemiología y resultados del paro cardiorrespiratorio pediátrico en la unidad de recuperación cardiovascular de un hospital público en Argentina / Epidemiology and outcomes of pediatric cardiac arrest in a cardiovascular unit at a public hospital in Argentina

Resumen Antecedentes: Los niños con cardiopatías congénitas experimentan paro cardiorrespiratorio (PCR) con mayor frecuencia que la población pediátrica general. Se desconoce la epidemiología exacta del PCR en nuestro medio, al igual que el riesgo de mortalidad y los factores que influyen en la evolución neurológica. Objetivo: Describir la epidemiología y los resultados asociados con la reanimación cardiopulmonar pediátrica en una unidad de recuperación cardiovascular. El criterio de valoración primario fue la supervivencia al momento del alta hospitalaria; los secundarios fueron el retorno de la circulación espontánea, la supervivencia a las 24 horas y la condición neurológica en el largo plazo. Método: Estudio de cohorte longitudinal, descriptivo, prospectivo, en menores de 18 años que requirieron reanimación cardiopulmonar entre 2016 y 2019. Se analizaron las variables demográficas y las características del paro cardiorrespiratorio y de la reanimación, así como su resultado. Se realizaron análisis de una y múltiples variables para comparar a los pacientes sobrevivientes con los fallecidos. Resultados: De los 1,842 pacientes internados, el 4.1% experimentó PCR. Se analizaron 50 pacientes con expedientes completos. Se logró el retorno de la circulación espontánea en el 78% (39), con una supervivencia alta del 46%. La reanimación > 6 min y el uso de fármacos vasoactivos fueron factores predictivos de mortalidad; se realizó el seguimiento de 16/23 pacientes, 10 de ellos con desarrollo normal para la edad luego de seis meses, seis tenían trastorno generalizado del desarrollo. Conclusiones: El 4.1% de los pacientes presentó un PCR, con una tasa de 3.4 PCR por 1,000 días-paciente. La supervivencia al egreso hospitalario (n = 50) fue del 46%. La reanimación > 6 min y la utilización de fármacos vasoactivos fueron factores predictivos independientes de mortalidad. Luego de seis meses, el 63% tenía desarrollo neurológico normal para la edad.

Abstract Background: Children with congenital heart disease present a higher frequency of cardiorespiratory arrest (CRA) than the general pediatric population. The epidemiology of CRA is not exactly known in our setting, nor are the mortality risk or the neurological evolution factors. Objective: To describe the epidemiology and outcomes associated with pediatric cardiopulmonary resuscitation in a cardiovascular recovery unit. The primary endpoint was the survival to discharge and the secondary endpoints were the return to spontaneous circulation, the survival at 24 hours and the remote neurological condition. Methods: Descriptive, prospective, longitudinal cohort study in children under 18 years of age who required cardiopulmonary resuscitation between 2016 and 2019. Demographic variables, characteristics of cardiopulmonary arrest, resuscitation and outcome were analyzed. An uni- and multivariate analysis was performed comparing survivors and deceased. Results: Out of 1,842 hospitalized patients, 4.1% presented CRA. Fifty patients with complete records were analyzed. Seventy-eight percent (39) returned to spontaneous circulation with a high survival rate of 46%. Resuscitation > 6 min and the use of vasoactive drugs were predictors of mortality; 16/23 patients were followed up, 10 of them with normal development for age at 6 months, six had pervasive developmental disorder. Conclusions: 4.1% of patients presented CRA, with a rate of 3.4 CRA per 1,000 patient-days. Survival at hospital discharge (n = 50) was 46%. Resuscitation > 6 min and the use of vasoactive drugs were independent predictors of mortality. At six months, 63% had normal neurological development for age.

Síndrome de Eisenmenger secundario a comunicación interatrial tipo ostium secundum en mujer adulta / Eisenmenger Syndrome Associated with an ostium secundum Atrial Septal Defect in an Adult Woman

El síndrome de Eisenmenger es la forma más severa de presentación de hipertensión arterial pulmonar secundaria a defectos cardíacos congénitos no reparados, aunque su prevalencia es baja, continúa siendo un reto para los sistemas de salud de los países en vías de desarrollo por su complejidad en el manejo. Presentación del caso. Paciente femenina sin antecedentes médicos conocidos quien consulta por disnea relacionada a los esfuerzos y policitemia. Intervención terapéutica. Se realiza ecocardiograma transesofágico que arroja la presencia de defecto interatrial tipo ostium secundum e hipertensión arterial pulmonar severa, con cortocircuito de derecha a izquierda, se inicia oxigenoterapia y terapia farmacológica. Evolución clínica. Paciente permaneció ingresada presentando notable mejora a la disnea, se le dio de alta con referencia a la clínica de cardiopatías congénitas del adulto en Hospital Nacional Rosales.

Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension secondary to an unrepaired congenital heart disease. Despite the low prevalence, it remains a challenge for the public health service of developing countries due to the complexity of the treatment. Case presentation. A female patient without known medical history, who consults with dyspnea on exertion and polycythemia. Treatment. A transesophageal echocardiogram was performed, showing an ostium secundum atrial septal defect and severe pulmonary arterial hypertension with a right-left shunt. Supplemental oxygen was administrated and pharmacological treatment was started. Outcome. The patient presented remarkable clinical improvement to dyspnea, she was discharged with medical reference to the Adult Congenital Heart Disease clinic at Rosales National Hospital.

Profile of cardiovascular diseases and physiotherapeutic intervention in a hospital emergency service / Perfil das doenças cardiovasculares e atuação fisioterapeutica em um serviço de emergência hospitalar

Abstract Introduction Cardiovascular disease (CVD) is the lead-ing cause of death globally, with a high proportion of hospitalizations and costs. In view of this, it is essential to understand the main CVDs in patients admitted to hospital emergency services and the role of physiotherapists, in order to plan and direct health services, and to denote participation and encourage specific physiotherapy training in the context of tertiary care. Objective To outline the profile of cardiovascular emergencies and to evaluate physiotherapy in adult patients in the emergency department of a hospital in the interior of the state of São Paulo. Methods This was an observational study which analyzed 1,256 on-call records over a period of eight months. The data collected included age, gender, cardiovascular diagnostic hypothesis and physiotherapy treatment carried out. Results A total of 75 patients with cardiovascular emergencies were included, the most prevalent of which were: heart failure (n = 21), acute coronary syndrome (n = 14), acute myocardial infarction (n = 13), bradyarrhythmia (n = 6) and hypertensive crisis (n = 5). Regarding physiotherapeutic actions and their applications, the most frequent were invasive mechanical ventilation management (n = 34), lung re-expansion maneuvers (n = 17), orotracheal intubation assistance (n = 17), non-invasive mechanical ventilation (n = 14), bronchial hygiene maneuvers (n = 12), kinesiotherapy (n = 10) and sedation (n = 10). Conclusion Heart failure and acute coronary syndrome were the cardiovascular diseases that caused the most admissions to the hospital emergency department and that the procedures with an emphasis on the respiratory system were the most applied.

Resumo Introdução As doenças cardiovasculares (DCV) repre-sentam a principal causa de morte global, destacando-se em internações e gastos. Diante disso, é essencial compreender as principais DCV em pacientes admitidos em serviços de emergência hospitalar e a atuação do fisioterapeuta para planejamento e direcionamento dos serviços de saúde e para denotar a participação e incentivar formações fisioterapêuticas específicas no contexto da atenção terciária. Objetivo Traçar o perfil de emergências cardiovasculares e avaliar a atuação fisioterapêutica em pacientes adultos de serviço de emergência de um hospital no interior do estado de São Paulo. Métodos Trata-se de um estudo observacional, em que foram analisadas 1.256 fichas de passagem de plantão, no período de oito meses. Os dados coletados foram idade, sexo, hipótese diagnóstica cardiovascular e tratamento fisioterapêutico realizado. Resultados Foram incluídos 75 pacientes que apresentavam o perfil de emergências cardiovasculares, sendo as mais prevalentes: insuficiência cardíaca (n = 21), síndrome corona-riana aguda (n = 14), infarto agudo do miocárdio (n = 13), bradarritmia (n = 6) e crise hipertensiva (n = 5). Em relação à atuação fisioterapêutica e suas aplicações, as mais frequentes foram manejo da ventilação mecânica invasiva (n = 34), manobras de reexpansão pulmonar (n = 17), auxílio a intubação orotraqueal (n = 17), ventila-ção mecânica não invasiva (n = 14), manobras de higiene brônquica (n = 12), cinesioterapia (n = 10) e sedestação (n = 10). Conclusão A insuficiência cardíaca e a síndrome coronária aguda foram as doenças cardiovasculares que mais ocasionaram internação no serviço de emergência hospitalar e as condutas com ênfase no aparelho respiratório foram as mais aplicadas.

Patients' prognosis with congenital heart disease followed by ten years: survival and associated factors / Prognóstico de pacientes com cardiopatia congênita seguidos por dez anos: sobrevida e fatores associados

ABSTRACT Objective: To evaluate the prognosis and influence of associated factors in patients with congenital heart disease admitted for the first time to the Intensive Care Unit of the Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, especially those factors associated with death. Methods: Patients were prospectively and consecutively allocated over a period of one year (August 2005 to July 2006). Now, 15 years after the initial selection, we collected data from these patients in the database of the Cytogenetics Laboratory of the Universidade Federal de Ciências da Saúde de Porto Alegre and in the medical records of the hospital. Results: Of the 96 patients, 11 died and 85 were alive until 20 years old. Four patients died in the Intensive Care Unit. The survival probability up to 365 days of life was 95.8%. The survival assessment identified that the deaths occurred mainly before the patients completed one thousand days of life. We found that complex heart disease was independently associated with an odds ratio of 5.19 (95% confidence interval — CI:1.09-24.71; p=0.038) for death. Conclusions: Knowledge about the factors that interfere with the prognosis can be crucial in care practice planning, especially considering that congenital heart disease is an important cause of mortality in the first year of life.

RESUMO Objetivo: Avaliar o prognóstico e a influência de fatores associados em pacientes com cardiopatia congênita internados pela primeira vez na Unidade de Terapia Intensiva do Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, principalmente aqueles fatores associados ao óbito. Métodos: Os pacientes foram alocados prospectiva e consecutivamente por um período de um ano (agosto de 2005 a julho de 2006). Agora, 15 anos após a seleção inicial, coletamos dados desses pacientes no banco de dados do Laboratório de Citogenética da Universidade Federal de Ciências da Saúde de Porto Alegre e nos prontuários do hospital. Resultados: Dos 96 pacientes, 11 faleceram e 85 permaneceram vivos até completar 20 anos. Quatro pacientes morreram na Unidade de Terapia Intensiva. A probabilidade de sobrevida até 365 dias de vida foi de 95,8%. A avaliação da sobrevida identificou que os óbitos ocorreram principalmente antes de os pacientes completarem mil dias de vida. Verificamos que a doença cardíaca complexa foi independentemente associada a um odds ratio de 5,19 (intervalo de confiança — IC95% 1,09-24,71; p=0,038) para morte. Conclusões: O conhecimento dos fatores que interferem no prognóstico pode ser fundamental no planejamento da prática assistencial, principalmente considerando-se que as cardiopatias congênitas são importante causa de mortalidade no primeiro ano de vida.

Patau and Edwards Syndromes in a University Hospital: beyond palliative care / Síndrome de Edwards e Patau em um hospital universitário: além do paliativo

ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

RESUMO Objetivo: Descrever a população de recém-nascidos com síndrome de Patau (T13) e Edwards (T18) portadores de cardiopatias congênitas, que permaneceram em Unidades de Terapia Intensiva (UTI) de um complexo hospitalar quaternário, com relação a conduta cirúrgica ou não, cuidados paliativos e seus desfechos. Métodos: Série de casos de pacientes internados entre janeiro de 2014 a dezembro de 2018, com análise dos prontuários de portadores de T13 ou T18 que permaneceram internados em UTI que recebem neonatos nesse hospital quaternário. Utilizou-se análise estatística descritiva. Resultados: Foram identificados 33 prontuários para análise — 27 T18 (81,8%) e seis T13 (18,2%); 64% do sexo feminino e 36% do sexo masculino. Oito foram prematuros, nascidos com 30 a 36 semanas (24,2%), e apenas quatro nasceram com mais de 2500 g (12,1%). Quatro pacientes foram submetidos a cirurgia cardíaca e um deles foi a óbito. A mortalidade intra-hospitalar foi de 83% para T13 e 59% para T18. A maioria apresentava outras malformações e foi submetida a outras cirurgias. Cuidados paliativos foram oferecidos a 54% dos pacientes. A mediana do tempo de hospitalização para T18 e T13 foi respectivamente de 29 dias (variação: 2-304) e 25 dias (13-58). Conclusões: Pacientes com T13 e T18 cursam com alta morbimortalidade e longa permanência hospitalar em UTI. São necessários estudos multicêntricos para melhor análise de aspectos importantes para a criação de protocolos que, buscando proporcionalidade terapêutica, tragam melhor qualidade de vida para os pacientes e suas famílias.