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

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

Coronary Artery Fistula: Association between Pathway Patterns, Clinical Features and Congenital Heart Disease. / Fístula da Artéria Coronária: Associação entre Padrões de Trajetos, Características Clínicas e Cardiopatias Congênitas.

BACKGROUND: Coronary artery fistula (CAF) is a direct connection of one or more coronary arteries to cardiac chambers or a large vessel; it may be associated with congenital heart disease. OBJECTIVE: To establish CAF pathway patterns from echocardiographic data and to correlate them with clinical aspects and congenital heart disease. METHODS: A total of 7.183 medical records of children under the age of five years with cardiac disease submitted to color Doppler echocardiography and Spearman's Correlation test were used to associate signs and symptoms and cardiopathy to CAF with a significance level of 5%. RESULTS: Twenty six children (0.0036%) presented CAF: from the right coronary artery (RCA) to the right ventricle (RV) 26.92%, from the left coronary artery (LCA) to the RV 23.08%, from the anterior interventricular branch (AIVB) to RV 23.08%, RCA to right atrium (RA) 11.54%, LCA for pulmonary trunk (PT) 7.69% or AIVB for PT 7.69%. In 57.69% of the patients, there was a positively correlated symptomatology to CAF with p=0.445 related to dyspnea or cyanosis (53.84%); in 96.15%, congenital heart disease associated with CAF, mainly interventricular communication (IVC) or interatrial communication (IAC) in 34.62% positively correlated to CAF with p=0.295. CAF pathway was represented in three dimensions by software modeling, texturing and animation Cinema 4D R19. CONCLUSION: CAF is an uncommon anatomical entity that presents a clinical picture compatible with dyspnea and cyanosis, and this is associated with congenital heart disease, mainly with IVC or IAC. According to echocardiographic analyzes, fistulas in RCA, LCA, or AIVB represent about one-third of the patients, with a priority pathway for right heart chambers.

FUNDAMENTO: A fístula da artéria coronária (FAC) é uma conexão direta entre uma ou mais artérias coronárias e câmaras cardíacas ou um grande vaso; pode estar associada à cardiopatia congênita. OBJETIVO: Estabelecer os padrões de trajetos de FAC a partir de dados ecocardiográficos e correlacioná-los com aspectos clínicos e cardiopatias congênitas. MÉTODOS: Um total de 7.183 prontuários médicos de crianças menores de 5 anos de idade com cardiopatia submetidas a ecodopplercardiograma colorido foram analisados utilizando o teste de correlação de Spearman para associar sinais, sintomas e cardiopatia à FAC, com nível de significância de 5%. RESULTADOS: Vinte e seis crianças (0,0036%) apresentaram FAC, nos seguintes trajetos: da artéria coronária direita para o ventrículo direito (26,92%), da artéria coronária esquerda para o ventrículo direito (23,08%), do ramo interventricular anterior para o ventrículo direito (23,08%), da artéria coronária direita para o átrio direito (11,54%), da artéria coronária esquerda para o tronco pulmonar (7,69%) e do ramo interventricular anterior para o tronco pulmonar (7,69%). Em 57,69% dos pacientes, houve uma correlação positiva entre sintomas e a FAC (p = 0,445), relacionada à dispneia ou cianose (53,84%). Em 96,15%, a cardiopatia congênita estava associada à FAC; principalmente, a comunicação interventricular e a comunicação interatrial, em 34,62% dos casos, correlacionaram-se positivamente com a FAC (p = 0,295). O trajeto da FAC foi representado em três dimensões pelo software de modelagem, texturização e animação Cinema 4D R19. CONCLUSÃO: A FAC é uma entidade anatômica incomum que apresenta quadro clínico compatível com dispneia e cianose e está associada a cardiopatias congênitas, principalmente com a CIV ou a CIA. De acordo com as análises ecocardiográficas, as fístulas na ACD, na ACE ou no RIVA representam aproximadamente um terço dos pacientes, com trajeto prioritário para as câmaras cardíacas direitas.

Fístula da Artéria Coronária: Associação entre Padrões de Trajetos, Características Clínicas e Cardiopatias Congênitas / Coronary Artery Fistula: Association between Pathway Patterns, Clinical Features and Congenital Heart Disease

Resumo Fundamento A fístula da artéria coronária (FAC) é uma conexão direta entre uma ou mais artérias coronárias e câmaras cardíacas ou um grande vaso; pode estar associada à cardiopatia congênita. Objetivo Estabelecer os padrões de trajetos de FAC a partir de dados ecocardiográficos e correlacioná-los com aspectos clínicos e cardiopatias congênitas. Métodos Um total de 7.183 prontuários médicos de crianças menores de 5 anos de idade com cardiopatia submetidas a ecodopplercardiograma colorido foram analisados utilizando o teste de correlação de Spearman para associar sinais, sintomas e cardiopatia à FAC, com nível de significância de 5%. Resultados Vinte e seis crianças (0,0036%) apresentaram FAC, nos seguintes trajetos: da artéria coronária direita para o ventrículo direito (26,92%), da artéria coronária esquerda para o ventrículo direito (23,08%), do ramo interventricular anterior para o ventrículo direito (23,08%), da artéria coronária direita para o átrio direito (11,54%), da artéria coronária esquerda para o tronco pulmonar (7,69%) e do ramo interventricular anterior para o tronco pulmonar (7,69%). Em 57,69% dos pacientes, houve uma correlação positiva entre sintomas e a FAC (p = 0,445), relacionada à dispneia ou cianose (53,84%). Em 96,15%, a cardiopatia congênita estava associada à FAC; principalmente, a comunicação interventricular e a comunicação interatrial, em 34,62% dos casos, correlacionaram-se positivamente com a FAC (p = 0,295). O trajeto da FAC foi representado em três dimensões pelo software de modelagem, texturização e animação Cinema 4D R19. Conclusão A FAC é uma entidade anatômica incomum que apresenta quadro clínico compatível com dispneia e cianose e está associada a cardiopatias congênitas, principalmente com a CIV ou a CIA. De acordo com as análises ecocardiográficas, as fístulas na ACD, na ACE ou no RIVA representam aproximadamente um terço dos pacientes, com trajeto prioritário para as câmaras cardíacas direitas.

Abstract Background Coronary artery fistula (CAF) is a direct connection of one or more coronary arteries to cardiac chambers or a large vessel; it may be associated with congenital heart disease. Objective To establish CAF pathway patterns from echocardiographic data and to correlate them with clinical aspects and congenital heart disease. Methods A total of 7.183 medical records of children under the age of five years with cardiac disease submitted to color Doppler echocardiography and Spearman's Correlation test were used to associate signs and symptoms and cardiopathy to CAF with a significance level of 5%. Results Twenty six children (0.0036%) presented CAF: from the right coronary artery (RCA) to the right ventricle (RV) 26.92%, from the left coronary artery (LCA) to the RV 23.08%, from the anterior interventricular branch (AIVB) to RV 23.08%, RCA to right atrium (RA) 11.54%, LCA for pulmonary trunk (PT) 7.69% or AIVB for PT 7.69%. In 57.69% of the patients, there was a positively correlated symptomatology to CAF with p=0.445 related to dyspnea or cyanosis (53.84%); in 96.15%, congenital heart disease associated with CAF, mainly interventricular communication (IVC) or interatrial communication (IAC) in 34.62% positively correlated to CAF with p=0.295. CAF pathway was represented in three dimensions by software modeling, texturing and animation Cinema 4D R19. Conclusion CAF is an uncommon anatomical entity that presents a clinical picture compatible with dyspnea and cyanosis, and this is associated with congenital heart disease, mainly with IVC or IAC. According to echocardiographic analyzes, fistulas in RCA, LCA, or AIVB represent about one-third of the patients, with a priority pathway for right heart chambers.

Development and Evaluation of a Continuing Education Program for Nursing Technicians at a Pediatric Cardiac Intensive Care Unit in a Developing Country.

BACKGROUND: The nursing profession faces continuous transformations demonstrating the importance of professional continuing education to extend knowledge following technological development without impairing quality of care. Nursing assistants and technicians account for nearly 80% of nursing professionals in Brazil and are responsible for uninterrupted patient care. Extensive knowledge improvement is needed to achieve excellence in nursing care. The objective was to develop and evaluate a continuing education program for nursing technicians at a pediatric cardiac intensive care unit (PCICU) using a virtual learning environment entitled EDUCATE. METHODS: From July to September 2015, a total of 24 nursing technicians working at the PCICU at a children's hospital located in the northwestern region of São Paulo state (Brazil) fully participated in the continuing education program developed in a virtual learning environment using Wix platform, allowing access to video classes and pre- and post-training theoretical evaluation questionnaires outside the work environment. The evaluation tools recorded participants' knowledge evolution, technological difficulties, educational, and overall rating. RESULTS: Knowledge development was descriptively presented as positive in more than 66.7%. Content and training were considered "excellent" by most participants and 90% showed an interest in the use of technological resources. Technical difficulties were found and quickly resolved by 40% of participants including Internet access, login, and lack of technical expertise. CONCLUSION: The continuing education program using a virtual learning environment positively contributed to the improvement in theoretical knowledge of nursing technicians in PCICU.

Transposition of the Great Arteries in the Developing World: Surgery and Outcomes.

BACKGROUND: Little has been published regarding surgery for transposition of the great arteries (TGA) in the developing world. OBJECTIVES: This study sought to identify patient characteristics, surgical interventions, institutional characteristics, risk factors for mortality, and outcomes among patients undergoing surgery for TGA in this setting. METHODS: Developing world congenital heart surgical programs submitted de-identified data to a novel international collaborative database as part of a quality improvement project. We conducted a retrospective cohort study that included all cases of TGA with intact ventricular septum and TGA with ventricular septal defect performed from 2010 to 2013. Demographic, surgical, and institutional characteristics and their associations with in-hospital mortality were identified. RESULTS: There were 778 TGA operations performed at 26 centers, 480 (62%) for TGA with intact ventricular septum and 298 (38%) for TGA with ventricular septal defect. Most (80%) were single-stage arterial switch operations, but 20% were atrial baffling procedures (atrial switch operation) or 2-stage repairs (pulmonary artery band followed by arterial switch operation). Age at operation was >30 days in one-half of the cases and did not vary significantly with operation type. Survival was 85% and did not significantly vary with age at operation or operation type. Preceding septostomy was infrequently reported (16%) and was not associated with surgical mortality. Mortality was associated with lower World Health Organization weight/body mass index-for-age percentile and lower institutional volume of TGA repair. CONCLUSIONS: Surgical repair of TGA performed in the developing world is associated with an early survival of 85%. Type of surgical repair and age at operation varied considerably, but no associations with mortality were identified. In contrast, poor nutrition and small surgical volume were most strongly associated with mortality. Multicenter collaborative quality improvement efforts may benefit patients with TGA in the developing world.

A trileaflet "mitral valve" with three papillary muscles: brand new echocardiographic finding.

Congenital mitral valve malformations are rare, but are well known and described entities. Mitral valve malformations involve mitral valve apparatuses (leaflets and annulus) and subvalvar apparatuses (chordae and papillary muscle). Case reports of accessory mitral leaflets were already described, but were usually an appendix of the normal valve. We describe here a case report and present the images of a trileaflet mitral valve sustained by three papillary muscles in a young girl with subaortic stenosis.

A relação da equipe de enfermagem com a criança e a família em pós-operatório imediato de cardiopatias congênitas / The nursing team relationship with the child and the family in immediate post operative period of congenital heart defects

Cardiopatia congênita refere-se a anormalidades do coração e de grandes vasos presentes no nascimento. Sua etiologia é por vezes desconhecida, podendo relacionar-se a fatores pré-natais e genéticos. Há uma incidência de 8/1000 crianças nascidas vivas. A evolução cirúrgica exige assistência de enfermagem no pós-operatório imediato (POI) continuamente aperfeiçoada e atualizada nos aspectos técnico e científico, bem como manutenção e recuperação das funções orgânicas. A doença e a hospitalização na infância afetam toda família, fazendo com que a equipe de enfermagem conforte além da criança, os pais. Este foi um estudo qualitativo e descritivo que teve como objetivo verificar o conhecimento do enfermeiro ao cuidar do paciente pediátrico e sua família em POI de cardiopatia congênita. Foram entrevistadas onze enfermeiras da Unidadede Terapia Intensiva (UTI) Pediátrica Cardiológica de um hospital-escola do interior do estado de São Paulono período de dezembro de 2006 a fevereiro de 2007. Foram extraídos os seguintes temas sobre POI: ações,equipe multidisciplinar/interdisciplinar, monitorização hemodinâmica, recursos materiais, educação permanente ou conhecimento específico, ações exclusivas do enfermeiro e família. Todas entrevistadas citaram como principais ações os cuidados com o ventilador mecânico, monitorização cardíaca, coleta de exames, drogas eexame físico; 18,2% destacaram a importância de equipe estruturada. Quanto às ações exclusivas do enfermeiro, todas chamaram atenção para a coleta de exames e 91% para a monitorização. Sobre ações desenvolvidas com familiares 45,7% não se manifestaram, 27% desconhecem, 18,2% não sabem responder sobre tais atividades e 9,1% relataram que existem. Assim, o POI em cardiopatias congênitas aliado aos tratamentos inovadores exige do enfermeiro capacitação, buscando continuamente aprimorar seu conhecimento...

Congenital heart defects refer to heart and great vessels abnormalities seen at birth. Its etiology is sometimes unknown, and it can be related to prenatal and genetic factors. Eight in one thousand live born children are affected. The surgical evolution demands continuously improved, technically and scientifically updated nursing assistance during the immediate post operative period (POI), as well as maintenance and recovery ofthe organic functions. Illness and hospitalization during the childhood affect the entire family, thus the nursing team must be a comfort to the child and parents. This is a qualitative and descriptive study which aimed at verifying the nurses’ know-how regarding the care of pediatric patients and its family with congenital heart defects in POI. Eleven nurses from the Cardiologic Pediatric Intensive Care Unit in a school hospital ofin the countryside of the state of São Paulo were interviewed from December 2006 to February 2007. The following themes regarding POI were extracted from the interviews: Actions, Multidisciplinary/Interdisciplinary Team, Hemodynamic Monitoring, Material Resources, Continued Education or Specific Knowledge, Nurse’s Exclusive Actions and Family. All of the interviewed professionals mentioned as main actions the care with the mechanical ventilator, cardiac monitoring, exams collection, drugs and physical examination; 18.2%reported the importance of a structuralized team. Regarding nurses’ exclusive actions, 100% focused onexams collection and 91% focused on monitoring. Regarding mutual actions involving the family, 45.7% didnot mention anything, 27% reported not to know such actions, 18.2% reported not to be able to assess thistopic, and 9.1% reported to be aware such actions exist. Thus, POI in congenital heart defects allied to theinnovative treatments demand continuous nurse qualification for knowledge improvement...

Distúrbios respiratórios em crianças com síndrome de Down / Respiratory disorders in children with Down syndrome

A síndrome de Down é uma condição genética caracterizada pela presença de um cromossoma a mais nas células de quem é portador e acarreta variável grau de retardo no desenvolvimento motor, físico e mental, sendo geralmente reconhecida por uma constelação de anormalidades ssociadas. O estudo teve como objetivo analisar os distúrbios pulmonares em crianças com síndrome de Down, pois é de fundamental importância relevar que essas crianças possuem más-formações congênitas e, que a presença da anormalidade cromossômica em si, pode aumentar o risco de certas complicações, como a predisposição a comprometimentos respiratórios. Mais da metade das causas de admissão hospitalar de crianças com síndrome de Down são problemas respiratórios, sendo que do total das admissões, 10% são admitidas diretamente na unidade de terapia intensiva pediátrica. Descobriu-se esta revisão que os principais distúrbios que interferem no sistema respiratório são obstrução das vias aéreas superiores, doença das vias respiratórias inferiores, cardiopatias congênitas, hipertensão pulmonar, hipoplasia pulmonar, apnéia obstrutiva do sono, imunodeficiência, obesidade relativa e hipotonia. No entanto, a maioria dos textos não faz referência a esses problemas, e há falta de pesquisa sobre o assunto

Ampliaçäo ostial coronariana bilateral com veia safena em paciente portador de aortite sifilítica / Coronary bilateral ostial enlargement using the saphenous vein in a patient with syphilitic aortitis

A patient with tertiary syphilis presenting with bilateral coronary ostial lesions and aortic regurgitation underwent surgical reconstruction of the coronary ostia by the anterior approach with autogenous saphenous vein grafting and substitution of the aortic valve with a bovine bioprosthesis. The procedure was easily performed and had good outcomes both early and late. The rarity of the association of a lesion in both coronary ostia with aortic regurgitation in syphilis and the surgical technique employed are discussed.