Double shunt technique for hybrid palliation of hypoplastic left heart syndrome: a case report

We report a technique to palliate hypoplastic left heart syndrome, with no PDA stenting, but with double polytetrafluoroethylene shunt from pulmonary artery to ascending and descending aorta by combined thoracotomies. A 30-day-old female was operated with this technique. Five months after first operation, the child was submitted to Norwood/Glenn operation. Good hemodinamic recovery and initial clinical evolution was observed. The child was extubated in 8th post operatory day and reentubated in the next day due to pulmonary infection. Despite antibiotic treatment, the child died after systemic infectious complications

I Diretriz brasileira de cardio-oncologia pediátrica da Sociedade Brasileira de Cardiologia

Sociedade Brasileira de Oncologia PediátricaUniversidade Federal de São Paulo (UNIFESP) Instituto de Oncologia Pediátrica GRAACCUniversidade Federal de São Paulo (UNIFESP)Universidade de São Paulo Faculdade de Medicina Instituto do Coração do Hospital das ClínicasUniversidade Federal do Rio Grande do Sul Hospital de Clínicas de Porto AlegreInstituto Materno-Infantil de PernambucoHospital de Base de BrasíliaUniversidade de Pernambuco Hospital Universitário Oswaldo CruzHospital A.C. CamargoHospital do CoraçãoSociedade Brasileira de Cardiologia Departamento de Cardiopatias Congênitas e Cardiologia PediátricaInstituto Nacional de CâncerHospital Pequeno PríncipeSanta Casa de Misericórdia de São PauloInstituto do Câncer do Estado de São PauloUniversidade Federal de São Paulo (UNIFESP) Departamento de PatologiaHospital Infantil Joana de GusmãoUNIFESP, Instituto de Oncologia Pediátrica GRAACCUNIFESP, Depto. de PatologiaSciEL

Diretriz sobre Diagnóstico e Tratamento da Cardiomiopatia Hipertrófica – 2024

Hypertrophic cardiomyopathy (HCM) is a form of genetically caused heart muscle disease, characterized by the thickening of the ventricular walls. Diagnosis requires detection through imaging methods (Echocardiogram or Cardiac Magnetic Resonance) showing any segment of the left ventricular wall with a thickness > 15 mm, without any other probable cause. Genetic analysis allows the identification of mutations in genes encoding different structures of the sarcomere responsible for the development of HCM in about 60% of cases, enabling screening of family members and genetic counseling, as an important part of patient and family management. Several concepts about HCM have recently been reviewed, including its prevalence of 1 in 250 individuals, hence not a rare but rather underdiagnosed disease. The vast majority of patients are asymptomatic. In symptomatic cases, obstruction of the left ventricular outflow tract (LVOT) is the primary disorder responsible for symptoms, and its presence should be investigated in all cases. In those where resting echocardiogram or Valsalva maneuver does not detect significant intraventricular gradient (> 30 mmHg), they should undergo stress echocardiography to detect LVOT obstruction. Patients with limiting symptoms and severe LVOT obstruction, refractory to beta-blockers and verapamil, should receive septal reduction therapies or use new drugs inhibiting cardiac myosin. Finally, appropriately identified patients at increased risk of sudden death may receive prophylactic measure with implantable cardioverter-defibrillator (ICD) implantation.La miocardiopatía hipertrófica (MCH) es una forma de enfermedad cardíaca de origen genético, caracterizada por el engrosamiento de las paredes ventriculares. El diagnóstico requiere la detección mediante métodos de imagen (Ecocardiograma o Resonancia Magnética Cardíaca) que muestren algún segmento de la pared ventricular izquierda con un grosor > 15 mm, sin otra causa probable. El análisis genético permite identificar mutaciones en genes que codifican diferentes estructuras del sarcómero responsables del desarrollo de la MCH en aproximadamente el 60% de los casos, lo que permite el tamizaje de familiares y el asesoramiento genético, como parte importante del manejo de pacientes y familiares. Varios conceptos sobre la MCH han sido revisados recientemente, incluida su prevalencia de 1 entre 250 individuos, por lo tanto, no es una enfermedad rara, sino subdiagnosticada. La gran mayoría de los pacientes son asintomáticos. En los casos sintomáticos, la obstrucción del tracto de salida ventricular izquierdo (TSVI) es el trastorno principal responsable de los síntomas, y su presencia debe investigarse en todos los casos. En aquellos en los que el ecocardiograma en reposo o la maniobra de Valsalva no detecta un gradiente intraventricular significativo (> 30 mmHg), deben someterse a ecocardiografía de esfuerzo para detectar la obstrucción del TSVI. Los pacientes con síntomas limitantes y obstrucción grave del TSVI, refractarios al uso de betabloqueantes y verapamilo, deben recibir terapias de reducción septal o usar nuevos medicamentos inhibidores de la miosina cardíaca. Finalmente, los pacientes adecuadamente identificados con un riesgo aumentado de muerte súbita pueden recibir medidas profilácticas con el implante de un cardioversor-desfibrilador implantable (CDI).A cardiomiopatia hipertrófica (CMH) é uma forma de doença do músculo cardíaco de causa genética, caracterizada pela hipertrofia das paredes ventriculares. O diagnóstico requer detecção por métodos de imagem (Ecocardiograma ou Ressonância Magnética Cardíaca) de qualquer segmento da parede do ventrículo esquerdo com espessura > 15 mm, sem outra causa provável. A análise genética permite identificar mutações de genes codificantes de diferentes estruturas do sarcômero responsáveis pelo desenvolvimento da CMH em cerca de 60% dos casos, permitindo o rastreio de familiares e aconselhamento genético, como parte importante do manejo dos pacientes e familiares. Vários conceitos sobre a CMH foram recentemente revistos, incluindo sua prevalência de 1 em 250 indivíduos, não sendo, portanto, uma doença rara, mas subdiagnosticada. A vasta maioria dos pacientes é assintomática. Naqueles sintomáticos, a obstrução do trato de saída do ventrículo esquerdo (OTSVE) é o principal distúrbio responsável pelos sintomas, devendo-se investigar a sua presença em todos os casos. Naqueles em que o ecocardiograma em repouso ou com Manobra de Valsalva não detecta gradiente intraventricular significativo (> 30 mmHg), devem ser submetidos à ecocardiografia com esforço físico para detecção da OTSVE.   Pacientes com sintomas limitantes e grave OTSVE, refratários ao uso de betabloqueadores e verapamil, devem receber terapias de redução septal ou uso de novas drogas inibidoras da miosina cardíaca. Por fim, os pacientes adequadamente identificados com risco aumentado de morta súbita podem receber medida profilática com implante de cardiodesfibrilador implantável (CDI)

Fechamento de canal arterial por minitoracotomia: técnica e resultados Patent ductus arteriosus (PDA) closure with minithoracotomy: technique and results

CASUÍSTICA E MÉTODOS: No período de novembro de 1996 a dezembro de 1997, 15 crianças portadoras de canal arterial (CA), sendo 12 do sexo feminino, com idade média de 2,7 anos, peso médio de 13,9 kg foram submetidas a fechamento do CA por minitoracotomia. O ecodopplercardiograma confirmou o diagnóstico em todos o casos e mostrou o diâmetro do CA entre 2 mm e 10 mm, com média de 4,06 mm. A indicação cirúrgica foi eletiva em todos os casos. A operação consistiu de minitoracotomia esquerda no 4º espaço intercostal de 2,5 cm a 3,0 cm, seguida de dissecção do CA e clipagem do mesmo com 2 clips metálicos. Não foi utilizada drenagem pleural em nenhum dos casos. RESULTADOS: Todos os pacientes receberam alta em média no 4º dia de pós-operatório, sem nenhum escape pelo CA ao ecodopplercardiograma. CONCLUSÃO: O fechamento de CA por minitoracotomia é uma alternativa de tratamento que reduz o período de internação, bom efeito cosmético e baixo índice de complicações.<br>OBJECTIVE:The purpose of this study was to describe a new technique for closure of patent ductus arteriosus (PDA) by minithoracotomy (2.5 a 3.0 cm) and clipping the PDA with titanium clips. MATERIAL AND METHODS: From November 1996 to December 1997, 15 children with PDA underwent surgical closure. The mean age at the time of operation was 2.7 years, mean weight was 13.9 kg. The procedure was through a left minithoracotomy at the 4º intercostal space. The ductus was identified, dissected and isolated. Interruption of ductal flow was performed by direct clipping with two clips. The chest was closed without a chest drain. Unless the patient was ventilator dependent before the closure, the child usually was extubated in the operating room. RESULTS: Color doppler echocardiography demonstrated total occlusion of the ductus in all patients. All 15 patients were discharged from the hospital on the 4º postoperative day (mean). CONCLUSION: We conclude that surgical closure of patent ductus arteriosus with minitoracotomy, without chest tube drainage can be accomplished safely and with low incidence of complications

Incidence and Treatment of Chylothorax in Children Undergoing Corrective Surgery for Congenital Heart Diseases

Abstract Introduction: Chylothorax is a lymphatic extravasation into pleural cavity and its incidence is 0.25%-5.3% in children undergoing cardiac surgery. Objective: To evaluate the incidence of chylothorax in pediatrics patients operated, linking it in each surgical intervention. Evaluate treatment types and efficiency. Methods: Retrospective study using medical records of children undergoing cardiac surgery in the Hospital do Coração between 2004 and 2014. For statistical analysis, qualitative variables by absolute frequency and relative frequency; quantitative variables, by median of 25 and 75 percentiles, as they did not present normal distribution (Shapiro-Wilk, P<0.05). The Chi-square test was used for the association between type of treatment and result. The adopted confidence level was 95%. Results: Incidence of chylothorax was 2.1% (0.9% in intracardiac surgery, 1.7% correction of patent ductus arteriosus and aortic coarctation, 8.3% Glenn's surgery, 11.8% total cavopulmonary surgery and 3% in others). Among treatments, fasting associated with total parenteral nutrition (TPN) resolved 51% of the cases. Hypoglossal diet had failed treatment and surgical referral in 22% of the cases. Fasting with TPN associated with octreotide had success in the treatment of chylothorax in a period exceeding 15 days in 78% of cases, and 3.7% were referred for surgery. Conclusion: According to the results, incidence of chylothorax was 2.18%. Treatment with fasting and TPN leads to resolutions in 86.5%, and the association with octreotide was successful in 85.1% of cases, showing an efficient option, while the treatment with hypoglossal diet had therapeutic failure in 22% of the cases in which it was used