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)

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

Prevalence and surgical approach of supravalvular pulmonary stenosis after jatene operation for transposition of great arteries

FUNDAMENTO: A transposição das grandes artérias é a cardiopatia congênita cianogênica mais freqüente no período neonatal, correspondendo a 7% de todas as cardiopatias congênitas. Dentre as operações para tratamento cirúrgico, a operação de Jatene, com correção arterial, é o tratamento escolhido. Durante a evolução pós-operatória tardia, alguns problemas foram observados, sendo o mais comum a ocorrência de estenose supravalvar na neopulmonar, independentemente do tipo da técnica cirúrgica utilizada. OBJETIVO: Estudar e analisar a prevalência da estenose, bem como descrever o tratamento cirúrgico e propor manobras técnicas para prevenir seu aparecimento. MÉTODOS: Dentre 553 pacientes operados, 409 tiveram alta hospitalar e 281 seguidos tardiamente; 59 (20,9%) apresentaram diferentes graus de estenose supravalvar pulmonar e 21 gradiente médio superior a 60 mmHg, necessitando tratamento cirúrgico. Dependendo da localização e da anatomia da estenose, o tratamento cirúrgico constou de aplicação de diferentes técnicas, como ampliação das áreas de estenose com remendos de pericárdio bovino, ressecção de áreas estenóticas e anastomose término-terminal, substituição de remendos retraídos e de tubos sintéticos. RESULTADOS: Houve boa evolução em 20 pacientes, com óbito em um dos casos. CONCLUSÃO: Conclui-se que a estenose supravalvar pulmonar pós-operação de Jatene para transposição das grandes artérias teve prevalência de 20,9%. Uma vez identificada e com indicação de tratamento, pode ser tratada cirurgicamente com baixa mortalidade, mediante diferentes técnicas cirúrgicas. Para prevenir a ocorrência de estenose, propõem-se ampla dissecção e liberação dos ramos pulmonares, anastomoses amplas, remendos amplos de pericárdio autólogo e cuidado na reconstrução da neoaorta, evitando compressão da neopulmonar.BACKGROUND: The Transposition of the Great Arteries is the most frequent congenital cyanogenic cardiopathy in the neonatal period, corresponding to 7% of all congenital cardiopathies. Among the operations for surgical treatment, the Jatene operation, with arterial correction, is the treatment of choice. During the late postoperative evolution, some problems were observed, with the most common being the occurrence of supravalvular stenosis at the neopulmonary, regardless of the type of surgical technique used. OBJECTIVE: To study and analyze the prevalence of stenosis, as well as describe the surgical treatment and propose technical maneuvers to prevent its onset. METHODS: Of the 553 patients that underwent surgery, 409 were discharged from the hospital and 281 had late follow-up; 59 (20.9%) presented different degrees of supravalvular pulmonary stenosis and 21 had a mean gradient > 60 mmHg, needing surgical treatment. Depending on the location and anatomy of the stenosis, the surgical treatment consisted of the use of different techniques, such as the enlargement of stenosis areas with bovine pericardium patches, resection of stenotic areas and termino-terminal anastomosis, replacement of retracted patches and synthetic tubes. RESULTS: Twenty patients presented good evolution and only one patient died. CONCLUSION: It can be concluded that the supravalvular pulmonary stenosis, post-Jatene operation for Transposition of Great Arteries, had a prevalence of 20.9%; once identified and with indication for treatment, it can be treated surgically with low mortality levels, through different surgical techniques; to prevent the occurrence of stenosis, ample dissection and release of the pulmonary branches, double anastomoses, large patches of autologous pericardium and careful reconstruction of the aorta are proposed, which prevents the compression of the neopulmonary