Caso Clínico: Tetralogía de Fallot Extrema / Case Report: Extreme Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot representa la cardiopatía congénita cianógena más frecuente después del primer año de vida, ocurre aproximadamente en 1 de cada 3 500 nacimientos y representa el 7 al 10% de todas las Enfermedades Cardiacas Congénitas. Su variante extrema que se acompaña de atresia pulmonar representa el 1 a 2% de las cardiopatías congénitas. Presentamos una Tetralogía de Fallot Extrema Ductus Dependiente de manejo complejo. CASO CLÍNICO: Se trata de un neonato a término de 12 días de vida, adecuado para la edad gestacional, sin factores de riesgo prenatales, ni antecedentes patológicos familiares, el cual es diagnosticado de Tetralogía de Fallot Extrema por ecocardiograma. EVOLUCIÓN: Paciente inicialmente tratado con infusión de prostaglandina E1. Posteriormente mediante fístula Blalock-Taussig izquierda. CONCLUSIONES: Para mejorar el pronóstico y sobrevida de un paciente con Tetralogía de Fallot Extrema el diagnóstico prenatal oportuno y el tratamiento apropiado es de vital importancia, sin embargo el tratamiento definitivo con derivaciones pulmonares sistémicas representan complicaciones con riesgo de mortalidad. El presente caso tuvo un desenlace desfavorable.

BACKGROUND: The Tetralogy of Fallot represents the most common cyanogenic heart disease after the first year of life. It occurs in 1 of 3 500 live births and accounts as 7 to 10% of all Congenital Heart Diseases. Its extreme variant is accompanied by pulmonary atresia and accounts 1 to 2% of the Congenital Heart Diseases. We present an Extreme Tetralogy of Fallot­ Ductus Dependent of complex management. CLINICAL CASE: A full term 12 day neonate, adequate for gestational age, without prenatal risk factors or pathologic family antecedents, is diagnosed with Extreme Tetralogy of Fallot by echocardiogram. EVOLUTION: The patient was treated initially with an infusion of prostaglandin E1. Then, a left Blalock-Taussig shunt was performed. CONCLUSIONS: To improve the prognosis and survival rates of patients with Extreme Tetralogy of Fallot , a timely diagnosis and treatment are of vital importance. Definitive treatment with systemic pulmonary shunts carries high risk complications. The present case had an unfavorable evolution.

Indicações atuais para anticoagulacão em crianças com Cardiopatias Congênitas / Current indications for anticoagulation in children with Congenital Heart Disease

Os avanços nas técnicas cirúrgicas e manejo clínico aumentaram a sobrevida das crianças com cardiopatia congênita. Eventos tromboembólicos representam importante causa de morbimortalidade nestes pacientes, principalmente no pós-operatório da cirurgia de Fontan e implante de próteses, acrescido do risco adicional do seu tratamento. Particularidades próprias da idade, fatores hemodinâmicos e limitações na investigação de trombose contribuem para a dificuldadede definir diretrizes para anticoagulação em crianças. Os medicamentos mais usados na fase aguda são: heparina não fracionada e de baixo peso molecular, enquanto em longo prazo os antagonistas da vitamina K, principalmente a varfarina. O manejo seguro de anticoagulação nesta faixa etária é dificultado pela estreita faixa terapêutica, interação alimentar, uso simultâneo de vários medicamentos, além da dificuldade de punção venosa frequente e, com frequência os níveis desejáveis não são atingidos. Ainda faltam dados que deem suporte para recomendação de anticoagulação em crianças. Os novos anticoagulantes como os inibidores diretos da trombina e os inibidores seletivos do fator Xa, são empregados em adultos, principalmente com fibrilação atrial, o uso pediátrico ainda não está definido. Esta revisão sumariza as indicações atuais do uso de anticoagulantes e antiagregantes plaquetários em crianças com cardiopatia congênita.

Advances in surgical techniques and clinical management have led to increased survival ofchildren with congenital heart disease. Thromboembolic events represent a major cause of morbidity and mortality in these patients, particularly following Fontan surgery and the implantation of prosthesis, added to the risk of their treatment. Characteristics such as age, hemodynamic factors and limitations in the investigation of thrombosis have contributed to the difficulty of defining guidelines for anticoagulation in children. The most commonly used medications in the acute phase are and low molecular weight heparin, and in the long term, vitamin K antagonists, particularly warfarin. The safe management of anticoagulation in this age range is hindered by the narrow therapeutic range, food interactions, simultaneous use of other medications, and the difficulty of frequent venous puncture. As a result, the desired levels are often not reached. There is also a lack of data to support the recommendation of anticoagulation in children. The pediatric use of the new anticoagulants, such as direct thrombin inhibitors and selective factor Xa inhibitors used in adults, particularly with atrial fibrillation, is not yet defined. This review summarizes the current indications of the use of anticoagulants and platelet antiaggregants in children with congenital heart disease.

Resultados imediatos e de médio prazo da intervenção com stent ductal em recém-nascidos e lactentes / Immediate and medium-term outcomes of ductal stenting in neonates and infants

O implante de stents para manter o ducto arterial patente na cardiopatia congênita cianótica éuma alternativa à cirurgia de Blalock-Taussig modificada (BTm) em pacientes de alto risco. Descrevemos osresultados imediatos e de médio prazo do implante de stent em neonatos e lactentes com circulação pulmonarducto-dependente. Métodos: Trata-se de estudo descritivo e prospectivo, que incluiu diferentes cardiopatias congênitas cianóticas tratadas entre 2014 e 2015.Resultados: Avaliamos 14 pacientes, com média de idade de 46 dias e pesando 4,5 kg, sendo a atresia pulmonar associada à comunicação interventricular a cardiopatia mais tratada. A abordagem pela artéria femoral ocorreu em 70% dos procedimentos e, nos demais, por via carotídea. Stents de 3,5 × 12 mm foram usados na maioria dos casos, e o sucesso do implante foi obtido em 78% das intervenções (11/14). Os casos de insucesso foram encaminhados para cirurgia − uma delas em situação de urgência, que resultou em óbito. Ocorreu espasmo ductal < 48 horas em três pacientes que necessitaram de BTm, com evolução favorável. Complicações após a alta e nos primeiros 30 dias incluíram trombose de stent (2/11), uma delas controlada com redilatação e outra que evoluiu para óbito, e uma morte súbita (1/11). A mortalidade total foi de 21,4% (3/14). A patência do ducto arterial nos primeiros 6 meses foi obtida em 5 casos que foram submetidos à cirurgia paliativa.Conclusões: A experiência inicial de implante de stent ductal mostrou resultados imediatos favoráveis, e, emmédio prazo, mais de um terço dos pacientes com circulação pulmonar ducto-dependente manteve seus canaispatentes...

The implantation of stents to keep the ductus arteriosus patent in cyanotic congenital heart disease is an alternative to the modified Blalock-Taussig surgery (mBT) in high-risk patients. This study describes theimmediate and medium-term outcomes of stent implantation in neonates and infants with duct-dependentpulmonary circulation. Methods: This was a descriptive and prospective study including different cyanotic congenital heart diseases treated between 2014 and 2015. Results: Fourteen patients with a mean age of 46 days, and mean weight of 4.5 kg were assessed, andpulmonary artresia with interventricular communication was the most treated condition. The femoral artery approach was used in 70% of procedures; carotid approach was used in the remaining cases. Stents of 3.5 x 12 mm were used in most cases, and implant success was achieved in 78% of interventions (11/14). The failed cases were referred to surgery – one of them was an emergency, which resulted in death. Ductal spasm occurred in < 48 hours in three patients who required mBT, with favorable outcome. Complicationsafter discharge and within the first 30 days included stent thrombosis (2/11), one of which was controlled with redilation, another progressed to death, and one sudden death (1/11). The overall mortality was 21.4% (3/14). A patent ductus arteriosus in the first 6 months was present in five cases, which underwent palliative surgery.Conclusions: The initial experience of ductal stenting showed favorable immediate outcomes, but in the mediumterm, little more than a third of the cases maintained a patent ductus arteriosus within 6 months...

Laparoscopic colectomy in an adult with single ventricle physiology: Anesthetic implications and management.

Increasing numbers of adult patients with complex congenital heart conditions are presenting for noncardiac surgery later in life. These disorders can present challenges for surgical and anesthesia providers. Specifically, single ventricle lesions offer anatomic and physiologic concerns during the perioperative period. Single ventricle physiology represents a delicate balance between systemic and pulmonary blood flow. Any alterations in blood flow through these systems can produce undesirable hemodynamic changes, especially during the perioperative period. We present a case of an adult patient with a single left ventricle who presented for laparoscopic total colectomy due to inflammatory bowel disease. His abnormal anatomy coupled with the hemodynamic disruptions caused by laparoscopy presented significant anesthetic challenges. We highlight the anesthetic concerns of single ventricle physiology, specifically pertaining to laparoscopic surgery. We provide recommendations for safely managing these patients perioperatively. With detailed preoperative evaluation and close hemodynamic monitoring during the perioperative period, these patients can experience successful surgical and anesthetic outcomes.

Modified Blalock Taussig shunt: Comparison between neonates, infants and older children.

Objective: The aim was to compare various pre-and post-operative parameters and to identify the predictors of mortality in neonates, infants, and older children undergoing Modifi ed Blalock Taussig shunt (MBTS). Materials and Methods: Medical records of 134 children who underwent MBTS over a period of 2 years through thoracotomy were reviewed. Children were divided into three groups-neonates, infants, and older children. For analysis, various pre-and post-operative variables were recorded, including complications and mortality. Results: The increase in PaO2 and SaO2 levels after surgery was similar and statistically signifi cant in all the three groups. The requirement of adrenaline, duration of ventilation and mortality was signifi cantly higher in neonates. The overall mortality and infant mortality was 4.5% and 8%, respectively. Conclusion: Neonates are at increased risk of complications and mortality compared with older children. Age (<30 days), weight (<3 kg), packed red blood cells transfusion >6 ml/kg, mechanical ventilation >24 h and post shunt increase in PaO2 (PDiff) <25% of baseline PaO2 are independent predictors of mortality in children undergoing MBTS.