ABSTRACT
La técnica quirúrgica de Fontan consiste en transmitir el flujo de la vena cava inferior a través de una anastomosis cavopulmonar para evitar que se mezcle la sangre oxigenada con la no oxigenada, en caso de atresia tricuspídea asociada a ventrículo hipoplásico. Las alteraciones fisiológicas que produce esta nueva circulación tienen varias consecuencias a nivel cardiaco y extracardiaco, incluido el hígado, con múltiples descripciones de hepatopatía crónica de origen vascular, e incluso de carcinoma hepatocelular en hígado cirrótico a edades tempranas. Con el objetivo de conocer un poco más sobre las afectaciones hepáticas que produce este procedimiento, se describen dos casos clínicos y se realiza una revisión de la literatura. A pesar de que la hepatopatía asociada a Fontan es una entidad poco frecuente, los hepatólogos deben tenerla presente y aprender a reconocerla, ya que un adecuado seguimiento puede ser la diferencia al momento de seleccionar de manera eficaz, aquellos pacientes que se beneficiarían de un trasplante cardiaco único o combinado de hígado y corazón, además de permitir la detección de manera oportuna de complicaciones tan devastadoras como un carcinoma hepatocelular en estadios avanzados.
Fontan surgery diverts the flow of the inferior vena cava through a cavopulmonary anastomosis, to avoid mixing the oxygenated and non-oxygenated blood, in the presence of tricuspid atresia associated with hypoplastic ventricle. The physiological changes produced by this new circulation have several consequences at cardiac and extracardiac level, including the liver, with multiple descriptions of chronic liver disease of vascular origin, and even hepatocellular carcinoma in cirrhotic liver at an early age. With the aim of a better understanding of liver complications caused by this procedure, two clinical cases are described and a literature review is presented. Despite the fact that Fontan-associated liver disease is a rare entity, hepatologists must keep it in mind and learn how to recognize it, since an adequate follow-up can make the difference when effectively selecting those patients who would benefit from a single or combined liver and/or heart transplant, as well as allowing the early detection of devastating complications such as advanced stage hepatocellular carcinoma.
Subject(s)
Humans , Fontan Procedure , Liver Diseases , Carcinoma, Hepatocellular , Univentricular Heart , Liver CirrhosisABSTRACT
Se realizó un estudio transversal en escolares con corazón univentricular en estadio pos-bypass total de ventrículo derecho con el objetivo de determinar la capacidad funcional basal mediante el test de marcha en 6 minutos e identificar posibles factores determinantes. Participaron 30 pacientes con una mediana de edad de 12 años. Dieciocho pacientes fueron de sexo masculino. La mediana de distancia recorrida fue de 551,3 metros, un 84 % de la distancia teórica para población pediátrica sana. Las variables talla, presión arterial sistólica pretest y saturación arterial de oxígeno de reposo se asociaron significativamente con la distancia recorrida en el modelo de regresión lineal múltiple. No hubo asociación significativa en los metros caminados respecto de las variables sexo, estado nutricional, dignóstico cardiológico inicial, número de cirugías previas y edad al momento del bypass total de ventrículo derecho
A cross-sectional study was done in students with univentricular heart after undergoing total cavopulmonary connection (Fontan procedure) to establish their baseline functional capacity based on the six-minute walk test and identify potential determining factors. Thirty patients were included; their median age was 12 years old. Eighteen patients were males. The median distance walked was 551.3 meters, 84 % of the theoretical distance for a healthy pediatric population. Height, pre-test systolic blood pressure, and resting arterial oxygen saturation showed a significant association with the distance walked in the multiple linear regression model. No significant association was observed in the meters walked in terms of the following outcome measures: sex, nutritional status, baseline cardiological diagnosis, number of prior surgeries, and age at the time of Fontan procedure
Subject(s)
Humans , Male , Female , Child , Adolescent , Univentricular Heart/diagnosis , Students , Cross-Sectional Studies , Exercise Tolerance , Fontan Procedure , Walk Test , Cardiac Rehabilitation , GaitABSTRACT
Abstract Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.
Resumo Fundamento: O procedimento de Fontan-Kreutzer (FK) foi amplamente realizado no passado, mas a longo prazo gerou muitas complicações, resultando em falha na circulação univentricular. A conversão para conexão cavopulmonar total (CCPT) é uma das opções de tratamento. Objetivo: Avaliar os resultados da conversão de FK para CCPT. Métodos: Revisão retrospectiva de prontuários de pacientes submetidos à conversão de FK para CCPT no período de 1985 a 2016. Significância p < 0,05. Resultados: Operações do tipo Fontan foram realizadas em 420 pacientes durante este período: CCPT foi realizada em 320, técnica de túnel lateral em 82 e FK em 18. Dez casos do grupo FK foram eleitos para conversão em CCPT. Todos os pacientes submetidos à conversão de Fontan foram incluídos neste estudo. Em nove pacientes, a indicação deveu-se a arritmia não controlada e em um devido à enteropatia perdedora de proteínas. A morte foi observada nos dois primeiros casos. O tempo médio de internação na unidade de terapia intensiva (UTI) foi de 13 dias e o tempo médio de internação hospitalar foi de 37 dias. Uma classe funcional pela melhora da New York Heart Association (NYHA) foi observada em 80% dos pacientes em NYHA I ou II. Cinquenta e sete por cento das conversões devido a arritmias tiveram melhora das arritmias; quatro casos foram curados. Conclusões: A conversão é um procedimento complexo e requer que um hospital terciário experiente seja realizado. A conversão melhorou a classe funcional da NYHA, apesar de uma resolução insatisfatória da arritmia.