Fontan-associated liver disease current status and transplantation consideration / 中华肝脏病杂志

Fontan-associated liver disease (FALD) is one of the main complications after the Fontan procedure, manifesting mostly as liver fibrosis and even cirrhosis, with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis. The specific cause is unknown, although it is considered to be associated with long-term elevated central venous pressure, impaired hepatic artery blood flow, and other relevant factors. The absence of association between laboratory tests, imaging data, and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult. A liver biopsy is the gold standard for diagnosing and staging liver fibrosis. The most important risk factor for FALD is time following the Fontan procedure; therefore, it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma. Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.

Echocardiographic and Ultrasonographic Parameters Associated with Protein-losing Enteropathy in Patients with Fontan Physiology: a Systematic Review with Meta-analysis

Abstract Background: Fontan circulation can be associated with significant morbidity, especially Protein-Losing Enteropathy (PLE). Echocardiographic parameters can provide valuable diagnostic information about a patient's risk of developing PLE after Fontan surgery. Objectives: To describe echocardiographic/ultrasonographic parameters associated with PLE in patients after Fontan surgery through a systematic review with meta-analysis. Methods: A literature search was performed in electronic databases to identify relevant studies about echocardiographic parameters and PLE prediction in children after Fontan surgery. The search terms used were: "echocardiography", "ultrasonography", "Fontan," and "protein-losing enteropathy". A p < 0.05 was considered statistically significant. Results: A total of 653 abstracts were obtained from electronic databases and bibliographic references. From these, six articles met criteria to be included in the qualitative analysis and three in the quantitative (meta-analysis). The resistance index in the superior mesenteric artery was described in three studies, and the quantitative analysis showed statistical significance (p < 0.001). Other echocardiographic and ultrasonographic parameters were also described, albeit in single studies not allowing a meta-analysis. Conclusion: This systematic review with meta-analysis identified echocardiographic and ultrasonographic parameters related to PLE in patients with Fontan physiology. Vascular ultrasonography seems to play a prominent role in this aspect, but additional studies are needed to increase the degree of evidence.

Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation / Resultados da Conversão da Cirurgia de Fontan-Kreutzer para Cavo-Pulmonar Total Devido Falência da Circulação Univentricular

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.

Major intrahepatic veno-venous fistula after fontan operation treated by transcatheter implantation of amplatzer septal occluder through internal jugular vein

Abstract Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay. Three weeks after the first intervention, a second procedure was performed to retrieve the first device and to close the fistula distally. Multiple attempts with different types of gooseneck snares and a bioptome catheter failed to retrieve the first device, so a telescopic method was used to re-screw it. Using a Mullins long sheath and delivery sheath, the delivery cable was manipulated to fit into the slot of the end screw, and the cable was rotated gently in a clockwise direction to re-screw the device. Then, another Amplatzer septal occluder was placed at the distal end of the fistula. In conclusion, distal transcatheter occlusion of intrahepatic veno-venous fistulas might lead to better clinical outcomes in selected patients. Amplatzer septal occluder device can be retrieve without any complication within three weeks.

Enteropatía perdedora de proteínas en pacientes con corazón univentricular / Protein-losing enteropathy found in patients with univentricular heart

INTRODU: la enteropatía perdedora de proteínas puede aparecer en la evolución de los pacientes con corazón univentricular que sobreviven a la derivación cavopulmonar total. Una vez que se diagnostica, la mortalidad es alta. OBJETIVO: identificar los posibles factores de riesgo de esta complicación. MÉTODOS: se realizó un estudio de cohorte prospectivo de la evolución en 74 pacientes con derivación cavopulmonar total, intervenidos en el Cardiocentro Pediátrico "William Soler", desde enero de 1992 hasta enero de 2011. RESULTADOS: el tiempo promedio de evolución fue de 8 años. Sufrió enteropatía perdedora de proteínas 8,1 % de los pacientes. Se presentó con mayor frecuencia en los operados con la técnica intratrial, en los operados con más de 6 años de edad, y en quienes sufrieron derrames pleurales persistentes en el posoperatorio inmediato. Se encontró relación significativa entre la enteropatía y la disfunción ventricular posoperatoria, con RR= 11,45 (IC: 95 %: 2,37 a 55,16). El análisis multivariado identificó a la disfunción ventricular como factor de riesgo. CONCLUSIÓN: la detección de disfunción ventricular en la evolución del paciente con derivación cavopulmonar debe orientar el tratamiento, en aras de evitar la aparición de enteropatía perdedora de proteínas.

INTRODUCTION: protein-losing enteropathy may occur in the progression of patients with univentricular heart, who survived total cavopulmonary shunt. Once diagnosed, the mortality rate of the condition is high. ONJECTIVE: to identify the possible risk factor of this complication. METHODS: a prospective cohort study of the progression of 74 patients with total cavopulmonary shunt was conducted from January 1992 through January 2011. They had been operated on at "William Soler" pediatric cardiac center. RESULTS: the average time of progression was 8 years. In this group, 8.1 % of patients suffered protein-losing enteropathy that was more frequently seen in patients operated on by the intraatrial technique, aged over 6 years and in those suffering persistent pleural effusion in the immediate postoperative period. Significant statistical relation was found between enteropathy and postoperative ventricular dysfunction with RR= 11.45 (CI: 95 %: 2.37 to 55.16). The multivariate analysis showed that the ventricular dysfunction was a risk factor. CONCLUSIONS: Detection of the ventricular dysfunction in the progression of a patient with cavopulmonary shunt should guide the treatment to avoid occurrence of protein-losing enteropathy.

Estudo comparativo entre o uso do antiagregante plaquetário e do anticoagulante oral na profilaxia de trombose em pacientes submetidos à operação cavopulmonar total com tubo extracardíaco: análise ecocardiográfica, angiotomográfica, cintilográfica, laboratorial e clínica / Comparative trial of the use of antiplatelet and oral anticoagulant in thrombosis prophylaxis in patients undergoing total cavopulmonary operation with extracardiac conduit: echocardiographic, tomographic, scintigraphic, clinical and laboratory analysis

Objetivo: Comparar a eficácia do AAS e da Varfarina na profilaxia da trombose em pacientes submetidos a operação cavopulmonar total. Avaliar se fatores de coagulação (VII, VIII e Proteína C), dados clínicos, fenestração ou fatores hemodinâmicos, interferem na trombose no pós-operatório. Métodos: Estudo prospectivo e randomizado de 30 pacientes, randomizados em Grupo I (Varfarina) e Grupo II (AAS), submetidos à derivação cavopulmonar total com tubo extracardíaco, entre 2008 e 2011, com seguimento de dois anos. Foram realizadas consultas clínicas que avaliavam efeitos colaterais e aderência. Realizado ecocardiograma transesofágico no pós-operatório imediato, 3, 6,12 e 24 meses; angiotomografia aos 6, 12 e 24 meses de pós-operatório para avaliação de alterações na parede interna do tubo ou trombos e cintilografia pulmonar, para avaliar possível TEP. Resultados: Dois óbitos no grupo I; 33,3% dos pacientes apresentaram trombo (46,7% no Grupo II). A ocorrência prévia de trombo e baixos níveis de proteína C da coagulação foram os únicos fatores que influenciaram no tempo livre de trombo (P=0,035 e 0,047). Avaliação angiotomográfica: 35,7% dos pacientes do grupo II tinham atapeteamento maior que 2 mm (P=0,082). Cintilografia: dois pacientes apresentaram TEP no grupo II. Cinco pacientes tiveram dificuldade de aderência, 4 no grupo I com INR variando de 1 a 6,4. Conclusão: A ocorrência prévia de trombo é um fator de risco para trombose no pós-operatório. Pacientes em uso de AAS tendem a depósito de material na parede do tubo. O número reduzido da amostra não permitiu concluir qual a droga mais eficaz na prevenção da trombose na população estudada. .

Objective: To compare the efficacy of aspirin and warfarin for prophylaxis of thrombosis in patients undergoing total cavopulmonary anastomosis. Evaluate whether coagulation factors (VII, VIII and protein C), clinical data, fenestration or hemodynamic factors, interfere with postoperative thrombosis. Methods: A prospective, randomized study of 30 patients, randomized into Group I (Warfarin) and Group II (AAS), underwent total cavopulmonary shunt with extracardiac conduit, between 2008 and 2011, with follow-up by clinical visits to evaluate side effects and adhesion. Performed transesophageal echocardiography in post operatory time, 3, 6,12 and 24 months; angiotomography at 6, 12 and 24 months to evaluate changes in the internal tube wall or thrombi and pulmonary scintigraphy to evaluate possible PTE. Results: Two deaths in group I; 33.3% of patients had thrombus (46.7% in Group II). The previous occurrence of thrombus and low levels of coagulation protein C were the only factors that influenced the time free of thrombus (P=0.035 and 0.047). Angiotomographic evaluation: 35.7% in group II presented material accumulation greater than 2 mm (P=0.082). Scintigraphy: two patients had PTE in group II. Five patients had difficulty to comply with the treatment, 4 in group I with INR ranging from 1 to 6.4. Conclusion: The previous occurrence of thrombus is a risk factor for thrombosis in the postoperative period. Patients using AAS tend to deposit material in the tube wall. The small sample size did not allow to conclude which is the most effective drug in the prevention of thrombosis in this population. .

"Air embolism during fontan operation".

In patients with a right to left intracardiac shunt, air embolism results in an obligatory systemic embolization. Nonembolization of entrained air is described in a child with a single ventricle physiology who had earlier undergone bidirectional Glenn shunt construction and Damus–Kaye–Stansel anastomosis. The air entrainment was detected by intra-operative transesophageal echocardiography. The combined effect of a “diving bell” phenomenon and mild aortic valve regurgitation are suggested as the reasons for the confi nement of air into the ventricle preventing catastrophic systemic embolization.

Cirugía de Fontan: complicaciones posquirúrgicas: Medellín, Colombia / Fontan surgery: complications postsurgical: Medellin, Colombia

El manejo del corazón univentricular ha mejorado laexpectativa de vida de los pacientes con la cirugía deFontan.Objetivo: identificar las complicacionesposquirúrgicas más frecuentes de la cirugía de Fontanen pacientes de una institución privada de la ciudadde Medellín.Metodología: estudio descriptivo del tipo de seriesclínicas. La población estuvo compuesta porpacientes sometidos a la cirugía de Fontan. Lainformación sobre las variables sociodemográficas yclínicas fue tomada directamente de las historiasclínicas. La investigación contó con la aprobación delComité de ética. Se empleó el programa SPSS®versión 17.0 (SPSS Inc; Chicago, Illinois, EE.UU.) parael procesamiento y análisis de la información. Para elmanejo estadístico de los datos se empleó laestadística descriptiva.Resultados: participaron 21 pacientes, la mediana deedad fue de 5 años (mínimo 3 y máximo 10); el 66,7%fueron de sexo masculino. El 100% de los niños tenían antecedentes de cirugía paliativa previa, la másfrecuente fue el Glenn bidireccional. En cuanto a lascomplicaciones más frecuentes fueron derramepleural (52,4%), disfunción miocárdica e infección enherida (19%).Conclusiones: las complicaciones que sepresentaron coinciden con lo reportado en la literatura, cabe resaltar que ningún paciente refirió estenosissubaórtica ni hemorragias.

Univentricular heart management has improved thelife expectancy of patients with Fontan surgery.Objective: identify the most common postoperative complications of the Fontan surgery in patientsfrom a private institution from Medellin.Methodology: descriptive clinical series. Thepopulation was patients undergoing the Fontansurgery. Information about sociodemographic andclinical variables was taken directly from themedical records. The research was approved bythe Ethics Committee. We used SPSS ® version17.0 (SPSS Inc, Chicago, Illinois, USA) forprocessing and analysis of information. For statistical handling of data is used for descriptiv estatistics. Results: 21 patients participated, the median agewas 5 years (minimum 3, maximum 10), 66.7% were male. 100% of the children had a history of priorpalliative surgery, the most common was the bidirectional Glenn. As for the most common complications were pleural effusion (52.4%), myocardial dysfunction and wound infection (19%). Conclusions: the complications presented agree with those reported in the literature, it is notable that no patient reported sub aortic stenosis and bleeding.

Add-on treatment with nebulized hypertonic saline in a child with plastic bronchitis after the Glenn procedure / Tratamento adjuvante com nebulização de salina hipertônica em uma criança com bronquite plástica após a operação de Glenn

Plastic bronchitis (PB), although a rare cause of airway obstruction, has mortality rates up to 50% in children after Fontan-type cardiac surgery. We present the case of an 18-month-old female patient with PB following pneumonia. At 6 months of age, the patient underwent the Glenn procedure due to functionally univentricular heart. Fiberoptic bronchoscopy revealed complete blockage of the left bronchus by mucoid casts. Pharmacotherapy consisted of glucocorticosteroids, azithromycin, and enalapril maleate. The child also received nebulized 3% NaCl solution, which proved to be beneficial. In children submitted to Fontan-type procedures, physicians must be alert for PB, which can be triggered by respiratory tract infection.

A bronquite plástica (BP), embora uma causa rara de obstrução de vias aéreas, apresenta taxas de mortalidade de até 50% em crianças submetidas a cirurgia cardíaca do tipo Fontan. Apresentamos o caso de uma menina de 18 meses de idade com BP secundária a pneumonia. Aos 6 meses de idade, a paciente havia sido submetida à operação de Glenn devido a coração funcionalmente univentricular. A fibrobroncoscopia revelou obstrução completa do bronco esquerdo por moldes mucoides. A farmacoterapia consistiu em glicocorticosteroides, azitromicina e maleate de enalapril. Adicionalmente, a criança recebeu nebulização de solução de NaCl a 3%, que provou ser benéfica. Em crianças submetidas a operações do tipo Fontan, devemos nos manter alerta quanto à BP, que pode ser desencadeada por infecção do trato respiratório.

Implante de stents em conduto cirúrgico cavopulmonar: relato de dois casos / Stent implantation in surgical cavopulmonary conduit: report of two cases / Implante de stents en conducto quirúrgico cavopulmonar: dos casos clínicos

Apesar dos avanços na cirurgia de Fontan, obstruções nos condutos extracardíacos podem ocorrer e causar deterioração clínica. Relatamos dois casos em que foram realizados implante de stent para correção de estenose na cirurgia de Fontan. Ascite era o sinal clínico comum; um paciente tinha enteropatia perdedora de proteínas.Todos os procedimentos obtiveram sucesso angiográfico e clínico.

Despite advances in Fontan surgery, obstruction in extracardiac ducts may occur and cause clinical deterioration. We report two cases of stent implantation for correction of stenosis in the Fontan surgery. Ascites was a common clinical sign; a patient was protein losing enteropathy. All procedures were angiographically and clinically successful.

Pese a los avances en la cirugía de Fontan, obstrucciones en los conductos extracardíacos pueden producirse y ocasionar deterioro clínico. Relatamos dos casos en que se llevaron a cabo implante de stent para la corrección de estenosis en la cirugía de Fontan. Ascitis era o el signo clínico común; un paciente tenía enteropatía perdedora de proteínas. Todos los procedimientos obtuvieron éxito angiográfico y clínico.

Associação de bronquite plástica com enteropatia perdedora de proteínas após operação de Fontan / Association of plastic bronchitis to protein-losing enteropathy after fontan operation

Relatamos um caso incomum de associação de bronquite plástica (BP) com enteropatia perdedora de proteínas (EPP) em menina de 4 anos e 9 meses de idade. com dupla via de entrada de ventrículo único tipo esquerdo e concordância ventrículo-arterial submetida à cirurgia cavopulmonar total. com túnel lateral intracardíaco aos três anos. Tornaram-se chamativas a eliminação de molde brônquico de fibrina de 10 cm (BP) e a elevação de alfa-1-antitripsina de 52 mg/g de fezes. Em uso de sildenafila. programou-se. em caso de continuidade do processo. a ligadura do ducto torácico e transplante cardíaco.

We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.

Operação de Fontan: uma técnica em evolução / Fontan operation: a technique in evolution

Objetivo: Estudos recentes de fluxo com modelos experimentais de anastomoses cavopulmonares totais (ACPTs) baseados em ressonância magnética e angiografia demonstram que este é um procedimento bem estabelecido para o tratamento de várias cardiopatias, mas o melhor arranjo espacial continua controverso. Nosso intuito é apresentar os resultados imediatos com três diferentes técnicas de ACPTs. Métodos: Ensaio clínico de ACPTs realizadas no período de janeiro de 2005 a julho de 2008 com 40 pacientes, com idade média de 6,4 ± 3,2 anos, com Glenn prévio. Os pacientes foram divididos em três grupos, dependendo da técnica cirúrgica empregada: Grupo 1 (G1) - túnel lateral; Grupo 2 (G2) - conduto extracardíaco; Grupo 3 (G3) - conduto intracardíaco dirigido para o ramo esquerdo de artéria pulmonar, todos com fenestração. Foram avaliadas variáveis pré e pós-operatórias. Resultados: Foram incluídos 11 pacientes no G1, 10 no G2 e 19 no G3. As variáveis pré-operatórias foram semelhantes nos três grupos (P>0,05). A mortalidade foi maior nos Grupos 1 e 2 (9,1 por cento e 10,0 por cento, respectivamente), comparadas ao Grupo 3 (zero), porém sem significância estatística (P=0,3841). Efusão pleural foi ausente no Grupo 3, diferença significativa (P=0,0128) em relação aos outros grupos (40,0 por cento e 33,3 por cento). A mediana do tempo de hospitalização pós-operatória foi menor no Grupo 3 (8 dias), em relação aos grupos 1 e 2 (18 e 13 dias, respectivamente) (P=0,0164). Conclusão: A técnica de conduto intracardíaco foi associada a menor morbidade pós-operatória, sendo a opção atual do nosso serviço na anastomose cavopulmonar total.

Objectives: Recent experimental flow studies based on angiography and magnetic resonance have shown that total cavopulmonary anastomosis (TCPA) is a valid concept for surgical treatment of many congenital heart defects, but there is not agreement of the best surgical arrangement. Our purpose is to analyze the immediate results with three different techniques of TCPA. Methods: We sought to analyze retrospectively all TCPA realized from January 2005 to July 2008; there were 40 patients, all with previous Glenn anastomosis, with mean age of 6.4 ± 3.2 years. Three different techniques were employed: Group 1 (G1) lateral tunnel, Group 2 (G2) extracardiac conduits, Group 3 (G3) intracardiac conduit directed to the left pulmonary artery. All patients had a fenestration done. Results: G1 had 11 patients, G2 10 patients and G3 19 patients. Preoperative data were similar in the 3 groups (P>0.05). Surgical mortality was higher in Groups 1 and 2 (9.1 percent and 10 percent) compared to Group 3 (0 percent) but there was no statistical significance (P=0.3841). Pleural effusion was absent in Group 3 (0 percent), which was statistically significant in relation to the other groups (P=0.0128). The hospitalization time was also significantly lower in G3 (8 days) in relation to G1 (18 days) and G2 (13 days) (P=0.0164). Conclusion Intracardiac TCPA was associated with lower postoperative morbidity and is currently our preferred technique.

Cirugía de Fontan: Factores de riesgo a corto y mediano plazo / Fontan operation: factors influencing early and late outcomes

Se presenta un análisis retrospectivo de pacientes sometidos a cirugía de Fontan, de enero de 1989 a diciembre 2003, con el fin de evaluar los resultados, así como las variables clínicas y hemodinámicas que inciden en su morbilidad y mortalidad. Se estudiaron un total de 81 pacientes con los siguientes diagnósticos: 53 con atresia tricuspídea, 14 con atresia pulmonar con septum intacto, 11 con conexión atriouniventricular y 3 con anomalía de Ebstein. A 61 pacientes se les realizó Fontan con túnel intra-atrial y en 20 se utilizó un tubo extracardíaco, fenestrados en 53 pacientes. La mortalidad operatoria (< 30 días) fue 13% para atresia tricuspídea y 25% en el resto, siendo básicamente ésta por choque cardiogénico y arritmias graves. La sobrevida global fue de 71% en 55 meses de seguimiento. Hubo que desmantelar el Fontan en 5 casos. De las diversas variables analizadas se encontró 2.8 veces más riesgo de morir en pacientes sin fenestración y 3.6 veces cuando a las 72 hrs postquirúrgicas la presión de atrio izquierdo fue > 10 mmHg y la presión media de la arteria pulmonar > 20 mmHg. Las complicaciones más relevantes fueron: arritmias (38%), enteropatía perdedora de proteínas (8%) y eventos trombóticos cerebrales (1 %). El 52% de los pacientes han reingresado siendo las principales causas falla hemodinámica e infecciones.

The purpose of this retrospective study was to determine the outcome of patients who underwent a Fontan procedure at National Heart Institute "Ignacio Chavez", Mexico, from January 1989 to December 2003. We had 81 patients with a mean age of 7 years old: 53 with tricuspid atresia, 14 with pulmonary atresia and intact septum, 11 with univentricular atrioventricular connection and 3 with Ebstein's anomaly. An intra-atrial tunnel was performed on 61 patients and an extracardiac conduit on the rest. Of all, 53 underwent a fenestration. The surgical mortality was 13% fortricuspid atresia and 25% for the other diseases. We had an overall mortality of 28.4%, being the main causes cardiogenic shock and arrhythmia. The global survival was 71% in 55 months of following. Takedown was indicated on 5 patients. The patients without fenestration showed to have 2.8 times more risk of death. The mean pulmonary artery pressure > 20 mm Hg plus the left atrium pressure > 10 mmHg increased the death risk 3.6 times. Of the 52% who required readmission, the main causes were hemodynamical failure and infections. The more relevant complications were: arrhythmia (38%), protein-losing enteropathy (8%) and thrombotic cerebral events (1.4%).

Mapeo electroanatómico 3D y ablación percutánea transtorácica de taquicardia auricular en pacientes post cirugía de Fontan / Electro anatomic 3-D mapping and percutaneous transthoracic ablation of atrial tachycardia after Fontan surgery

Introducción: La incidencia de taquicardias auriculares (TA) luego de la operación de Fontan es alta. El acceso a la aurícula pulmonar (AuP) requiere de una punción transeptal o acceso aórtico retrógrado. Se presentan resultados iniciales con una técnica percutánea que permite el acceso a la AuP para mapeo y ablación por radiofrecuencia (ARF). Métodos: Seis EEF con mapeo 3D electroanatómico (CARTO) y ARF se efectuaron en 5 pacientes (1.2 a 17 años). Todos tenían un Fontan (túnel lateral) y TA. Vía transtoráxica se posicionó en la AuP un catéter Navistar para mapeo, estimulación y ARF. Se indujo TA con estimulación programada e isoproterenol. La secuencia de activación, áreas de bloqueo eléctrico y anatómico, y la ubicación del electrograma de His fueron definidas con mapeo 3D electroanatómico. Las zonas de interés fueron estudiadas con técnicas de “entrainment”. Luego de la ARF, el éxito se definió como la imposibilidad de reinducir TA. Resultados: Se encontró taquicardia por reentrada auricular en 5 casos y taquicardia ectópica en uno. La ARF fue exitosa en todos ellos. Hubo 1 pneumotórax y hemotórax en dos casos. En un paciente se observó recurrencia de la TA a los 3 meses, que requirió una segunda ARF, sin nueva recidiva. Ningún paciente esta recibiendo tratamiento antiarrítmico. Resumen: El acceso transtorácico percutáneo a la AuP parece apropiado para mapear y tratar las taquicardias auriculares en pacientes con cirugía de Fontan.

Balloon-expandable stenting, percutaneous coil embolization, and amplatzer septal occluder; treatments after complicated lateral tunnel Fontan: a case report.

The authors report a 7-year-old girl with univentricular heart physiology who developed prolonged pleural effusion due to discrete narrowing of the proximal right pulmonary artery, and progressive cyanosis which resulted from leakage of the atrial baffle, multiple veno-venous collaterals after the lateral tunnel Fontan operation. Percutaneous balloon-expandable stent implantation was used to correct the right pulmonary artery stenosis with an excellent result. Cyanosis was improved by coil embolization of the collaterals and occlusion of the baffle leakage with Amplatzer septal occluder. This is the first successful report in Thailand.