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.

Cardiovascular Risk Factors in Children and Adolescents with Fontan Circulation

Abstract Background: Long-term outcomes of patients with Fontan circulation are uncertain regarding the prevalence and role of risk factors (RFs) such as increased body mass index (BMI), arterial hypertension, and hypercholesterolemia. Objectives: To describe the prevalence of RFs in patients with univentricular heart, with variable follow-up times. Methods: This mixed cohort study was performed with 66 patients, who underwent blood count, fasting blood glucose, C-reactive protein (CRP), and lipid profile tests; systolic/diastolic blood pressure (SBP/DBP) measurements; and anthropometric and sociodemographic data collection. Cardiovascular RFs among first-degree relatives and physical activity habits were also assessed. Prevalence was described using proportions, with a 95% confidence interval. Continuous variables (height, weight, age, SBP, DBP) were described as means and standard deviations (m±SD). Associations between RFs were assessed using chi-squared or Fisher's exact tests. Spearman's correlation was used for analyzing CRP and the presence of 2 or more RFs. The Shapiro-Wilk test was used to check for data normality. Statistical significance considered p<0.05. Results: In our population, 19.7% were overweight, mean SBP was 89.44±37.4, and mean DBP was 60.0±26.08. The most prevalent diseases in the interviewees' families were systemic arterial hypertension (30.3%), obesity (16.7%), and 2 or more cardiovascular RFs among first-degree relatives (13.8%). We observed a trend towards significance between the presence of 2 familial RFs and overweight, as well as a risk profile for cardiovascular disease. There was an association between the BMI percentile, the presence of 2 or more RFs (p<0.05), and CRP (p<0.01). Conclusions: Overweight is common in patients with univentricular heart, being related to more than 2 cardiovascular RFs among first-degree relatives; physical inactivity and changes in lipid profiles are also frequent.

Clinical efficacy and safety of Treprostinil in the treatment of children with early decompensation after the Fontan procedure / 中华实用儿科临床杂志

Objective:To investigate the efficacy and safety of Treprostinil in the treatment of children with early decompensation after the Fontan procedure.Methods:A retrospectively analysis was performed on the clinical data of 16 children with early decompensation after the Fontan procedure treated with Treprostinil injection from December 2017 to June 2020 at Fuwai Central China Cardiovascular Hospital.A total of 16 patients were included, including 5 boys (31.2%) and 11 girls (68.8%). The age was (4.6 ±1.2) years, the weight was (16.0±2.1) kg.The changes of central venous pressure (CVP), heart rate (HR), systolic blood pressure (SBP), central venous oxygen saturation (ScvO 2), lactic acid (Lac), oxygenation index and B-type natriuretic peptide (BNP) were recorded at the infusion of Treprostinil and 3 hours, 24 hours, 48 hours and 72 hours after the infusion.The short-term efficacy of Treprostinil was observed[mortality, mechanical ventilation time, and length of intensive care unit (ICU) stay]; paired t-test was used to analyze the above indexes at different time points.The adverse reactions during the administration were also recorded. Results:Of the 16 children, the median mechanical ventilation time was 9 (5, 22) h, and the median ICU stay time was 2 (1, 12)days.After 72 hours of drug administration, CVP, Lac, BNP and HR decreased: CVP decreased from(16±5) mmHg (1 mmHg=0.133 kPa) to (11±2) mmHg ( P<0.001), Lac decreased from(6.8±3.2) mmol/L to (3.2±1.2) mmol/L ( P=0.002), BNP decreased from(980±223) ng/L to (250±120) ng/L( P<0.001), HR decreased from(150±20) times/min to (125±16) times/min( P=0.002); SBP, ScvO 2 and oxygenation index increased: SBP increased from(83±10) mmHg to (98±12) mmHg( P<0.001), ScvO 2 increased from 0.53±0.13 to 0.65±0.11 ( P=0.003), oxygenation index increased from (200±72) mmHg to (298±13) mmHg ( P<0.001), and the differences were statistically significant(all P< 0.05). One case died (6.3%), 2 cases (12.5%) had transient blood pressure drop and 1 case (6.3%) had nausea and vomiting.Besides, no other treatment-related complications were observed. Conclusions:As for children with early decompensation after the Fontan procedure, the intravenous application of Treprostinil can reduce pulmonary artery pressure rapidly, effectively improve circulatory status and oxygenation and ultimately improve the prognosis.

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.

Hepatopatía crónica asociada a Fontan: a propósito de dos casos y revisión de la literatura / Fontan-associated liver disease: report of two cases and literature review

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.

Hemodynamic Study on Non-Newtonian Properties of Fontan Procedure / 医用生物力学

Objective To investigate the effects of non-Newtonian properties on hemodynamic characteristics of Fontan procedure in computational simulation. Methods A Fontan vascular model was reconstructed based on patient-specific medical images. The boundary conditions were set according to velocity profiles measured by real-time echocardiography. The Newtonian model and two non-Newtonian (Casson and Carreau) models were applied to analyze the hemodynamic parameters, including flow ratio (FR), energy loss (EL), wall shear stress (WSS) as well as non-Newtonian importance factor, so as to compare flow differences between the Newtonian model and non-Newtonian models. Results The rheological models had small effects on the FR. The EL of non-Newtonian models was higher than that of the Newtonian model, and the EL of Casson model was the highest. Flow recirculation and flow disturbance accompanied with low WSS were observed in inferior vena cava (IVC). At low inflow velocity, flow disturbance in IVC of Newtonian model was more obvious. The calculations of non-Newtonian importance factor suggested significant non-Newtonian viscosity in IVC. Conclusions Non-Newtonian viscosity is significant in IVC where low velocity and flow recirculation are observed. Non-Newtonian properties of the blood should be considered in patient-specific hemodynamic simulation of Fontan procedure.

Evaluación de la capacidad funcional en escolares con fisiología de corazón univentricular / Assessment of functional capacity among students with univentricular heart

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

A simple predictive factor for mortality in fontan surgery: serum hypo-osmolality

Abstract Objective: Close follow-up is important after the Fontan procedure, which is a palliative surgical method for a single ventricle. In this period, serum osmolality is an important parameter with the advantages of easy to obtain and poor outcome prediction. Methods: Patients who had undergone Fontan operation between May 2011 and February 2017 were retrospectively evaluated. Patients were divided into three groups based on their serum osmolality values: hypoosmolar (Group 1), isosmolar (Group 2), and hyperosmolar (Group 3). Demographics, clinical information and postoperative data of the groups were compared. Results: Forty-three patients had undergone extracardiac Fontan operation in the study period. There were 8, 19 and 16 patients in Groups 1, 2 and 3, respectively. Among the three groups, postoperative intubation and length of hospital stay, prolonged pleural effusion, need for inotropic support and mortality were statistically significantly higher in Group 1. Conclusion: After the Fontan procedure, one of the determinants of cardiac output might be affected by serum osmolality. Decreased serum osmolality might be associated with poor prognosis after Fontan procedure. Serum osmolality monitoring may be beneficial to improve postoperative outcomes in these patients.

Anesthetic management for non-cardiac surgery in a patient with Fontan palliation: case report / Manejo anestésico para cirugía no cardiaca en paciente con paliación de Fontán: Reporte de caso

Abstract Survival of patients with Fontan palliation has improved significantly over the years and it constitutes a condition frequently found nowadays in the perioperative setting for non-cardiac surgery. A history of this disease condition implies complex physiologic and multiorgan considerations for the anesthetist who may need to resort to special measures in order to maintain homeostasis and avoid complications. In this paper we present the case of a patient with a history of Fontan, and describe successful anesthetic management during laparoscopic surgery.

Resumen En los últimos años la sobrevida de pacientes con paliación de Fontán ha aumentado significativamente, y hoy es una condición frecuente en el escenario perioperatorio para cirugía no cardiaca. Este antecedente supone complejas consideraciones fisiológicas y multiorgánicas para el anestesiólogo, quien puede requerir medidas especiales para mantener la homeostasia y evitar complicaciones. En este artículo presentamos el caso de un paciente con antecedente de Fontán y describimos el manejo anestésico exitoso en cirugía laparoscópica.

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

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.

Characterization of Flow Efficiency, Pulsatility, and Respiratory Variability in Different Types of Fontan Circuits Using Quantitative Parameters

PURPOSE: Details on the hemodynamic differences among Fontan operations remain unclear according to respiratory and cardiac cycles. This study was undertaken to investigate hemodynamic characteristics in different types of Fontan circulation by quantification of blood flow with the combined influence of cardiac and respiratory cycles. MATERIALS AND METHODS: Thirty-five patients [10 atriopulmonary connections (APC), 13 lateral tunnels (LT), and 12 extracardiac conduits (ECC)] were evaluated, and parameters were measured in the superior vena cava, inferior vena cava (IVC), hepatic vein (HV), baffles, conduits, and left and right pulmonary artery. Pulsatility index (PIx), respiratory variability index (RVI), net antegrade flow integral (NAFI), and inspiratory/expiratory blood flow (IQ/EQ) were measured by intravascular Doppler echocardiography. RESULTS: The PIx between APC and total cavopulmonary connection (TCPC; LT and ECC) showed significant differences at all interrogation points regardless of respiratory cycles. The PIxs of HVs and IVCs in APC significantly increased, compared with that in LT and ECC, and the RVI between APC and TCPC showed significant differences at all interrogation points (p < 0.05). The NAFI and IQ/EQ between APC and TCPC showed significant differences at some interrogation points (p < 0.05). CONCLUSION: Patients with different types of Fontan circulation show different hemodynamic characteristics in various areas of the Fontan tract, which may lead to different risks causing long-term complications. We believe the novel parameters developed in this study may be used to determine flow characteristics and may serve as a clinical basis of management in patients after Fontan operations.

Numerical simulation of a self-powered Fontan based on venturi effect / 中国胸心血管外科临床杂志

@#Objective    To investigate the effects of a self-powered conduit in different patients’ models who underwent extracardiac Fontan procedure. Methods    Four children who underwent extracardiac Fontan procedure in Shanghai Children's Medical Center from 2011 to 2017 year were selected. Venae cavae and pulmonary arteries were reconstructed using Mimics 19.0®. In silico, a venturi conduit was introduced to the anastomosis of venae cavae and pulmonary artery. Then computational fluid dynamics simulation was performed using patients’ clinical data. Results    When inferior venae cavae were directly to or to the left of superior venae cavae, the venturi conduit could assist the return of venous blood and reduce the pressures of venae cavae about 0.5 mm Hg. And the pressure differences between venae cavae and pulmonary arteries were about –0.7 mm Hg, which suggested that the conduit could generate right ventricle-like effect. Conclusion    The venturi conduit can reduce the pressure of venae cavae, increase pulmonary circulation flow and improve Fontan hemodynamics.

Long term evaluaction of cardiopulmonary function in 20 years after Fontan surgery / 中华胸心血管外科杂志

To evaluate the long term cardiopulmonary function after Fontan operation. Methods 10 patients who received Fontan operation were followed for(26 ±4) years in our hospital. 7 males and 3 females,(30. 7 ± 13. 2) years old(20 -60 years). All patients were diagnosed by echocardiography, CTA, 24 h ECG, cardiac catheterization, pulmonary angiography, radionuclide pulmonary blood perfusion and cardiopulmonary exercise test. Results Echocardiography shows: LVEF(0. 49 ± 0. 04), more than moderate atrioventricular valve regurgitation in 7 cases. CTA shows: there was no anastomotic stenosis, arteriovenous fistula in 4 cases. 24 h ECG shows: sinus rhythm in 7 cases, frequent paroxysmal atrial tachycardia in 1 case, atrial flutter in one and atrial fibrillation in one. Cardiac catheterization and pulmonary angiography show: the average pulmonary artery pressure(17. 8 ±2. 0)mmHg, average pulmonary resistance(4. 17 ±0. 56) woods. Radionuclide pulmonary blood perfusion shows: pulmonary blood of upper lung was reduced significantly with unbalanced distribution. Cardiopulmonary exercise test shows: both of lung ventilation function and heart reserve function decreased moderately or severely. Protein losing enteropathy was found in 1 case. Conclusion The decrease of cardiopulmonary function is found after Fontan operation during the long-term follow up for 20 years, especially for cardiac function. More attention should be paid to the early postoperative atrioventricular valve regurgitation. Unbalanced distribution of pulmonary blood is Found in long term. The incidence of arrhythmia after extra conduit Fontan operation is lower than other methods.

Late follow-up of patients submitted to total cavopulmonary derivation: clinical aspects, reinterventions, and complications interfering in morbidity

Abstract Objective: To identify main complications in outpatient follow-up, as well as factors before or during operation that may interfere in patient's evolution. Methods: Retrospective study of patients submitted to total cavopulmonary shunt with extracardiac conduit from 2000 to 2014 at the Hospital do Coração (São Paulo, Brazil) and who underwent clinical follow-up at this institution. Results: One hundred and fifty surgeries were performed and 59 patients maintained outpatient follow-up. The mean age of these patients at the time of surgery was 4.45 years (median of 45 months) and 70.2% of them were males. Among the patients undergoing outpatient follow-up, postoperative time at evaluation ranged from 10 days to 145 months; 30 (50.8%) patients had single left ventricle and 29 (49.2%) had single right ventricle (48.2% of these presented with hypoplastic left heart syndrome [HLHS]). Patients with single left ventricle had a higher percentage of reintervention-free survival, but without statistically significant difference. 40% of the patients had no complications and 35% of them presented with thrombosis at some point in the follow-up period, with ventricular dysfunction being the second most frequently found complication (15% of cases), mainly among patients with single right ventricle morphology (P=0.04). Between the patients currently under follow-up, 20 (35%) of them had been evaluated by ultrasonography and had some degree of hepatic congestion and/or hepatomegaly. 16.7% of the patients with such alteration had HLHS (P=0.057). Conclusion: Except for the right ventricular morphology, no other factor has been shown to interfere in late evolution after total cavopulmonary shunt.

Assessment of the effect of two regimens of milrinone infusion in pediatric patients undergoing fontan procedure: A randomized study

Objective: The aim of the study was to compare the effect of two different regimens of milrinone on hemodynamics and oxygen saturation in pediatric patients undergoing Fontan procedure. Design: This was a randomized study. Setting: Cardiac centers. Patients: This study included 116 patients undergoing Fontan procedure. Material and Methods: Group E: Milrinone was started as infusion 0.5 μg/kg/min without a loading dose at the beginning of cardiopulmonary bypass (CPB) followed by infusion 0.5–0.75 μg/kg/min in the pediatric cardiac surgical intensive care unit (PSICU). Group L: Milrinone was started as a loading dose 50 μg/kg over 10 min before weaning from CPB followed by infusion 0.5–0.75 μg/kg/min in the PSICU. Measurements: Heart rate, mean arterial blood pressure, central venous pressure, transpulmonary pressure, cardiac index, pharmacological support, lactate level, urine output, oxygen saturation, ICU, and hospital length of stay. Main Results: There were no changes in the heart rate and mean arterial blood pressure (P > 0.05). The increase in the postoperative central venous pressure, transpulmonary pressure and lactate level was lower in Group E than Group L (P < 0.05). The increase in the postoperative cardiac index, oxygen saturation, and urine output was higher in Group E than Group L (P < 0.05). The requirement for pharmacological support was lower in the Group E (P < 0.05). The ICU and hospital length of stay were shorter in the Group E than Group L (P < 0.05). Conclusion: Early use of milrinone during Fontan procedure facilitated the weaning from CPB, decreased the elevation in the central venous pressure, transpulmonary gradient pressure, and the requirement for pharmacological support. Furthermore, it increased the cardiac index and arterial oxygen saturation.

Heart Transplantation in Patients with Superior Vena Cava to Pulmonary Artery Anastomosis: A Single-Institution Experience

BACKGROUND: Heart transplantation (HTx) can be a life-saving procedure for patients in whom single ventricle palliation or one-and-a-half (1½) ventricle repair has failed. However, the presence of a previous bidirectional cavopulmonary shunt (BCS) necessitates extensive pulmonary artery angioplasty, which may lead to worse outcomes. We sought to assess the post-HTx outcomes in patients with a previous BCS, and to assess the technical feasibility of leaving the BCS in place during HTx. METHODS: From 1992 to 2017, 11 HTx were performed in patients failing from Fontan (n=7), BCS (n=3), or 1½ ventricle (n=1) physiology at Asan Medical Center. The median age at HTx was 12.0 years (range, 3–24 years). Three patients (27.3%) underwent HTx without taking down the previous BCS. RESULTS: No early mortality was observed. One patient died of acute rejection 3.5 years after HTx. The overall survival rate was 91% at 2 years. In the 3 patients without BCS take-down, the median anastomosis time was 65 minutes (range, 54–68 minutes), which was shorter than in the patients with BCS take-down (93 minutes; range, 62–128 minutes), while the postoperative central venous pressure (CVP) was comparable to the preoperative CVP. CONCLUSION: Transplantation can be successfully performed in patients with end-stage congenital heart disease after single ventricle palliation or 1½ ventricle repair. Leaving the BCS in place during HTx may simplify the operative procedure without causing significant adverse outcomes.

Management of Coronary Sinus Ostial Atresia during a Staged Operation of a Functional Single Ventricle

Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.

Extracardiac Conduit Fontan Operation with Reduction Aortoplasty for Left Pulmonary Artery Compression after a Norwood Procedure in a Patient with Double-Inlet Left Ventricle / 대한흉부외과학회지

Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.