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Background: The benefit of additional pulmonary blood flow (APBF) in Fontan pathway remains controversial. The aim of the study was to analyze the systemic arterial saturation, ventricular and atrioventricular (AV) valve function, development of pulmonary arteries and postoperative course after Fontan operation in children with preserved or eliminated APBF between stages 2 and 3 of the Fontan pathway. Methods: A group of 180 consecutive children (median age: 2.92(1.83–16.83) years) with single ventricle underwent extracardiac conduit Fontan operation. The patients were retrospectively analyzed with respect to the presence of APBF between stages 2 and 3. Results: Preoperative echocardiography showed no differences in ventricular function (good – 105[95%] vs. 65[98.5%]; reduced – 9[5%] vs 1[1.5%]; p = 0.07) or AV valve function (p = 0.78). There was no difference in oxygen saturation at admission (p = 0.48), in preoperative blood gas analysis (p = 0.62), or at the discharge (p = 0.73). There was no difference in size of the left pulmonary artery (9.5(0–16.2) vs 10.6 (4.9–21.7) mm; p = 0.13), however the right pulmonary artery was significantly larger (11.2(6.5–19.6) vs. 12.8(7.8–2.5) mm; p = 0.048) in APBF group. In this group the mean pressure in the pulmonary artery was significantly higher as well (8[4–17.5] vs 9.5[4–17.3] mmHg; p = 0.03). Conclusions: APBF does not result in higher oxygen saturation, both before and after Fontan completion. APBF can stimulate the growth of the pulmonary arteries and increase the pulmonary artery pressure. The presence of APBF does not affect the post-operative course after Fontan operation, however it can increase the need of AV valve reconstruction. [ABSTRACT FROM AUTHOR]

Abstract Background The benefit of additional pulmonary blood flow (APBF) in Fontan pathway remains controversial. The aim of the study was to analyze the systemic arterial saturation, ventricular and atrioventricular (AV) valve function, development of pulmonary arteries and postoperative course after Fontan operation in children with preserved or eliminated APBF between stages 2 and 3 of the Fontan pathway. Methods A group of 180 consecutive children (median age: 2.92(1.83–16.83) years) with single ventricle underwent extracardiac conduit Fontan operation. The patients were retrospectively analyzed with respect to the presence of APBF between stages 2 and 3. Results Preoperative echocardiography showed no differences in ventricular function (good – 105[95%] vs. 65[98.5%]; reduced – 9[5%] vs 1[1.5%]; p = 0.07) or AV valve function (p = 0.78). There was no difference in oxygen saturation at admission (p = 0.48), in preoperative blood gas analysis (p = 0.62), or at the discharge (p = 0.73). There was no difference in size of the left pulmonary artery (9.5(0–16.2) vs 10.6 (4.9–21.7) mm; p = 0.13), however the right pulmonary artery was significantly larger (11.2(6.5–19.6) vs. 12.8(7.8–2.5) mm; p = 0.048) in APBF group. In this group the mean pressure in the pulmonary artery was significantly higher as well (8[4–17.5] vs 9.5[4–17.3] mmHg; p = 0.03). Conclusions APBF does not result in higher oxygen saturation, both before and after Fontan completion. APBF can stimulate the growth of the pulmonary arteries and increase the pulmonary artery pressure. The presence of APBF does not affect the post-operative course after Fontan operation, however it can increase the need of AV valve reconstruction.

The single ventricle is a group of congenital heart defects, which even today remains the most difficult for surgical treatment and further patient care. The survival rate of patients with this defect remains relatively low despite a number of staged surgical interventions. A large percentage of mortality is observed in the interstage period due to the development of heart failure, the treatment of which is an urgent problem because standard therapy does not meet expectations. This article reviews the world literature on the application of an innovative method of heart failure correction – stem cell therapy. The main types of stem cells, their sources, mechanism of action, methods of their delivery to myocardium are also considered.

Elevated central venous pressure (CVP) and decreased arterial oxygen saturation (SaO 2) are the characteristics of patients after Fontan operations and determine morbidity and mortality in the long-term. Oxygen inhalation therapy theoretically increases SaO 2 and may decrease the elevated CVP in these patients. However, there is no previous study to support this hypothesis. This study aimed to determine the acute effects of oxygen inhalation on the hemodynamics of adult patients late after Fontan operations using cardiac catheterization. This study enrolled 58 consecutive adult patients (median age, 30 years; female, n = 24) who had undergone Fontan operations. We assessed the hemodynamic changes during oxygen inhalation (2 L/min) with a nasal cannula in cardiac catheterization. We divided the studied patients into two groups according to the reduction in CVP during oxygen inhalation using the median value: responders (>2 mmHg) and non-responders (≤2 mmHg). Clinical characteristics of the responders to oxygen inhalation were investigated with uni- and multivariate analyses. SaO 2 increased from 93.3 % (91.3–94.5 %) to 97.5 % (95.2–98.4 %) (p < 0.001) and CVP decreased from 12 mmHg (11–14 mmHg) to 10 mmHg (9–12 mmHg) (p < 0.001) after oxygen inhalation. There was a weak but significant correlation between the increase in SaO 2 and the decrease in CVP (R = 0.29, p = 0.025). Pulmonary blood flow increased from 4.1 L/min (3.5–5.0 L/min) to 4.4 L/min (3.7–5.3 L/min) (p = 0.007), while systemic blood flow showed no significant changes. A multivariate analysis revealed that high baseline CVP was associated with a larger decrease in CVP (>2 mmHg) after oxygen inhalation. Oxygen inhalation increased SaO 2 and decreased CVP, especially in patients with high baseline CVP. Further studies with home oxygen therapy are needed to investigate the long-term effects of oxygen inhalation in adult patients who underwent Fontan operations. This study enrolled 58 consecutive adult patients (median age, 30 years; female, n = 24) who had undergone Fontan operations. We assessed the hemodynamic changes after oxygen inhalation with a nasal cannula using cardiac catheterization. After oxygen inhalation, SaO2 increased from 93.3 % (91.3–94.5 %) to 97.5 % (95.2–98.4 %) (p < 0.001), and CVP decreased from 12 mmHg (11–14 mmHg) to 10 (9–12 mmHg) mmHg (p < 0.001). CVP, central venous pressure; SaO2, arterial oxygen saturation. [Display omitted] • We investigated the acute hemodynamic effect of oxygen inhalation in Fontan patients. • Arterial oxygen saturation increased and central venous pressure decreased after oxygen inhalation. • The decrease in central venous pressure was correlated with the increase in arterial oxygen saturation. • High baseline central venous pressure was a predictor of the responder to oxygen inhalation. [ABSTRACT FROM AUTHOR]

OBJECTIVES The evaluation of Fontan-associated liver disease is often challenging. Diffusion-weighted magnetic resonance imaging can detect hepatic fibrosis from capillary perfusion and diffusion abnormalities from extracellular matrix accumulation. This study investigated its role in the evaluation of liver disease in Fontan patients and explored possible diagnostic methods for early detection of advanced liver fibrosis. METHODS Stable adult Fontan patients who could safely be examined with magnetic resonance imaging were enrolled, and blood biomarkers, transient elastography were also examined. RESULTS Forty-six patients received diffusion-weighted imaging; and 58.7% were diagnosed with advanced liver fibrosis (severe liver fibrosis, 37.0%, and cirrhosis 21.7%). Two parameters of hepatic dysfunction, platelet counts (Spearman's ρ: –0.456, P  = 0.001) and cholesterol levels (Spearman's ρ: –0.383, P  = 0.009), decreased with increasing severity of fibrosis. Using transient elastography, a cut-off value of 14.2 kPa predicted the presence of advanced liver fibrosis, but with a low positive predictive value. When we included platelet count, cholesterol, post-Fontan years and transient elastography values as a composite, the capability of predicting advanced liver fibrosis was the most satisfactory (C statistic 0.817 ± 0.071, P  < 0.001). A cut-off value of 5.0 revealed a sensitivity of 78% and a specificity of 82%. CONCLUSIONS In Fontan patients, diffusion-weighted imaging was helpful in detecting liver fibrosis that was correlated with hepatic dysfunction. A simple score was proposed for long-term surveillance and early detection of advanced liver disease in adult Fontan patients. For adult Fontan patients with a calculated score > 5.0, we may consider timely diffusion-weight imaging and early management for liver complications. [ABSTRACT FROM AUTHOR]

The fenestration in Fontan operation consists in creation of a communication between venous circulation and the pulmonary venous atrium, allowing direct passage of blood from the venous to the systemic circulation (right-to-left shunt). There is no general agreement concerning the mean pulmonary artery pressure (mPAP) level above which fenestration is indicated. There are no strict guidelines for the indications for fenestration closure as well. Here we present our data about invasive hemodynamic assessment before and after temporary closure of the fenestration in patients after Fontan operation. Our recommendations regarding comprehensive evaluation of patients with fenestrations after Fontan surgery are also presented.

Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation. Demographics, anatomic characteristics, and early and late outcomes, including the length of stay, mortality, and surgical or catheter reinterventions, were analyzed. Results: Among 250 patients, 150 (60%) underwent biventricular repair. In-hospital mortality was 7.6% (n = 19). Median follow-up was 5.2 (range, 0-16) years. Among survivors to discharge, mortality was 19% (n = 44) and reintervention was 52% (n = 120). Patients with moderate/severe atrioventricular valve regurgitation were older (32 vs 16 months, P = .02), were more likely to experience adverse events during their index surgical admission (72% vs 46%, P

This article summarizes a case-control study on hepatocellular carcinoma (HCC) in adult patients who have undergone the Fontan operation for congenital heart disease. The study found that liver injury is common in these patients and can lead to fibrosis and cirrhosis. Risk factors for HCC in Fontan patients are not fully understood, but the study identified associations between HCC and factors such as prior Fontan revision, valve surgery, and certain liver function markers. The estimated prevalence of HCC in adult Fontan patients was 1.8%, and the one-year survival rate after HCC diagnosis was 81%. The study recommends regular screenings for HCC in adult Fontan patients. [Extracted from the article]

Introduction: Hepatic dysfunction is a well-known complication and is frequently referred to as Fontan-associated liver disease. The aim of the study is to assess liver dysfunction in children and adolescents with Fontan circulation. Material and methods: The study was an observational analytical study conducted on 30 patients who underwent Fontan operation more than 2 years before inclusion in the study and who were followed up at the Paediatric Cardiology Unit, Cairo University Children's Hospital. All patients were subjected to echocardiography, laboratory investigations, abdominal ultrasound, and liver elastography. Results: The median patient age was 13 years (inter-quartile range [IQR] 10-16.32), and the median follow-up duration after the Fontan operation was 5 years (IQR 3.83-9). Global longitudinal strain was lower in 26 (86.7%), and the single ventricle ejection fraction was reduced in 23 (76.7%); however, no correlation was found with time interval since Fontan. None of the patients had ascites clinically or by ultrasound examination. Gamma-glutamyl transferase was elevated in 28 (93.33%), which was significantly higher in the systemic right ventricle group. Abdominal ultrasound revealed periportal enhancement in 6 (20%), which was significantly higher in the hepatitis C virus exposed group (p = 0.006). Superior mesenteric artery resistance index was decreased in 27 (90%), which was significantly lower in the systemic left ventricle group (p = 0.01). The liver stiffness values did not correlate with time interval since Fontan (p = 0.09). Conclusions: Liver disease is prevalent in Fontan patients. Non-invasive measures such as laboratory tests, ultrasound, and elastography should be implemented with consideration for hepatology consultation. Fontan-associated liver disease screening is important in the monitoring of Fontan patients. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Heart transplantation for adult congenital heart disease is complicated and associated with challenging pretransplant support, long waiting and high early post-transplant mortality. We explored if surgical and medical advances and allocation system changes have affected outcomes. METHODS From United Network for Organ Sharing database, adults with congenital heart disease listed for heart transplantation were queried. To explore practice and outcome trends, patients were divided into 4 eras (eras 1–3: nearly 3 equal periods from 1992 to 2018, era 4: after 2018, corresponding with new allocation system). Univariate and multivariable analysis was performed to evaluate outcomes. RESULTS A total of 2737 patients were listed. There was gradual increase in listed and transplanted patients, along with significant increase in use of mechanical support, simultaneous kidney and liver transplantation. While proportion of transplanted remained constant, there was decrease in proportion delisted/died after listing (P  = 0.01) and waiting list duration (P  = 0.01), especially in era 4. Thirty-day post-transplant mortality remains high; however, it has significantly improved starting era 3 (P  = 0.01). Current survival at 1-year and 5-years is 85% and 65%, with improvement mainly related to decreased early death. On multivariable analysis, factors associated with survival were lower glomerular filtration rate (hazard ratio = 0.99, P  = 0.042), bilirubin (hazard ratio = 1.17, P <0.001) and mechanical ventilation (hazard ratio = 2.3, P =0.004) CONCLUSIONS Heart transplantation in adults with congenital heart disease is increasing, along with added complexity, higher usage of pretransplant mechanical support and simultaneous organ transplantation. Despite that, more complex patients do not experience worse outcomes. Early mortality improved but remains high. New donor allocation system allowed shorter waiting time and higher proportion transplanted without altering early mortality. [ABSTRACT FROM AUTHOR]

The gold standard for determining the severity of liver disease in Fontan patients is now liver biopsy. Since it is an invasive procedure, this study determined the possibility of applying mitochondrial function from isolated peripheral blood mononuclear cells (PBMCs) as a non‐invasive indicator of liver fibrosis. Fontan patients (n = 37) without known liver disease were analysed cross‐sectionally. Patients were classified according to their histology using the METAVIR score as follows; F0/F1—no/mild fibrosis; F2—moderate fibrosis; and F3/F4—cirrhosis. Peripheral blood mononuclear cells were assessed for mitochondrial activity and apoptosis. This study did not find any significant differences in cardiac function among the groups according to liver histology. Interestingly, our findings indicated a significant decrease in maximal respiration and spare respiratory capacity, in both the moderate (F2) and cirrhosis (F3/F4) groups compared with the group without significant fibrosis (F0/F1). Moreover, the cirrhosis group exhibited higher levels of apoptosis and lower levels of live cells, compared with both the moderate and no significant fibrosis groups. In conclusion, the degree of liver fibrosis in Fontan patients is strongly correlated with mitochondrial dysfunction in PBMCs. Mitochondrial function and apoptosis could potentially serve as novel markers for tracking the progression of liver fibrosis in these patients. [ABSTRACT FROM AUTHOR]

Sinus node dysfunction with concomitant junctional rhythm (JR) is frequently observed among Fontan patients and has been recognized as a contributor to heart failure. The impact and management of JR is unclear. A survey was mailed to all members of the Pediatric and Congenital Electrophysiology society (PACES) and members were asked to forward the questionnaire to their non-electrophysiology colleagues. Responses were received from 154 physicians (88 electrophysiologists (EP's) and 66 non-EP's (46 pediatric cardiologists and 20 adult congenital cardiologists). There were few differences in the response between EP's and non-EP's. Overall, 57% recommended an annual ambulatory ECG (AECG). A significant majority (80%) opted to continue to follow patients with significant periods of JR on AECG as long as the patients were asymptomatic, and showed no echocardiographic signs of cardiac decompensation. However, 84% would place a pacemaker in a patient with JR who was having open chest surgery for other reasons. Finally, pacemaker placement would be performed by 91% if a patient with JR showed signs of heart failure. Most congenital cardiologists would not recommend pacemaker placement in asymptomatic Fontan patients with JR. Further studies are needed on the Fontan population to determine the impact of SND and JR on longer term outcomes and to determine the role and optimal timing of pacemaker placement in these patients. [ABSTRACT FROM AUTHOR]

The OBJECTIVE was to study the prevalence and nature of congenital and acquired anatomical features of the pulmonary vascular bed in patients with single ventricle and to evaluate their impact on staged hemodynamic correction.METHODS AND MATERIALS. The study was a retrospective cohort study based on the study of the experience of surgical treatment of patients with a single ventricle from 2005 to 2022. 208 patients, 95 females and 113 males met the inclusion criteria. Depending on the type of the first stage of correction, all patients were divided into three groups: 1 – patients after the Norwood operation (n=84), 2 – patients after the isolated systemic-to-pulmonary shunts (n=74), 3 – patients after pulmonary artery band or those who did not need the first stage of surgical treatment (n=50).RESULTS. From all patients who survived the Stage-I (158 patients), 47 (30 %) patients underwent 72 reconstructive interventions on the pulmonary arteries: 8 % – before Stage-II, 46 % – at Stage-II, 17 % – between stages II and III, 11 % – at Stage-III and 18 % – after Stage-III. When comparing freedom from stenosis of the pulmonary arteries, statistically significant differences were found between groups of patients (p=0.005), with the least freedom from stenosis in group 1 (after the Norwood operation) and the greatest freedom from stenosis in group 3 (after pulmonary artery band or without first stage of correction).CONCLUSIONS. Patients with a functionally single ventricle are at risk of developing pulmonary artery stenosis throughout the entire period of staged hemodynamic correction and after its completion. The identified narrowing needs immediate correction, since a long-term existing narrowing can lead to hypoplasia of the pulmonary vascular bed and become an obstacle to the timely Fontan completion. More extensive use of tomographic imaging techniques is recommended in order to fully assess the degree of narrowing.

Ventricular dysfunction is the most frequent complication in adult patients post-Fontan completion. Through this work, we aim to evaluate ventricular systolic function by conventional echographic parameters and by global longitudinal strain (GLS) to determine the prediction of early ventricular systolic dysfunction. This is a prospective monocentric study enrolling 15 clinically stable adult Fontan patients with preserved ejection fraction (EF). Myocardial deformation study by GLS with speckle tracking technique in addition to a standard Doppler transthoracic echocardiography (TTE) was performed. Cardiac magnetic resonance imaging (CMR) was also performed. A comparison of echocardiographic and CMR parameters was made. In comparison to CMR-derived EF, we found a significant correlation with GLS and TTE-derived EF (P=0.003 and 0.014). We divided our population into two groups based on the cut-off value of 50% of CMR derived EF. Comparison of GLS in both groups showed a significant correlation (P=0.003). A cut-off value of -13.3% showed sensitivity of 67% and specificity of 100%. GLS has a moderate diagnostic value for systolic myocardial dysfunction in the population of adult patients with Fontan circulation.

Introduction: The Fontan operation is a surgical technique used to treat cyanotic heart diseases with single ventricle physiology. Although it has undergone several modifications since its inception in 1971, it is still associated with significant morbidity and mortality. Aim: To examine the outcomes of the Fontan operation and identify various risk factors associated with it. Materials and Methods: This retrospective observational study was conducted at the Postgraduate Institute of Medical Education and Research (PGIMER) in Chandigarh, India from January 2014 to December 2017. All patients who underwent the Fontan operation at PGIMER were included in the study. The study analysed outcomes such as survival, arrhythmias, neurological complications, heart failure, cirrhosis, and Protein Losing Enteropathy (PLE) following the Fontan operation. Various risk factors that could potentially influence the outcome of the Fontan operation were assessed, including preoperative, intraoperative, and postoperative factors. Statistical analysis was performed using Statistical Package for the Social Sciences (SPSS) software, version 25.0. The Chi-square test was utilised to determine predictors of survival and other longterm outcomes, with a p-value of

Ventricular dysfunction is the most frequent complication in adult patients post-Fontan completion. Through this work, we aim to evaluate ventricular systolic function by conventional echographic parameters and by global longitudinal strain (GLS) to determine the prediction of early ventricular systolic dysfunction. This is a prospective monocentric study enrolling 15 clinically stable adult Fontan patients with preserved ejection fraction (EF). Myocardial deformation study by GLS with speckle tracking technique in addition to a standard Doppler transthoracic echocardiography (TTE) was performed. Cardiac magnetic resonance imaging (CMR) was also performed. A comparison of echocardiographic and CMR parameters was made. In comparison to CMR-derived EF, we found a significant correlation with GLS and TTE-derived EF (P = 0.003 and 0.014). We divided our population into two groups based on the cut-off value of 50% of CMR derived EF. Comparison of GLS in both groups showed a significant correlation (P = 0.003). A cut-off value of -13.3% showed sensitivity of 67% and specificity of 100%. GLS has a moderate diagnostic value for systolic myocardial dysfunction in the population of adult patients with Fontan circulation. [ABSTRACT FROM AUTHOR]

A simplified technique of performing the extracardiac Fontan operation on cardiopulmonary bypass is described. The advantages of this technique are briefly discussed. [ABSTRACT FROM AUTHOR]

BACKGROUND: Total cavopulmonary connection (TCPC) is associated with a lower risk of incident atrial arrhythmias as compared to atriopulmonary Fontan, but the risk of recurrent atrial arrhythmias is unknown in this population. The purpose of this study was to determine the incidence and risk factors for recurrent atrial arrhythmias and thromboembolic complications in patients with TCPC. METHODS: This is a retrospective multicenter study conducted by the Alliance for Adult Research in Congenital Cardiology (AARCC), 2000-2018. The inclusion criteria were TCPC patients (age > 15 years) with prior history of atrial arrhythmia. RESULTS: A total of 103 patients (age 26 ± 7 years; male 58 [56%]) met inclusion criteria. The mean age at initial arrhythmia diagnosis was 13 ± 5 years, and atrial arrhythmias were classified as atrial flutter/tachycardia in 85 (83%) and atrial fibrillation in 18 (17%). The median duration of follow-up from the first episode of atrial arrhythmia was 14.9 (12.1-17.3) years, and during this period 64 (62%) patients had recurrent atrial arrhythmias (atrial flutter/tachycardia 51 [80%] and atrial fibrillation 13 [20%]) with annual incidence of 4.4%. Older age was a risk factor for arrhythmia recurrence while the use of a class III anti-arrhythmic drug was associated with a lower risk of recurrent arrhythmias. The incidence of thromboembolic complication was 0.6% per year, and the cumulative incidence was 4% and 7% at 5 and 10 years respectively from the time of first atrial arrhythmia diagnosis. There were no identifiable risk factors for thromboembolic complications in this cohort. CONCLUSIONS: Although TCPC provides superior flow dynamics and lower risk of incident atrial arrhythmias, there is a significant risk of recurrent arrhythmias among TCPC patients with a prior history of atrial arrhythmias. These patients may require more intensive arrhythmia surveillance as compared to other TCPC patients.

Abstract Background Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes. Methods This was a retrospective, single-center study of patients with Fontan circulation who underwent CMR. Frequency matching of age at CMR was used to construct a comparison group of patients with repaired tetralogy of Fallot (rTOF). TD characteristics included maximum diameter and a qualitative assessment of tortuosity. Clinical outcomes included protein-losing enteropathy (PLE), plastic bronchitis, listing for heart transplantation, and death. A composite outcome was defined as presence of any of these events. Results The study included 189 Fontan patients (median age 16.1 years, IQR 11.0–23.2 years) and 36 rTOF patients (median age 15.7 years, IQR 11.1–23.7 years). The TD diameter was larger (median 2.50 vs. 1.95 mm, p = 0.002) and more often well visualized (65% vs. 22%, p

Background The Fontan operation is a well-described palliative procedure for functional single-ventricle patients. This population has an increased risk of thrombo-embolic events. Adequate imaging, and therefore diagnosis, requires an understanding of the unique anatomy and physiology of a Fontan. Optimal strategies for the prevention and treatment of thrombo-embolic complications in the Fontan population are poorly defined. Case summary A 28-year-old female with a history of Ebstein anomaly of the tricuspid valve status post-Fontan presented with chest pain and acute hypoxia. Computed tomographic angiography (CTA) reported a submassive pulmonary embolism (PE). She was initiated on a heparin drip. Catheterization demonstrated elevated Fontan pressures and a large thrombus in the right lower pulmonary artery that was removed with an aspiration device. The patient was transitioned to a direct oral anticoagulant (DOAC) following the procedure. Discussion Thrombotic complications are common in the adult Fontan population. Given the morbidity and mortality associated with this complication, the use of proper imaging techniques is imperative. Traditional CTA imaging for PE in Fontan patients often has contrast filling defects related to their anatomy and physiology. Utilization of adequate imaging techniques helps decrease cost and additional radiation exposure. In addition, it avoids inappropriate hospitalization, need for anticoagulation, and potential need for catheterization toconfirm or exclude the presence of PE. For Fontan patients with a thrombus, prior event, and no contraindications, current guidelines recommend oral anticoagulation with a vitamin K antagonist; however, there are increasing data on the use of DOACs in this population. [ABSTRACT FROM AUTHOR]

Background: By rerouting the systemic venous return into the pulmonary circulation, the Fontan operation is perhaps the most effective palliative procedure in patients with complex congenital heart disease. The Fontan, however, still comes with several significant complications and morbidities, one of them being postoperative prolonged pleural effusion. In our center from 2017 to 2021, 10% of all our patients who underwent Fontan operation developed prolonged pleural effusion. With this study, we aimed to analyze the preoperative use of sildenafil for 6 months and its role in prolonged pleural effusion after the Fontan operation. Materials and Methods: In total, 158 patients were included in the analysis. The cohort was divided into patients who developed prolonged pleural effusion after Fontan surgery (n = 17) and those who did not (n = 141). We analyzed several risk factors and the history of sildenafil use in each group in the cohort. We found that patients who used sildenafil for at least 6 months prior to Fontan surgery versus those who did not use (n = 112 vs. n = 46) were found to be at lower risk of developing prolonged pleural effusion (odds ratio [OR]: 0.32 [confidence interval 95%: 0.11-0.88], P = 0.027). Conclusion: Preoperative administration of sildenafil for at least 6 months before Fontan might reduce the risk of postoperative prolonged pleural effusion. [ABSTRACT FROM AUTHOR]

[Display omitted] [ABSTRACT FROM AUTHOR]

Background : By rerouting the systemic venous return into the pulmonary circulation, the Fontan operation is perhaps the most effective palliative procedure in patients with complex congenital heart disease. The Fontan, however, still comes with several significant complications and morbidities, one of them being postoperative prolonged pleural effusion. In our center from 2017 to 2021, 10% of all our patients who underwent Fontan operation developed prolonged pleural effusion. With this study, we aimed to analyze the preoperative use of sildenafil for 6 months and its role in prolonged pleural effusion after the Fontan operation. Materials and Methods : In total, 158 patients were included in the analysis. The cohort was divided into patients who developed prolonged pleural effusion after Fontan surgery (n = 17) and those who did not (n = 141). We analyzed several risk factors and the history of sildenafil use in each group in the cohort. We found that patients who used sildenafil for at least 6 months prior to Fontan surgery versus those who did not use (n = 112 vs. n = 46) were found to be at lower risk of developing prolonged pleural effusion (odds ratio [OR]: 0.32 [confidence interval 95%: 0.11–0.88], P = 0.027). Conclusion : Preoperative administration of sildenafil for at least 6 months before Fontan might reduce the risk of postoperative prolonged pleural effusion.

Abstract Background The Fontan operation has improved the survival of children born with congenital heart disease with single ventricle physiology. The most widely adopted variations of the Fontan procedure are the extracardiac conduit, the lateral tunnel ve the intra/extracardiac conduit with fenestration. Despite advances in the treatment and prevention of early and late complications that may develop after Fontan surgery, morbidity still remains an important problem. Methods 304 patients who underwent Fontan surgery in our center between 1995 and 2022 were included in our study. The complications that developed in patients who underwent primary Fontan or lateral tunnel surgery and extracardiac conduit Fontan application were compared. Results Classic Fontan surgery and lateral tunnel surgery were performed in 26 of the patients, and extracardiac Fontan surgery was performed in 278 patients. 218 of 304 cases were patients with single ventricular pathology. 86 cases were patients with two ventricular morphologies but complex cardiac pathology. Fenestration was performed in only 6 patients, other patients did not require fenestration. The mean follow-up period of our patients was 12 years (3 months–27 years). When the complications between Fontan procedures were compared in our study, it was found that the length of hospital stay and mortality were statistically significantly reduced in patients who underwent extracardiac Fontan surgery. There was no significant difference in terms of complications that can be seen after Fontan surgery and the length of stay in the intensive care unit. Conclusion Fontan complex is a palliative surgery for children with complex heart disease. Palliative surgical operations aimed at the preparation of the Fontan circulation lead to the preparation of the pulmonary vascular bed and the preservation of ventricular function. The techniques applied in Fontan surgery affect the early and long-term complications and the survival of the patients. In our study, when we examined the patients who extracardiac conduit Fontan procedure for the non-cardiac route, we found that mortality and morbidity were minimal.

Abstract Background Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. Methods This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (mass i ), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. Results Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2–9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (− 0.61%/year vs. − 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). Conclusions Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.

Abstract Background Little is known about the social life in adult patients after Fontan palliation. The study aimed to assess the long-term social life of patients after Fontan surgery, including school achievement, employment, and marital status. Results We conducted a cross-sectional study on patients aged 18 years or above who had a Fontan operation for a single ventricle pathology. Our outcomes were the academic performance and marital and employment status of adult Fontan patients. Patients or their families were interviewed directly or by phone, and a set of questions were asked to address their educational level, employment, and marital status. Ninety-nine patients were included in the study. Their median age was 21 years (min–max: 18–41), and 60% were females (n= 59). The most common diagnoses were double inlet left ventricle (n= 24, 24%), tricuspid atresia (n= 21, 21%), and the unbalanced atrioventricular canal (n= 21, 21%). Fourteen patients (14%) were married, and 6 of them had children (two were females). Eleven percent were college graduates (either diploma or bachelor’s degree), and 47% were high school graduates. Conclusions Fontan operation could negatively affect the social life of the patients. It may affect employment patterns, educational levels, and marital status.

The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation. Pulmonary complications are common and may contribute to both circulatory and pulmonary insufficiency, leading ultimately to Fontan failure. These complications include restrictive lung disease, sleep abnormalities, plastic bronchitis, and cyanosis. Cyanosis post-Fontan procedure can be attributed to multiple causes including systemic to pulmonary venous collateral channels and pulmonary arteriovenous malformations. This review presents the unique cardiopulmonary interactions in the Fontan circulation. Understanding the cardiopulmonary interactions along with improved recognition and treatment of pulmonary abnormalities may improve the long-term outcomes in this growing patient population. Interventions focused on improving pulmonary function including inspiratory muscle training and endurance training have shown a promising effect post-Fontan procedure. [Display omitted] • Pulmonary complications are common post-Fontan procedure, including restrictive lung disease, sleep abnormalities, PB, and cyanosis. • Pulmonary complications may contribute to Fontan circulatory failure by worsening pulmonary vascular resistance, hypoxia, and exercise capacity. • Respiratory muscle and exercise training may improve pulmonary function and exercise capacity after Fontan surgery. [ABSTRACT FROM AUTHOR]

Background: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes. Methods: This was a retrospective, single-center study of patients with Fontan circulation who underwent CMR. Frequency matching of age at CMR was used to construct a comparison group of patients with repaired tetralogy of Fallot (rTOF). TD characteristics included maximum diameter and a qualitative assessment of tortuosity. Clinical outcomes included protein-losing enteropathy (PLE), plastic bronchitis, listing for heart transplantation, and death. A composite outcome was defined as presence of any of these events. Results: The study included 189 Fontan patients (median age 16.1 years, IQR 11.0–23.2 years) and 36 rTOF patients (median age 15.7 years, IQR 11.1–23.7 years). The TD diameter was larger (median 2.50 vs. 1.95 mm, p = 0.002) and more often well visualized (65% vs. 22%, p < 0.001) in Fontan patients vs. rTOF patients. TD dimension increased mildly with age in Fontan patients, R = 0.19, p = 0.01. In Fontan patients, the TD diameter was larger in those with PLE vs. without PLE (age-adjusted mean 4.11 vs. 2.72, p = 0.005), and was more tortuous in those with NYHA class ≥ II vs. class I (moderate or greater tortuosity 75% vs. 28.5%, p = 0.02). Larger TD diameter was associated with a lower ventricular ejection fraction that was independent of age (partial correlation = − 0.22, p = 0.02). More tortuous TDs had a higher end-systolic volume (mean 70.0 mL/m2 vs. 57.3 mL/m2, p = 0.03), lower creatinine (mean 0.61 mg/dL vs. 0.70 mg/dL, p = 0.04), and a higher absolute lymphocyte count (mean 1.80 K cells/µL vs. 0.76 K cells/µL, p = 0.003). The composite outcome was present in 6% of Fontan patients and was not associated with TD diameter (p = 0.50) or tortuosity (p = 0.09). Conclusions: The TD is well visualized in two-thirds of patients with Fontan circulation on 3D-bSSFP images. Larger TD diameter is associated with PLE and increased TD tortuosity is associated with an NYHA class ≥ II. [ABSTRACT FROM AUTHOR]

Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic hemodynamic abnormalities resulting from multiple defects in patients with cardiac malposition should be the focus of treatment. At the time of the first presentation, treating the pathophysiologic aberrations caused by the defect complex, whether it is by improving the pulmonary blood flow or restricting it, is the first step. Some patients with simpler or single defects are amenable to surgical or transcatheter therapy and should be treated accordingly. Other associated defects should also be treated appropriately. Biventricular or univentricular repair dependent on the patient's cardiac structure should be planned. Complications in-between Fontan stages and after conclusion of Fontan surgery may occur and should be promptly diagnosed and addressed accordingly. Several other cardiac abnormalities unrelated to the initially identified heart defects may manifest in adulthood, and they should also be treated. [ABSTRACT FROM AUTHOR]

Background Fontan circulation is associated with kidney injury and dysfunction, often unappreciated until Fontan circulatory failure. We hypothesized that cystatin C‐estimated glomerular filtration rate (eGFR) would identify chronic kidney disease more frequently and that urine kidney injury biomarkers would be higher with declining Fontan physiological features. Methods and Results We enrolled 100 ambulatory individuals. Blood and urinary laboratory measurements were compared with demographics and clinically obtained data. Different eGFR equations were used for individuals aged ≥19 years and

The Fontan operation is performed in patients with a single ventricle. As the systemic venous return is directly connected to the pulmonary circulation during this procedure, chronic hepatic congestion is induced, leading to Fontan-associated liver disease (FALD) including liver cirrhosis and hepatocellular carcinoma (HCC). In this report, we present a case of HCC diagnosed in a patient who underwent the Fontan operation 30 years ago. The patient underwent regular surveillance for FALD, which revealed a 4 cm-sized hepatic mass with elevated serum alpha-fetoprotein. After surgical treatment, there was no evidence of HCC recurrence during 3 years of follow-up. As the risk of HCC and Fontan-associated liver cirrhosis increases with the duration elapsed since the operation, regular surveillance should be emphasized. Serial follow-up of serum alpha-fetoprotein levels and abdominal imaging are necessary to achieve early and accurate diagnosis of HCC in post-Fontan patients.

It has been 50 years since the Fontan operation was introduced and it is still used in clinical praxis as the final interventional solution for complex congenital heart disease with single ventricle physiology. As a results of its modifications, improvements in patient selection and perioperative care, survival has steadily increased. A growing number of longterm survivors is discovering the challenges of this non-physiologic circuit. The absence of a subpulmonary ventricle is characterized by chronically elevated systemic venous pressures and decreased cardiac output. Systolic and more importantly diastolic dysfunction, arrhythmia, thromboembolism and different forms of lymphatic failure such as plastic bronchitis or protein-losing enteropathy are the most frequent complications of the Fontan circulation. Liver fibrosis, gradual decline of renal function and abnormalities in growth and bone structure are also consequences of this abnormal hemodynamic state. Oral medical therapies of the Fontan circulatory failure have little data demonstrating benefit. It is important to look proactively for optimalization of the Fontan circuit with catheter-based or surgical interventions. The only widely prescribed medication for this population is antithrombotic prophylaxis. Fontan circulatory failure is a new clinical phenotype for most adult cardiologists, and therefore the principles of acute and long-term management of the most common complications are highlighted. The survival and quality of life of this growing population can be even more favourable in the near future by implementing innovative approaches such as lymphatic interventions, mechanical circulatory support or medical treatment of diastolic dysfunction. Fig. 6, Ref. 49, on-line full text (Free, PDF) www.cardiologyletters.sk [ABSTRACT FROM AUTHOR]

Background: The Fontan operation has improved the survival of children born with congenital heart disease with single ventricle physiology. The most widely adopted variations of the Fontan procedure are the extracardiac conduit, the lateral tunnel ve the intra/extracardiac conduit with fenestration. Despite advances in the treatment and prevention of early and late complications that may develop after Fontan surgery, morbidity still remains an important problem. Methods: 304 patients who underwent Fontan surgery in our center between 1995 and 2022 were included in our study. The complications that developed in patients who underwent primary Fontan or lateral tunnel surgery and extracardiac conduit Fontan application were compared. Results: Classic Fontan surgery and lateral tunnel surgery were performed in 26 of the patients, and extracardiac Fontan surgery was performed in 278 patients. 218 of 304 cases were patients with single ventricular pathology. 86 cases were patients with two ventricular morphologies but complex cardiac pathology. Fenestration was performed in only 6 patients, other patients did not require fenestration. The mean follow-up period of our patients was 12 years (3 months–27 years). When the complications between Fontan procedures were compared in our study, it was found that the length of hospital stay and mortality were statistically significantly reduced in patients who underwent extracardiac Fontan surgery. There was no significant difference in terms of complications that can be seen after Fontan surgery and the length of stay in the intensive care unit. Conclusion: Fontan complex is a palliative surgery for children with complex heart disease. Palliative surgical operations aimed at the preparation of the Fontan circulation lead to the preparation of the pulmonary vascular bed and the preservation of ventricular function. The techniques applied in Fontan surgery affect the early and long-term complications and the survival of the patients. In our study, when we examined the patients who extracardiac conduit Fontan procedure for the non-cardiac route, we found that mortality and morbidity were minimal. [ABSTRACT FROM AUTHOR]

Background and aims of the study A functionally single ventricle (FSV) refers to a group of congenital heart defects that are not amenable for biventricular correction. The Fontan operation is utilized as surgical treatment for most of FSV patients. The evaluation of FSV function is extremely difficult due to its unique pathophysiology. This study aimed to explore the efficacy of speckle tracking echocardiography (STE) parameters measured at rest and during exercise for comprehensive assessment of univentricular heart. We enrolled 37 patients with a functionally single ventricle after the Fontan operation, hospitalized in the Department of Congenital Heart Defects between years 2019 and 2021.The echocardiographic stress tests were performed in the Echocardiography Laboratory of the Congenital Heart Defects Department. The study was conducted on a bicycle ergometer in a semi-recumbent position. The parameters obtained by speckle tracking echocardiography (STE): the longitudinal strain of the FSV free wall (Ɛ) and the longitudinal strain of myocardial layers: subendocardial, medial and subepicardial were analyzed. A transmural longitudinal strain gradient (TG) was calculated as the difference between longitudinal deformation of the subendocardial and subepicardial layers. Current results of cardiac magnetic resonance imaging (CMR) and cardio-pulmonary test (CPET) were also incorporated. Demographic data, past interventions, pharmacological treatment and comorbidities were extracted from medical records. Ɛ at rest and during exercise were not related to the parameters of physical capacity obtained on CPET nor to the CMR results. The transmural strain gradient was dependent on physical performance parameter- peak oxygen uptake- and related to the FSV ejection fraction calculated by magnetic resonance imaging. The transmural strain gradient and FSV free wall strain are readily measurable and suitable for evaluating single ventricle function. The TG is positively correlated with peak oxygen uptake during the cardiopulmonary test and with the ejection fraction derived from cardiac magnetic resonance imaging. The applicability of these findings in patients undergoing the Fontan procedure warrants further exploration. [Display omitted] • Speckle tracking echocardiography is an appropriate method for assessing the function of a functionally single ventricle (FSV). • The longitudinal strain of the functionally single ventricle wall is a novel parameter that is easy to obtain and overcomes the difficulties in assessing global FSV strain. • The transmural strain gradient is a novel parameter of myocardial structure. It expresses the difference between the longitudinal strains of the subendocardial and subepicardial layers. • The transmural strain gradient correlates with the ejection fraction from FSV cardiac magnetic resonance imaging and the results of cardiopulmonary tests. [ABSTRACT FROM AUTHOR]

Background : Various operative strategies are described for the Fontan procedure. In this study, we describe our short-term results and technique of Fontan procedure on cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Methods : This was a retrospective study of 32 patients, median age of 6 years (4–19 years) and median weight of 20 kg (13–51 kg), who underwent Fontan procedure on CPB and DHCA from July 2016 to July 2021. Results : The median CPB time was 125 min (77–186 min), the median DHCA time was 42 min (27–50 min), and the median Fontan pressure was 14 mmHg (10–18 mmHg). The median time to extubation was 4 h (1–20 h), the duration of chest tube drainage was 8 days (5–24 days), and the median intensive care unit stay was 4 days (3–8 days). The presence of heterotaxy was associated with longer duration of pleural drainage (P = 0.01). There was no operative mortality and no major adverse events such as seizures, gross neurological deficits, or arrhythmias in the postoperative period. Conclusions : Fontan procedure can be safely performed on CPB and DHCA with good operative results. This operative strategy may be used in special circumstances like in patients with situs and systemic venous anomalies and those requiring repair of a complex intracardiac defect. Long-term follow-up will be required to evaluate if this strategy has any impact on the neurodevelopmental outcome and the long-term sequelae of Fontan.

Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center. Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed. Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era. Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.

Background: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. Methods: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. Results: Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2–9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (− 0.61%/year vs. − 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). Conclusions: Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance. [ABSTRACT FROM AUTHOR]

There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation. We divided the patients into two groups according to the change in the pulmonary artery index before and after the Fontan operation: the increased pulmonary artery index group (n = 51) and decreased pulmonary artery index group (n = 43). The pulmonary artery index after the Fontan operation was significantly higher in the increased pulmonary artery index group than decreased pulmonary artery index group (p < 0.0001). The central venous pressure (p < 0.0001) and pulmonary vascular resistance (p = 0.007) were significantly lower in the increased pulmonary artery index group. The mixed venous oxygen saturation was significantly higher in the increased pulmonary artery index group (p < 0.0001). The pulmonary artery index was more increased in the patients who were administered a phosphodiesterase type 5 inhibitor than in those who were not (p = 0.03). The change in the pulmonary artery index reflected the hemodynamics after a Fontan operation during the short term. In that respect, phosphodiesterase type 5 inhibitors might be involved in the process. [ABSTRACT FROM AUTHOR]

Background: Little is known about the social life in adult patients after Fontan palliation. The study aimed to assess the long-term social life of patients after Fontan surgery, including school achievement, employment, and marital status. Results: We conducted a cross-sectional study on patients aged 18 years or above who had a Fontan operation for a single ventricle pathology. Our outcomes were the academic performance and marital and employment status of adult Fontan patients. Patients or their families were interviewed directly or by phone, and a set of questions were asked to address their educational level, employment, and marital status. Ninety-nine patients were included in the study. Their median age was 21 years (min–max: 18–41), and 60% were females (n= 59). The most common diagnoses were double inlet left ventricle (n= 24, 24%), tricuspid atresia (n= 21, 21%), and the unbalanced atrioventricular canal (n= 21, 21%). Fourteen patients (14%) were married, and 6 of them had children (two were females). Eleven percent were college graduates (either diploma or bachelor's degree), and 47% were high school graduates. Conclusions: Fontan operation could negatively affect the social life of the patients. It may affect employment patterns, educational levels, and marital status. [ABSTRACT FROM AUTHOR]

Background: The traditional outcomes of the Fontan operation (FO) in endocardial cushion defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect, and do not directly compare short‐term outcomes of FO in ECD patients with non‐ECD patients. Our study aims to address these shortcomings. Methods: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non‐ECD diagnosis. The data were abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. Results: Three thousand three hundred eighty patients underwent the FO of which 360 patients (11%) were FO‐ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non‐ECD patients. FO‐ECD had a higher discharge‐mortality (2.84% vs. 0.45%, p =.04). The length of stay (16 vs. 13 days, p =.05) and total charges incurred ($283, 280 vs. $234, 106, p =.03) for the admission were higher in the FO‐ECD as compared to non‐ECD patients. In multivariable analysis, ECD diagnosis, cardiac arrest, acute kidney injury, and postoperative hemorrhage were predictors of mortality. Conclusion: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than in non‐ECD patients. The occurrence of postoperation complications and a diagnosis of ECD were predictive of a negative outcome. [ABSTRACT FROM AUTHOR]

The optimal management of functionally univentricular hearts with a significant unbalanced pulmonary blood flow is unclear. The role of cavopulmonary shunt in the rehabilitation of hypoplastic pulmonary arteries is limited. Intrapulmonary artery septation is a technique, wherein a systemic-to-pulmonary artery shunt provides an additional source of blood flow to rehabilitate the hypoplastic pulmonary artery. We report a case of functionally univentricular heart with hypoplastic left pulmonary artery following a cavopulmonary shunt in whom intrapulmonary artery septation resulted in adequate growth of the left pulmonary artery. [ABSTRACT FROM AUTHOR]

A 33-year-old man, who had undergone atriopulmonary connection Fontan procedure (AP Fontan) for double outlet right ventricle, suffered from heart failure due to atrial tachycardia at 27 years old. Atrial tachycardia was suppressed after amiodarone administration. At 32 years old, atrial tachycardia recurred, and short palpitations gradually increased. Cardiac computed tomography showed that coronary sinus (CS) was perfused into the pulmonary venous atrium, and catheter insertion to CS from the systemic venous atrium was impossible. We performed an electrophysiology study (EPS) and radiofrequency catheter ablation (RFCA) under local anesthesia. An esophageal electrode catheter was inserted as a potential reference for ultra-high density three-dimensional (3D) mapping system. Two types of atrial tachycardia were induced by EPS. Ultra-high-density 3D mapping system revealed an intra-atrial reentrant pattern around the scar area on the lower right atrium in both atrial tachycardias; therefore, we diagnosed intra-atrial reentrant tachycardia (IART). The low voltage area and inferior vena cava during IART were ablated linearly, and IART was terminated. In conclusion, a CS electrode catheter cannot be inserted in a patient with AP Fontan, and ultra-high-density 3D mapping using the esophageal electrode catheter as a potential reference enables accurate and rapid mapping and is very effective for RFCA. The incidence of intra-atrial reentrant tachycardia (IART) is high in patients with late after Fontan procedure, and the treatment may be difficult. Ultra-high-density three-dimensional mapping can perform accurate mapping of IART and rapid detection of low voltage areas effective for radiofrequency catheter ablation. An esophageal electrode catheter can be the reference potential for accurate activation mapping in Fontan patients where coronary sinus electrode catheter insertion may be impossible from a systemic venous atrium. [ABSTRACT FROM AUTHOR]

Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended. [ABSTRACT FROM AUTHOR]