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Background Heart failure phenotyping in single‐ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross‐sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e’/end‐diastolic volume) was >90th percentile (≥0.26 mL ‐1 ). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO 2 . Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P <0.01. DD patients had lower predicted peak VO 2 compared with controls (52% [20] versus 67% [23], P <0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P <0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients.

Background: Aortopulmonary collateral arteries (APCAs) are common pulmonary blood supply in the group of congenital heart disease with functionally single ventricle. APCAs causes loss of aortic outflow, increase in pulmonary arterial hydrostatic pressure, reducing blood flow from the SVC and IVC to the pulmonary artery (in patients after Glenn and Fontan operations). Therefore, the closure of APCAs is vital in the management of single ventricle patients before and during Fontan operation. The technique of percutaneous APCAs occlusion with coils is a new, effective and better method of control of APCAs, excluding the need for surgical ligation of the APCAs.. Objectives: To evaluate results of aortopulmonary collateral arteries coil embolization  in pre-fontan patients Methods: This is the descriptive clinical and retrospective study Result: 29 patients with average age of 7.9 underwent cardiac catheterization with APCAs coil embolization successfully before the Fontan  surgery. Complication rate was very low. This method effectively reduced mean pulmonary artery pressure with statistical significance. The results of Fontan surgery of this group were better than that in other studies in the rate of diaphragmatic paralysis and chylous effusion Conclusions: The technique of percutaneous APCAs occlusion with coils before Fontan surgery is safe, effective in reducing mean pulmonary artery pressure and improving results of Fontan surgery.

AbstractComputational fluid dynamics (CFD) modeling may aid in planning of invasive interventions in Fontan patients. Clinical application of current CFD techniques is however limited by complexity and long computation times. Therefore, we validated a “lean” CFD method to magnetic resonance imaging (MRI) and an “established” CFD method, ultimately aiming to reduce complexity to enable predictive CFD during ongoing interventions. Fifteen Fontan patients underwent MRI for CFD modeling. The differences between lean and established approach, in hepatic and total flow percentage to the left pulmonary artery (%LPA), power loss and relative wall shear stress area were 1.5 ± 4.0%, -0.17 ± 1.1%, -0.055 ± 0.092 mW and 1.1 ± 1.4%. Compared with MRI, the lean and established method showed a bias in %LPA of -1.9 ± 3.4% and -1.8 ± 3.1%. Computation time was for the lean and established approach 3.0 ± 2.0 min and 7.0 ± 3.4 h, respectively. We conclude that the proposed lean method provides fast and reliable results for future CFD support during interventions. Graphical abstract

Objectives: Fontan-associated liver disease (FALD) is the most common end-organ dysfunction affecting up to 70–80% of the Fontan population. The clinical significance of FALD is incompletely understood and no unambiguous correlation between hepatic function and FALD severity has been established. In this study, we sought to evaluate maximal liver function capacity with liver maximum function capacity test (LiMAx®) in adult Fontan patients.Methods: Thirty-nine adult Fontan patients (median age: 29.4 years [IQR 23.4; 37.4], median follow-up after Fontan operation: 23.9 years [IQR 17.8;26.4]) were analyzed in a cross-sectional observational study using LiMAx® test (Humedics GmbH, Berlin, Germany), laboratory testing, transient elastography (TE) and hepatic ultrasound. The LiMAx® test is based on the metabolism of 13C-methacetin, which is administered intravenously and cleaved by the hepatic cytochrome P4501A2 to paracetamol and 13CO2, which is measured in exhaled air and correlates with maximal liver function capacity.Results: Maximal liver function capacity assessed by LiMAx® test was normal in 28 patients (&gt;315 μg/h*kg) and mildly to moderately impaired in 11 patients (140–314 μg/h*kg), while no patient displayed severe hepatic impairment (&lt;139 μg/kg*h). No correlation was found between maximal liver function capacity and hepatic stiffness by TE (r2 = −0.151; p = 0.388) or the presence of sonographic abnormalities associated with FALD (r2 = −0.204, p = 0.24). There was, however, an association between maximal liver function capacity and the laboratory parameters bilirubin (r2 = −0.333, p = 0.009) and γ-glutamyl transferase (r2 = −0.367; p = 0.021). No correlation was detected between maximal liver function capacity and the severity of FALD (r2 = −0.235; p = 0.152).Conclusion: To the best of our knowledge, this is the first study to evaluate maximal liver function capacity using LiMAx® test in Fontan patients, which is a useful complementary diagnostic instrument to assess chronic hepatic injury. Maximal liver function capacity was preserved in most of our adult Fontan patients despite morphologic evidence of FALD. Moreover, maximal liver function capacity does not correlate with the extent of FALD severity evaluated by sonography or laboratory analysis. Thus, the development and progression of FALD in Fontan patients is not a uniform process and diagnostics of chronic hepatic injury during follow-up should encompass various modalities.

Abstract Aims Fontan operation represents the surgical palliative option for congenital heart disease with single ventricle physiology. With the improvement of surgical and percutaneous technique, we are facing a growing population of patients with an unique pathophysiology and potential complications. Methods and results Patients that underwent Fontan palliation in our centre between 1993 and 2016 were included in this prospective study. We excluded patients with major congenital renal anomalies, those that underwent cardiac transplantation, and redo-Fontan patients. All the subjects underwent clinical evaluation, laboratory exams with complete renal and hepatic function, transient hepatic elastography, and complete cardiac evaluation. We used Schwartz equation for estimating glomerular filtration rate in patients younger than 18 years, and CDK-EPI equation for adult patients. We enrolled 35 patients, 46% female (N = 16), and 54% male (N = 19). Medium age was 17 years old, median age 15 years old (range: 10–31 years old). Medium time from Fontan completion was 160 months (range: 57–340 months). Regarding to cardiac anatomy, 10 patients had functional single left ventricle (FSLV, 28.5%) and 21 a functional single right ventricle (FSRV, 60%); 4 patients had undetermined single ventricle (11.5%). Total cavo-pulmonary connection (TCPC) with intracardiac lateral tunnel was performed in 7 patients (20%, N = 7), whereas 28 patients had TCPC with external conduit (80%). Data from echocardiographic evaluation showed a medium EF established with Simpson’s method of 60% in patients with FSLV; patients with a FSRV or undetermined single ventricle had a medium FAC of 41.1%, with 15.1% having a reduced FAC &lt; 35%. No FSLV patients had an EF &lt; 50%. When using creatinine-based formula, data about renal function in our population showed a stage 2 chronic kidney disease (eGFR: 60–89 ml/min 1.73 mq) in 11% of total population (N = 4), that became 26% when using cystatin C-based equation (N = 9), with one patient showing a moderate reduced loss of kidney function (eGFR: 40–59 ml/min 1.73 mq). Urinalysis showed 29% (N = 10) of patients having microalbuminuria (microalbumin/creatinine ratio between 30 and 300 mg/g). Statistical analysis demonstrated a negative correlation between systolic function (TAPSE for FSRV) and cystatin C blood levels (Pearson’s R −0.428, P = 0.053), and between systolic function (FAC and Simpson) and microalbuminuria (Pearson’s R −0.414 with P = 0.049 and Pearson’s R −0.754 with P = 0.019, respectively). Transient elastography reported 10 patients (29.4%) with abnormal hepatic stiffness for Fontan patients. That condition appeared to be more frequent in patients with higher grade of AV valve insufficiency (P &lt; 0.05). Conclusions Our population showed an higher prevalence of FSRV Fontan patients, with an expected lower systolic function compared with FSLV. 2D evaluation of systolic function showed a linear inverse correlation with renal function, suggesting that Fontan patients need a closer renal monitoring. Hepatic stiffness, which is a warning sign of potential hepatic cirrhosis need to be monitored in all Fontan patients, especially those with a worse AV valve insufficiency.

Abstract Aims In Fontan patients, the pathophysiology of diastolic function and its relationship with systemic complications are still not well understood. Methods and results This is a prospective study including patients who underwent Fontan completion in our centre between 1993 and 2016. We excluded patients with major congenital renal anomalies, those who underwent cardiac transplantation and redo-Fontan patients. All the subjects underwent clinical evaluation, laboratory exams with complete renal and hepatic function, transient hepatic elastography, and complete cardiac evaluation. We used Schwartz equation for estimating glomerular filtration rate in patients younger than 18 years, and CDK-EPI equation for adult patients. We enrolled 35 patients, 46% female (N = 16), and 54% male (N = 19). Medium age was 17 years old (range: 10–31 years old). Medium time from Fontan completion was 160 months (range: 57–340 months). Ten patients had a functional single left ventricle (FSLV, 28.5%) and 21 a functional single right ventricle (FSRV, 60%); four patients had an undetermined single ventricle (11.5%). Data from renal function assessment showed a prevalence of stage 2 chronic kidney disease (eGFR: 60–89 ml/min 1.73 mq). Of those, 11% with creatinine-based equation and 26% (N = 9) when using cystatin C-based equation, with one patients showing a moderate reduced loss of kidney function (eGFR: 40–59 ml/min/1.73 mq). Most of the patients with reduced eGFR measured with cystatin C were FSRV (89%). None had laboratory markers of acute tubular damage, but four patients had signs of chronic tubular dysfunction with elevation of beta 2 microglobulin (13%). Echocardiographic evaluation of diastolic function showed two patients with baseline E/A &lt; 1 (6%, tot N = 33) and 11/33 (33%) pts with abnormal E/E′ (&gt;12). All of them were FSRV patients (100%). Interestingly, statistical correlation between diastolic parameters and renal function showed a significant association between tubular damage parameters, such as alfa1microglobulin and beta2microglobulin, and E/E′ (Pearson’s R 0.4 and 0.48, respectively, P &lt; 0.05), both for FSLV and FSRV patients. Diastolic function appeared to be associated also with glomerular filtration: we found a statistically significant direct correlation between diastolic pulmonary wave deceleration time (dt D wave) and creatinine value (Pearson’s R 0.49, P &lt; 0.05). Supporting the role of diastolic function in Fontan systemic complications is the linear correlation we found with hepatic tests: higher values of aspartate aminotransferase and of gamma-glutamyltransferase were associated with worse diastolic ventricular filling (longer dt D wave and E wave deceleration time, lower TDI early diastolic wave; Pearson’s R 0.45, 0.5, and −0.41, respectively, P &lt; 0.05). Conclusions Fontan-related nephropathy is associated with worsening diastolic function, which was more represented in FSRV patients. Diastolic function is also associated with liver disease in Fontan patients. Those data suggest renal and liver function should be closely monitored in patients with impaired diastolic function.