Repair of complex transposition of great arteries: What is the best technique to avoid outflow tract obstructions?

OBJECTIVES: This study aimed to evaluate the short-/mid-term outcome of patients with complex dextro (d)-/levo (l)-transposition of the great arteries (TGA), ventricular septal defect and left ventricular outflow tract obstructions. METHODS: A single-centre, retrospective review of all complex dextro-TGA (n = 85) and levo-TGA (n = 22) patients undergoing different surgeries [Arterial switch operation + left ventricular outflow tract obstruction-resection (ASO-R), half-turned truncal switch/Mair (HTTS), Nikaidoh and Rastelli] between May 1990 and September 2022 was performed. Groups were analysed using Kruskal-Wallis test with post hoc pairwise comparison and Kaplan-Meier time-to-event models. RESULTS: A total of 107 patients [ASO-R (n = 20), HTTS (n = 23), Nikaidoh (n = 21), Rastelli (n = 43)] were included, with a median age of 1.0 year (0.5-2.5) and surgical repair median follow-up was 3.8 years (0.3-10.5). Groups did not differ in respect to early postoperative complications/early mortality. Five-year overall survival curves were comparable: ASO-R 78.9% (53.2-91.5), HTTS 75.3% (46.8-89.9), Nikaidoh 85% (60.4-94.9) and Rastelli 83.9% (67.5-92.5), P = 0.9. Highest rates of right ventricular outflow tract (RVOT) reinterventions [33.3% and 32.6% (P = 0.04)] and reoperations [28.6% and 32.6% (P = 0.02)] occurred after Nikaidoh and Rastelli procedures. However, overall freedom from RVOT reinterventions and RVOT reoperations at 5 years did not differ statistically significantly between the groups (ASO-R, HTTS, Nikaidoh and Rastelli): 94.4% (66.6-99.2), 69.1% (25.4-90.5), 67.8% (34-86.9), 64.4% (44.6-78.7), P = 0.2, and 90.0% (65.6-97.4), 91% (50.8-98.7), 65.3% (32.0-85.3) and 67.0% (47.4-80.6), P = 0.3. CONCLUSIONS: Surgical repair of complex dextro-/levo-TGA can be performed with satisfying early/mid-term survival. RVOT reinterventions/reoperations were frequent, with highest rates after Nikaidoh and Rastelli procedures. Left ventricular outflow tract obstruction reoperations were rare with zero events after Nikaidoh and HTTS procedures.

EASL-ERN position paper on liver involvement in patients with Fontan-type circulation.

Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants born with a univentricular heart. The resulting long-term haemodynamic changes promote liver damage, leading to Fontan-associated liver disease (FALD), in virtually all patients with Fontan circulation. Owing to the lack of a uniform definition of FALD and the competitive risk of other complications developed by Fontan patients, the impact of FALD on the prognosis of these patients is currently debatable. However, based on the increasing number of adult Fontan patients and recent research interest, the European Association for The Study of the Liver and the European Reference Network on Rare Liver Diseases thought a position paper timely. The aims of the current paper are: (1) to provide a clear definition and description of FALD, including clinical, analytical, radiological, haemodynamic, and histological features; (2) to facilitate guidance for staging the liver disease; and (3) to provide evidence- and experience-based recommendations for the management of different clinical scenarios.

Mid-term performance of decellularized equine pericardium in congenital heart surgery.

OBJECTIVE: The aim was to report mid-term performance of decellularized equine pericardium used for repair of various congenital heart defects in the pediatric population. METHODS: A retrospective review of all patients undergoing patch implantation between 2016 - 2020 was performed. Patch quality, surgical handling, hemostasis and early patch-related complications were studied on all patients. Mid-term performance was observed in patients with ≥12 months follow-up and intact patch at discharge (without reoperation/stent implantation). RESULTS: A total of 201 patients with median age of 2.5 years [interquartile range (IQR): 0.6-6.5] underwent 207 procedures at 314 implant locations. The patch was used in following numbers/locations: 171 for pulmonary artery (PA) augmentation, 36 for aortic repair, 35 for septal defect closure, 22 for valvular repair and 50 at other locations. Early/30-day mortality was 6.5%. Early patch-related reoperations/stent implantations occurred in 28 locations (8.9%). No patch-related complications were noted except for bleeding from implant site in three locations (1%). Follow-up ≥ 12 months was available for 132 patients/200 locations. During a median follow-up of 29.7 months [IQR: 20.7-38.3], 53 patch-related reoperations/catheter reinterventions occurred (26.5%) with the majority in PA position (88.7%, 47/53). Overall 12- and 24-months freedom from patch-related reoperation/catheter reintervention per location was 91.5% (95% CI: 86.7%-94.6%) and 85.2% (95% CI: 78.9%-89.6%) respectively. CONCLUSION: Decellularized equine pericardium used for repair of various congenital heart defects showed acceptable mid-term performance. Reoperation/reintervention rates were in a range as observed with other xenogeneic materials previously reported articles, occurring most frequently after PA augmentation.

Functional hepatic deterioration determined by 13C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults.

Background: Despite improved survival a substantial number of Fontan patients eventually develop late failure. Fontan-associated liver disease (FALD) is the most frequent end-organ dysfunction. Although impaired hemodynamics and Fontan failure correlate with FALD severity, no association between hepatic functional metabolic impairment and Fontan hemodynamics has been established. Hypothesis: Metabolic liver function measured by liver maximum function capacity test (LiMAx®) correlates with Fontan hemodynamics and Fontan failure. Methods: From 2020 to 2022, 58 adult Fontan patients [median age: 29.3 years, IQR (12.7), median follow-up time after Fontan operation: 23.2 years, IQR (8.7)] were analyzed in a cross-sectional study. Hemodynamic assessment included echocardiography, cardiopulmonary exercise testing and invasive hemodynamic evaluation. Fontan failure was defined based on commonly applied clinical criteria and our recently composed multimodal Fontan failure score. Results: LiMAx® test revealed normal maximum liver function capacity in 40 patients (>315 µg/h*kg). In 18 patients a mild to moderate impairment was detected (140-314 µg/h*kg), no patient suffered from severe hepatic deterioration (≤ 139 µg/kg*h). Fontan failure was present in 15 patients. Metabolic liver function was significantly reduced in patients with increased pulmonary artery pressure (p = 0.041. r = -0.269) and ventricular end-diastolic pressure (p = 0.033, r = -0.325), respectively. In addition, maximum liver function capacity was significantly impaired in patients with late Fontan failure (289.0 ± 99.6 µg/kg*h vs. 384.5 ± 128.6 µg/kg*h, p = 0.007). Conclusion: Maximum liver function capacity as determined by LiMAx® was significantly reduced in patients with late Fontan failure. In addition, elevated pulmonary artery pressure and end-diastolic ventricular pressure were associated with hepatic functional metabolic impairment.

Virtual treatment planning in three patients with univentricular physiology using computational fluid dynamics-Pitfalls and strategies.

Background: Uneven hepatic venous blood flow distribution (HFD) to the pulmonary arteries is hypothesized to be responsible for the development of intrapulmonary arteriovenous malformations (PAVM) in patients with univentricular physiology. Thus, achieving uniform distribution of hepatic blood flow is considered favorable. However, no established method for the prediction of the post-interventional hemodynamics currently exists. Computational fluid dynamics (CFD) offers the possibility to quantify HFD in patient-specific anatomies before and after virtual treatment. In this study, we evaluated the potential benefit of CFD-assisted treatment planning. Materials and methods: Three patients with total cavopulmonary connection (TCPC) and PAVM underwent cardiovascular magnetic resonance imaging (CMR) and computed tomography imaging (CT). Based on this imaging data, the patient-specific anatomy was reconstructed. These patients were considered for surgery or catheter-based intervention aiming at hepatic blood flow re-routing. CFD simulations were then performed for the untreated state as well as for different surgical and interventional treatment options. These treatment options were applied as suggested by treating cardiologists and congenital heart surgeons with longstanding experience in interventional and surgical treatment of patients with univentricular physiology. HFD was quantified for all simulations to identify the most viable treatment decision regarding redistribution of hepatic blood flow. Results: For all three patients, the complex TCPC anatomy could be reconstructed. However, due to the presence of metallic stent implants, hybrid models generated from CT as well as CMR data were required. Numerical simulation of pre-interventional HFD agreed well with angiographic assessment and physiologic considerations. One treatment option resulting in improvement of HFD was identified for each patient. In one patient follow-up data after treatment was available. Here, the virtual treatment simulation and the CMR flow measurements differed by 15%. Conclusion: The combination of modern computational methods as well as imaging methods for assessment of patient-specific anatomy and flow might allow to optimize patient-specific therapy planning in patients with pronounced hepatic flow mismatch and PAVM. In this study, we demonstrate that these methods can also be applied in patients with complex univentricular physiology and extensive prior interventions. However, in those cases, hybrid approaches utilizing information of different image modalities may be required.

A Multimodal Score Accurately Classifies Fontan Failure and Late Mortality in Adult Fontan Patients.

Objectives: Despite the outstanding success of the Fontan operation, it is a palliative procedure and a substantial number of patients experience late failure of the Fontan circulation. Clinical presentation and hemodynamic phenotypes of Fontan failure are considerably variable. While various parameters have been identified as risk factors for late Fontan failure, a feasible score to classify Fontan failure and possibly allow timely risk stratification is lacking. Here, we explored the possibility of developing a score based on hemodynamic, clinical and laboratory parameters to classify Fontan failure and mortality. Methods: We performed a retrospective study in our cohort of adult Fontan patients from two institutions [n = 198, median follow-up after Fontan 20.3 (IQR 15.6-24.3) years], identifying those patients with clinical Fontan failure (n = 52, 26.3%). Various hemodynamic, echocardiographic, laboratory and clinical data were recorded and differences between patients with and without Fontan failure were analyzed. We composed a Fontan Failure Score containing 15 parameters associated with Fontan failure and/or mortality and assessed its accuracy to discriminate between patients with and without late Fontan failure as well as late mortality and survival. Results: Late failure occurred at a median of 18.2 (IQR 9.1-21.1) years after Fontan completion. Mortality associated with Fontan failure was substantial (25/52, 48.1%) with freedom of death/transplantation/take-down of 64% at 5 years and 36% at 10 years after onset of Fontan failure, respectively. Patients with Fontan failure had a significantly higher median Fontan Failure Score compared to non-failing Fontan patients [8 points (IQR 5-10) vs. 2 points (IQR 1-5), p < 0.001]. The score accurately classifies Fontan failure as well as mortality as assessed with receiver operating characteristic analysis. Area under the curve of the Fontan Failure Score was 0.963 (95% CI 0.921; 0.985, p < 0.001) to discriminate failure and 0.916 (95% CI 0.873; 0.959, p < 0.001) to classify mortality. Conclusion: We have developed an uncomplex yet remarkably accurate score to classify Fontan failure and late mortality in adult Fontan patients. Prospective validation and most likely refinement and calibration of the score in larger and preferably multi-institutional cohorts is required to assess its potential to predict the risk of Fontan failure and late mortality.

Anatomic Repair of Congenitally Corrected Transposition: Reappraisal of Eligibility Criteria.

Several criteria to identify suitable candidates for anatomic repair in congenitally corrected transposition (cc-TGA) have been proposed. The purpose of this study was to critically re-evaluate adequacy of these recommendations in our patient cohort. All cc-TGA patients undergoing anatomic repair between 2010 and 2019 were reviewed. Evaluated eligibility criteria for repair included age ≤ 15 years, LV mass index ≥ 45-50 g/m2, LV mass/volume ratio > 0.9-1.5 and systolic LV to right ventricle pressure ratio > 70-90% among others. Repair failure was defined as postoperative early mortality or LV dysfunction requiring mechanical circulatory support. Twenty-five patients were included (median [interquartile range] age at surgery 1.8 years [0.7;6.6]; median postoperative follow-up 3.2 years [0.7;6.3]). Median preoperative LV ejection fraction was 60% [56;64], indexed LV mass 48.5 g/m2 [43.7;58.1] and LV mass/volume ratio 1.5 [1.1;1.6], respectively. A total of 12 patients (48%) did not meet at least one of the previously recommended criteria, however, all but two patients (92%) experienced favorable early outcome. Of 7 patients (28%) with indexed LV mass < 45 g/m2, 6 were successfully operated. There were two early repair failures (8%) with LV dysfunction: one patient died and one required mechanical circulatory support but recovered well. Surgery was performed successfully in patients with LV mass and volume Z-scores as low as - 2 and - 2.5, respectively. Anatomic correction for cc-TGA can be performed with excellent early outcome and is feasible even in patients with LV mass below previously recommended cut-offs. The use of LV mass and volume Z-scores might help to refine eligibility criteria.

Late Fontan failure in adult patients is predominantly associated with deteriorating ventricular function.

BACKGROUND: The Fontan operation is a palliative procedure and a substantial number of patients eventually experiences late Fontan circulation failure. Previous concepts of Fontan failure implicate increasing pulmonary vascular resistance (PVR) as a key contributor to late circulatory failure. However, data to support this assumption are sparse. We sought to characterize longitudinal hemodynamic and echocardiographic findings in adult failing Fontan patients. METHODS: We performed a retrospective cohort study in adult Fontan patients, identifying patients with Fontan failure. Hemodynamic, echocardiographic and clinical data were recorded. RESULTS: Of 173 adult patients (median follow-up after Fontan 20.2 years [IQR 15.7-24.3]), 48 (28%) showed signs of clinical Fontan failure. Thirty-seven patients (77.1%) exhibited ventricular dysfunction (systolic dysfunction defined by ejection fraction ≤45%, n = 22, or diastolic dysfunction defined by systemic ventricular end-diastolic pressure (SVEDP) ≥12 mmHg, n = 15). Elevated indexed PVR (≥2.5 WU*m2) was only observed in 9 (18.8%) patients. Ejection fraction declined from 60% [IQR 55-65] to 47% [IQR 35-55] during follow-up (p < 0.001). Mean pulmonary artery pressure and SVEDP increased from 11 mmHg [IQR 9-15] to 15 mmHg [IQR 12-18] and from 7 mmHg [IQR 4-10] to 11 mmHg [IQR 8-15] (both p < 0.001), respectively, while indexed PVR did not change significantly (2.1 [IQR 1.1-2.4] vs. 1.7 [IQR 1.1-2.5] WU*m2, p = 0.949). Fontan failure-associated mortality during follow-up was substantial (23/48; 48%). CONCLUSIONS: Systolic and diastolic ventricular dysfunction are frequent features in late Fontan failure in adults, while increases in PVR were rarely observed. The intricate interplay between hemodynamic compromises in Fontan failure deserves further research to optimize treatment strategies and outcome.

Persisting and reoccurring cyanosis after Fontan operation is associated with increased late mortality.

OBJECTIVES: The Fontan operation aims at reducing cyanosis and cardiac volume overload in patients with complex univentricular heart malformations. However, persisting or reoccurring cyanosis is frequently observed. We sought to systematically determine the prevalence and clinical consequences of persisting and secondary cyanosis after Fontan operation. METHODS: A total of 331 Fontan patients, operated between 1984 and 2016 with a median postoperative follow-up of 7.9 (interquartile range 2.6-15.8) years, were studied retrospectively. Cyanosis was defined as transcutaneous oxygen saturation ≤93% at rest measured by pulse oximetry. Prevalence of cyanosis was analysed at 3 different time points (t1 = post-Fontan operation, t2 = post-Fontan cardiac catheterization, t3 = last follow-up) and the association of cyanosis with mortality was examined. RESULTS: Prevalence of cyanosis was 50% at t1 and 39% at t3. Fenestration was patent in 71% and 33% of all cyanotic patients at t1 and t3, respectively. In patients with clinical indication for catheterization (t2; n = 178/331), prevalence of cyanosis was 72%. At t2, patent fenestration (33%), veno-venous collaterals (24%) or both (32%) were present. Thirty-six (11%) patients died during follow-up. In a time-varying multivariable Cox regression analysis, cyanosis was the strongest predictor for late mortality (P < 0.001, hazard ratio 12.2, 95% confidence interval 3.7-40.5). CONCLUSIONS: Prevalence of cyanosis was considerable during long-term follow-up after Fontan operation and-as a surrogate parameter for unfavourable Fontan haemodynamics-is associated with increased late mortality. Accordingly, particular attention should be directed towards the persistence or reoccurrence of cyanosis during follow-up since it may indicate haemodynamic attrition and development of Fontan failure.

Can Left Atrioventricular Valve Reduction Index (LAVRI) Predict the Surgical Strategy for Repair of Atrioventricular Septal Defect?

Despite improved survival, surgical treatment of atrioventricular septal defect (AVSD) remains challenging. The optimal technique for primary left atrioventricular valve (LAVV) repair and prediction of suitability for biventricular approach in unbalanced AVSD are still controversial. We evaluated the ability of our recently developed echocardiographic left atrioventricular valve reduction index (LAVRI) in predicting LAVV reoperation rate and surgical strategy for unbalanced AVSD. Retrospective echocardiographic analysis was available in 352 of 790 patients with AVSD treated in our institution and included modified atrioventricular valve index (mAVVI), ventricular cavity ratio (VCR), and right ventricle/left ventricle (RV/LV) inflow angle. LAVRI estimates LAVV area after complete cleft closure and was analyzed with regard to surgical strategy in primary LAVV repair and unbalanced AVSD. Of the entire cohort, 284/352 (80.68%) patients underwent biventricular repair and 68/352 (19.31%) patients underwent univentricular palliation. LAVV reoperation was performed in 25/284 (8.80%) patients after surgical correction of AVSD. LAVRI was significantly lower in patients requiring LAVV reoperation (1.92 cm2/m2 [IQR 1.31] vs. 2.89 cm2/m2 [IQR 1.37], p = 0.002) and significantly differed between patients receiving complete and no/partial cleft closure (2.89 cm2/m2 [IQR 1.35] vs. 2.07 cm2/m2 [IQR 1.69]; p = 0.002). Of 82 patients diagnosed with unbalanced AVSD, 14 were suitable for biventricular repair (17.07%). mAVVI, LAVRI, VCR, and RV/LV inflow angle accurately distinguished between balanced and unbalanced AVSD and predicted surgical strategy (all p < 0.001). LAVRI may predict surgical strategy in primary LAVV repair, LAVV reoperation risk, and suitability for biventricular approach in unbalanced AVSD anatomy.

Morphologic Alterations Precede Functional Hepatic Impairment as Determined by 13C-Methacetin Liver Function Breath Test in Adult Fontan Patients.

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 (>315 µg/h*kg) and mildly to moderately impaired in 11 patients (140-314 µg/h*kg), while no patient displayed severe hepatic impairment (<139 µg/kg*h). No correlation was found between maximal liver function capacity and hepatic stiffness by TE (r 2 = -0.151; p = 0.388) or the presence of sonographic abnormalities associated with FALD (r 2 = -0.204, p = 0.24). There was, however, an association between maximal liver function capacity and the laboratory parameters bilirubin (r 2 = -0.333, p = 0.009) and γ-glutamyl transferase (r 2 = -0.367; p = 0.021). No correlation was detected between maximal liver function capacity and the severity of FALD (r 2 = -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.

Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation-Initial Results From a Prospective Study.

BACKGROUND: Patients with congenital heart disease (CHD)-including those after Fontan operation-are encouraged to be physically active. AIM: To prospectively determine the effects of an individually adapted, home-based cycle ergometer endurance training in combination with inspiratory muscle training (IMT) in pediatric and adult Fontan patients. We, herein, report the results of the initial 10-months follow-up (phase 1). METHODS: 18 patients (median age 16.5 years; range 10-43 years) completed baseline check-ups, and 4 and 10 months follow-up visits, which each included cardiopulmonary exercise testing (CPET), bodyplethysmography (including measurement of respiratory muscle strength), and a quality of life questionnaire (PedsQL™). The training program consisted of a home-based cycle ergometer endurance training on a "Magbike® AM-5i/3i" (DKN Technology®, Clermont-Ferrand, France) and IMT with a handheld "POWERbreathe® Medic plus" device. Patients performed 90 min of endurance training per week in addition to IMT (30 breaths per day, 6-7 times per week). After the first 4 months, patients underwent additional interval training. RESULTS: After 10 months of training, we observed significant increases in maximum relative workload (W/kg, p = 0.003) and in maximum inspiratory (MIP, p = 0.002) and expiratory (MEP, p = 0.008) pressures. Peak VO2 values did not increase significantly as compared to baseline (p = 0.12) in the entire cohort (n = 18), but reached statistical significance in a subgroup analysis of teenage/adult patients (n = 14; p = 0.03). Patients' subjective quality of life did not show any significant changes after 10 months of training. DISCUSSION: In Fontan patients, an individually adapted home-based training is safe and associated with improvements in some CPET variables. However, these improvements did not translate into an improved QoL after 10 months. With an unclear, but most likely negative, impact of the COVID-19 pandemic, improvements in QoL may become evident during further follow-up (phase 2 of the study).

Mismatch between self-estimated and objectively assessed exercise capacity in patients with congenital heart disease varies in regard to complexity of cardiac defects.

AIM: Regular evaluation of physical capacity takes a crucial part in long-term follow-up in patients with congenital heart disease (CHD). This study aims to examine the accuracy of self-estimated exercise capacity compared to objective assessments by cardiopulmonary exercise testing in patients with CHD of various complexity. METHODS: We conducted a single centre, cross-sectional study with retrospective analysis on 382 patients aged 8-68 years with various CHD who completed cardiopulmonary exercise tests. Peak oxygen uptake was measured. Additionally, questionnaires covering self-estimation of exercise capacity were completed. Peak oxygen uptake was compared to patient's self-estimated exercise capacity with focus on differences between complex and non-complex defects. RESULTS: Peak oxygen uptake was 25.5 ± 7.9 ml/minute/kg, corresponding to 75.1 ± 18.8% of age- and sex-specific reference values. Higher values of peak oxygen uptake were seen in patients with higher subjective rating of exercise capacity. However, oxygen uptake in patients rating their exercise capacity as good (mean oxygen uptake 78.5 ± 1.6%) or very good (mean oxygen uptake 84.8 ± 4.8%) was on average still reduced compared to normal. In patients with non-complex cardiac defects, we saw a significant correlation between peak oxygen uptake and self-estimated exercise capacity (spearman-rho -0.30, p < 0.001), whereas in patients with complex cardiac defects, no correlation was found (spearman-rho -0.11, p < 0.255). CONCLUSION: The mismatch between self-estimated and objectively assessed exercise capacity is most prominent in patients with complex CHD.Registration number at Charité Universitätsmedizin Berlin Ethics Committee: EA2/106/14.

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications.

OBJECTIVES: Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients. METHODS: From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated. RESULTS: There were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up. CONCLUSION: Adult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.

Evaluation of Fontan failure by classifying the severity of Fontan-associated liver disease: a single-centre cross-sectional study.

OBJECTIVES: Fontan-associated liver disease (FALD) is a hallmark of the failing Fontan circulation, but no general classification of FALD severity exists. In this study, we propose a scoring system to grade the severity of FALD and analyse its applicability for evaluation of Fontan failure. METHODS: From 2017 to 2019, a total of 129 successive Fontan patients received a comprehensive hepatic assessment. The FALD score was based on results from laboratory testing, hepatic ultrasound and transient elastography by assigning scoring points for each abnormality detected. FALD severity was graded mild, moderate and severe. Haemodynamic assessment was performed using echocardiography, cardiopulmonary exercise testing and catheterization. RESULTS: FALD was graded absent/ mild, moderate and severe in 53, 26 and 50 patients, respectively. Cardiopulmonary capacity was significantly impaired in patients with severe FALD compared to patients with absent/mild FALD (P = 0.001). The FALD score significantly correlated with pulmonary artery pressure (P = 0.001), end-diastolic ventricular pressure (P < 0.001), hepatic venous pressure (P = 0.004) and wedged hepatic venous pressure (P = 0.009). Fontan failure was present in 21 patients. FALD was graded moderate in 2 and severe in 19 of these patients. The FALD score accurately discriminated patients with and without Fontan failure (sensitivity 90.5%, specificity 71.3%). CONCLUSIONS: The FALD score significantly correlates with impaired Fontan haemodynamics. A cut-off value ≥6.0 has a high diagnostic accuracy in detecting Fontan failure. CLINICAL TRIAL REGISTRY: DRKS (GCTR, German clinical trial registry). CLINICAL TRIAL REGISTRATION NUMBER: DRKS00015039.

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients.

OBJECTIVE: Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality. MATERIAL AND METHODS: We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986-2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed. RESULTS: Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001-0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival. CONCLUSIONS: Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.

Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period.

OBJECTIVES: The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season. METHODS: We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October). RESULTS: Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06). CONCLUSION: Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics.

Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest®, n = 101), liver ultrasound (n = 117) and transient elastography (FibroScan®, n = 61). The median patient age was 16.0 years [IQR 14.2], and the median duration of the Fontan circulation was 10.3 years [IQR 14.7]. Hemodynamic assessment was performed using echocardiography, cardiopulmonary exercise capacity testing and cardiac catheterization. Liver ultrasound revealed hepatic parenchymal changes in 83 patients (70.9%). Severe liver cirrhosis was detectable in 20 patients (17.1%). Median liver stiffness measured by FibroScan® was 27.7 kPa [IQR 14.5], and the median Fibrotest® score was 0.5 [IQR 0.3], corresponding to fibrosis stage ≥ 2. Liver stiffness values and Fibrotest® scores correlated significantly with Fontan duration (P1 = 0.013, P2 = 0.012). Exercise performance was significantly impaired in patients with severe liver cirrhosis (P = 0.003). Pulmonary artery pressure and end-diastolic pressure were highly elevated in cirrhotic patients (P1 = 0.008, P2 = 0.003). Multivariable risk factor analysis revealed Fontan duration to be a major risk factor for the development of FALD (P < 0.001, OR 0.77, CI 0.68-0.87). In the majority of patients, hepatic abnormalities suggestive of FALD were detectable by liver ultrasound, transient elastography and laboratory analysis. The severity of FALD correlated significantly with Fontan duration and impaired Fontan hemodynamics. A detailed hepatic assessment is indispensable for long-term surveillance of Fontan patients.

Bilateral Pulmonary Artery Banding before Norwood Procedure: Survival of High-Risk Patients.

BACKGROUND: This study reports midterm results of high-risk patients with hypoplastic left ventricle treated with initial bilateral pulmonary artery banding (PAB) before secondary Norwood procedure (NP). METHODS: Retrospective study of 17 patients admitted between July 2012 and February 2017 who underwent this treatment strategy because diagnosis or clinical status was associated with high risk for NP. Survival was compared with that of patients who underwent primary NP. RESULTS: Mean Aristotle comprehensive complexity score for NP would have been 19.7 ± 2.6. Risk factors included obstructed pulmonary venous return (n = 9), body weight < 2.5 kg (n = 7), total anomalous pulmonary venous connection (n = 3), and necrotizing enterocolitis (n = 1). Ten patients had a score ≥ 19.5. Early survival after PAB was 82.4% (14/17). NP was performed in 14 patients after improvement of clinical condition at a median age of 56 days and a weight ≥2,500 g. There was no 30-day mortality, but one interstage death. One patient died later after Glenn operation. One-year survival after primary PAB followed by NP was 70.6 ± 11.1%. During the same period, 35 patients with overall lower risk factors underwent primary NP; early postoperative survival and 1-year survival were 88.6 ± 5.4% and 68.6 ± 7.8%, respectively. There was no significant difference in survival between the two groups (p = 0.83) despite higher risk in the secondary Norwood group (p <0.0001). CONCLUSIONS: PAB before NP in high-risk patients constituted salvage management. Primary PAB provided enough time for stabilization and control of most risk factors. It allowed midterm survival equivalent to the survival after primary NP in lower risk neonates.