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Objective We aimed to analyze somatic growth of patients after total cavopulmonary connection (TCPC) as well as to identify factors influencing postoperative catch-up growth. Methods A total of 309 patients undergoing TCPC at 4 years old or less between 1994 and 2021 were included. Weight for age z-score (WAZ) and height for age-z-score (HAZ) at TCPC and at postoperative time between 1 and 3 years were calculated. Factors influencing somatic growth were analyzed. Results Most frequent diagnosis and initial palliation were hypoplastic left heart syndrome (HLHS) (34%) and the Norwood procedure (51%), respectively. Median age and weight at TCPC were 2.0 (IQR: 1.7–2.5) years and 11.3 (10.5–12.7) kg, respectively. Median 519 days after TCPC, a significant increase in WAZ (−0.4 to −0.2, p < 0.001) was observed, but not in HAZ (−0.6 to −0.6, p = 0.38). Older age at TCPC (p < 0.001, odds ratio [OR]: 2.6) and HLHS (p = 0.007, OR: 2.2) were risks for low WAZ after TCPC. Older age at TCPC (p = 0.009, OR: 1.9) and previous Norwood procedure (p = 0.021, OR: 2.0) were risks for low HAZ after TCPC. Previous bidirectional cavopulmonary shunt (BCPS) was a protective factor for both WAZ (p = 0.012, OR: 0.06) and HAZ (p = 0.028, OR: 0.30) at TCPC. Conclusion In patients undergoing TCPC at the age of 4 years or less, a significant catch-up growth was observed in WAZ after TCPC, but not in HAZ. Previous BCPS resulted to be a protective factor for a better somatic development at TCPC. HLHSs undergoing Norwood were considered as risks for somatic development after TCPC. [ABSTRACT FROM AUTHOR]

Right-sided mechanical support of the Fontan circulation by existing devices has been compounded by the cross-sectional design of vena cava anastomosis to both pulmonary arteries. Our purpose was to investigate whether increasing inferior vena cava (IVC) flow with a rotary blood pump in the IVC only in an ovine animal model of Fontan would lead to acceptable superior vena cava (SVC) pressure. To achieve this, a Fontan circulation was established in four female sheep by anastomosing the SVC to the main pulmonary artery (MPA) and by interposing a Dacron graft between the IVC and the MPA. A rotary blood pump was then introduced in the graft, and the effect of incremental flows was observed at increasing flow regimen. Additionally, to stimulate increased pulmonary resistance, the experience was repeated in each animal with the placement of a restrictive band on the MPA distally to the SVC and Dacron graft anastomosis. Circulatory support of IVC flow alone increased the systemic cardiac output significantly, both with and without banding, indicating the feasibility of mechanical support of the Fontan circulation by increasing the flow only in the inferior vena cava. The increase in SVC pressure remained within acceptable limits, indicating the potential effectiveness of this mode of support. The findings suggest that increasing the flow only in the inferior vena cava is a feasible method for mechanical support of the Fontan circulation, potentially leading to an increase in cardiac output with acceptable increases in superior vena cava pressure. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the exercise capacity in patients following Fontan–Kreutzer, Fontan–Björk, and total cavopulmonary connection (TCPC). Methods: Patients who performed exercise capacity tests at least once after the Fontan procedure between 1979 and 2007 were included. Patients after Fontan–Björk procedure were divided into 2 groups according to the pulmonary blood flow (PBF) pattern: patients with pulsatile PBF and those without. Peak oxygen uptake (VO2) was measured and percent-predicted VO2 was calculated. Results: A total of 227 patients were nominated. The types of Fontan procedure included Fontan–Kreutzer in 48 (21.1%) patients, Fontan–Björk in 38 (16.7%); 11 (4.8%) with pulsatile PBF and 27 (11.9%) without pulsatile PBF; and TCPC in 141 (62.1%). Median age at the Fontan procedure was 4.5 years (interquartile range, 2.1-8.2 years). A total of 978 cardiopulmonary exercise tests were performed at median follow-up of 17.7 years (interquartile range, 11.3-23.4 years) postoperatively. Analysis using linear mixed-effects models demonstrated that percent-predicted VO2 was greater in patients with pulsatile PBF after Fontan–Björk compared with patients after other types of Fontan procedure (P

Open in new tab Download slide OBJECTIVES The 2 opposing inflows and 2 outflows in a total cavopulmonary connection make mechanical circulatory support (MCS) extremely challenging. We have previously reported a novel convergent cavopulmonary connection (CCPC) Fontan design that improves baseline characteristics and provides a single inflow and outflow, thus simplifying MCS. This study aims to assess the feasibility of MCS of this novel configuration using axial flow pumps in an in vitro benchtop model. METHODS Three-dimensional segmentations of 12 single-ventricle patients (body surface area 0.5–1.75 m2) were generated from cardiovascular magnetic resonance images. The CCPC models were designed by connecting the inferior vena cava and superior vena cava to a shared conduit ascending to the pulmonary arteries, optimized in silico. The 12 total cavopulmonary connection and their corresponding CCPC models underwent in vitro benchtop characterization. Two MCS devices were used, the Impella RP® and the PediPump. RESULTS MCS successfully and symmetrically reduced the pressure in both vena cavae by >20 mmHg. The devices improved the hepatic flow distribution balance of all CCPC models (Impella RP® P  = 0.045, PediPump P  = 0.055). CONCLUSIONS The CCPC Fontan design provides a feasible MCS solution for a failing Fontan by balancing hepatic flow distribution and symmetrically decompressing the central venous pressure. Cardiac index may also improve with MCS. Additional studies are needed to evaluate this concept for managing Fontan failure. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Effects of aortopulmonary collaterals (APCs) on outcomes after the total cavopulmonary connection (TCPC) are unclear. This study evaluated the incidence of APCs before and after TCPC and analysed the impacts of APCs on adverse outcomes. METHODS A total of 585 patients, who underwent TCPC from 1994 to 2020 and whose preoperative angiographies were available, were included. Pre-TCPC angiograms in all patients were used for the detection of APCs, and post-TCPC angiograms were evaluated in selected patients. Late adverse events included late death, protein-losing enteropathy (PLE) and plastic bronchitis (PB). RESULTS The median age at TCPC was 2.3 (1.8–3.4) years with a body weight of 12 (11–14) kg. APCs were found in 210 patients (36%) before TCPC and in 81 (14%) after TCPC. The closure of APCs was performed in 59 patients (10%) before TCPC, in 25 (4.2%) at TCPC and in 59 (10%) after TCPC. The occurrences of APCs before and after TCPC were not associated with short-term or mid-term mortality. The APCs before TCPC were associated with chylothorax (P  = 0.025), prolonged chest tube duration (P  = 0.021) and PB (P  = 0.008). The APCs after TCPC were associated with PLE (P  < 0.001) and PB (P  < 0.001). With APCs following TCPC, freedom from PLE and PB was lower than without (P  < 0.001, P  < 0.001). CONCLUSIONS APCs before TCPC were associated with chylothorax, prolonged chest tube duration and PB. APCs after TCPC were associated with both PLE and PB. The presence of APCs might affect the lymph drainage system and increase the incidence of chylothorax, PLE and PB. [ABSTRACT FROM AUTHOR]

This study aims to clarify the relation of development of aortopulmonary collateral arteries (APCs) with anatomical sub-types and the shunt types at Norwood procedure in patients with hypoplastic left heart syndrome (HLHS). A total of 140 patients with HLHS who completed 3 staged palliation between 2003 and 2019 were included. Incidence of APCs and corresponding interventions were examined using angiogram by cardiac catheterization, with respect to the anatomical sub-types and shunt types. Totally, APCs were observed in 87 (62%) of the patients; pre-stage II in 32 (23%), pre-stage III in 64 (46%), and after stage III in 40 (29%). The incidence of APCs before stage II was significantly higher in patients with aortic atresia/mitral atresia (AA/MA) compared with other sub-types (P = 0.022). Patients with right ventricle to pulmonary artery conduit (RVPAC) had a higher incidence of APCs originating from the descending aorta, compared with those with modified Blalock-Taussig shunt (20% vs 2%, P = 0.002). Interventions for APCs were performed in 58 (41%) patients; before stage II in 10 (7%), after stage II in 7 (5%), before stage III in 22 (16%), and after stage III in 32 (23%). Patients with AA/MA had more interventions before stage II (P = 0.019), and patients with aortic stenosis/mitral stenosis (AS/MS) had a lower incidence of interventions after stage III (P = 0.047). More than half of the patients with HLHS developed APCs. Before stage II, patients with AA/MA sub-type had a higher incidence of APCs, and those with RVPAC had significantly more APCs from the descending aorta. [Display omitted] [ABSTRACT FROM AUTHOR]

Right-sided mechanical support of the Fontan circulation by existing devices has been compounded by the cross-sectional design of vena cava anastomosis to both pulmonary arteries. Our purpose was to investigate whether increasing inferior vena cava (IVC) flow with a rotary blood pump in the IVC only in an ovine animal model of Fontan would lead to acceptable superior vena cava (SVC) pressure. To achieve this, a Fontan circulation was established in four female sheep by anastomosing the SVC to the main pulmonary artery (MPA) and by interposing a Dacron graft between the IVC and the MPA. A rotary blood pump was then introduced in the graft, and the effect of incremental flows was observed at increasing flow regimen. Additionally, to stimulate increased pulmonary resistance, the experience was repeated in each animal with the placement of a restrictive band on the MPA distally to the SVC and Dacron graft anastomosis. Circulatory support of IVC flow alone increased the systemic cardiac output significantly, both with and without banding, indicating the feasibility of mechanical support of the Fontan circulation by increasing the flow only in the inferior vena cava. The increase in SVC pressure remained within acceptable limits, indicating the potential effectiveness of this mode of support. The findings suggest that increasing the flow only in the inferior vena cava is a feasible method for mechanical support of the Fontan circulation, potentially leading to an increase in cardiac output with acceptable increases in superior vena cava pressure.

Total cavopulmonary connection (Fontan) without using cardiopulmonary bypass (CPB) may be superior to Fontan on CPB. In the experience of many, a Fontan operation without CPB may be associated with a reduced need for inotropic support, improved outcomes in the postoperative phase including faster time to termination of mechanical ventilatory support, reduced intensive care unit stay, lower volume of pleural and peritoneal effusions, and decreased hospital stay, thereby rendering it more cost-effective. However, the operation is technically more difficult to perform than Fontan on CPB and requires significant modifications of surgical technique and alteration in overall management strategy. In this article, an alternative technique of performing total cavopulmonary connection without CPB is described. Its advantages 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.

Open in new tab Download slide OBJECTIVES This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). METHODS In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. RESULTS The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P  = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P  = 0.007, 20 years: P  = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P  < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P  = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P  < 0.001). CONCLUSIONS There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV. [ABSTRACT FROM AUTHOR]

Total cavopulmonary connection (Fontan) without using cardiopulmonary bypass (CPB) may be superior to Fontan on CPB. In the experience of many, a Fontan operation without CPB may be associated with a reduced need for inotropic support, improved outcomes in the postoperative phase including faster time to termination of mechanical ventilatory support, reduced intensive care unit stay, lower volume of pleural and peritoneal effusions, and decreased hospital stay, thereby rendering it more cost-effective. However, the operation is technically more difficult to perform than Fontan on CPB and requires significant modifications of surgical technique and alteration in overall management strategy. In this article, an alternative technique of performing total cavopulmonary connection without CPB is described. Its advantages are briefly discussed.

Total cavopulmonary connection, commonly referred to as the Fontan procedure, is the established destination therapy for univentricular hearts. While the procedure permits a longer survival of these patients, this circulation involves several compromises from normal human circulation and poses several challenges with increasing age after surgery. We present an instance of acute ST-elevation myocardial infarction with Guillain-Barre syndrome in an adult after Fontan palliation and discuss the challenges in management.

Open in new tab Download slide OBJECTIVES To date, it is not known if 16–20-mm extracardiac conduits are outgrown during somatic growth from childhood to adolescence. This study aims to determine total cavopulmonary connection (TCPC) haemodynamics in adolescent Fontan patients at rest and during simulated exercise and to assess the relationship between conduit size and haemodynamics. METHODS Patient-specific, magnetic resonance imaging-based computational fluid dynamic models of the TCPC were performed in 51 extracardiac Fontan patients with 16–20-mm conduits. Power loss, pressure gradient and normalized resistance were quantified in rest and during simulated exercise. The cross-sectional area (CSA) (mean and minimum) of the vessels of the TCPC was determined and normalized for flow rate (mm2/l/min). Peak (predicted) oxygen uptake was assessed. RESULTS The median age was 16.2 years (Q1–Q3 14.0–18.2). The normalized mean conduit CSA was 35–73% smaller compared to the inferior and superior vena cava, hepatic veins and left/right pulmonary artery (all P  < 0.001). The median TCPC pressure gradient was 0.7 mmHg (Q1–Q3 0.5–0.8) and 2.0 (Q1–Q3 1.4–2.6) during rest and simulated exercise, respectively. A moderate–strong inverse non-linear relationship was present between normalized mean conduit CSA and TCPC haemodynamics in rest and exercise. TCPC pressure gradients of ≥1.0 at rest and ≥3.0 mmHg during simulated exercise were observed in patients with a conduit CSA ≤ 45 mm2/l/min and favourable haemodynamics (<1 mmHg during both rest and exercise) in conduits ≥125 mm2/l/min. Normalized TCPC resistance correlated with (predicted) peak oxygen uptake. CONCLUSIONS Extracardiac conduits of 16–20 mm have become relatively undersized in most adolescent Fontan patients leading to suboptimal haemodynamics. [ABSTRACT FROM AUTHOR]

Objective: This study applied preoperative computed tomography angiography (CTA) and computational fluid dynamics (CFD) simulation to predicte and verify the outcome of Y-shaped extracardiac conduits Fontan for functional single ventricle. Methods: Based on the preoperative CTA data of functional single ventricle (FSV), 4 types of spatial structures of extracardiac conduits were designed for 4 experimental groups: Group A, a traditional TCPC group (20 mm); Group B, a diameter-preserving Y-shaped TCPC (YCPC) group (branch 10 mm); Group C, YCPC group (branch 12 mm); and Group D, an area-preserving YCPC group (branch14 mm). Four indicators including flow velocity, pressure gradient (PG), energy efficiency and inferior vena cava (IVC) blood flow distribution were compared. The optimal procedure was applied. The radionuclide lung perfusion, CTA, echocardiography, cardiovascular angiography and catheterization were performed postoperatively. Results: There were the lowest PG, the lowest flow velocity of branches, the highest energy efficiency, and a relatively balanced and stable distribution of IVC flow for group D. Subsequently, the group D, a handcrafted Y-shaped conduit (14 mm) was used for the YCPC procedure. There was no postoperative PG between the conduit and pulmonary artery with normal pressure and resistance. IVC flow was distributed uniformly. Conclusion: CTA-based CFD provided more guidance for the clinical application of TCPC. A comprehensive surgical design could bring good postoperative outcome. Area-preserving YCPC has more advantages than TCPC and the diameter-preserving YCPC. The study effectively improved the feasibility of clinical applications of YCPC. [ABSTRACT FROM AUTHOR]

Background: A small proportion of congenital heart disease cases are united under the diagnosis of a functionally single ventricle. The only management strategy for this group of patients is a series of palliative surgical procedures creating a unique situation of the so-called Fontan circulation. Currently, total cavopulmonary connection (TCPC) is the target intervention in which the superior vena cava is connected directly to the pulmonary artery branch and the inferior vena cava via a valveless intracardiac or extracardiac conduit. The heart is excluded from the pulmonary circulation and the functionally single ventricle is connected only to the systemic circulation. Patients and methods: We aimed to analyse the number of cardiac surgical and catheterisation procedures in a population of patients with TCPC (N = 407) treated in a single paediatric centre covering the whole region of the Czech Republic. Results: The 10-year and 20-year survival probability rates in our study group were 96% and 92%, respectively. A total of 1,128 cardiac surgical procedures, 299 catheterisation procedures, and 738 diagnostic catheterisations were performed. Conclusion: Overall, long-term survival in the population of TCPC patients in the Czech Republic is favourable. However, this group of patients requires a high number of repeated cardiac interventions. [ABSTRACT FROM AUTHOR]

Abstract Aims Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long‐term survival is negatively affected by the absence of sub‐pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease‐specific reference V̇O2peak dataset. Methods and results A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter‐quartile range) of 3.0 (2.0–5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2peak decline correlated negatively with the trend of body mass index z‐score (P = 0.006) and was faster in women than men (P = 0.008). Conclusions Total cavopulmonary connection patients have significantly reduced exercise capacity. The age‐related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

To evaluate the prevalence of veno-venous collaterals (VVCs) after total cavopulmonary connection (TCPC) and analyze their impact on outcomes. Patients undergoing TCPC between 1994 and 2022 were evaluated. VVCs were identified using angiograms of cardiac catheterizations and their impact on outcomes was analyzed. A total of 635 patients were included. Median age at TCPC was 2.3 (interquartile ranges (IQR): 1.8–3.3) years. The most frequent diagnosis was hypoplastic left heart syndrome in 173 (27.2%) patients. Prior bidirectional cavopulmonary shunt was performed in 586 (92.3%) patients at a median age of 5.3 (3.6–9.9) months. VVCs were found in 94 (14.8%) patients at a median of 2.8 (0.1–11.8) years postoperatively. The prevalence of VVCs was similar between the dominant right and left ventricle (14.7 vs. 14.9%, p = 0.967). Mean pulmonary artery pressure (16.2 vs. 16.0 mmHg, p = 0.902), left atrial pressure (5.5 vs. 5.7 mmHg, p = 0.480), transpulmonary gradient (4.0 vs. 3.8 mmHg, p = 0.554) and oxygen saturation (81.4 vs. 82.6%, p = 0.103) before TCPC were similar between patients with and without VVCs. The development of VVCs did not affect survival after TCPC (p = 0.161). Nevertheless, VVCs were a risk for the development of plastic bronchitis (PB, p < 0.001). Interventional closure of VVCs was performed in 60 (9.4%) patients at a median of 8.9 (0.6–15.1) years after TCPC, and improvement of oxygen saturation was observed in 66% of the patients. The prevalence of VVCs after TCPC was 15%. VVCs had no impact on survival following TCPC but were associated with a high prevalence of PB. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: This single-center, retrospective study aims to determine the association between alanine aminotransferase (ALT) and outcomes in pediatric patients undergoing total cavopulmonary connection (TCPC). Methods: In total, 256 pediatric patients undergoing TCPC were included and divided into a normal-ALT group and a high-ALT group. Clinical data were collected for comparisons between groups, and risk factors of high postoperative ALT were identified by univariate and multivariate analysis. A ROC analysis of the predictive value of postoperative ALT was conducted. Results: Compared to the normal-ALT group, the members of the high-ALT group were 1.6 years older and had significantly higher preoperative creatinine and direct bilirubin levels. The high-ALT group had increased fluid overload, higher vasoactive inotropic drug scores, and inferior central venous pressure. The short-term outcomes in the high-ALT group were markedly worse: they suffered a longer duration of mechanical ventilation (MV), had a higher ICU and hospital length of stay (LOS), and higher rates of mortality, infection, and reintubation. Prolonged ICU and hospital LOS, longer MV, and reintubation were identified as independent risk factors for high postoperative ALT. Postoperative ALT was of high value in predicting reintubation, MV, ICU LOS, and mortality. Conclusions: Elevated postoperative ALT levels are associated with poor short-term outcomes in pediatric patients undergoing TCPC. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to clarify the association of the aorto-pulmonary/veno-venous collaterals in patients with plastic bronchitis after the Fontan procedure., Methods: All patients who underwent total cavopulmonary connection from 1994 to 2022 were reviewed. Aorto-pulmonary/veno-venous collaterals were detected using angiograms. The impact of aorto-pulmonary/veno-venous collaterals as well as other variables on the onset of plastic bronchitis were evaluated., Results: A total of 635 patients were included. Plastic bronchitis was observed in 15 (2.4%) patients and the median duration between total cavopulmonary connection and the onset of plastic bronchitis was 1.1 years. Freedom from plastic bronchitis at 5 and 10 years was 97.5 and 96.5%, respectively. As for the association of PB and APCs/VVCs, patients with PB had an increased incidence of post-TCPC APCs (60 vs. 14%, p<0.001) and VVCs (53 vs. 14%, p<0.001). The freedom from plastic bronchitis was the lowest in the patients who were associated with both aortopulmonary collaterals and veno-venous collaterals after total cavopulmonary connection. Aortopulmonary collaterals after total cavopulmonary connection (p=0.002, hazard ratio: 5.729), veno-venous collaterals after total cavopulmonary connection (p=0.016, hazard ratio: 3.527), and chylothorax (p=0.019, hazard ratio: 3.662) were identified as independently associated factors with the onset of plastic bronchitis., Conclusions: The occurrence of post-Fontan aortopulmonary and veno-venous collaterals was significantly associated with the development of plastic bronchitis. Treatment or mitigation of collaterals may represent an opportunity to prevent the development of plastic bronchitis, a remaining source of post-Fontan excess morbidity and mortality., (Copyright © 2024. Published by Elsevier Inc.)

Background: Failing Fontan poses a significant clinical challenge. This study aims to improve patients' outcomes by comprehensively understanding the incidence, pathophysiology, risk factors, and treatment of failing Fontan after total cavopulmonary connection., Methods: We performed a retrospective analysis of patients who underwent total cavopulmonary connection at the German Heart Center Munich between 1994 and 2022. The onset of failing Fontan was defined as: protein-losing enteropathy, plastic bronchitis, NYHA class IV, NYHA class III for > one year, unscheduled hospital admissions for heart failure symptoms, and evaluation for heart transplantation., Results: Among 634 patients, 76 patients presented with failing Fontan, and the incidence was 1.48 per 100 patient-years. Manifestations included protein-losing enteropathy (n = 34), hospital readmission (n = 28), NYHA III (n = 18), plastic bronchitis (n = 16), evaluation for heart transplantation (n = 14), and NYHA IV (n = 4). Risk factors for the onset of failing Fontan were dominant right ventricle (p = 0.010) and higher pulmonary artery pressure before total cavopulmonary connection (p = 0.004). A total of 72 interventions were performed in 59 patients, including balloon dilatation/stent implantation in the total cavopulmonary connection pathway (n = 49) and embolization of collaterals (n = 24). Heart transplantation was performed in four patients. The survival after the onset of Fontan failure was 77% at 10 years. Patients with failing Fontan revealed significantly higher zlog-NT-proBNP levels after onset compared to those without (p = 0.021)., Conclusions: The incidence of Fontan failure was 1.5 per 100 patient years. Dominant right ventricle and higher pulmonary artery pressure before total cavopulmonary connection were significant risks for the onset of failing Fontan. Zlog-NT-proBNP is only a late marker of Fontan failure.

Objective: We have left antegrade pulmonary blood flow (APBF) at bidirectional cavopulmonary shunt (BCPS) only for high-risk patients. This study evaluates the indication and the outcomes of patients with APBF, compared to those without APBF., Methods: Patients with APBF after BCPS were identified among patients who underwent BCPS between 1997 and 2022. Outcomes of patients with and without APBF after BCPS were compared., Results: APBF was open in 38 (8.2%) of 461 patients. Median age (7.7 versus 6.3 months, p = 0.55) and weight (5.6 versus 6.1 kg, p = 0.75) at BCPS were similar in both groups. The most frequent indication for APBF was high pulmonary artery pressure (PAP) in 14 patients, followed by hypoxaemia in 10, and hypoplastic left pulmonary artery in 8. The source of APBF was the pulmonary trunk in 10 patients and the aortopulmonary shunt in 28. Median hospital stay after BCPS was longer (22 versus 14 days, p = 0.018) and hospital mortality was higher (10.5 versus 2.1%, p = 0.003) in patients with APBF compared to those without APBF. However, 448 hospital survivors showed similar survival after discharge following BCPS (p = 0.224). Survival after total cavopulmonary connection (TCPC) was similar between the groups (p = 0.753), although patients with APBF were older at TCPC compared to those without (3.9 versus 2.2 years, p = 0.010)., Conclusion: APBF was left in 8% following BCPS in high-risk patients, mainly due to preoperative high PAP. Hospital survivors after BCPS demonstrated comparable survival in patients with and without APBF. Adding APBF at BCPS might be a useful option for high-risk patients.

Total cavopulmonary connection, commonly referred to as the Fontan procedure, is the established destination therapy for univentricular hearts. While the procedure permits a longer survival of these patients, this circulation involves several compromises from normal human circulation and poses several challenges with increasing age after surgery. We present an instance of acute ST-elevation myocardial infarction with Guillain-Barre syndrome in an adult after Fontan palliation and discuss the challenges in management. [ABSTRACT FROM AUTHOR]

Patients palliated with Total Cavopulmonary Connection have a lower muscle mass and a lower exercise capacity. We assessed calf muscle oxidative metabolism during and after heel raise exercise to exhaustion in young patients with TCPC compared to healthy peers. Near-infrared spectroscopy was used for measuring oxygen metabolism in the medial portion of the gastrocnemius muscle. Forty-three patients with TCPC, aged 6–18 years, were compared with 43 age and sex-matched healthy control subjects. Subgroups were formed to include children (6–12 years) and adolescents (13–18 years) to determine if these age groups influenced the results. During exercise, for the patients compared to controls there was a lower increase in deoxygenated hemoglobin (oxygen extraction) (5.13 ± 2.99au vs. 7.75 ± 4.15au, p = 0.001) and a slower rate of change in total hemoglobin (blood volume) (0.004 ± 0.015au vs 0.016 ± 0.01au, p = 0.001). Following exercise, patients exhibited a slower initial increase in tissue oxygenation saturation index (0.144 ± 0.11au vs 0.249 ± 0.226au, p = 0.007) and a longer half-time to maximum hyperemia (23.7 ± 11.4 s vs 16.8 ± 7.5 s, p = 0.001). On the subgroup level, the adolescents differed compared to healthy peers, whereas the children did not. Young patients with TCPC had impaired oxidative metabolism during exercise and required a longer time to recover. In that the differences were seen in the adolescent group and not in the children group may indicate a declining function with age. [ABSTRACT FROM AUTHOR]

Aims: Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long‐term survival is negatively affected by the absence of sub‐pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease‐specific reference V̇O2peak dataset. Methods and results: A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter‐quartile range) of 3.0 (2.0–5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2peak decline correlated negatively with the trend of body mass index z‐score (P = 0.006) and was faster in women than men (P = 0.008). Conclusions: Total cavopulmonary connection patients have significantly reduced exercise capacity. The age‐related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients. [ABSTRACT FROM AUTHOR]

Abstract Objective(s) The Fontan procedure is a common palliative intervention for sufferers of single ventricle congenital heart defects that results in an anastomosis of the venous return to the pulmonary arteries called the total cavopulmonary connection (TCPC). In patients with palliated single ventricular heart defects, the Fontan circulation passively directs systemic venous return to the pulmonary circulation in the absence of a functional sub-pulmonary ventricle. Therefore, the Fontan circulation is highly dependent on favourable flow and energetics, and minimal energy loss is of great importance. The majority of in vitro studies, to date, employ a rigid TCPC model. Recently, few studies have incorporated flexible TCPC models, without the inclusion of commercially available conduits used in these surgical scenarios. Method The methodology set out in this study successfully utilizes patient-specific phantoms along with the corresponding flowrate waveforms to characterise the flow haemodynamic performance of extracardiac Gore-Tex conduits. This was achieved by comparing a rigid and flexible TCPC models against a flexible model with an integrated Gore-Tex conduit. Results The flexible model with the integrated Gore-Tex graft exhibited greater levels of energy losses when compared to the rigid walled model. With this, the flow fields showed greater levels of turbulence in the complaint and Gore-Tex models compared to the rigid model under ultrasound analysis. Conclusion This study shows that vessel compliance along with the incorporation of Gore-Tex extracardiac conduits have significant impact on the flow haemodynamics in a patient-specific surgical scenario.

Background: Chylothorax is a severe complication after total cavopulmonary connection (TCPC) in children. This study was performed to evaluate the incidence, risk factors, and short- and long-term prognosis for chylothorax.Methods: We retrospectively reviewed the electronic records of patients who underwent TCPC between January 2008 and December 2020 in Fuwai Hospital. Patients were divided into two groups based on the occurrence of post-operative chylothorax. Univariate and multivariate analyses were performed to identify risk factors, and long-term survival was estimated by the Kaplan–Meier method.Results: Of 386 patients included in our study, chylothorax occurred in 60 patients (15.5%). Compared with the non-chylothorax group, the prevalence of prolonged intensive care unit (ICU) stay (p = 0.000) and post-operative hospital stay (p = 0.000) were greater in patients with chylothorax. Post-operative adverse events in terms of infection (p = 0.002), ascites (p = 0.001), prolonged pleural effusion (p = 0.000), and diaphragmatic paralysis (p = 0.026) were more frequent in chylothorax patients. The median follow-up duration was 4.0 (2.0, 6.8) years. The chylothorax group had significantly lower survival rates at 1 year (92.4 vs. 99.3%, p < 0.001) and 10 years (84.6 vs. 91.6%, p < 0.001), respectively. Having a right dominant ventricle [odds ratio (OR) = 2.711, 95% confidence interval (CI) = 1.285–5.721, p = 0.009] and a higher peak central venous pressure (CVP) on post-operative day (POD) 0 (OR = 1.116, 95% CI = 1.011–1.233, p = 0.030) were the risk factors for the development of chylothorax after TCPC operation.Conclusion: The incidence of chylothorax in patients undergoing TCPC is lower than previously reported but is associated with poor early- and long-term survival. Having a right dominant ventricle and a higher peak CVP on POD 0 are the risk factors for chylothorax after TCPC operation.

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The aim of this study was to identify the impact of bilateral bidirectional Glenn (BBDG) anastomosis on staged Fontan strategy and late Fontan circulation. METHODS Of 267 patients who underwent bidirectional Glenn prior to Fontan completion between 1989 and 2013, 62 patients (23%) who underwent BBDG were enrolled in this study. Age at operation was 0.84 years (25th–75th percentile: 0.58–1.39). Thirty-three patients had heterotaxy syndrome (53%). The mean follow-up period was 12.7 ± 8.1 (max. 30.6) years. RESULTS The overall survival rate at 15 years was 73%. Although 49 patients (79.0%) went on to Fontan completion, 12 patients (19.4%) died without achieving it. Thrombus formation and poor development in a central pulmonary artery were not observed, but obstruction of the superior vena cava (SVC) occurred in 8 patients (13%), mainly those with right atrial isomerism (P  =   0.037). SVC obstruction was not, however, a risk factor for mortality (P  =   0.097) or Fontan completion (P  =   0.41). The shape of BBDG anastomosis, symmetricity of pulmonary blood flow, impingement of caval blood flow returning from the superior and inferior vena cavae or coexisting interrupted inferior vena cava with azygos or hemi-azygos continuation did not affect late Fontan outcomes, such as overall survival, freedom from protein-losing enteropathy or pulmonary arterio-venous malformation rates. CONCLUSIONS SVC obstruction after BBDG frequently occurred, mainly in patients with right atrial isomerism; however, its direct impact on prognosis or achieving Fontan completion was not identified. Once Fontan circulation was established, the arrangement of the Fontan pathway did not affect late Fontan outcomes. [ABSTRACT FROM AUTHOR]

Aims We evaluated pulmonary arterial (PA) vasa vasorum (VV) in Fontan candidate patients with a novel three-dimensional (3D) imaging technique using optical coherence tomography (OCT). Methods and results This prospective study assessed the development of adventitial VV in the distal PA of 10 patients with bidirectional Glenn circulation (BDG group, 1.6 ± 0.3 years) and Fontan circulation (Fontan group, 3.3 ± 0.3 years), and in 20 children with normal PA haemodynamics and morphology (Control group, 1.5 ± 0.3 years). We assessed the PA VV with two-dimensional (2D) cross-sectional, multi-planar reconstruction (MPR), and volume rendering (VR) imaging. VV development was evaluated by the VV area/volume ratio, defined as the VV area/volume divided by the adventitial area/volume. Compared to the control group, the observed VV number and diameter on 3D images of MPR and VR were significantly higher, and curved and torturous-shaped VV were more frequently observed in the BDG and Fontan groups (P  < 0.001, all). The median VV volume ratio was significantly greater in the BDG than in the control group (3.38% vs. 0.61%; P  < 0.001). Although the VV volume ratio decreased significantly after the Fontan procedure (2.64%, P  = 0.005 vs. BDG), the ratio remained higher than in the control group (P  < 0.001 vs. control). Conclusion 3D OCT imaging is a novel method that can be used to evaluate adventitial PA VV and may provide pathophysiological insight into the role of the PA VV in these patients. [ABSTRACT FROM AUTHOR]

Background Protein‐losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast‐enhanced magnetic resonance lymphangiography (IH‐DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH‐DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single‐center retrospective study of IH‐DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH‐DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH‐DCMRL than patients without PLE (P

An anomalous left hepatic venous (LHV) connection is an extremely rare cardiac malformation, and left hepatic venous route abnormalities not associated with other cardiac lesions do not require surgical treatment because they are physiologically benign. However, when venous route abnormalities exist with associated cardiac lesions, the conduct of the cardiac surgical repair must accommodate the abnormal venous anatomy, especially in total cavopulmonary connection patients. Herein, we present a rare case of a 7-year-old Chinese boy about 1 year post bilateral superior vena cava pulmonary anastomosis who presented with severe cyanosis and was referred to our department. However, the patient showed an unexpected gradual decrease in blood oxygen saturation to 60–70% after the extracardiac total cavopulmonary connection (ETCPC) operation. Emergency echocardiography and computed tomography confirmed that the LHV entered the right atrium. Subsequently, the patient undergone completion of a staged TCPC with intra-atrial tunnel technique. This illustrative report highlights the essence of improving the preoperative accurate diagnosis to avoid unplanned reoperation in China, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. Failure to identify anomalous LHV connection, in this case, will delay effective treatment past the optimal treatment time.

Congenital heart disease is the most common birth defect and functionally univentricular heart defects represent the most severe end of this spectrum. The Fontan circulation provides an unique solution for single ventricle patients, by connecting both caval veins directly to the pulmonary arteries. As a result, the pulmonary circulation in Fontan palliated patients is characterized by a passive, low-energy circulation that depends on increased systemic venous pressure to drive blood toward the lungs. The absence of a subpulmonary ventricle led to the widely believed concept that respiration, by sucking blood to the pulmonary circulation during inspiration, is of great importance as a driving force for antegrade blood flow in Fontan patients. However, recent studies show that respiration influences pulsatility, but has a limited effect on net forward flow in the Fontan circulation. Importantly, since MRI examination is recommended every 2 years in Fontan patients, clinicians should be aware that most conventional MRI flow sequences do not capture the pulsatility of the blood flow as a result of the respiration. In this review, the unique flow dynamics influenced by the cardiac and respiratory cycle at multiple locations within the Fontan circulation is discussed. The impact of (not) incorporating respiration in different MRI flow sequences on the interpretation of clinical flow parameters will be covered. Finally, the influence of incorporating respiration in advanced computational fluid dynamic modeling will be outlined.

Background: This single-center, retrospective study aims to determine the association between alanine aminotransferase (ALT) and outcomes in pediatric patients undergoing total cavopulmonary connection (TCPC). Methods: In total, 256 pediatric patients undergoing TCPC were included and divided into a normal-ALT group and a high-ALT group. Clinical data were collected for comparisons between groups, and risk factors of high postoperative ALT were identified by univariate and multivariate analysis. A ROC analysis of the predictive value of postoperative ALT was conducted. Results: Compared to the normal-ALT group, the members of the high-ALT group were 1.6 years older and had significantly higher preoperative creatinine and direct bilirubin levels. The high-ALT group had increased fluid overload, higher vasoactive inotropic drug scores, and inferior central venous pressure. The short-term outcomes in the high-ALT group were markedly worse: they suffered a longer duration of mechanical ventilation (MV), had a higher ICU and hospital length of stay (LOS), and higher rates of mortality, infection, and reintubation. Prolonged ICU and hospital LOS, longer MV, and reintubation were identified as independent risk factors for high postoperative ALT. Postoperative ALT was of high value in predicting reintubation, MV, ICU LOS, and mortality. Conclusions: Elevated postoperative ALT levels are associated with poor short-term outcomes in pediatric patients undergoing TCPC.

Background: Acute kidney injury (AKI) after cardiac surgery contributes to adverse outcomes. We aimed to assess the incidence and identify the predictors for persistent AKI after total cavopulmonary connection (TCPC) surgery.Methods: A retrospective study, including 465 children undergoing TCPC surgery from 2010 to 2019, was conducted. We used pRIFLE criteria to define AKI and defined persistent AKI as AKIs occurring between post-operative day1 (POD1) and POD3 and sustaining at least on POD7. Univariate and multivariate logistic regressions were applied to analyze the predictors for persistent AKI.Results: A total of 35.3% patients developed AKI between POD1 to POD3 and 15.5% patents had persistent AKI after TCPC. Patients with persistent AKI had prolonged mechanical ventilation and ICU stay, and had higher rates of renal replacement treatment and reintubation, which was associated with higher hospitalization costs and in-hospital mortality. The independent predictors for persistent AKI were peripheral oxygen saturation (SpO2) upon admission, intraoperative fluid balance, POD0 maximal lactic acid, renal perfusion pressure (RPP), POD0 estimated glomerular filtration rate and POD0 total bilirubin. The areas under receiver operating characteristic curve (AUC) in the total cohort and the subgroup undergoing TCPC surgery after 2017 were 0.75 (95% CI, 0.66–0.82) and 0.87 (95% CI, 0.77–0.97), respectively. The acceptable AUCs (nearly 0.7) were achieved in other 5 subgroups and good calibration ability (p ≥ 0.05) were achieved in the total cohort and all six subgroups.Conclusions: Persistent AKI after TCPC was common and strongly associated with poorer in-hospital outcomes in Chinese pediatric patients. Six perioperative variables, including SpO2, intraoperative fluid balance, POD0 maximal lactic acid, RPP, POD0 moderate-to-severe kidney injury and POD0 total bilirubin, were identified as independent predictors for persistent AKI. Our findings may help to perform an early risk stratification for these vulnerable patients and improve their outcomes.

Arrhythmia is related to heart rate variability (HRV), which reflects the autonomic nervous regulation of the heart. We hypothesized that autonomic nervous ganglia, located at the junction of the superior vena cava's entrance to the heart, may be affected during the bidirectional Glenn procedure (BDG), resulting in reduced HRV. We aimed to investigate changes in heart rate and HRV in a cohort of children with univentricular heart defects, undergoing stepwise surgery towards total cavopulmonary connection (TCPC), and compare these results with healthy controls. Twenty four hours Holter-ECG recordings were obtained before BDG (n = 47), after BDG (n = 47), and after total cavopulmonary connection (TCPC) (n = 45) in patients and in 38 healthy controls. HRV was analyzed by spectral and Poincaré methods. Age-related z scores were calculated and compared using linear mixed effects modeling. Total HRV was significantly lower in patients before BDG when compared to healthy controls. The mean heart rate was significantly reduced in patients after BDG compared to before BDG. Compared to healthy controls, patients operated with BDG had significantly reduced heart rate and reduced total HRV. Patients with TCPC showed reduced heart rate and HRV compared with healthy controls. In patients after TCPC, total HRV was decreased compared to before TCPC. Heart rate was reduced after BDG procedure, and further reductions of HRV were seen post-TCPC. Our results indicate that autonomic regulation of cardiac rhythm is affected both after BDG and again after TCPC. This may be reflected as, and contribute to, postoperative arrhythmic events. [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.

With the surgical improvement of congenital heart disease, Fontan operation has been applied to many complicated patients in recent years. This is the first report of a child with panhypopituitarism who underwent Fontan operation. A 5-year-old boy was scheduled for Fontan operation. He previously underwent Blalock-Taussig shunt and bidirectional Glenn operations for univentricular heart with double-outlet right ventricle and pulmonary atresia. He was receiving hydrocortisone and 1-desamino-8-D-arginine vasopressin (DDAVP) for panhypopituitarism secondary to removal of craniopharyngioma performed at the age of three years. Although urine output and serum sodium concentration were adequately controlled by adjustment of vasopressin infusion rate during surgery, massive pleural effusions and ascites developed postoperatively, which required several days for control by adjusting the dose of oral DDAVP and normalize the serum sodium level. Intraoperative management of Fontan operation for a patient with panhypopituitarism was controllable by appropriate hormone replacement. However, postoperative fluid management was complicated by the clinical features of panhypopituitarism and Fontan physiology. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study is to evaluate the incidence and outcomes regarding tachyarrhythmia in patients after total cavopulmonary connection., Methods: A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 at our institution was performed. Incidence of tachyarrhythmia was depicted, and results after onset of tachyarrhythmia were evaluated. Factors associated with the onset of tachyarrhythmia were identified., Results: A total of 52 (8%) patients presented with tachyarrhythmia that required medical therapy. Onset during hospital stay was observed in 27 patients, and onset after hospital discharge was observed in 32 patients. Freedom from late tachyarrhythmia following total cavopulmonary connection at 5, 10, and 15 years was 97, 95, and 91%, respectively. The most prevalent late tachyarrhythmia was atrial flutter (50%), followed by supraventricular tachycardia (25%) and ventricular tachycardia (25%). Direct current cardioversion was required in 12 patients, and 7 patients underwent electrophysiological study. Freedom from Fontan circulatory failure after onset of tachyarrhythmia at 10 and 15 years was 78% and 49%, respectively. Freedom from occurrence of decreased ventricular systolic function after the onset of tachyarrhythmia at 5 years was 85%. Independent factors associated with late tachyarrhythmia were dominant right ventricle (hazard ratio, 2.52, p = 0.02) and weight at total cavopulmonary connection (hazard ratio, 1.03 per kilogram; p = 0.04). Type of total cavopulmonary connection at total cavopulmonary connection was not identified as risk., Conclusions: In our large cohort of 620 patients following total cavopulmonary connection, the incidence of late tachyarrhythmia was low. Patients with dominant right ventricle and late total cavopulmonary connection were at increased risk for late tachyarrhythmia following total cavopulmonary connection.

Aims This study aims to retrospectively quantify skeletal muscle mass from cardiovascular imaging studies in total cavopulmonary connection (TCPC) patients and to correlate calculated muscle mass with clinical outcomes. Materials and methods Ninety-one TCPC patients at a mean age of 24.0 ±5.5 years (37 women; 40.7%) who underwent chest computed tomography (CT) or cardiac magnetic resonance imaging (MRI) as part of their follow-up were identified in a single-center database. The cross-sectional skeletal muscle index (SMI) at the Th4 and Th12 levels was calculated from CT images, and the dorsal skeletal muscle area (SMA) at the Th12 level was measured from an MRI. Results Calculated SMI at Th12 level was 38.0 (34.5; 42.0) cm 2 .m -2 or 89.6 (81.9; 101.6) % of predicted values. The median follow-up from CT was 5.9 (3.1; 8.5) years, and the composite endpoint (death N=5, heart transplant N=6) was reached in a total of 11 (26.8%) patients. Patients with SMI (Th12) less than 90% of predicted values had a hazard ratio of 5.8 (95% CI: 1.2; 28.3) (p=0.03) for endpoint achievement. In the MRI group, dorsal SMA at the Th12 level was 27.6 ±5.1 cm 2 in men and 20.0 ±5.8 cm 2 in women. Correlations were found between SMA/kg and peak oxygen uptake (VO 2  peak) (r=0.48, p=0.0005) and fat-free mass (r=0.63, p<0.0001), respectively. Conclusions A low SMI at the Th12 level was associated with a higher risk of death or cardiac transplantation. Evaluation of skeletal muscle mass using cardiovascular imaging methods allows rapid identification of individuals at risk of sarcopenia., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Illinger et al.)

Background: Brady-arrhythmia requiring pacemaker implantation remains one of the Fontan-specific complications before and after total cavopulmonary connection., Methods: A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 was performed to evaluate the incidence of brady-arrhythmia and the outcomes after pacemaker implantation. Factors associated with the onset of brady-arrhythmia were identified., Results: A total of 52 patients presented with brady-arrhythmia and required pacemaker implantation. Diagnosis included 16 sinus node dysfunctions, 29 atrioventricular blocks, and 7 junctional escape rhythms. Pacemaker implantation was performed before total cavopulmonary connection (n = 16), concomitant with total cavopulmonary connection (n = 8), or after total cavopulmonary connection (n = 28, median 1.8 years post-operatively). Freedom from pacemaker implantation following total cavopulmonary connection at 10 years was 92%. Twelve patients needed revision of electrodes due to lead dysfunction (n = 9), infections (n = 2), or dislocation (n = 1). Lead energy thresholds were stable, and freedom from pacemaker lead revision at 10 years after total cavopulmonary connection was 78%. Congenitally corrected transposition of the great arteries (odds ratio: 6.6, confidence interval: 2.0-21.5, p = 0.002) was identified as a factor associated with pacemaker implantation before total cavopulmonary connection. Pacemaker rhythms for Fontan circulation were not a risk factor for survival (p = 0.226), protein-losing enteropathy/plastic bronchitis (p = 0.973), or thromboembolic complications (p = 0.424)., Conclusions: In our cohort of patients following total cavopulmonary connection, freedom from pacemaker implantation at 10 years was 92% and stable atrial and ventricular lead energy thresholds were observed. Congenitally corrected transposition of the great arteries was at increased risk for pacemaker implantation before total cavopulmonary connection. Having a pacemaker in the Fontan circulation had no adverse effect on survival, protein-losing enteropathy/plastic bronchitis, or thromboembolic complications.

Objectives: The 18- and 16-mm conduits in extracardiac total cavopulmonary connection (eTCPC) were reported to be optimal based on energy loss and flow stagnation at the relatively early phase. However, because the artificial conduit lacks growth potential, we have recently encountered some cases in which the conduit needs to be changed several years after eTCPC. These cases prompted us to reconsider the surgical strategy for eTCPC., Methods: We reviewed our 20-year single-centre experience with eTCPC patients (n = 256) to compare the 18-mm conduit (n = 195) and 16-mm conduit (n = 61) in terms of mortality and morbidity., Results: The 16-mm conduit was used significantly more frequently in patients whose main chamber was right ventricle (P < 0.001). There was also a significant difference in preoperative inferior vena cava pressure (P = 0.008). There was a significant difference in the actuarial rate of freedom from late-occurring complications, including mortality, between the 2 groups (P = 0.003). There was a significant difference in the actuarial rate of reoperation-free survival (P = 0.042); however, there was no significant difference in resurgical intervention for the conduit (P = 0.333). In multivariate analysis, preoperative inferior vena cava pressure was an independent predictor for late-occurring complications (hazard ratio 1.19; P = 0.026). Conduit size (18 or 16 mm) itself was not an independent predictive factor for late-occurring complications (P = 0.690)., Conclusions: The mid-term clinical outcomes in patients who underwent eTCPC were excellent with low mortality. Preoperative inferior vena cava pressure was the only predictive risk factor for postoperative morbidity, and the 16 mm conduit was not predictive thereof., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

In order to improve the surgical treatment of the congenital heart disease patient with single ventricle defect, two axial flow blood pumps, one with diffuser and the other without diffuser, were designed and virtually implanted into an idealized total cavopulmonary connection (TCPC) model to form two types of Pump-TCPC physiological structure. Computational fluid dynamics (CFD)simulationswere performed to analyze the variations of the hemodynamic characteristics, such as flow field, wall shear stress (WSS), oscillatory shear index (OSI), relative residence time (RRT), between the two Pump-TCPC models. Numerical results indicate that the Pump-TCPC with diffuser has better flow field stability, less damage on endothelial cell of vessel wall, and lower risk of vascular injury and thrombosis formation than that without diffuser. [ABSTRACT FROM AUTHOR]

Background: Prolonged pleural effusion (PPE) contributes to adverse outcomes after total cavopulmonary connection (TCPC) completion. We aimed to identify risk factors for PPE following TCPC surgery.Methods: We studied a retrospective cohort of 525 who undergoing TCPC surgery from 2010 to 2019. We defined PPE as the duration of pleural effusion exceeding 14 days. Logistic regression was applied to identify risk factors for PPE and Cox regression was used to identify risk factors for predicting the duration of pleural effusion. The impacts of PPE on the short-term outcomes were evaluated.Results: The rate of PPE was 27.4% in our study and independent risk factors for PPE included: young age, no fenestration, low postoperative total protein, prolonged mechanical ventilation and chylothorax. These predictors were also achieved in the Cox regression for predicting the duration of pleural effusion. The applicability of the model was acceptable in different subgroups, which derived from the total cohort. Patients with PPE were associated with more renal replacement treatment, longer length of ICU and hospital stay, more hospitalization costs and a higher rate of in-hospital mortality.Conclusions: PPE in our study occurs at a relatively lower rate than that reported in previous studies and patients with PPE was associated with higher rate of in-hospital mortality when compared to patients without PPE. Young age, no fenestration, low postoperative total protein, prolonged mechanical ventilation, and chylothorax were identified as independent risk factors to predict PPE. A preventive strategy that targets the identified risk factors to reduce the incidence of PPE following TCPC surgery could be beneficial for in-hospital outcomes, and the model needs further validation before its application.

Extracardiac total cavopulmonary connection (EC TCPC) is the end stage of hemodynamic correction in patients with a functional single ventricle (SV). The dilatation of the aortic root and proximal portion of the ascending aorta (Ao) has a progressive nature in this cohort of patients. The purpose of this article is to analyse the dynamics of root and proximal Ao dilatation in patients after EC TCPC in the long-term period. Material and methods. In the period from 2005 to 2016, 137 patients with hemodynamic SV underwent EC TCPC surgery, with hospital mortality being 2.3% (n = 3). Pre- and postoperative data of the aortic root and ascending Ao dilatation have been studied in the group of 116 (84.7%) patients. Result and discussion. According to the statistical evidence, Ao dilatation has been observed mainly in older patients (older than 10 years) at the time of hemodynamic correction end stage, with longer exposure to chronic hypoxia, respectively. Transposition of the great arteries and/or pulmonary artery atresia with the left type of SV proved to be major risk factors. Conclusions. Aortic root and ascending Ao dilatation has a progressive nature in patients with functional SV after the end stage of hemodynamic correction. The patient’s age, initial SV anatomy (anatomy of the great arteries and SV morphological type) are predictors of Ao dilatation.

Simulation of the human body normal operating conditions is the important issue in the engineering process of designing biomedical devices intended for implantation. As an example of such process the Fontan procedure aims to support the human body function. It is a standard palliative treatment method for patients with a functionally univentricular heart. Nevertheless, this procedure has significant drawbacks. For instance, overload of the only functional ventricle leads to the inevitability of the heart transplantation. Herein, we perform simulation and experimental characteristics of the pediatric total cavopulmonary connection (TCPC) influence on the blood flow. We investigate and design three different types of pediatric TCPC configurations; we detect fluorescent particles via a high-speed camera in order to analyze distribution of the blood flow velocity modulus in different types of TCPCs. Finally, we evaluate hydraulic power losses for various cases. This work is particularly relevant for the improvement of existing TCPCs quality that can extend the life of Fontan patients. Moreover, it also applies to the reduction of morbidity and mortality of the patients waiting for a heart transplantation. [ABSTRACT FROM AUTHOR]

Objective(s): The Fontan procedure is a common palliative intervention for sufferers of single ventricle congenital heart defects that results in an anastomosis of the venous return to the pulmonary arteries called the total cavopulmonary connection (TCPC). In patients with palliated single ventricular heart defects, the Fontan circulation passively directs systemic venous return to the pulmonary circulation in the absence of a functional sub-pulmonary ventricle. Therefore, the Fontan circulation is highly dependent on favourable flow and energetics, and minimal energy loss is of great importance. The majority of in vitro studies, to date, employ a rigid TCPC model. Recently, few studies have incorporated flexible TCPC models, without the inclusion of commercially available conduits used in these surgical scenarios.Method: The methodology set out in this study successfully utilizes patient-specific phantoms along with the corresponding flowrate waveforms to characterise the flow haemodynamic performance of extracardiac Gore-Tex conduits. This was achieved by comparing a rigid and flexible TCPC models against a flexible model with an integrated Gore-Tex conduit.Results: The flexible model with the integrated Gore-Tex graft exhibited greater levels of energy losses when compared to the rigid walled model. With this, the flow fields showed greater levels of turbulence in the complaint and Gore-Tex models compared to the rigid model under ultrasound analysis.Conclusion: This study shows that vessel compliance along with the incorporation of Gore-Tex extracardiac conduits have significant impact on the flow haemodynamics in a patient-specific surgical scenario. [ABSTRACT FROM AUTHOR]