Total Cavopulmonary Connection Research Articles

Relationship of Aortopulmonary Collaterals and Pulmonary Artery Development During Staged Single Ventricle Reconstruction.

To evaluate the relationship of aortopulmonary collaterals and the development of central pulmonary arteries during staged palliation. A total of 287 patients, who underwent staged palliation with bidirectional cavopulmonary shunt and total cavopulmonary connection between 2008 and 2019, had available angiography. Pulmonary artery index was calculated using pulmonary angiography as described by Nakata and colleagues. Aortopulmonary collaterals were observed in 47 (16%) patients at stage II palliation, in 131 (46%) at total cavopulmonary connection, and afterwards in 49 (7%). The interventional closure of aortopulmonary collaterals was performed before stage II in 12 (4%) patients, before Fontan completion in 38 (13%), and afterwards in 39 (14%). Presence of aortopulmonary collaterals before stage II was not associated with the pulmonary artery index (129 vs. 150 mm2/m2, p = 0.176) at stage II. In contrast, aortopulmonary collaterals before the Fontan completion were associated with lower pulmonary artery index (154 vs. 172 mm2/m2, p = 0.005), and right pulmonary artery index (99 vs. 106 mm2/m2, p = 0.006). Patients who underwent interventional closure of aortopulmonary collaterals before total cavopulmonary connection had lower pulmonary artery index (141 vs. 169 mm2/m2, p < 0.001), lower right pulmonary artery index (93 vs. 106 mm2/m2, p = 0.007), and left pulmonary artery index (54 vs. 60 mm2/m2, p = 0.013) at Fontan completion. The presence of aortopulmonary collaterals did not influence pulmonary artery size by the time of stage II. However, presence of aortopulmonary collaterals was associated with under-developed pulmonary arteries at Fontan completion, especially in patients who needed interventional closure of aortopulmonary collaterals.

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics. The effect of age-related changes in caval inflows on HFD was evaluated using cardiac magnetic resonance data and patient-specific computational fluid dynamics modeling. SVC and IVC flow from 414 patients with Fontan were collected to establish a relationship between SVC:IVC flow ratio and age. Computational fluid dynamics modeling was performed in 60 (30 extracardiac and 30 lateral tunnel) patient models to quantify the HFD that corresponded to patient ages of 3, 8, and 15 years, respectively. SVC:IVC flow ratio inverted at ≈8 years of age, indicating a clear shift to lower body flow predominance. Our data showed that variation of HFD in response to age-related changes in caval inflows (SVC:IVC, 2, 1, and 0.5 corresponded to ages, 3, 8, and 15+, respectively) was not significant for extracardiac but statistically significant for lateral tunnel cohorts. For all 3 caval inflow ratios, a positive correlation existed between the IVC flow distribution to both the lungs and the HFD. However, as the SVC:IVC ratio changed from 2 to 0.5 (age, 3-15+) years, the correlation's strength decreased from 0.87 to 0.64, due to potential flow perturbation as IVC flow momentum increased. Our analysis provided quantitative insights into the impact of the changing caval inflows on Fontan's long-term HFD, highlighting the importance of SVC:IVC variations over time on Fontan's long-term hemodynamics. These findings broaden our understanding of Fontan hemodynamics and patient outcomes.

Cardiovascular Outcomes in Fontan Patients With Right vs Left Univentricular Morphology: A Multicenter Study

BackgroundThere is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle. ObjectivesThe purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV. MethodsThe Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America. All components of the composite outcome, that is, atrial arrhythmias, thromboembolic events, cardiac transplantation, and death, were reviewed and classified by a blinded adjudicating committee. Time-to-event analyses were performed that accounted for competing risks. ResultsA total of 384 patients were followed for 10.5 ± 5.9 years. The composite outcome occurred in 3.7 vs 1.7 cases per 100 person-years for uRV (N = 171) vs uLV (N = 213), respectively (P < 0.001). In multivariable analyses, uRV conferred a greater than twofold higher risk of the composite outcome (HR: 2.17, 95% CI: 1.45-3.45, P < 0.001). In secondary analyses of components of the primary outcome, uRV was significantly associated with a greater risk of cardiac transplantation or death (HR: 9.09, 95% CI: 2.17-38.46, P < 0.001) and atrial arrhythmias (HR: 2.17, 95% CI: 1.20-4.00, P = 0.010) but not thromboembolic events (HR: 1.64, 95% CI: 0.86-3.16, P = 0.131). ConclusionsFontan patients with uRV vs uLV morphology have a higher incidence of adverse cardiovascular events, including atrial arrhythmia, cardiac transplantation, and all-cause mortality.

In vitro hemodynamic performance of a blood pump for self-powered venous assist in univentricular hearts

Univentricular heart anomalies represent a group of severe congenital heart defects necessitating early surgical intervention in infancy. The Fontan procedure, the final stage of single-ventricle palliation, establishes a serial connection between systemic and pulmonary circulation by channeling venous return to the lungs. The absence of the subpulmonary ventricle in this peculiar circulation progressively eventuates in failure, primarily due to chronic elevation in inferior vena cava (IVC) pressure. This study experimentally validates the effectiveness of an intracorporeally-powered venous ejector pump (VEP) in reducing IVC pressure in Fontan patients. The VEP exploits a fraction of aortic flow to create a jet-venturi effect for the IVC, negating the external power requirement and driveline infections. An invitro Fontan mock-up circulation loop is developed and the impact of VEP design parameters and physiological conditions is assessed using both idealized and patient-specific total cavopulmonary connection (TCPC) phantoms. The VEP performance in reducing IVC pressure exhibited an inverse relationship with the cardiac output and extra-cardiac conduit (ECC) size and a proportional relationship with the transpulmonary pressure gradient (TPG) and mean arterial pressure (MAP). The ideal VEP with fail-safe features provided an IVC pressure drop of 1.82 ± 0.49, 2.45 ± 0.54, and 3.12 ± 0.43 mm Hg for TPG values of 6, 8, and 10 mm Hg, respectively, averaged over all ECC sizes and cardiac outputs. Furthermore, the arterial oxygen saturation was consistently maintained above 85% during full-assist mode. These results emphasize the potential utility of the VEP to mitigate elevated venous pressure in Fontan patients.

Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study.

Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. This nationwide cohort study included all children undergoing operation with single-ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow-up was 98.7% complete (10 patients emigrated). Mean follow-up was 11.3 years (maximum, 26.7 years). Long-term survival was significantly higher in patients with left ventricular compared with RV dominance (10-year survival: 91.0% [95% CI, 87.3%-93.6%] versus 71.1% [95% CI, 66.4%-75.2%]). RV dominance had a significant impact on outcomes after first-stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology. This study provides clinically relevant knowledge about the long-term prognosis in patients with different underlying cardiac anomalies undergoing single-ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation. URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.

Cardiovascular Imaging-Derived Skeletal Muscle Mass Correlates With Fitness and Survival in Patients With Univentricular Circulation.

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) cm2.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 cm2 in men and 20.0 ±5.8 cm2 in women. Correlations were found between SMA/kg and peak oxygen uptake (VO2peak) (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.

Quantification of ventricular stress in univentricular hearts during early childhood using age-independent zlog-NT-proBNP

BackgroundChildren with univentricular hearts (UVH) undergo up to three palliative surgical procedures to achieve complete circulatory separation (Fontan circulation). As a marker of cardiac wall stress, NT-proBNP is a promising tool to assess systemic ventricular load in these patients. However, different reference intervals (RI) apply to each stage, as NT-proBNP is highly age-dependent. MethodsChildren undergoing systemic-to-pulmonary (SP) shunt placement (stage 1), bidirectional cavopulmonary shunt (BCPS, stage 2) or total cavopulmonary connection (TCPC, stage 3) between 2011 and 2021 with NT-proBNP measurement within 7 days before surgery were included. Furthermore, outpatients after TCPC with NT-proBNP measurement were enrolled. Biomarker levels were evaluated using its age-adjusted z-score (“zlog-NT-proBNP”; age-independent RI, −1.96 to +1.96), allowing comparison between different stages and revealing changes in systemic ventricular load independent of the marked physiological decline in RI with age. ResultsOverall, 289 children (227 before, 62 after TCPC) met the eligibility criteria. Median time between blood sampling and surgery (SP shunt/BCPS/TCPC) was 2 [1–3] days and 3.2 [2.0–4.5] years after TCPC. Age-adjusted zlog-NT-proBNP levels were 3.47 [2.79–3.93] in children with native UVH (before SP shunt), 3.10 [1.89–3.58] at stage 1 (before BCPS), 1.08 [0.51–1.88] at stage 2 (before TCPC), and 1.09 [0.72–1.75] at stage 3 (after TCPC/Fontan completion). Consequently, BCPS revealed the strongest decrease (median − 2.02 logarithmized standard deviations, p < 0.001). ConclusionsIn children with UVH undergoing staged Fontan palliation, zlog-NT-proBNP is a highly promising tool for course assessment of systemic ventricular load, independent of the age-related decline in physiological NT-proBNP concentration.

Cardiovascular magnetic resonance reference values of right ventricular volumetric variables in patients with hypoplastic left heart syndrome

Cardiovascular magnetic resonance (CMR) has established itself as the gold standard for serial assessment of systemic right ventricular (RV) performance but due to the lack of standardized RV reference values for hypoplastic left heart syndrome (HLHS) patients, the interpretation of RV volumetric data in HLHS remains difficult. Therefore, this study aimed to close this gap by providing CMR reference values for the systemic RV in HLHS patients. CMR scans of 160 children, adolescents, and young adults (age range 2.2-25.2years, 106 males) with HLHS were retrospectively evaluated. All patients were studied following total cavopulmonary connection. Short-axis stacks were used to measure RV end-diastolic and end-systolic volumes (RVEDV, RVESV), RV stroke volume (RVSV), RV ejection fraction (RVEF), and RV end-diastolic myocardial mass (RVEDMM). Univariable and multiple linear regression analyses were performed to assess associations between RV parameters and demographic and anthropometric characteristics. Following the results of the regression analysis, reference graphs and tables were created with the Lambda-Mu-Sigma method. Multiple linear regression analysis showed strong associations between body height and RVEDV, RVESV as well as RVSV. Age was highly associated with RVEDMM. Therefore, percentile curves and tables were created with respect to body height (RVEDV, RVESV, RVSV) and age (RVEDMM). The influence of demographic and anthropometric parameters on RVEF was mild, thus no percentile curves and tables for RVEF are provided. We were able to define CMR reference values for RV volumetric variables for HLHS patients. These data might be useful for the assessment and interpretation of CMR scans in these patients and for research in this field.

Predicting the effects of surgically determined parameters on exercise tolerance in Fontan patients

The Fontan physiology is a surgically created circulation for patients with a single functioning ventricle. Patients with this circulation tend to have lower exercise tolerance compared to those with a normal circulation. Important computational and experimental work has been done to investigate this reduction in exercise tolerance. However, there are few systematic modeling studies that focus on the effect of several surgically determined parameters within the same framework. We propose a mathematical model to describe the Fontan circulation under exercise. We then formulate a heuristic based on clinical data from Fontan patients to estimate exercise tolerance. The model is used to investigate the effect of three important surgically determined parameters on exercise tolerance: the systemic arterial compliance, the systemic-venous to pulmonary-venous fenestration, and the resistance of the total cavopulmonary connection.

Impact of the Rudimentary Chamber on Outcomes in Fontan Patients.

Background: We sought to evaluate the influence of the rudimentary ventricle on long-term outcomes after the Fontan operation, focusing on exercise capacity and cardiac performance. Methods: Between 1995 and 2021, 290 patients underwent a total cavopulmonary connection. "Two-ventricle" Fontan circulation was defined as a rudimentary ventricle >30% of the dominant ventricle or >50% of its predicted normal value. This cohort was compared with patients with single-ventricle Fontan circulation. The primary endpoint was Fontan failure, and the secondary endpoints were VO2 and cardiac catheterization data at ten years postoperatively. Results: The median follow-up after the Fontan operation was 7.9 years (interquartile range: 1.9-13.9). No significant difference was found in Fontan failure-free survival between the "two-ventricle" Fontan circulation group and the single-ventricle Fontan circulation group (83/91, 93% vs 156/199, 78%), respectively at 20 years; P = .11). No significant difference was found in VO2 or cardiac performance except cardiac index, with 2.9 (2.3-4.8) versus 2.5 (2.3-4.3) L/min/m2 (P = .047). Fifty-one patients in the "two-ventricle" Fontan circulation group were followed up for over ten years after the Fontan operation. In the subgroup analysis of this cohort, the rudimentary ventricular end-diastolic volume/dominant ventricular end-diastolic volume ratio showed a significant positive correlation with dominant ventricular end-diastolic pressure (r = 0.58 [95% CI 0.35-0.74], P = .002) and a significant negative correlation with VO2 (r = -0.61 [95% CI -0.80 to -0.28], P = .001). Conclusions: The rudimentary ventricle was not significantly associated with any clinical disadvantages regarding Fontan failure. However, a large rudimentary ventricle was significantly associated with higher end-diastolic pressure and lower exercise capacity.

The effect of the conduit size on middle-term outcomes in patients with extracardiac total cavopulmonary connection.

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. 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. 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). 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.

Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

Impact of Fontan Fenestration on Adverse Cardiovascular Outcomes: A Multicentre Study

BackgroundFenestrating a Fontan baffle has been associated with improved perioperative outcomes in patients with univentricular hearts. However, longer-term potential adverse effects remain debated. We sought to assess the impact of a fenestrated Fontan baffle on adverse cardiovascular events including all-cause mortality, cardiac transplantation, atrial arrhythmias, and thromboemboli. MethodsA multicentre North American retrospective cohort study was conducted on patients with total cavopulmonary connection Fontan baffle, with and without fenestration. All components of the composite outcome were independently adjudicated. Potential static and time-varying confounders were taken into consideration, along with competing risks. ResultsA total of 407 patients were followed for 10.4 (7.1-14.4) years; 70.0% had fenestration of their Fontan baffle. The fenestration spontaneously closed or was deliberately sealed in 79.9% of patients a median of 2.0 years after Fontan completion. In multivariable analysis in which a persistent fenestration was modelled as a time-dependent variable, an open fenestration did not confer a higher risk of the composite outcome (hazard ratio, 1.18; 95% confidence interval, 0.71-1.97; P = 0.521). In secondary analyses, an open fenestration was not significantly associated with components of the primary outcome: that is, mortality or transplantation, atrial arrhythmias, or thromboemboli. However, sensitivity analyses to assess the possible range of error resulting from imprecise dates for spontaneous fenestration closures could not rule out significant associations between an open fenestration and atrial arrhythmias or thromboemboli. ConclusionsIn this multicentre study, no significant association was identified between an open fenestration in the Fontan baffle and major adverse cardiovascular events.

Noninvasive Advanced Cardiovascular Magnetic Resonance-Derived Fontan Hemodynamics Are Associated With Reduced Kidney Function But Not Albuminuria.

Kidney disease is the most important predictor of death in patients with a Fontan circulation, yet its clinical and hemodynamic correlates have not been well established. A total of 53 ambulatory patients with a Fontan circulation (median age, 16.2 years, 52.8% male patients) underwent advanced cardiovascular magnetic resonance assessment, including 4-dimensional flow imaging and computational fluid dynamics. Estimated glomerular filtration rate (eGFR) <90 mL/min per 1.73 m2 was observed in 20.8% and albumin-to-creatinine ratio >3 mg/mmol in 39.6%. The average eGFR decline rate was -1.83 mL/min per 1.73 m2 per year (95% CI, -2.67 to -0.99; P<0.001). Lower eGFR was associated with older age, larger body surface area at examination, longer time since Fontan procedure, and lower systemic ventricular ejection fraction. Higher albumin-to-creatinine ratio was associated with absence of fenestration at the Fontan operation, and older age and lower systemic ventricular ejection fraction at the assessment. Lower cross-sectional area of the Fontan conduit indexed to flow (r=0.32, P=0.038), higher inferior vena cava-conduit velocity mismatch factor (r=-0.35, P=0.022), higher kinetic energy indexed to flow in the total cavopulmonary connection (r=-0.59, P=0.005), and higher total cavopulmonary connection resistance (r=-0.42, P=0.005 at rest; r=-0.43, P=0.004 during exercise) were all associated with lower eGFR but not with albuminuria. Kidney dysfunction and albuminuria are common among clinically well adolescents and young adults with a Fontan circulation. Advanced cardiovascular magnetic resonance-derived metrics indicative of declining Fontan hemodynamics are associated with eGFR and might serve as targets to improve kidney health. Albuminuria might be driven by other factors that need further investigation.

"Quality of life" analysis in the long-term follow-up after "Fontan" palliation for CHDs-a single-centre experience.

Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated. Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire "Pediatric quality of life inventory", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results. The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.

Impact of early postoperative haemodynamic and laboratory parameters on outcome after the Fontan procedure.

To identify early postoperative haemodynamic and laboratory parameters predicting outcomes following total cavopulmonary connection. Patients who underwent total cavopulmonary connection between 2012 and 2021 were evaluated. Serial values of mean pulmonary artery pressure, mean arterial pressure, peripheral oxygen saturation, and lactate levels were collected. The influence of these variables on morbidities was analyzed. Cut-off values were calculated using the receiver operating characteristic analysis. A total of 249 patients were included. All patients had previous bidirectional cavopulmonary shunt. Median age and weight at total cavopulmonary connection were 2.2 (1.8-2.7) years and 11.7 (10.7-13.4) kg, respectively. All patients were extubated in the ICU at a median of 3 (2-5) hours after ICU admission. Postoperative pulmonary artery pressure, around 12 hours after extubation, was significantly associated with chest tube drainage (p = 0.048), chylothorax (p = 0.021), ascites (p = 0.016), and adverse events (p = 0.028). Receiver operating characteristic analysis revealed a cut-off value of 13-15 mmHg for chest tube drainage and chylothorax and 17 mmHg for ascites and adverse events. Mean arterial pressure 1 hour after extubation was associated with prolonged chest tube drainage (p = 0.015) and adverse events (p = 0.008). Peripheral oxygen saturation 6 hours after extubation (p = 0.003) was associated with chest tube duration and peripheral oxygen saturation 1 hour after extubation (p < 0.001) was associated with ascites. Lactate levels on 2nd postoperative day (p = 0.022) were associated with ascites and lactate levels on 1st postoperative day (p = 0.009) were associated with adverse events. Higher pulmonary artery pressure, lower mean arterial pressure, lower peripheral oxygen saturation, and higher lactate in early postoperative period, around 12 hours after extubation, predicted in-hospital and post-discharge adverse events following total cavopulmonary connection.

Ablation of atrial flutter in a patient after Fontan procedure: a case report of challenging access to the common atrium

Abstract Background In patients with a total cavopulmonary connection in Fontan circulation, the access to the common atrium (CA) during a catheter ablation can be challenging, even in the presence of fenestration in an intra-atrial lateral tunnel (IALT). In our department, the fenestration is typically marked with metal clips (MCs). To the best of our knowledge, there is no previous report of balloonoplasty of clipped fenestration. Case summary A 19-year-old male with hypoplastic left heart syndrome (HLHS) was scheduled for catheter ablation of recurrent atrial tachycardia. He was diagnosed with HLHS prenatally and underwent a stepwise surgical palliation. Fontan circulation was completed with the creation of a fenestrated IALT. The fenestration was marked by four MCs. During the ablation procedure, the passage of the steerable sheath with mapping catheter to the CA was prevented by a small fenestration size and rigidness of the edges of the fenestration caused by the MCs. Multiple attempts to dilate the fenestration using a peripheric angioplasty balloon failed. Only angioplasty with the ‘balloon-against-dilator’ technique was finally successful. Activation map showed a counterclockwise atrial flutter in the CA; successful ablation was performed. Discussion We present a case of challenging access to the CA through a clipped fenestration in a polytetrafluoroethylene baffle for atrial tachycardia ablation. Even though a tunnel fenestration in Fontan patients facilitates access to the CA, the passage of a steerable introducer with a mapping catheter may be challenging due to diameter mismatch and the rigidity of its edges caused by MCs. The balloon-against-dilator technique might be helpful when conventional balloon angioplasty fails.

In vitro investigation of axial mechanical support devices implanted in the novel convergent cavopulmonary connection Fontan.

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. 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. 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). 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.

Kiosk 3R-TA-07 - Anatomical Subtypes of Hypoplastic Left Heart Syndrome Do Not Impact on Right Ventricular Size and Function During Serial Follow up After Completion of the Total Cavopulmonary Connection

Kiosk 3R-TA-07 - Anatomical Subtypes of Hypoplastic Left Heart Syndrome Do Not Impact on Right Ventricular Size and Function During Serial Follow up After Completion of the Total Cavopulmonary Connection

Converting the Fontan Circulation: Challenges and Evolution.

The Fontan operation, the final palliative step after a series of complex operations in patients with univentricular hearts, has undergone multiple modifications throughout the last decades, with the goal of finding the method which combines the optimal hemodynamic effects of the Fontan circulation with minimal long term side effects. An understanding of the operative evolution and subsequent side effects, as well as the management thereof seems imperative. Since its inception by Francis Fontan the, now obsolete, initial atriopulmonary connection has passed through several milestones into having now reached the era of total cavopulmonary connection. However, recently published results bring to light the new option of biventricular conversion which comes to challenge the management of Fontan patients as we know it. Currently, there is no consensus as to whether to continue with palliation in this challenging group of patients or proceed with heart transplantation. In this collective review, we provide a historic overview of the Fontan evolution as well as future insights, discussing the perspectives of options for patients with failing Fontan, including the latest addition of biventricular correction.