Total Cavopulmonary Connection Patients Research Articles

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.

Cardiac Function and Serum Biomarkers throughout Staged Fontan Palliation: A Prospective Observational Study.

Fontan patients undergo multiple cardiothoracic surgeries in childhood. Following these procedures, ventricular function is temporarily decreased, and recovers over months. This is presumably related to cardiopulmonary bypass, but this is incompletely understood. Throughout the Fontan palliation, cardiac function is also affected by volume unloading. We aimed to gain insight into the biological processes related to impaired ventricular function and recovery following Fontan palliations using a panel of biomarkers. Furthermore, we described changes in ventricular function across the Fontan palliation due to volume unloading. We performed a prospective multicenter observational study in patients undergoing partial (PCPC) or total cavo-pulmonary connection (TCPC). Patients underwent assessment-including echocardiography and blood sampling-before surgery (T1), at first follow-up (T2), and 1 year after their procedures (T3). Blood samples were analyzed using a biomarker panel (OLINK CVD-III). Ninety-two biomarkers were expressed as principal components (PC) to limit multiple statistical testing. We included 32 PCPC patients aged 7.2 [5.3-10.3] months, and 28 TCPC patients aged 2.7 [2.2-3.8] years. The single ventricular longitudinal strain (SV GLS) temporarily decreased for PCPC patients at T2 (-15.1 ± 5.6 (T1) to -13.5 ± 5.2 (T2) to -17.3 ± 4.5 (T3), p < 0.047 for all differences), but not following TCPC. The serum biomarkers were expressed as 4 PCs. PC1, including biomarkers of cell-cell adhesion, was not related to any patient characteristic. PC2, including biomarkers of superoxide anion regulation, increased at T2. PC3, including biomarkers of cardiovascular development, related to the stage of Fontan palliation. PC4 was of uncertain biological or clinical significance. No PC was found that related to ventricular performance. The SV GLS was temporarily diminished following PCPC, but not following TCPC. Several biomarkers were related to post-operative stress and adaptation to the PCPC or TCPC circulation, but none were related to the outcome.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Fontan procedure.

This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. 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). 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.

Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records...

Recurrent sustained atrial arrhythmias and thromboembolism in Fontan patients with total cavopulmonary connection.

BackgroundTotal 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.MethodsThis 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.ResultsA 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.ConclusionsAlthough 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.

Path ahead for ‘low risk’ adolescents living with a Fontan circulation

ObjectiveA high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time...

Abstract 14695: What Lies Ahead? Project Survival Estimates in the Setting of a ’Low Risk’ Fontan Circulation

The Fontan procedure has enabled those born with a functionally single ventricle to survive well into early adulthood albeit with high rates of morbidity and mortality. The total cavopulmonary connection (TCPC) has superseded the atriopulmonary connection (APC) and is associated with improved outcomes. However, the chance of survival into late adulthood is unknown and outcomes specifically in young adults without high risk features are not well characterized. We therefore projected the late survival of ‘low risk’ Fontan patients. Patients were included from the Australian and New Zealand Fontan Registry if they survived to age 16y without transplantation, sustained arrhythmia, thromboembolic event, Fontan conversion or takedown, plastic bronchitis, protein losing enteropathy, NYHA class III/IV or ≥ moderate ventricular dysfunction or atrioventricular regurgitation. Estimates of projected survival were obtained via Weibull distribution (Stata software, V15, StataCorp LLC) using current survival curves and extrapolating different modelled scenarios. In ‘optimistic’, Fontan mortality risks continue on current trends until reaching all-cause general population mortality risks. In ‘conservative’, the ratio of mortality risk between Fontan survivors and the general population are maintained from last follow-up. ‘Pessimistic’ was modelled to consider the possibility the TCPC cohort experience significant deterioration, as observed in the APC cohort; here risks were increased to match the APC mortality risk from age 55y. There were 612 ‘low risk’ patients included; 152 APC and 460 TCPC. Survival at 40 years of age was 80% and 89% for APC and TCPC respectively. Projected survival estimates are shown in Table 1 . Projected survival for low-risk TCPC patients is far more encouraging than previously expected, with 49-67% reaching their seventh decade. There is a growing need for enhanced resources in the specialized area of adult congenital heart disease.

Cardiovascular complications in patients with total cavopulmonary connection: A nationwide cohort study

BackgroundLong-term outcomes are not well established at the population level after completion of the total cavopulmonary connection (TCPC) among patients with functional univentricular hearts. PurposeTo evaluate the incidence of cardiovascular events after TCPC completion. MethodsFrom a validated population-based cohort, we identified 178 patients with TCPC circulation completed after January 1, 1995. We established a comparison cohort by frequency-matching patients 1:200 on sex, and month and year of birth to the general Danish population (n = 35,600 population controls). We started follow-up at date of TCPC completion for cases and, for controls, at the index date of their matched case. The risks of cardiovascular events were assessed using cumulative incidence rates and Poisson regression models adjusted for age, sex, and calendar year. ResultsThe median age at TCPC completion was 3.3 years (interquartile range 2.6 to 5.3 years). Over a median follow-up of 12.5 years, 10 (5.6%) TCPC patients died and 7 (4.5%) had a heart transplantation compared with a 0.2% mortality in the matched population. In TCPC patients, 15.7% had an arrhythmia (11.8% supraventricular tachycardia), 3.4% had a stroke (all ischemic), and 21.4% of TCPC patients initiated a combination of a diuretic and a renin-angiotensin system (RAS)-inhibitor. These rates were >50-fold higher than in the comparison cohort: 0.2% arrhythmia, 0.06% stroke, and 0.04% starting a diuretic and a RAS-inhibitor. ConclusionsPatients with TCPC circulation face a high risk of cardiovascular events during intermediate term follow-up.

Conduit Route Selection for Total Cavopulmonary Connection in Patients With Apicocaval Juxtaposition

Apicocaval juxtaposition (ACJ) may complicate the selection of conduit route in patients with single ventricles when total cavopulmonary connection (TCPC) is performed. We reviewed our experience of pathway selection and evaluated the clinical results. Of 128 patients who underwent TCPC at our hospital between January 2009 and April 2016, 31 with ACJ were included in this study. In 24 patients, the conduit was placed between the inferior vena cava (IVC) and the ipsilateral pulmonary artery. To avoid compression of the conduit and pulmonary veins in 5 patients, the conduit was placed between the IVC and the contralateral pulmonary artery. In 2 patients, the tube graft was anastomosed with the IVC orifice within the atrium, then guided through the atrial free wall and anastomosed with the contralateral pulmonary artery outside the heart (intra/extracardiac Fontan). Patient demographics were compared with those of patients without ACJ. The mean age and body weight at surgery were 58.5 ± 32.4 months and 16.2 ± 6.0 kg, respectively. The mean postoperative pulmonary artery pressure was 15 ± 3 mm Hg. The postoperative data did not differ significantly from that of patients without ACJ who underwent extracardiac TCPC. One patient died of overwhelming infection. The mean follow-up was 17.5 ± 15.4 months (range, 1-65 months). There were no conduit-related early or late complications. TCPC in patients with ACJ can be performed with excellent early and midterm results. The route between the IVC and the ipsilateral pulmonary artery is our preference.

Functional outcome in contemporary children with total cavopulmonary connection – Health-related physical fitness, exercise capacity and health-related quality of life

ObjectiveChildren and adolescents with an univentricular heart after total cavopulmonary connection (TCPC) have functional impairments. This study assesses health-related physical fitness (HRPF) and exercise capacity, as well as their relation to health-related quality of life (HRQoL) in patients with an univentricular heart after total-pulmonary connection (TCPC). Patients and methodsBetween July 2014 and October 2016 a total of 78 children and adolescents with TCPC (12.0±3.2years, 21 female) performed a motor test including five tasks for strength and flexibility during their routine follow-up appointment. They also underwent a symptom limited cardio-pulmonary exercise test and filled in a HRQoL questionnaire (KINDL-R). Patients' data were compared to a recent sample of healthy children (n=1650, 12.6±2.4years, 49% female). ResultsMultivariable regressions corrected for sex, age and BMI showed that TCPC patients achieved 12.4 repetitions of curl-ups (p<0.001) and 2.6 push-ups (p=0.010) less than healthy counterparts. They had impairments in trunk (−8.5cm; p<0.001), shoulder (−7.5cm; p<0.001) and lower limb flexibility (−4.7cm; p<0.001). Peak oxygen uptake was reduced to 34.8±7.5ml/min/kg and 77.7% respectively, compared to peers (p<0.001). Ventilatory efficiency was also impaired (healthy: 27.5±2.9 vs. TCPC: 31.6±3.3; p<0.001). HRQoL did not differ significantly (p=0.233). ConclusionsChildren and adolescents with TCPC still present impaired HRPF and exercise capacity whereas HRQoL is similar to healthy peers. Since low HRPF may yield to worse motor competence and exercise capacity, early screening for HRPF and early treatment, if indicated, is recommended.

Outcomes of a total cavopulmonary connection in patients with impaired ventricular function.

Our aim was to evaluate outcomes following a total cavopulmonary connection (TCPC) in patients with preoperatively impaired ventricular function (VF). Of 483 consecutive TCPC patients, 44 (9.1%) had impaired VF (ejection fraction <50%, Group A), and 439 patients had normal VF (ejection fraction ≥50%, Group B). We compared the clinical outcomes between the groups. The median age at TCPC was 2.8 (interquartile range 1.9-8.3) years in Group A and 2.3 (1.8-3.5) years in Group B (P = 0.025). An atrioventricular valve (AVV) operation prior to (38.6 vs 27.1%, P < 0.001) and concomitant with (31.8 vs 12.1%, P < 0.001) the TCPC was performed more frequently in Group A. The median intensive care unit stay (7.0 vs 7.0 days, P = 0.737) and 30-day survival (97.7 vs 98.4%, P = 0.737) were not significantly different between groups. Freedom from death, transplantation (P = 0.115) and catheter intervention (P = 0.603) showed no difference between groups. However, freedom from cardiac reoperation was significantly lower in Group A (P < 0.001). VF was resolved in 22 of the 39 (56.4%) survivors in Group A. The recovered patients had a lower incidence of AVV reoperation (0 vs 6, P = 0.002) and pacemaker rhythm (0 vs 5, P = 0.006). In patients planned for TCPC, impaired VF is often associated with AVV regurgitation. TCPC can be performed with low risk and comparable clinical results except for cardiac reoperation in patients with impaired VF when compared to patients with normal VF. Following TCPC, VF recovers in half of the survivors. A competent AVV and sinus rhythm are prerequisites for recovery.

Aortopulmonary collateral flow quantification by MR at rest and during continuous submaximal exercise in patients with total cavopulmonary connection.

Aortopulmonary collateral flow is considered to have significant impact on the outcome of patients with single ventricle circulation and total cavopulmonary connection (TCPC). There is little information on collateral flow during exercise. To quantify aortopulmonary collateral flow at rest and during continuous submaximal exercise in clinical patients doing well with TCPC. Prospective, case controlled. Thirteen patients with TCPC (17 (11-37) years) and 13 age and sex-matched healthy controls (18 (11-38) years). 1.5T; free breathing; phase sensitive gradient echo sequence. Blood flow in the ascending and descending aorta and superior vena cava were measured at rest and during continuous submaximal physical exercise in patients and controls. Systemic blood flow (Qs ) was assumed to be represented by the sum of flow in the superior caval vein (Qsvc ) and the descending aorta (QAoD ) at the diaphragm level. Aortopulmonary collateral flow (Qcoll ) was calculated by subtracting Qs from flow in the ascending aorta (QAoA ). Mann-Whitney U-test and Wilcoxon test for comparison between groups and between rest and exercise. Absolute collateral flow in TCPC patients at rest was 0.4 l/min/m2 (-0.1-1.2), corresponding to 14% (-2-42) of Qs . Collateral flow did not change during exercise (difference -0.01 (-0.7-1.0) l/min/m2 , P = 0.97). TCPC patients had significantly lower Qs at rest (2.5 (1.6-4.1) vs. 3.5 (2.6-4.8) l/min/m2 , P = 0.001) and during submaximal exercise (3.2 (2.0-6.0) vs. 4.8 (3.3-6.9) l/min/m2 , P = 0.001), compared to healthy controls. The increase in Qs with exercise was also significantly lower in patients than in healthy controls (median 0.6 vs. 1.2 l/min/m2 , P < 0.02). Clinical patients doing well with TCPC have significant aortopulmonary collateral flow at rest (14% of Qs ) compared to healthy controls, which does not change during submaximal exercise. 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:1509-1516.

Impact of early Fontan completion on postoperative outcomes in patients with a functional single ventricle.

Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC ≤ 18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. Median age at TCPC was 1.4 (interquartile ranges: 1.3-1.5) years in group A and 2.5 (1.9-4.5) years in group B. Duration of intensive care unit stay (6 vs 7 days), hospital stay (20 vs 20 days), and 30-day survival (100 vs 98%) was not significantly different between groups. Estimated survival (95.3 vs 92.1%), freedom from reoperation (93.7 vs 86.3%), freedom from catheter intervention (60.1 vs 77.0%), and freedom from protein losing enteropathy (97.6 vs 93.8%) at 10 years was not significantly different between groups. At last follow-up, no patient in group A but 13 patients in group B exhibited reduced ventricular function ( P = 0.035). Exercise-capacity testing showed that both peak oxygen uptake (36.4 vs 28.6 ml/kg/min; P = 0.026) and its percentage of predicted value (82.9 vs 70.0%; P = 0.004) were significantly higher in group A ( n = 6, mean postoperative period: 8.9 years) than in group B ( n = 119, mean postoperative period: 8.7 years). Fontan completion for TCPC can be performed at an early age with a low perioperative risk and good intermediate results. Earlier unloading of the systemic ventricle and earlier elimination of chronic cyanosis by staged cavopulmonary connection might be advantageous for preservation of long-term ventricular function.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection

Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. The initial procedure was pulmonary artery banding in 9 patients, systemic-to-pulmonary shunt in 2 and Damus-Kaye-Stansel/Norwood procedure in 2. Median follow-up was 69 months (interquartile range 25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome ( P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left ( P > 0.05). A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.

Exercise Capacity and Quality of Life after Total Cavopulmonary Connection at Adults

Objective: The aim of this study was to evaluate the clinical and psychological profile of after total cavopulmonary connection procedure and to identify the significant determinants of quality of life. Methods: Data from 21 patients underwent total cavopulmonary connection procedure from January 2001 to December 2015 were retrospectively analyzed. Patients underwent an echocardiography and cardiopulmonary exercise testing. Used ST-36 questionnaire to perceived health status. The NT-pro BNP levels was test. Results: 21 patients aged between 19 to 36 years (mean 26.6 years) were enrolled. The mean follow-up time was 44.2 ± 29.9 months. The mean EF was 59.4 ± 6.2%, and the mean maximal oxygen consumption was 19.9 ± 3.6 ml/kg/min, predict value was 52.5 ± 8.9%. SF-36 score was well. Independent risk for impaired exercise capacity was interval time from surgery. Conclusions: The exercise capacity of total cavopulmonary connection patients was impaired. Identify more predict factors of the quality of these patients need further study.

Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection.

To assess quantitative measurement of mean pulmonary artery pressure (PAP) in extracardiac total cavopulmonary connection (TCPC) patients by noninvasive echocardiographic inferior vena cava collapsibility index (IVC-CI) and also correlation between the peripheral vein pressure and mean PAP. In 19 TCPC patients with at least 1-year follow-up after completion of TCPC, complete echocardiography including IVC-CI was recorded. All patients underwent cardiac catheterization for mean PAP pressure, peripheral vein pressure (PVP) and contrast study. Different cutoff points of mean PAP were analyzed, and based on the highest cutoff point, patients were categorized into two groups: mean PAP<17mmHg (acceptable) and >=17mmHg (high) for comparison analysis. Between 2006 and 2015, from 43 patients who had undergone TCPC, nineteen patients with a mean age 12.9±6.6years and mean follow-up time of 3.4±1.9years were included in this study. Mean PAP was 14.6±3.97 (range 8-22mmHg). Thirteen (68%) patients had PAP<17mmHg. No significant statistical difference was detected with respect to age, sex, type of congenital heart disease and fenestration between these two groups. But, right ventricular dominancy was more prevalent in the high PAP group (50% vs. 7.7% P value 0.03). IVC-CI had a correlation with mean PAP (r 0.67, P<0.001). IVC-CI<21.8% can predict PAP>=17mmHg with 83% sensitivity and 100% specificity. Regression analysis proposed an equation for PAP measurement: PAP (mmHg)=20.2097-0.1796×(IVC-CI), (r2=0.56). Peripheral vein pressure measurement also showed a good correlation with mean PAP and may be used to estimate PAP with the following equation: PAP (mmHg)=0. 8675×PVP, (r 0.90, P<0.0001). In conclusion, IVC-CI as noninvasive and peripheral vein pressure measurement as a minimal invasive method may be useful for quantitative estimation of PAP in patients with extracardiac TCPC.

Strategies for thromboprophylaxis in Fontan circulation: a meta-analysis

BackgroundThe Fontan circulation is associated with an increased risk of thromboembolic events (TEs). As many as 25% of these thrombotic events result in fatality. More subtle adverse effects on the...

NTproBNP after Fontan anastomosis: Early versus late post-operative value

NTproBNP assessment remains limited in congenital cardiology, particularly in patients with univentricular heart physiology who underwent total cavopulmonary connection (TCPC) where long-term prognosis is greatly related to ventricular function. To evaluate the NTproBNP after TCPC and seek a correlation with clinical and laboratory monitoring. A retrospective study was initiated on all TCPC patients born after 1991. Demographic, anatomical, echocradiography data and NTproBNP serum level were collected following the early post-operative period. Routine NTproBNP screening for these patients was initiated in our institution in 2008. NTproBNP Z-score was calculated based on our published equation from healthy children. Of a total of 46 patients who completed TCPC, 5 died post-operatively. All remaining 41 subjects had available NTproBNP beyond the immediate post-operative period and constituted the study population. NTproBNP was significantly elevated during the first post-operative year (Z-score 1.9 ± 1.17) compared to mid-term follow-up (3–5 years) (Z-score 1.23 ± 0.72), P = 0.03. Mean NTproBNP Z-score increased in the subsequent years to 1.6 ± 1.1 ( P = 0.21 vs year-1 post TCPC, and 0.06 vs mid-term). Z-scores > 2.0 were associated with a lower serum albumin (39.2 ± 3.3 vs 44.4 ± 4.4 mg/dL; P = 0.04), but not with other laboratory tests. There were however no identifiable predisposing clinical factors (i.e., ventricular morphology, age), preoperative hemodynamic data (PVRi, mean pulmonary artery pressure, Nakata index), surgical specifics (type of TCPC, presence of fenestration, pacemaker implantation), or postoperative events. The TCPC postoperative year is marked by a significant increase in the levels of NTproBNP. The observation does not seem to vary between left and right dominant ventricular anatomy, neither did it correlate with various parameters. NTproBNP improved in the mid-term follow-up.

Computational fluid dynamics in Fontan patients to evaluate power loss during simulated exercise

ObjectiveExercise intolerance is common in total cavopulmonary connection (TCPC) patients. It has been suggested that power loss (Ploss) inside the TCPC plays a role in reduced exercise performance. Our objective...

Exercise capacity in children after total cavopulmonary connection: Lateral tunnel versus extracardiac conduit technique

In patients with univentricular heart disease, the total cavopulmonary connection (TCPC) is the preferred treatment. TCPC can be performed using the intra-atrial lateral tunnel (ILT) or extracardiac conduit (ECC) technique. The purpose of the present study was to evaluate exercise capacity in contemporary TCPC patients and compare the results between the 2 techniques. A total of 101 TCPC patients (ILT, n=42; ECC, n=59; age, 12.2±2.6 years; age at TCPC completion, 3.2±1.1 years) underwent cardiopulmonary exercise testing. The patients were recruited prospectively from 5 tertiary referral centers. For the entire group, the mean peak oxygen uptake was 74%±14%, peak heart rate was 90%±8%, peak workload was 62%±13%, and slope of ventilation versus carbon dioxide elimination (VE/VCO2 slope) was 127%±30% of the predicted value. For the ILT and ECC groups, patient age, age at TCPC completion, body surface area, peak workload, and peak heart rate were comparable. The percentage of predicted peak oxygen uptake was lower in the ILT group (70%±12% vs 77%±15%; P=.040), and the percentage of predicted VE/VCO2 slope was greater in the ILT group (123%±36% vs 108%±14%; P=.015). In a subgroup analysis that excluded ILT patients with baffle leak, these differences were not statistically significant. Common exercise parameters were impaired in contemporary Fontan patients. Chronotropic incompetence was uncommon. The peak oxygen uptake and VE/VCO2 slope were less favorable in ILT patients, likely related to baffle leaks in some ILT patients. These results have shown that a reduced exercise capacity in Fontan patients remains an important issue in contemporary cohorts. The ECC had a more favorable exercise outcome at medium-term follow-up.