The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed.

Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16–26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4–42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m2) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test.

In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

Interstage monitoring programs for single ventricle disease have been developed to reduce morbidity and mortality. There is increased use of telemedicine and mobile application monitoring. It is unknown if there are disparities in use based on patient socio-demographic factors.

We conducted a retrospective cohort study of patients enrolled in the single ventricle monitoring program and KidsHeart application at a single centre from 4/21/2021 to 12/31/2023. We investigated the association of socio-demographic factors with telemedicine usage, mobile application enrollment and usage. We assessed resource utilisation and weight changes by program era.

There were 94 children in the cohort. Patients with Norwood and ductal stent had higher mean telemedicine visits per month (1.8 visits, p = 0.004), without differences based on socio-demographic factors. There were differences in application enrollment with more Black patients enrolled compared to White patients (p = 0.016). There were less Hispanic patients enrolled than Non-Hispanic patients (p = 0.034). There were no Spaish speaking patient’s enrolled (p = 0.0015). There were no patients with maternal education of less than high school enrolled and all those with maternal education of advanced degree were enrolled (p = 0.0016). There was decreased mobile application use in those from neighbourhoods in the lowest income quartile. There were decreased emergency department visits with mobile application monitoring. Mean weight-for-age z-scores had increased from start to completion of the program in all eras.

Differences were seen in mobile application enrollment and usage based on socio-demographic factors. Further work is needed to ensure that all patients have access to mobile application usage.

Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation.

This was a single-centre retrospective observational study conducted at a quaternary free-standing children’s hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation. 76 patients (42%) underwent early extubation, 59 (33%) standard extubation, and 45 (25%) delayed extubation. We measured time to extubation, post-operative outcomes, length of stay and utilised Fine gray models, Youden’s J statistic, cumulative incidence function, and logistic regression to analyse outcomes.

Delayed extubation group suffered significantly higher rates of mortality (31.1% vs. 6.8%), cardiac arrest (40.0% vs. 10.2%), stroke (37.8% vs. 11.9%), and need for catheter (28.9% vs. 5.1%) and surgical intervention (24.4% vs. 8.5%) (P < 0.001). Physiologic dead space fraction was significantly higher in the delayed extubation group and in non-survivors with a value of 0.3, which was found to be the discriminatory point by Youden’s J statistic. For a 0.1 unit increase in physiologic dead space fraction on post-operative day 1, the odds of a patient expiring increase by a factor of 2.26 (95% CI 1.41–3.97, p < 0.001) and by a factor of 3.79 (95% CI 1.65–11.7, p 0.01) on post-operative day 3.

Delayed extubation impacts morbidity and mortality in patients undergoing the comprehensive stage 2 operation. Increased physiologic dead space fraction in the first 60 hours after arrival to the ICU is associated with higher mortality.

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.

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.

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).

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.

Acute kidney injury is a common postoperative complication of paediatric cardiac surgery associated with increased morbidity and mortality. The purpose of this study is to characterise associations between haemodynamic parameters, clinical parameters, and medical interventions, on acute kidney injury.

Nine patients with univentricular physiology undergoing the Norwood procedure from a single-centre tertiary care paediatric cardiac ICU were included (September 2022 to March 2023). Patients were monitored with the T3 software. Data were analysed using a Fisher exact test, Mann–Whitney-U test, LASSO-based machine learning techniques, and receiver operator curve analyses.

Over 27,000 datapoints were included. Acute kidney injury occurred in 2 patients (22%) during this period. Net fluid balance and renal oxygen extraction were independently associated with acute kidney injury, while commonly used metrics of pressure (systolic, diastolic, or mean arterial blood pressure) were not. The resulting acute kidney injury risk score was (4.1 × fluid balance) + (1.9 × renal oxygen extraction). The risk score was significantly higher in acute kidney injury with a score of 32.9 compared to 7.9 (p < 0.01). Optimal cut-offs for fluid balance (7 mL/hr) and renal oxygen extraction (29%) were identified. Higher serum creatinine:baseline creatinine ratio was associated with a higher mean airway pressure, higher renal oxygen extraction, higher mean arterial blood pressure, higher vasoactive inotropic score, and fluid balance.

Among patients with univentricular physiology undergoing the Norwood procedure, renal oxygen extraction and a higher net fluid balance are independently associated with increased risk of acute kidney injury. Renal perfusion pressure is not significantly associated with acute kidney injury.

This study describes the illness burden in the first year of life for children with single-ventricle heart disease, using the metric of days alive and out of hospital to characterize morbidity and mortality.

This is a retrospective single-centre study of single-ventricle patients born between 2005 and 2021 who had their initial operation performed at our institution. Patient demographics, anatomical details, and hospitalizations were extracted from our institutional single-ventricle database. Days alive and out of hospital were calculated by subtracting the number of days hospitalized from number of days alive during the first year of life. A multivariable linear regression with stepwise variable selection was used to determine independent risk factors associated with fewer days alive and out of hospital.

In total, 437 patients were included. Overall median number of days alive and out of hospital in the first year of life for single-ventricle patients was 278 days (interquartile range 157–319 days). In a multivariable analysis, low birth weight (<2.5kg) (b = −37.55, p = 0.01), presence of a dominant right ventricle (b = −31.05, p = 0.01), moderate-severe dominant atrioventricular valve regurgitation at birth (b = −37.65, p < 0.05), index hybrid Norwood operation (b = −138.73, p < 0.01), or index heart transplant (b = −158.41, p < 0.01) were all independently associated with fewer days alive and out of hospital.

Children with single-ventricle heart defects have significant illness burden in the first year of life. Identifying risk factors associated with fewer days alive and out of hospital may aid in counselling families regarding expectations and patient prognosis.

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.

Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown.

Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated.

Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children. Superior caval vein flow ratio decreased in 14/15 patients after cavopulmonary anastomosis: mean 0.63 ± 0.12 before versus 0.43 ± 0.14 after. No linear correlation between intraoperative superior caval vein pressure and superior caval vein flow after cavopulmonary anastomosis was found. Neither Nakata index nor pulmonary vascular resistance measured at preoperative cardiac catheterisation correlated with intraoperative flows. None of patients died or required a take down.

The higher mean superior caval vein flow ratio before cavopulmonary anastomosis compared to healthy children suggests flow redistribution in univentricular physiology to protect brain and neurodevelopment. The decrease of superior caval vein flow ratio after cavopulmonary anastomosis may reflect the flow redistribution related to trans-pulmonary gradient. The lack of correlation between superior caval vein pressure and superior caval vein flow could be explained by limited sample size and multifactorial determinants of caval veins flow, although pressure remain essential. Larger sample of measurements are needed to find flow range potentially predictive for clinical failure. To authors’ knowledge, this is the first intraoperative flow measurement of both caval veins during cavopulmonary operations.

Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation.

We sought to validate the score in an external, modern cohort.

This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (Norwood type—Norwood/Blalock–Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, Opiates at discharge: 6, No Digoxin at discharge: 9, Arch Obstruction on discharge echo: 9, Tricuspid regurgitation ≥ moderate on discharge echo: 12; Extra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant.

In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4–89.5%).

In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.

Low pre-albumin, body mass index, and thiamine levels have been associated with poor nutritional status and cognitive/memory deficits in adult heart failure patients. However, the relationship of these nutritional/dietary intake biomarkers to cognition has not been assessed in adolescents post-Fontan procedure and healthy controls.

This is a cross-sectional study. Adolescents (14–21 years of age) post-Fontan completion were recruited from paediatric cardiology clinics and controls from the community. The Montreal Cognitive Assessment was administered (normal ≥ 26), and blood draw (thiamine [normal 70–110 nmol/L] and pre-albumin levels [adolescent normal 23–45 mg/dL]) and the Thiamine Food Frequency Questionnaire were completed by all participants.

Seventy subjects, 40 post-Fontan (mean age 16 ± 1.6, female 51%, Hispanic 44%, hypoplastic left heart syndrome 26%) and 30 controls (mean age 16.8 ± 1.9, female 52%, Hispanic 66%), were participated. Post-Fontan group had lower median total cognitive scores (23 versus 29, p < 0.001), pre-albumin levels (23 versus 27, p = 0.013), and body mass index (20 versus 24, p = 0.027) than controls. Post-Fontan group had higher thiamine levels than controls (127 versus 103, p = 0.033). Lower pre-albumin levels (< 23) and underweight body mass index were associated with abnormal total cognitive scores (p = 0.030). Low pre-albumin level (p = .038) was an independent predictor of worse cognition.

Lower pre-albumin was an independent predictor for worse cognition in adolescents post-Fontan. Lower pre-albumin levels may reflect chronic liver changes or protein-losing enteropathy seen in Fontan physiology. These findings highlight the possibility for nutrition-induced cognitive changes.

An alternative surgical approach for hypoplastic left heart syndrome is the Hybrid pathway, which delays the risk of acute kidney injury outside of the newborn period. We sought to determine the incidence, and associated morbidity, of acute kidney injury after the comprehensive stage 2 and the cumulative incidence after the first two operations in the Hybrid pathway.

A single centre, retrospective study was conducted of hypoplastic left heart patients completing the second-stage palliation in the Hybrid pathway from 2009 to 2018. Acute kidney injury was defined utilising Kidney Diseases Improving Global Outcomes criteria. Perioperative and post-operative characteristics were analysed.

Sixty-one patients were included in the study cohort. The incidence of acute kidney injury was 63.9%, with 36.1% developing severe injury. Cumulatively after the Hybrid Stage 1 and comprehensive stage 2 procedures, 69% developed acute kidney injury with 36% developing severe injury. The presence of post-operative acute kidney injury was not associated with an increase in 30-day mortality (acute kidney injury 7.7% versus none 9.1%; p = > 0.9). There was a significantly longer median duration of intubation among those with acute kidney injury (acute kidney injury 32 (8, 155) hours vs. no injury 9 (0, 94) hours; p = 0.018).

Acute kidney injury after the comprehensive stage two procedure is common and accounts for most of the kidney injury in the first two operations of the Hybrid pathway. No difference in mortality was detected between those with acute kidney injury and those without, although there may be an increase in morbidity.

Brady-arrhythmia requiring pacemaker implantation remains one of the Fontan-specific complications before and after total cavopulmonary connection.

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.

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).

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.

Enteral feeding prior to cardiac surgery has benefits in pre-operative and post-operative patient statuses. In 2020, to increase pre-operative feeding for single-ventricle patients prior to stage 1 palliation, an enteral feeding algorithm was created. The aim of this study is to monitor the impact of our practice change with the primary outcome of necrotising enterocolitis incidence from birth to 2 weeks following surgical intervention.

This is a single-site, retrospective cohort study including patients from 1 March, 2018 to 1 July, 2022. Variables assessed include demographics, age at cardiac surgery, primary cardiac diagnosis, necrotising enterocolitis pre-operative and 2 weeks post-operative cardiac surgery, feeding route, feeding type, volume of trophic enteral feeds, and near-infrared spectroscopy.

Following implementation of a pre-operative enteral feeding algorithm, the rate of neonates fed prior to surgery increased (39.5–75%, p = .001). The feedings included a mean volume of 28.24 ± 11.16 ml/kg/day, 83% fed breastmilk only, 44.4% tube fed, and 55.5% of infants had all oral feedings. Comparing enterally fed neonates and those not enterally fed, the necrotising enterocolitis incidence from birth to 2 weeks post-op was not significantly increased (p = 0.926).

As a result of implementing our feeding algorithm, the frequency of infants fed prior to stage I Norwood or Hybrid surgeries increased to 75%, and there was no significant change in the incidence of necrotising enterocolitis. This study confirmed that pre-operative enteral feeds are safe and are not associated with increased incidence of necrotising enterocolitis.

Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care.

We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician’s approach to single ventricle patients at different stages of surgical palliation.

Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Despite high survival after bidirectional cavopulmonary anastomosis, a considerable number of patients suffer significant post-operative morbidities related to prolonged length of stay.

A single-center retrospective cohort study of all consecutive patients undergoing a first-time bidirectional cavopulmonary anastomosis from 2006 to 2019.

Prolonged length of stay was defined as hospital stay greater than the 75th percentile for our cohort. Of 195 patients who met inclusion criteria, the median post-operative length of stay was 8 days (interquartile range, 4-15 days). Prolonged length of stay was defined as greater than 15 days. In multivariate analysis, greater than mild systemic atrioventricular valve regurgitation (odds ratio 3.7, 95% CI 1.05-13.068, p = 0.04), longer length of stay after the initial palliative procedure (odds ratio 1.028, 95% CI 1.004-1.05, p = 0.02), and pre-operative higher superior vena cava oxygen saturation (odds ratio 0.922, 95% CI 0.85-0.99, p = 0.04) maintained statistical significance as independent risk and protective factors for prolonged length of stay. A one-level increase in the severity of pre-operative systemic atrioventricular valve regurgitation was associated with a multiplicative change in the odds ratio of prolonged length of stay of 5.45 (p = 0.005) independent of the severity of systemic ventricular dysfunction.

Pre-operative characteristics with greater than mild systemic atrioventricular valve regurgitation, longer length of stay after the initial palliative procedure, and lower superior vena cava oxygen saturation were associated with prolonged length of stay after a first-time bidirectional cavopulmonary anastomosis.

We investigated the efficacy and complication profile of intranasal dexmedetomidine for transthoracic echocardiography sedation in patients with single ventricle physiology and shunt-dependent pulmonary blood flow during the high-risk interstage period.

A single-centre, retrospective review identified interstage infants who received dexmedetomidine for echocardiography sedation. Baseline and procedural vitals were reported. Significant adverse events related to sedation were defined as an escalation in care or need for any additional/increased inotropic support to maintain pre-procedural haemodynamics. Minor adverse events were defined as changes from baseline haemodynamics that resolved without intervention. To assess whether sedation was adequate, echocardiogram reports were reviewed for completeness.

From September to December 2020, five interstage patients (age 29–69 days) were sedated with 3 mcg/kg intranasal dexmedetomidine. The median sedation onset time and duration time was 24 minutes (range 12–43 minutes) and 60 minutes (range 33–60 minutes), respectively. Sedation was deemed adequate in all patients as complete echocardiograms were accomplished without a rescue dose. When compared to baseline, three (60%) patients had a >10% reduction in heart rate, one (20%) patient had a >10% reduction in oxygen saturations, and one (20%) patient had a >30% decrease in blood pressure. Amongst all patients, no significant complications occurred and haemodynamic changes from baseline did not result in need for intervention or interruption of study.

Intranasal dexmedetomidine may be a reasonable option for echocardiography sedation in infants with shunt-dependent single ventricle heart disease, and further investigation is warranted to ensure efficacy and safety in an outpatient setting.

Children with a single ventricle post-Fontan palliation are at increased risk of poor outcomes with peak oxygen consumption acting as a surrogate outcome marker. The purpose of this study is to evaluate the relationship between peak oxygen consumption and echocardiographic measures of ventricular function and deformation, including ventricular global longitudinal strain and dyssynchrony, in children and adolescents following Fontan palliation.

Patients (age 8–21 years) with single ventricle post-Fontan palliation were prospectively recruited and participated in an echocardiogram, including views optimised for two-dimensional speckle tracking, and a cardiopulmonary exercise test on a cycle ergometer to maximal volitional fatigue.

Thirty-eight patients (mean age 13.7 ± 2.3 years) post-Fontan palliation had either a single left ventricular (n = 20), single right ventricular (n = 14), or biventricular (n = 4) morphology. Peak oxygen consumption (24.9 ± 5.6 ml/kg/minute) was correlated with global longitudinal strain (r = −0.435, p = 0.007), a strain discoordination time to peak index (r = −0.48, p = 0.003), and the presence of an electro-mechanical dyssynchrony strain pattern (p = 0.008). On multivariate regression modelling, these three variables were associated with peak oxygen consumption independently of age and sex. The single right ventricular group had evidence of possible diastolic dysfunction by E/e’ compared to the single left ventricular and biventricular groups (p = 0.001).

Strain analysis measures are correlated with peak oxygen consumption in this cohort of children, adolescents, and young adults following Fontan palliation, suggesting that ventricular mechanics may influence the efficiency of the Fontan circulation.