Current research suggests that a small pulmonary artery can cause adverse events and reduce exercise capacity after the Fontan procedure. This study aimed to evaluate the impact of pulmonary artery size on early haemodynamic and laboratory variables after total cavopulmonary connection.

We reviewed all patients who underwent staged Fontan between 2012 and 2022. Pulmonary artery index before bidirectional cavopulmonary shunt and before total cavopulmonary connection was calculated according to Nakata and colleagues. We sought to analyse the impact of the pulmonary artery index on early haemodynamic and laboratory variables, including pulmonary artery pressure and mean arterial pressure 12 hours after extubation and lactate levels 6 hours after extubation.

A total of 263 patients were included. Median age and weight at total cavopulmonary connection were 2.2 (interquartile ranges: 1.8–2.7) years and 11.7 (interquartile range: 10.7–13.3) kg, respectively. Before that, all patients underwent bidirectional cavopulmonary shunt at a median age of 4.1 (interquartile range: 3.2–5.8) months. In the multivariable analysis, pre-bidirectional cavopulmonary shunt pulmonary artery index (p = 0.016, odds ratio 0.993), with a cut-off value of 154 mm2/m2 was an independent risk factor for a higher pulmonary artery pressure (> 17 mmHg). No variable was identified as a significant risk factor for lower mean arterial pressure (< 57 mmHg). Regarding lactate levels (> 4.5 mg/dl), pre-bidirectional cavopulmonary shunt right pulmonary artery index (p < 0.001, odds ratio 0.983), with a cut-off value of 70 mm2/m2 was identified as an independent risk factor.

In patients with staged Fontan palliation, a small pulmonary artery size before bidirectional cavopulmonary shunt and total cavopulmonary connection was a determinant factor associated with unfavourable early postoperative haemodynamics after total cavopulmonary connection.

This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.

Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms. The prevalence of veno-venous collaterals, the risks for the development of veno-venous collaterals, and the impact of veno-venous collaterals on outcomes were analysed.

In total, 586 patients were included. Veno-venous collaterals were found in 72 (12.3%) patients. Majority of veno-venous collaterals originated from the superior caval vein and drained into the inferior caval vein. Before bidirectional cavopulmonary shunt, mean pulmonary artery pressure (16.3 vs. 14.5 mmHg, p = 0.018), and trans-pulmonary gradient (9.5 vs. 8.0 mmHg, p = 0.030) were higher in patients with veno-venous collaterals compared to those without. Veno-venous collaterals intervention was performed in 32 (5.5%) patients, in a median of 29 (16–152) days after bidirectional cavopulmonary shunt. Before total cavopulmonary connection, pulmonary artery pressure (10.3 vs. 9.4 mmHg, p = 0.015) and ventricular end-diastolic pressure (8.4 vs. 7.6 mmHg, p = 0.035) were higher, and arterial oxygen saturation (SaO2, 80.6 vs. 82.6 %, p = 0.018) was lower in patients with veno-venous collaterals compared to those without. More palliations before total cavopulmonary connection (p < 0.001, odds ratio: 1.689) were an independent risk for the development of veno-venous collaterals. Veno-venous collaterals did not affect survival after total cavopulmonary connection (92.8 vs. 92.7% at 10 years, p = 0.600).

The prevalence of veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection was 12%. Veno-venous collaterals may be induced by the elevated pulmonary artery pressure and trans-pulmonary gradient, and also by more previous palliations. However, they had no impact on clinical outcomes following total cavopulmonary connection.

This study aims to assess the surgical outcome of borderline hypoplastic left ventricle before and after the induction of the left ventricle rehabilitation strategy.

A retrospective review investigated patients with borderline hypoplastic left ventricle who underwent surgical intervention between 2012 and 2022. The patient cohort was stratified into two groups based on the initiation of left ventricle rehabilitation: an early-era group (E group, 2012–2017) and a late-era group (L group, 2018–2022). Left ventricle rehabilitation was defined as palliation combined with other procedures aimed at promoting left ventricular growth such as restriction of atrial septal defect, relief of inflow/outflow obstructive lesions, and resection of endocardial fibroelastosis.

A total of 58 patients were included. Primary diagnosis included 12 hypoplastic left heart syndromes, 11 critical aortic valve stenosis, and others. A total of 9 patients underwent left ventricle rehabilitation, 8 of whom underwent restriction of atrial septal defect. As for clinical outcomes, 9 of 23 patients achieved biventricular repair in the E group, whereas in the L group, 27 of 35 patients achieved biventricular repair (39% vs. 77%, p = 0.004). Mortality did not differ statistically between the two groups (log-rank test p = 0.182). As for the changes after left ventricle rehabilitation, left ventricular growth was observed in 8 of 9 patients. The left ventricular end-diastolic volume index (from 11.4 to 30.1 ml/m2, p = 0.017) and left ventricular apex-to-right ventricular apex ratio (from 86 to 106 %, p = 0.014) significantly increased after left ventricle rehabilitation.

The introduction of the left ventricle rehabilitation strategy resulted in an increased proportion of patients achieving biventricular repair without a concomitant increase in mortality. Left ventricle rehabilitation was associated with enhanced left ventricular growth and the formation of a well-defined left ventricle apex. Our study underscores the significance of left ventricle rehabilitation strategies facilitating successful biventricular repair. The data suggest establishing restrictive atrial communication may be a key factor in promoting left ventricular growth.

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.

This study aimed to assess the impact of caloric intake and weight-for-age-Z-score after the Norwood procedure on the outcome of bidirectional cavopulmonary shunt.

A total of 153 neonates who underwent the Norwood procedure between 2012 and 2020 were surveyed. Postoperative daily caloric intake and weight-for-age-Z-score up to five months were calculated, and their impact on outcome after bidirectional cavopulmonary shunt was analysed.

Median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Modified Blalock-Taussig shunt was used in 95 patients and right ventricle to pulmonary artery conduit in 58. Postoperatively, total caloric intake gradually increased, whereas weight-for-age-Z-score constantly decreased. Early and inter-stage mortality before stage II correlated with low caloric intake. Older age (p = 0.023) at Norwood, lower weight (p < 0.001) at Norwood, and longer intubation (p = 0.004) were correlated with low weight-for-age-Z-score (< –3.0) at 2 months of age. Patients with weight-for-age-Z-score < –3.0 at 2 months of age had lower survival after stage II compared to those with weight-for-age-Z-score of –3.0 or more (85.3 versus 92.9% at 3 years after stage II, p = 0.017). There was no difference between inter-stage weight gain and survival after bidirectional cavopulmonary shunt between the shunt types.

Weight-for-age-Z-score decreased continuously throughout the first 5 months after the Norwood procedure. Age and weight at Norwood and intubation time were associated with weight gain. Inter-stage low weight gain (Z-score < –3) was a risk for survival after stage II.

The purpose of this study is to evaluate the incidence and outcomes regarding tachyarrhythmia in patients after total cavopulmonary connection.

A retrospective analysis of 620 patients who underwent total cavopulmonary connection between 1994 and 2021 at our institution was performed. Incidence of tachyarrhythmia was depicted, and results after onset of tachyarrhythmia were evaluated. Factors associated with the onset of tachyarrhythmia were identified.

A total of 52 (8%) patients presented with tachyarrhythmia that required medical therapy. Onset during hospital stay was observed in 27 patients, and onset after hospital discharge was observed in 32 patients. Freedom from late tachyarrhythmia following total cavopulmonary connection at 5, 10, and 15 years was 97, 95, and 91%, respectively. The most prevalent late tachyarrhythmia was atrial flutter (50%), followed by supraventricular tachycardia (25%) and ventricular tachycardia (25%). Direct current cardioversion was required in 12 patients, and 7 patients underwent electrophysiological study. Freedom from Fontan circulatory failure after onset of tachyarrhythmia at 10 and 15 years was 78% and 49%, respectively. Freedom from occurrence of decreased ventricular systolic function after the onset of tachyarrhythmia at 5 years was 85%. Independent factors associated with late tachyarrhythmia were dominant right ventricle (hazard ratio, 2.52, p = 0.02) and weight at total cavopulmonary connection (hazard ratio, 1.03 per kilogram; p = 0.04). Type of total cavopulmonary connection at total cavopulmonary connection was not identified as risk.

In our large cohort of 620 patients following total cavopulmonary connection, the incidence of late tachyarrhythmia was low. Patients with dominant right ventricle and late total cavopulmonary connection were at increased risk for late tachyarrhythmia following total cavopulmonary connection.

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.