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.

Growing evidence has emphasised the importance of ventricular performance in functionally single-ventricle patients, particularly concerning diastolic function. Cardiac MRI has been proposed as non-invasive alternative to pre-Fontan cardiac catheterisation in selected patients.

To identify clinical and cardiac magnetic resonance predictors of high pre-Fontan end-diastolic ventricular pressure.

In a retrospective single-centre study, 38 patients with functionally univentricular heart candidate for Fontan intervention, who underwent pre-Fontan cardiac catheterisation, beside a comprehensive cardiac MRI, echocardiographic, and clinical assessment were included. Medical and surgical history, cardiac magnetic resonance, cardiac catheterisation, echocardiographic, and clinical data were recorded. We investigated the association between non-invasive parameters and cardiac catheterisation pre-Fontan risk factors, in particular with end-diastolic ventricular pressure. Moreover, the impact of conventional invasive pre-Fontan risk factor on post-operative outcome as also assessed.

Post-operative complications were associated with higher end-diastolic ventricular pressure and Mayo Clinic indexes (p < 0.01 and p = 0.05, respectively). At receiver operating characteristic curve analysis end-diastolic ventricular pressure ≥ 10.5 mmHg predicted post-operative complications with a sensitivity of 75% and specificity of 88% (AUC: 0.795, 95% CI 0.576;1.000, p < 0.05). At multivariate analysis, both systemic right ventricle (OR: 23.312, 95% CI: 2.704–200.979, p < 0.01) and superior caval vein indexed flow (OR: 0.996, 95% CI: 0.993–0.999, p < 0.05) influenced end-diastolic ventricular pressure ≥ 10.5 mmHg.

A reduced superior caval vein flow, evaluated at cardiac magnetic resonance, is associated with higher end-diastolic ventricular pressure a predictor of early adverse outcome in post-Fontan patients.

The single- and double-patch repairs are undoubtedly the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in paediatric and adult patients, focusing in particular on the occurrence of ectopic atrial rhythm.

Seventy patients (male: 38, 54.2%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. Forty-nine patients (70%) underwent surgical repair in paediatric age (<16 years old), while 21 of (30%) patients were operated in adulthood. Thirty patients (42.8%) underwent single-patch repair and 39 patients (55.7%) underwent double-patch repair. In only one patient, the Warden procedure was performed (1.4%). Median follow-up time was 52 months (IQ 15.1–113).

The type of surgical technique didn’t affect the incidence of ectopic atrial rhythm (26.6% in single-patch group and 25.6% in double-patch groups, p = 0.9). At long-term follow-up, ectopic atrial rhythm, as an expression of sinoatrial node disturbance, was however significantly more frequent in the paediatric population (28.8% paediatric group and 4.7% adult group, p = 0.02).

The higher incidence of ectopic atrial rhythm in children is probably related to the closer position of the sinus node to the superior cavoatrial incision, which makes irreversible iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavoatrial junction should, therefore, be preferred for children undergoing partial anomalous pulmonary venous connection repair.