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• Atrial fibrosis is a key pathological change of atrial myopathy. • Progression of atrial fibrosis is common long after classic Fontan surgery. • Atrial fibrosis was more common in the persistent/permanent atrial tachyarrhythmia group. • Atrium enlargement was found to correlate with atrial fibrosis. • Long-term Fontan circulation contributed to atrial fibrosis progression. The progression of atrial fibrosis long after Fontan surgery is unclear. This study aimed to evaluate the degree of atrial fibrosis long after the classic Fontan procedure and to investigate the factors associated with atrial fibrosis. We obtained atrial free wall specimens resected at Fontan conversion from 43 patients (Fontan group) and studied the degree of atrial fibrosis, along with its association with atrial tachycardia/fibrillation (AT/AF) and other clinical parameters, compared with those of the control group without heart disease (n=6). The time after the initial Fontan procedure was 19.9 (15.9–25.3) years. Atrial fibrosis (%) was more common in the Fontan group than in the control group [24.3 (20.9–35.0)% vs. 6.2 (5.6–7.5)%, p <0.001]. The severity of atrial fibrosis was mild in 16% (n=7), moderate in 54% (n=23), and severe in 30% (n=13) of cases. Atrial fibrosis (%) was more common in the persistent/permanent AT/AF group than in the no AT/AF (p <0.001) and paroxysmal AT/AF (p <0.001) groups. The maximum atrial diameter on computed tomography (CT) (mm) significantly correlated with atrial fibrosis (%) (r=0.52, p <0.001). The maximum diameter of the right atrium (≥75 mm) on CT was a significant risk factor for severe atrial fibrosis on multivariate logistic analysis (hazard ratio=10.22, 95% confidence interval=1.04–254.8, p =0.04). Atrial fibrosis was prominent long after classic Fontan surgery, especially in patients with non-paroxysmal AT/AF and dilated right atrium. [Display omitted] [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Fontan patients are known to suffer from clinical attrition over the years, which has been characterized as Fontan failure. We sought to evaluate the clinical outcomes of such Fontan patients undergoing surgical management in a 25-year, single-centre experience. METHODS A retrospective single-centre analysis of patients undergoing surgical treatment for failing Fontan between 1995 and 2020, including any reoperations when ventricular function was preserved, or a heart transplant (HTx), when ventricular contractility was impaired. We analysed survival, indications for surgery and early and late complication rates. RESULTS We collected 30 patients (mean age 24.7 years) who required surgery after a mean time of 19.3 years from the original Fontan procedure: Fontan conversion in 21 (70%, extracardiac conduit in 19, lateral tunnel in 2), a HTx in 4 (13.3%) and other reoperations in 5 (16.7%). The most common indications for surgery were tachyarrhythmias (63.3%) and severe right atrial dilatation (63.3%). Overall survival at the 1-, 5-, 10- and 20-year follow-up examinations were 75.9% [95% confidence interval (CI): 91.4–60.4%], 75.9% (95% CI: 91.4–60.4%), 70% (95% CI: 78–52%) and 70% (95% CI: 78–52%), respectively. The most frequent complications were postoperative tachyarrhythmias (50%) and late Fontan-associated liver disease (56.5%). HTx and Fontan conversion provided comparably good outcomes compared to other reoperations (P  = 0.022). CONCLUSIONS Surgery for failing Fontan can be performed effectively with overall good long-term survival. However, early and late morbidities are still a significant burden. Because other reoperations performed when patients presented with contraindications for a HTx have carried high mortality, close clinical follow-up is mandatory, and an earlier indication for Fontan conversion or a HTx is advisable to optimize outcomes. [ABSTRACT FROM AUTHOR]

A 38-year-old man with a functional single ventricle secondary to hypoplastic left heart syndrome presented with exertional fatigue. His last palliation was an intra-atrial conduit Fontan procedure. Comprehensive evaluation showed elevated liver enzyme values and a small, calcified conduit. Successful conversion to a nonfenestrated extracardiac conduit Fontan was performed with normalization of his liver enzyme values., Competing Interests: Dr Said has served as a consultant for Artivion, Abbott, and Japanese Organization for Medical Device Development (JOMDD). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Objectives: Patients with univentricular hearts who require permanent pacing systems typically require placement of epicardial leads. It is frequently difficult to find a position with good thresholds due to epimyocardial fibrosis or fat. The goal of the study is to assess the progression of capture thresholds (CT), sensing parameters (P waves and R waves), and impedances (imp) of steroid eluting epicardial pacing leads in young adults who underwent Fontan conversion and a pacemaker implant. Methods: All patients undergoing Fontan conversion in two institutions were retrospectively identified. Demographic data, congenital heart defects, pacing leads used, and pacing parameters were analyzed at implant, at 6 weeks and 12 months after implant. Results: Twenty patients were identified (twelve males); mean age at conversion was 24.9 ± 5.4 years (range 18-35). Epicardial bipolar steroid eluting leads were used. The site of implant both in the atria and the ventricles varied depending on the parameters. At implant, mean atrial and ventricular impedances were 617 ± 171 O and 1061 ± 771 O, respectively, mean P wave amplitude was 2 ± 0.7 mV, and mean R wave amplitude was 12.5 ± 7.7 mV. Mean CT was 1.7 ± 0.8 V at 0.5 ms for the atrium and 2.2 ± 1.2 V at 0.5 ms for the ventricle. Ventricular CT and impedance showed an improvement within the first 12 months after implant, with four patients having a decrease in threshold of more than 2 V. Conclusion: In patients undergoing Fontan conversion, implant ventricular CT and impedances are frequently higher than expected but typically improve during follow-up. Acceptance of higher initial threshold values may be a potential strategy in this patient population. [ABSTRACT FROM AUTHOR]

• Fontan conversion can be suggested in asymptomatic patients. • Fontan conversion with the Maze procedure may prevent future tachyarrhythmia. • The outcome is compatible with that of primary total cavopulmonary connection. • The Maze procedure should be reserved for those with a history of tachyarrhythmia. • Sinus node dysfunction can occur after the prophylactic Maze procedure. : The indication of Fontan conversion (FC) from atriopulmonary connection (APC) to total cavopulmonary connection (TCPC) is unclear. We sought to analyze the mid-term outcome of prophylactic and therapeutic Fontan conversion compared with that of primary TCPC. : Patients with a univentricular heart who underwent cardiac catheterization at >18 years of age between July 2005 and July 2019 were included and divided into three groups: symptomatic APC patients who underwent therapeutic FC (t-FC, n = 13), asymptomatic APC patients after prophylactic FC (p-FC, n = 15), and patients who had primary TCPC procedure (pTCPC, n = 24). : The mean last follow up was at the age of 32.0 ± 7.8, 26.8 ± 3.8, and 27.3 ± 7 years (p = 0.07) in t-FC, p-FC, and pTCPC, respectively. There was no late death. All of t-FC and 12 (80%) of p-FC cases underwent concomitant arrhythmic surgery. Consequently, five and four patients in t-FC and p-FC groups required pacemaker implantations mostly due to sinus node dysfunction. Thromboembolism was seen in 2 cases in both t-FC (15%) and p-FC (13%), and 1 case in pTCPC (4%) (p = 0.50). The last cardiac catheterization was performed at the age of 29.5 ± 8.5, 24.6 ± 3.8, and 26.3 ± 7.1 years (p = 0.11) in t-FC, p-FC, and pTCPC patients, respectively. There was no significant difference in central venous pressure, aortic pressure, and cardiac index among the three groups. There was no late supraventricular tachyarrhythmic event seen in t-FC and p-FC, whereas two patients in pTCPC had newly developed atrial flutter. : FC is a safe and feasible procedure to bring APC patients back onto the same track of primary TCPC patients in terms of hemodynamics as well as arrhythmia. The antiarrhythmic procedure should be carefully chosen because sinus node dysfunction can frequently occur and FC itself would reduce the risk of arrhythmia. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9–29.2) and 17.8 years (15.4–20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P  = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P  < 0.0001) and cardiac index (2.0 ± 0.3 to 2.9 ± 0.6 l/min/m2, P  < 0.0001) significantly improved from preoperative evaluation to 1 year after the conversion, and these improvements were maintained during the entire follow-up period. Peak oxygen uptake remained unchanged from the preoperative evaluation (49.7 ± 11.5% predicted) to 1 year (52.5 ± 12.0%), 5 years (56.2 ± 9.6%) and 10 years (51.2 ± 9.4%) after conversion (P  = 0.19). CONCLUSIONS Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients. [ABSTRACT FROM AUTHOR]

Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To investigate the benefits of a strategy of early Fontan conversion. METHODS: Using the Australia and New Zealand Fontan Registry, retrospective analysis of their long-term follow-up data was performed. RESULTS: Between 1990 and 2014, a total of 39 patients underwent surgical conversion in 6 centres at a median age of 23.8 years (IQR: 19.3–28.2), 18.7 ± 5.0 years post-Fontan. One centre tended to perform conversion earlier: interval since first documented arrhythmia 2.9 ± 4.0 vs 4.0 ± 4.2 years, average NYHA Class 2 ± 0.4 vs 3 ± 0.9 (P = 0.008), mean number of preop anti-arrhythmics 1 ± 0.4 vs 2 ± 0.6 (P = 0.05). Two patients underwent conversion to an extracardiac conduit only, while 36 had concomitant right atrial cryoablation, of which 16 also had pacemaker implantation. Nine patients suffered major cardiac-related complications (7 low output syndrome, 3 ECMO, 3 acute renal failure, one stroke) (2/17 from the early conversion centre and 7/22 of the others; P = 0.14). Four patients died in hospital (10.3%) and 4 late after a median of 0.9 years [95% confidence interval (CI): 0.5–1] after conversion. An additional 2 patients needed transplantation at 1 and 8.8 years after conversion, respectively. The 10-year freedom from heart transplantation was 86% (95% CI: 51–97%). Outcomes from the centre with an early conversion strategy were significantly better: 8-year freedom from death or heart transplantation was 86% (95% CI: 53–96) vs 51% (95% CI: 22–74; log-rank P = 0.007). Eight additional patients required pacemaker implantation and 5 had arrhythmia recurrence. CONCLUSIONS: Fontan conversion is associated with lasting survival outcomes up to 10 years after conversion. A strategy of surgical conversion at earlier stage of failure may be associated with better survival free from transplantation. [ABSTRACT FROM AUTHOR]

OBJECTIVES We investigated the mid-term surgical results of Fontan conversion with antiarrhythmias surgery and permanent pacemaker implantation, which were complications of a previous Fontan operation. METHODS From January 1996 to November 2011, we performed Fontan conversion in 31 of 260 Fontan cases (M:F = 17:14, mean age = 19.0 years). The Fontan conversion from atriopulmonary connection (APC) to extracardiac conduit (ECC) was performed in 20 patients, APC to lateral tunnel in 5 and lateral tunnel to ECC in 6. The clinical outcomes and improvements of arrhythmias were analysed. The types of arrhythmias included atrial flutter in 21 patients, atrial fibrillation with flutter in 4 patients and junctional tachycardia in 3 patients. RESULTS Twenty-six patients (83.9%) required antiarrhythmia surgery (isthmus cryoablation = 9, right-sided maze = 13, bilateral maze = 4). In addition, 23 patients (74.2%) received a permanent pacemaker. The New York Heart Association functional class (NYHA Fc) was statistically improved after the surgery during the 6.5-year median follow-up duration (preoperative NYHA Fc = 1.77, postoperative NYHA Fc = 1.13, n = 15, P = 0.001). There were 4 late mortalities. Actuarial 5-year survival was 90.0 ± 5.5%. And freedom from arrhythmia was 91.8 ± 5.5%, at 5 years. Normal sinus rhythm was maintained in 12 patients (38.7%), pacing-dependent rhythm in 10 patients (32.3%) and intermittent pacing-dependent rhythm in 4 patients (12.9%). CONCLUSIONS Fontan conversion with antiarrhythmia surgery and permanent pacemaker implantation is safe and improves the clinical outcome and arrhythmias. [ABSTRACT FROM AUTHOR]

Abstract: Objective: In the long-term period after Fontan operation, atrial arrhythmia was one of the important factors to decide the postoperative quality of life. We reviewed the impact of Fontan conversion with arrhythmia surgery and pacemaker therapy. Methods: Thirty-eight patients underwent Fontan conversion using extracardiac conduit from 1992, and 22 patients with atrial arrhythmia underwent maze procedure simultaneously using cryoablation or radiofrequency ablation and epicardial DDD pacemaker implantation and 16 patients had regular ‘sinus’ rhythm before Fontan conversion. Mean follow-up period was 52 months. Pre- and postoperative clinical course were analyzed. Average weight, age at Fontan conversion, and years after first Fontan operation were 49.0kg, 25.8 years old, 14.7 years, respectively. Nineteen percent of patients were in New York Heart Association class I (NYHA I), and 74% of patients were in NYHA II, and 7% were in NYHA III, respectively. Results: Except three early deaths, actual survival rate at 1 year and 5 years were 80% and 64%, respectively. In survivors, 80% of the patients obtained regular heart rhythm including artificial pacemaker rhythm, although only 43% of the patients had regular ‘sinus’ rhythm before the Fontan conversion. Postoperative average cardiothoracic ratio and oxygen saturation (SpO2) were 50% and 94%, and 74% of patients were in NYHA I and 26% were in NYHA II, respectively, after Fontan conversion. Conclusions: Mid-term results of Fontan conversion with arrhythmia surgery and pacemaker therapy were acceptable. Restoration of regular rhythm might improve the postoperative NYHA status and the activity of the daily life. [Copyright &y& Elsevier]

Abstract: Objective: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with Fontan conversion and concomitant arrythmia surgery. Methods: Between January 1996 and February 2004, 16 patients underwent Fontan conversion and arrhythmia surgery. Mean age at the initial Fontan operation was 5.1±3.5 (range: 2–15) years and mean age at Fontan conversion was 17.0±5.8 (range: 6–30). The initial Fontan operations were atriopulmonary connections in 14 patients, extracardiac lateral tunnel in 1, and intracardiac lateral tunnel in 1. The types of arrhythmia included atrial flutter in 10 patients and atrial fibrillation in 3. Fontan conversion operation was performed with intracardiac lateral tunnel in 5 patients and extracardiac conduit in 11. Arrhythmia surgery included isthmus cryoablation in 10 patients and right-sided maze in 3. Results: There has been no mortality. At Fontan conversion operation, 7 patients required permanent pacemaker. All patients have improved to New York Heart Association class I or II. With a mean follow-up of 26.9±30.6 (range:1–87) months, 16 patients had sinus rhythm, 2 patients had transient atrial flutter which was well controlled, and 2 patients required permanent pacemaker during follow-up. Conclusions: Fontan conversion with concomitant arrhythmia surgery and permanent pacemaker placement is safe, improves New York Heart Association functional class, and has a low incidence of recurrent arrhythmias. In most patients, concomitant permanent pacemakers are needed. [Copyright &y& Elsevier]

Although Fontan conversion combined with cardiac resynchronization therapy appears to be an effective surgical solution for the management of failing Fontan circulation with refractory atrial arrhythmia and cardiac dysfunction due to dyssynchronous ventricular wall motion, limited data are available on the mid- to long-term results of this treatment. We report our successful experience with Fontan conversion combined with cardiac resynchronization therapy in a male patient with failing Fontan circulation who showed favourable outcomes 5 years after the operation. [ABSTRACT FROM AUTHOR]

The article offers information on the development of dilated atrial connection by atriopulmonary Fontan which leads to atrial arrhythmias and low cardiac output from inefficient venous flow. Topics discussed include quality of life and transplant-free survival of the patients; improvement in the outcomes of the operation; and development of ventricular dysfunction or multisystem organ failure.