Fontan-associated liver disease and total cavopulmonary anatomical flow effectors.

OBJECTIVE: We investigated a relationship between a composite index comprised of Fontan-circuit anatomical features and hepatic fibrosis scores from biopsy. METHODS: We identified living extracardiac Fontan patients, ≥7 years old and ≥5 but <20 years postoperative, that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2020. We divided patients into anatomical groups and applied a risk score to each patient. We compared average anatomical risk scores with average hepatic total fibrosis scores by group. RESULTS: We identified 111 patients that met inclusion criteria. After excluding four patients, we assigned 107 to one of 12 anatomical variant groups (n ≥ 3). For the 107, the average age at liver biopsy was 14 ± 6 years old. Of the 107, 105 (98%) were New York Heart Association Class 1. We found average anatomical risk scores by group correlated with average total fibrosis scores by group (R = 0.8; p = .005). An average Fontan duration to biopsy of 10 ± 1 years was similar for all 12 anatomical groups. We found no other clinical variables, laboratory, or hemodynamic values that trended with anatomical risk scores or hepatic total fibrosis scores. CONCLUSIONS: In a cohort of relatively young, stable extracardiac Fontan patients, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group. These findings suggest that some anatomical variants in extracardiac Fontan patients are associated with higher Fontan-associated liver disease (FALD)-related hepatic total fibrosis scores than others, despite similar Fontan durations.

Effect of Fenestration on Early Postoperative Outcome in Extracardiac Fontan Patients with Different Risk Levels.

Although fenestration is used to improve the postoperative course of Fontan patients, the effect of fenestration on the extracardiac Fontan seems controversial especially at early postoperation. One hundred and eighty-three patients from January 2004 to June 2013 undergoing an extracardiac Fontan operation were retrospectively selected for this study. We divided the patients into low risk (93 patients) and high risk (90 patients) groups according to the risk factors recognized by previous studies and then compared the perioperative data between the nonfenestrated and fenestrated patients in each group. In both groups there was no significant difference in preoperative and operative data between the nonfenestrated and fenestrated patients. The postoperative blood oxygen saturation of fenestrated patients was significantly lower (p < 0.01) in each group. In the high risk group the chest tube volume (1153 mL vs. 1739 mL, p = 0.021) and chest tube duration (11.9 days vs. 17.0 days, p = 0.028) of fenestrated patients were lower comparing to nonfenestrated patients, while the chest tube volume and chest tube duration were similar between the nonfenestrated and fenestrated patients in the low risk group. The morbidity and mortality of nonfenestrated and fenestrated patients were similar in both groups (p > 0.05). Although fenestration was associated with lower postoperative oxygen saturation, fenestration showed better postoperative outcomes regarding the chest tube volume and duration for the high-risk patients. Considering the similar early postoperative outcomes of nonfenestrated and fenestrated patients in low risk group, our data indicate that fenestration for the high-risk patients should be performed.

Outcomes of extracardiac Fontan operation: A single institution experience with 398 patients.

OBJECTIVE: The study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution. METHODS: We conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up. RESULTS: The median follow-up time was 10.3 years (interquartile range, 6.4-14.6). The overall survival was 96% and 86% at 10 and 20 years after extracardiac Fontan operation, respectively. There were 6 early deaths (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation was 88% at 10 years and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular dominance (hazard ratio, 4.7; P < .001; 95% CI, 2.1-10.5), aortic atresia (hazard ratio, 5.5; P < .001; 95% CI, 2.3-12.8), and elevated mean pulmonary artery pressure (hazard ratio, 2.3; P = .002; 95% CI, 1.2-6.7). CONCLUSIONS: Rates of failure of the Fontan circulation are low after the contemporary extracardiac Fontan operation. Risk factors for failure of the extracardiac Fontan circulation include right ventricular dominance, aortic atresia, and elevated pulmonary artery pressures.

Hepatic Fibrosis Risk Factors in Extracardiac-Fontan Patients: Observations From a Single Center.

Objective: We reviewed our experience with transvenous liver biopsy-derived hepatic fibrosis scores and possible associated risk factors in those postextracardiac Fontan patients. Methods: We identified extracardiac-Fontan patients with postoperative durations <20 years who underwent cardiac catheterizations with transvenous hepatic biopsies between April 2012 and July 2022. If a patient underwent two liver biopsies, we averaged the two total fibrosis scores and concurrent time, pressure, and oxygen saturation data. We grouped patients by the following factors: (1) sex, (2) venovenous collaterals, and (3) type of functionally univentricular heart. We identified potential hepatic fibrosis risk factors as the following: female, presence of venovenous collaterals, and a functional univentricle of right-ventricular type. For statistical analysis, we used Kruskal-Wallis nonparametric testing. Results: We identified 127 patients who underwent 165 transvenous biopsies, with 38 patients undergoing 2 biopsies. We found that females with two additional risk factors had the highest median total fibrosis scores, 4 (1-8); males with <2 risk factors had the lowest median total fibrosis scores, 2 (0-5); and females with <2 additional risk factors and males with two risk factors were in the middle, median total fibrosis score 3 (0-6), P =.002; and there were no statistical differences for the other demographic or hemodynamic variables. Conclusions: For extracardiac-Fontan patients with similar demographic and hemodynamic variables, identifiable risk factors are associated with the degree of hepatic fibrosis.

Transvenous pacing approach for atrioventricular block in fontan - Possibility of transvenous approach by electrophysiological assessment.

In extracardiac Fontan, an epicardial pacemaker implantation has many limitations, especially given that it is highly invasive and a high-risk procedure due to repeat thoracotomy. Herein we illustrate a case with the possibility of transvenous pacing in extracardiac Fontan being less invasive and lower risk transvenous dual-chamber pacemaker implantation by electrophysiological assessment. .

Completion of an interventional extracardiac Fontan.

The extracardiac Fontan can be completed via transcatheter perforation of the pericardial membrane created during the preparatory stage, thus establishing continuity between the inferior vena cava and the pulmonary artery. This step is followed by deployment of a covered stent to isolate the systemic and pulmonary circuits. The procedure avoids the morbidity associated with cardiac reoperation and is a safe option for patients who present late with prohibitive pulmonary artery pressures in whom primary Fontan completion may not be feasible.

Off-pump extracardiac Fontan completion: Surgical technique and pitfalls.

Completion of the extracardiac Fontan procedure is the final palliative stage for treating a functional single ventricle. It has been associated with a smaller incidence of atrial arrhythmias and more laminar flow in the Fontan pathway. We present our technique for the off-pump extracardiac Fontan procedure.

Off-Pump "Clamp and Sew" Extracardiac Fontan With Inverted Bifurcated Graft for Dextrocardia With Heterotaxy Syndrome.

Avoiding cardiopulmonary bypass during palliation of single ventricle has the advantages of minimizing transfusions, pulmonary vascular resistance, and avoiding the inflammatory response from cardiopulmonary bypass. It is however not always straightforward, and the technique may be faced with challenges.

Improved Technique for Interventional Extracardiac Fontan.

We have previously reported a simple technique for preparatory staging and subsequent interventional completion of an extracardiac Fontan procedure that anatomically and hemodynamically closely mimics a standard extracardiac Fontan. We describe herein modifications that simplify the original procedural sequence and that may allow wider application. Percutaneous completion can be achieved even without a radiofrequency probe, using electrocautery. Fenestration is also easy to perform if there is a clinical indication.

Systemic right ventricular morphology in the early postoperative course after extracardiac Fontan operation: is there still a need for special care?

Objectives: We aimed to compare early postoperative outcome after extracardiac (EC) Fontan operation between patients with right (RV) or left (LV) systemic ventricles. Methods: In total, 173 consecutive patients (median age 4 years, median weight 14 kg) underwent EC Fontan between 1995 and 2013. Pre- and intraoperative data as well as detailed postoperative haemodynamic variables were compared between patients with LV [ n = 109 (63%)] and RV [ n = 64 (37%)]. Results: : RV patients showed significantly lower mean arterial (median 55 vs 59 mmHg, P = 0.04), higher atrial (median 8 vs 6 mmHg, P = 0.03) and comparable pulmonary pressure (median 14 vs 14 mmHg, P = 0.7) as well as lower mean systemic perfusion pressure (median 39 vs 43 mmHg, P = 0.03) on Day 0 after EC Fontan. They suffered from longer intubation time (median 18 vs 12 h, P = 0.008), higher incidence of ascites (46% vs 28%, P = 0.04) and need for dialysis (21% vs 4%, P = 0.003). Prolonged inotropic support (25% vs 8%, P = 0.02) and pharmacological treatment to reduce pulmonary vascular resistance (71% vs 53%, P = 0.002) were more often used in RV patients and they showed more often supraventricular tachyarrhythmia (27% vs 5%, P < 0.001) and a longer intensive care unit-stay (median 4 vs 3 days, P = 0.03). However, early mortality, need for Fontan takedown, use of mechanical circulatory support, pleural effusions and hospital stay were not significantly different between both groups. Conclusions: Patients with systemic RV demonstrate higher morbidity in the early postoperative course compared with patients with systemic LV anatomy and require intensified postoperative management to avoid postoperative Fontan failure.

Easy Fenestration Technique for Extracardiac Fontan Operation.

During an extracardiac conduit type of Fontan operation, fenestration is not always technically easy, particularly when the right atrium is small and located posteriorly. We describe here an easy technique for performing an anastomosis of a polytetrafluoroethylene ringed graft to the atrium for fenestration in the setting of an extracardiac conduit Fontan operation. The surgical technique starts with a purse string suture that is customized to fit the atrium and utilizes a ringed graft that is inserted into the right atrium through the purse string, which is then tied.