Pacemaker Implantation Via Femoral Vein and Successful Arrhythmia Management in an Elderly Patient with Fontan Circulation: A Case Report

Background The frequency of arrhythmias increases after the Fontan operation over time; atrial tachycardia (AT) and sinus node dysfunction (SND) are frequently observed. Case summary Our patient was 63-year-old woman who underwent a lateral tunnel Fontan operation for double outlet right ventricle at age 36. She experienced paroxysmal AT for one year, and antiarrhythmic medication was not feasible due to symptomatic SND. Computed tomography revealed a 45 mm-sized thrombus in the high right atrium (RA). The patient had three coexisting conditions: paroxysmal AT, symptomatic SND and the RA thrombus, for which total cavopulmonary connection conversion and epicardial PMI would have been effective; however, given her age and comorbidities, surgical treatment was considered high-risk. Catheter ablation was avoided because of the RA thrombus. Finally, a transvenous pacemaker was implanted via the right femoral vein to avoid the RA thrombus and severe venous tortuosity from the left subclavian vein to the RA. After PMI, the patient was prescribed amiodarone and bisoprolol for AT suppression. AT occurred once in the third month after discharge. We increased the dose of amiodarone, and she has been tachycardia-free. Discussion Transvenous PMI must be considered in cases where open thoracic surgery or catheter ablation cannot be performed. This is the first report of transvenous PMI via the right femoral vein and successful AT and SND management in an elderly Fontan patient.

Surgical Planning and Optimization of Patient-Specific Fontan Grafts With Uncertain Post-Operative Boundary Conditions and Anastomosis Displacement

ObjectiveFontan surgical planning involves designing grafts to perform optimized hemodynamic performance for the patient’s long-term health benefit. The uncertainty of post-operative boundary conditions (BC) and graft anastomisis displacements may significantly affect the optimized graft designs and lead to undesired outcomes, especially for hepatic flow distribution (HFD). We aim to develop a computation framework to automatically optimize patient-specific Fontan grafts with the maximized possibility of keeping the post-operative results within clinical acceptable thresholds.MethodsThe uncertainties of BC and anastomosis displacements were modeled by using Gaussian distributions according to prior research studies. By parameterizing the Fontan grafts, we built surrogate models of hemodynamic parameters by taking the design parameters and BC as inputs. A two-phased reliability-based robust optimization (RBRO) strategy was developed by combining deterministic optimization (DO) and optimization under uncertainty (OUU) to reduce the computation cost.ResultsWe evaluated the performance of the RBRO framework by comparing it with the DO method on four Fontan patient cases. The results showed that the surgical plans computed from the proposed method yield up to 79.2% improvement on the reliability of HFD than those from the DO method (p < 0.0001). The mean values of iPL and %WSS satisfied the clinically acceptable thresholds.ConclusionThis study demonstrated the effectiveness of our RBRO framework to address uncertainties of BC and anastomosis displacements for Fontan surgical planning.SignificanceThe technique developed in this paper demonstrates a significant improvement in the reliability of predicted post-operative outcomes for Fontan surgical planning. This planning technique is immediately applicable as a building block to enable technology for optimal long-term outcomes for pediatric Fontan patients and can also be used to other pediatric and adult cardiac surgeries.

Quality of life and emotional vulnerability in a national cohort of adolescents living with Fontan circulation

Introduction: To investigate quality of life and mental health after Fontan completion, we aimed to characterise outcomes in a representative group of adolescent patients. The study was part of the pre-transition clinical work-up in adolescents with Fontan-type palliation of univentricular CHD. The programme covers the entire paediatric Fontan patient population in Norway. Methods: Our cross-sectional study included 42 adolescents with Fontan circulation aged 15–18. We recruited a control group of 29 healthy peers. Quality of life was measured by the Pediatric Quality of Life Inventory Questionnaire, while mental health was assessed with the Strength and Difficulties Questionnaire. Results: Fontan patients scored lower than healthy controls on the Pediatric Quality of Life Inventory total (p = 0.004), the physical (p < 0.001) and social (p = 0.001) functioning subscale, and the Strength and Difficulties Questionnaire subscale of emotional symptoms (p = 0.035). Compared to two of the healthy teens (7%), seven patients (16%) in the Fontan group scored as having impaired mental health (p = 0.224). The female/male ratio for individuals with impaired health was 7:2 (p = 0.003). Conclusions: Compared to healthy controls, adolescents after Fontan-type palliation in Norway have good health-related quality of life and mental health, despite having slightly lower score than healthy individuals, mainly in physical domains and school functioning. Compared to healthy controls and healthy teenagers, these adolescents have somewhat more emotional problems, and compared to male patients, female patients more often have impaired mental health.

Liver nodules after the Fontan operation: role of the computerised tomography scan

Currently, there is an increasing prevalence of liver nodules in patients following the Fontan operation. The appropriate non-invasive modalities have been applied to assess a diagnosis of Fontan-associated liver disease. The aims of this study were to determine the prevalence and associated factors for the presence of liver nodules using CT scan. A cross-sectional study of 34 patients older than 15 years of age was recruited. Ultrasound upper abdomen, ultrasound liver elastography, and CT scan of the upper abdomen were performed after the Fontan operation. The median age of patients was 20 years (range 14–36 years). The median age at the Fontan operation was 7 years (range 5–17 years) and the duration after the Fontan operation was 12 years (range 4–22 years). The prevalence of liver nodules was 62% as detected by CT scans. Hepatic vein pressure in patients with liver nodules was significantly higher than in those without liver nodules. Hepatic vein pressure above 13 mmHg was a factor associated with liver nodules. There was little agreement between the ultrasound of the upper abdomen and CT scan of the upper abdomen in the evaluation of liver nodules. Hepatic pressure was the only associated factor for the occurrence of liver nodules in patients following the Fontan operation. The prevalence of liver nodules was very high after the Fontan operation. The upper abdomen CT scan should be performed for the surveillance of liver nodules in every Fontan patient over the age of 15 years.

Fontan Fenestration and the Role of the Covered Stent

Key Points : • Interventional therapies directed at fenestration closure in the Fontan patient must rely on good hemodynamic data • The Large Optimus-CVSTM stent is an additional armamentarium for fenestration closure however, longer term follow up is needed • Multi institutional studies defining the long-term benefits of fenestration closure and outlining fenestration management guidelines may help improve the long-term morbidity and mortality in this group of patients.

Usefulness of a Radiofrequency Transseptal Needle in the Second Puncture of Fontan Extracardiac Conduit

We performed a second puncture of the extracardiac conduit in an 11-year-old Fontan patient to assess the patency of the stent previously deployed in the left pulmonary vein. For the first puncture, a mechanical Brockenbrough needle was selected to puncture the Gore-Tex conduit, an electrical insulator. For the second puncture, the location of that previous hole was detected as an indentation covered with atrial tissue, which is an electrical conductor. The second puncture was performed safely using a radiofrequency transseptal needle.