Search

Background: The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain biventricular circulation. Long-term complications are common, such as veno-venous collaterals (VVC). The aim of this study was to evaluate patient characteristics, percutaneous treatment strategy and (short-term) outcomes in adult Fontan patients with VVC, and review literature to date. Methods: In this single-centre retrospective observational cohort study, patients who underwent percutaneous VVC closure between 2017 and 2023 were identified. Results: Thirteen patients underwent percutaneous VVC closure (77 % female, age at intervention 24 ± 4 years, 77 % systemic left ventricle, 77 % extracardiac tunnel, median conduit size 16 [16–20]mm). Indications for closure were symptoms and/or significant exercise-related hypoxia. Mean Fontan pressure was 10±4 mmHg. The VVC originated from tributaries of the vena cava superior (VCS) and connected to pulmonary veins (8 VVC, 32 %), VCS to systemic atrium (3 VVC, 12 %), VCS to coronary sinus (3 VVC, 12 %) and tributaries of vena cava inferior to pulmonary veins (11 VVC, 44 %). Twenty-three VVC were occluded using coils and/or plugs. No periprocedural complications occurred. At first follow-up at least 6 months after closure (n = 11), 9 patients (82 %) reported symptom reduction. Saturation at rest and peak exercise increased significantly (96 ± 3 to 98 ± 1 %, p = 0.040; 89 ± 3 to 93 ± 5 %, p = 0.024, respectively). Exercise capacity remained unchanged. Conclusions: VVC typically connect the tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible, safe, and associated with symptom reduction and a significant rise in resting and exercise oxygen saturation.

Catheter interventions can offer patient tailored solutions in high-risk congenital heart disease patients. A 21-year-old male with a Fontan circulation in the setting of unbalanced atrioventricular septal defect with a hypoplastic left ventricle and an aortic homograft connecting the right ventricular outflow tract to the ascending aorta, developed failure of the heavily calcified homograft with severe regurgitation and stenosis. He underwent three sequential transcatheter aortic valve-in-valve implantations to address the homograft failure and the subsequent paravalvular regurgitation, with satisfactory result and improved hemodynamics.

AimsGiven the compelling evidence on the effectiveness of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in the conventional heart failure population, SGLT2i deserve exploration in systemic right ventricular (sRV) failure. The initial experience with dapagliflozin in sRV failure patients is described, with a focus on tolerability and short-term effects on clinical outcomes.Methods and resultsTen patients (70% female, median age 50 years [46.5–52]) with symptomatic sRV failure who received dapagliflozin 10 mg per day on top of optimal medical therapy between 04–2021 and 01–2023 were included. Within 4 weeks, no significant changes in blood pressure, electrolytes, or serum glucose occurred. Creatinine and estimated glomerular filtration rate (eGFR) showed a slight decline (88 ± 17 to 97 ± 23 µmol/L, p = 0.036, and 72 ± 14 vs. 66 ± 16 ml/min/1.73m2, p = 0.020, respectively). At 6 months follow-up (n = 8), median NT-proBNP decreased significantly from 736.6 [589.3–1193.3] to 531.6 [400.8–1018] ng/L (p = 0.012). Creatinine and eGFR recovered to baseline levels. There were no significant changes in echocardiographic systolic sRV or left ventricular function. New York Heart Association class improved significantly in 4 out of 8 patients (p = 0.046), who also showed an improvement in the 6-minute walk test or bicycle exercise test performance. One female patient developed an uncomplicated urinary tract infection. No patients discontinued treatment.ConclusionDapagliflozin was well-tolerated in this small cohort of sRV failure patients. While the early results on the reduction of NT-proBNP and clinical outcome parameters are encouraging, large-scale prospective studies are warranted to thoroughly evaluate the effects of SGLT2i in the growing sRV failure population.

Abstract In congenitally corrected transposition of the great arteries, the morphological right ventricle supports the systemic circulation. This chronic exposure to pressure overload ultimately leads to systemic right ventricular (sRV) dysfunction and heart failure. Pharmacological options for the treatment of sRV failure are poorly defined and no solid recommendations are made in the most recent guidelines. Sodium‐glucose cotransporter 2 (SGLT‐2) inhibitors are a new class of antihyperglycaemic drugs that have been demonstrated to significantly reduce the risk of worsening heart failure and death from cardiovascular causes in patients with chronic heart failure with reduced left ventricular ejection fraction, yet no data are available in sRV patients. We report on the treatment and clinical follow‐up of a patient with advanced heart failure and poor sRV function in the context of congenitally corrected transposition of the great arteries, who did not tolerate sacubitril/valsartan and had a high burden of heart‐failure‐related hospitalizations. Treatment with dapagliflozin was well tolerated and resulted in (small) subjective and objective functional and echocardiographic improvement and a reduction in heart‐failure‐related hospitalizations.

BackgroundPatients with transposition of the great arteries (TGA) after an atrial switch or congenitally corrected TGA (ccTGA) are prone to systemic right ventricular (sRV) failure. Tricuspid valve (TV) regurgitation aggravates sRV dysfunction. Timely TV surgery stabilizes sRV function, yet the development of atrioventricular (AV)-conduction disturbances in the course of sRV failure can contribute to sRV dysfunction through pacing-induced dyssynchrony. This study aims to explore the incidence, timing, and functional consequences of AV-block requiring ventricular pacing after TV surgery in patients with sRV.MethodsConsecutive adolescent and adult patients with an sRV who underwent TV surgery between 1989 and 2020 and followed-up at our center were included in this observational cohort study.ResultsThe data of 28 patients (53% female, 57% ccTGA, and a mean age at surgery 38 ± 13 years) were analyzed. The mean follow-up was 9.7 ± 6.8 years. Of the remaining 22 patients at the risk of developing high degree AV-block after TV surgery, 9 (41%) developed an indication for chronic ventricular pacing during follow-up, of which 5 (56%) within 24 months postoperatively (3 prior to hospital discharge). The QRS duration, a surrogate marker for dyssynchrony, was significantly higher in patients with chronic left ventricular pacing than in patients with native AV-conduction (217 ± 24 vs. 116 ± 23 ms, p = 0.000), as was the heart failure biomarker NT-pro-BNP [2,746 (1,242–6,879) vs. 495 (355–690) ng/L, p = 0.004] and the percentage of patients with ≥1 echocardiographic class of deterioration of systolic sRV function (27 vs. 83%, p = 0.001). Of the patients receiving chronic subpulmonary ventricular pacing (n = 12), 9 (75%) reached the composite endpoint of progressive heart failure [death, ventricular assist device implantation, or upgrade to cardiac resynchronization therapy (CRT)]. Only 4 (31%) patients with native AV-conduction (n = 13) reached this composite endpoint (p = 0.027).ConclusionPatients with a failing sRV who undergo TV surgery are prone to develop AV-conduction abnormalities, with 41% developing an indication for chronic ventricular pacing during 10 years of follow-up. Patients with chronic subpulmonary ventricular pacing have a significantly longer QRS complex duration, have higher levels of the heart failure biomarker NT-pro-BNP, and are at a higher risk of deterioration of systolic sRV function and progressive heart failure.