Percutaneous closure of veno-venous collaterals in adult patients with univentricular physiology after Fontan palliation: Single centre experience and systematic review

Abstract

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.

Keywords: Adult congenital heart disease; Fontan circulation; Long term complications; Transcatheter interventions; Univentricular heart; Veno-venous collaterals.

Fig. 1

Prisma flow diagram illustrating the selection of studies on prevalence of VVC, associated factors, percutaneous closure strategy and outcomes VVC: veno-venous collaterals.

Fig. 2

Angiographic projection showing: (A) collateral flow from left subclavian vein to left inferior pulmonary venous return. (B) collateral flow (black arrow) from inferior vena cava to right and left inferior pulmonary venous return. (C) collateral flow from left subclavian vein to coronary sinus. (D, E) after coiling (red circle) there is reduced distal contrast opacification and (F) after closure with an Amplatzer Vascular plug (blue circle) APC: pulmonary trunk; APD: right pulmonary artery, APS: left pulmonary artery, CS: coronary sinus, LIPV: left inferior pulmonary vein, PL: posterolateral, RIPV: right inferior pulmonary vein. Black arrow: collateral, blue arrow: catheter, blue circle: Amplatzer Vascular plug II (Abbott), red circle: vortX pushable coils (Boston Scientific).

Fig. 3

Pie chart visualizing the origin and insertion of the veno-venous collaterals. CS: coronary sinus, PV: pulmonary veins, VCI: tributaries of the vena cava inferior, SVC: tributaries of the vena cava superior, VVC: veno-venous collaterals.

Fig. 4

Box and whisker plot showing the median peripheral oxygen saturation at ambient air (horizontal line) with 25th and 75th percentiles (box) and lower and upper extremes (whiskers) pre- and post-closure of the veno-venous collaterals (A) at rest, (B) at peak exercise. * statistically significant (p < 0.05).

Fig. 5

Forest plot for (A) oxygen saturation at rest directly after the procedure, and (B) peripheral saturation at rest at (first) follow-up SD: standard deviation. * patients without simultaneous fenestration closure.