Percutaneous Closure of Veno-venous Collaterals in Adult Patients with Univentricular Physiology After Fontan Palliation: Single Centre Experience and Systematic Review

Overview

Journal Int J Cardiol Congenit Heart Dis

Date 2024 Dec 23

PMID 39712986

Authors

Marieke Nederend

Anastasia D Egorova

Frank van der Kley

Philippine Kies

Arno A W Roest

Martin J Schalij

Monique R M Jongbloed

Affiliations

Soon will be listed here.

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.

References

Schwartz M, Glatz A, Rome J, Gillespie M . The Amplatzer vascular plug and Amplatzer vascular plug II for vascular occlusion procedures in 50 patients with congenital cardiovascular disease. Catheter Cardiovasc Interv. 2010; 76(3):411-7. DOI: 10.1002/ccd.22370. View

FONTAN F, Baudet E . Surgical repair of tricuspid atresia. Thorax. 1971; 26(3):240-8. PMC: 1019078. DOI: 10.1136/thx.26.3.240. View

de Leval M, Kilner P, Gewillig M, Bull C . Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg. 1988; 96(5):682-95. View

Patan S . Vasculogenesis and angiogenesis. Cancer Treat Res. 2004; 117:3-32. DOI: 10.1007/978-1-4419-8871-3_1. View

Rijnberg F, van Assen H, Hazekamp M, Roest A, Westenberg J . Hemodynamic Consequences of an Undersized Extracardiac Conduit in an Adult Fontan Patient Revealed by 4-Dimensional Flow Magnetic Resonance Imaging. Circ Cardiovasc Imaging. 2021; 14(8):e012612. DOI: 10.1161/CIRCIMAGING.121.012612. View

Baba K, Kondo M, Eitoku T, Shigemitsu Y, Hirai K, Otsuki S . Vascular occlusion with 0.035-inch hydrogel expandable coils in congenital heart diseases and vascular anomalies. J Cardiol. 2022; 80(3):249-254. DOI: 10.1016/j.jjcc.2022.04.005. View

Rychik J, Atz A, Celermajer D, Deal B, Gatzoulis M, Gewillig M . Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation. 2019; 140(6):e234-e284. DOI: 10.1161/CIR.0000000000000696. View

Nederend M, Egorova A, Vliegen H, Roest A, Ruijter B, Korteweg T . Case report of the broad spectrum of late complications in an adult patient with univentricular physiology palliated by the Fontan circulation. Eur Heart J Case Rep. 2022; 6(2):ytac067. PMC: 8867817. DOI: 10.1093/ehjcr/ytac067. View

Stamm C, Friehs I, Duebener L, Zurakowski D, Mayer Jr J, Jonas R . Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg. 2003; 74(6):1967-77; discussion 1978. DOI: 10.1016/s0003-4975(02)04124-3. View

10.

Raimondi F, Martins D, Coenen R, Panaioli E, Khraiche D, Boddaert N . Prevalence of Venovenous Shunting and High-Output State Quantified with 4D Flow MRI in Patients with Fontan Circulation. Radiol Cardiothorac Imaging. 2021; 3(6):e210161. PMC: 8686005. DOI: 10.1148/ryct.210161. View

11.

Sugiyama H, Yoo S, Williams W, Benson L . Characterization and treatment of systemic venous to pulmonary venous collaterals seen after the Fontan operation. Cardiol Young. 2003; 13(5):424-30. View

12.

Kaulitz R, Ziemer G, Paul T, Peuster M, Bertram H, Hausdorf G . Fontan-type procedures: residual lesions and late interventions. Ann Thorac Surg. 2002; 74(3):778-85. DOI: 10.1016/s0003-4975(02)03756-6. View

13.

Ohuchi H, Mori A, Nakai M, Fujimoto K, Iwasa T, Sakaguchi H . Pulmonary Arteriovenous Fistulae After Fontan Operation: Incidence, Clinical Characteristics, and Impact on All-Cause Mortality. Front Pediatr. 2022; 10:713219. PMC: 9218217. DOI: 10.3389/fped.2022.713219. View

14.

Rijnberg F, van t Hul L, Hazekamp M, van den Boogaard P, Juffermans J, Lamb H . Haemodynamic performance of 16-20-mm extracardiac Goretex conduits in adolescent Fontan patients at rest and during simulated exercise. Eur J Cardiothorac Surg. 2022; 63(1). PMC: 9972516. DOI: 10.1093/ejcts/ezac522. View

15.

Douglas Y, Jongbloed M, DeRuiter M, Gittenberger-de Groot A . Normal and abnormal development of pulmonary veins: state of the art and correlation with clinical entities. Int J Cardiol. 2010; 147(1):13-24. DOI: 10.1016/j.ijcard.2010.07.004. View

16.

Evans W, Acherman R, Mayman G, Galindo A, Rothman A, Ciccolo M . Fontan venovenous collaterals and hepatic fibrosis. J Card Surg. 2020; 35(11):2974-2978. DOI: 10.1111/jocs.14951. View

17.

Ozawa H, Hoashi T, Ohuchi H, Kurosaki K, Ichikawa H . Long-Term Outcomes After Fenestration Closure in High-Risk Fontan Candidates. Pediatr Cardiol. 2021; 42(6):1356-1364. DOI: 10.1007/s00246-021-02619-9. View

18.

Girisch M, Sieverding L, Rauch R, Kaulitz R, Gass M, Ziemer G . Recanalisation of bilateral superior vena cava after total cavopulmonary connection. Interventional occlusion with the Amplatzer VSD Occluder. Z Kardiol. 2005; 94(7):469-73. DOI: 10.1007/s00392-005-0250-1. View

19.

Stout K, Daniels C, Aboulhosn J, Bozkurt B, Broberg C, Colman J . 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2018; 139(14):e637-e697. DOI: 10.1161/CIR.0000000000000602. View

20.

Goldstein B, Aiyagari R, Bocks M, Armstrong A . Hydrogel expandable coils for vascular occlusion in congenital cardiovascular disease: a single center experience. Congenit Heart Dis. 2011; 7(3):212-8. DOI: 10.1111/j.1747-0803.2011.00583.x. View