Contemporary Management Strategies of Baffle Leaks in Adults with a Failing Systemic Right Ventricle Late After Atrial Switch: A Case Series and Literature Overview

Overview

Journal J Cardiovasc Dev Dis

Specialty Cardiology & Vascular Diseases

Date 2023 Mar 28

PMID 36975893

Authors

Ralph M L Neijenhuis

Madelien V Regeer

Frank van der Kley

Hubert W Vliegen

Monique R M Jongbloed

Philippine Kies

Martin J Schalij

J Wouter Jukema

Anastasia D Egorova

Affiliations

Soon will be listed here.

Abstract

Baffle leaks are a frequently encountered and often overlooked complication after the atrial switch procedure for transposition of the great arteries. Baffle leaks are present in up to 50% of non-selected patients, and while they initially may not cause clear symptoms, they can complicate the hemodynamic course and influence the prognosis in this complex patient group. A shunt from the pulmonary venous atrium (PVA) to the systemic venous atrium (SVA) can lead to pulmonary overflow and subpulmonary left ventricular (LV) volume overload, while a shunt from the SVA to the PVA can result in (exercise-associated) cyanosis and paradoxical embolism. We report three cases of baffle leaks in patients with systemic right ventricular (sRV) failure late after the atrial switch procedure. Two symptomatic patients who presented with exercise-associated cyanosis due to SVA to PVA shunting over the baffle leak underwent successful percutaneous baffle leak closure with a septal occluder device. One patient with overt sRV failure and signs of subpulmonary LV volume overload due to PVA to SVA shunting was managed conservatively, as baffle leak closure was expected to lead to an increase in sRV end-diastolic pressure and aggravation of sRV dysfunction. These three cases illustrate the considerations made, challenges faced, and necessity of a patient-tailored approach when addressing baffle leaks.

References

Bradley E, Cai A, Cheatham S, Chisolm J, Sisk T, Daniels C . Mustard baffle obstruction and leak - How successful are percutaneous interventions in adults?. Prog Pediatr Cardiol. 2018; 39(2 Pt B):157-163. PMC: 5856449. DOI: 10.1016/j.ppedcard.2015.10.018. View

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: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2018; 139(14):e698-e800. DOI: 10.1161/CIR.0000000000000603. View

Couperus L, Vliegen H, Zandstra T, Kies P, Jongbloed M, Holman E . Long-term outcome after atrial correction for transposition of the great arteries. Heart. 2018; 105(10):790-796. DOI: 10.1136/heartjnl-2018-313647. View

SENNING A . Surgical correction of transposition of the great vessels. Surgery. 1959; 45(6):966-80. View

Woudstra O, Alban F, Bijvoet G, de Bruin-Bon R, Planken R, Leiner T . Baffle Complications in Adults After Atrial Switch for Transposition of the Great Arteries. Can J Cardiol. 2021; 38(1):68-76. DOI: 10.1016/j.cjca.2021.09.034. View

Hoffman J, Kaplan S . The incidence of congenital heart disease. J Am Coll Cardiol. 2002; 39(12):1890-900. DOI: 10.1016/s0735-1097(02)01886-7. View

Woudstra O, Zandstra T, Vogel R, van Dijk A, Vliegen H, Kies P . Clinical Course Long After Atrial Switch: A Novel Risk Score for Major Clinical Events. J Am Heart Assoc. 2021; 10(5):e018565. PMC: 8174274. DOI: 10.1161/JAHA.120.018565. View

Miranda W, Jain C, Borlaug B, Connolly H, Egbe A . Exercise invasive hemodynamics in adults post-Fontan: A novel tool in understanding functional limitation and liver disease. J Heart Lung Transplant. 2022; 41(6):704-707. DOI: 10.1016/j.healun.2022.02.023. View

De Pasquale G, Bonassin Tempesta F, Santos Lopes B, Babic D, Oxenius A, Seeliger T . High prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries. Eur Heart J Cardiovasc Imaging. 2017; 18(5):531-535. DOI: 10.1093/ehjci/jew276. View

10.

Morrison M, Grant B, McCrossan B, Sands A, Owens C, Spence M . 32 year follow up of patients following atrial redirection surgery for transposition of the great arteries. Congenit Heart Dis. 2019; 14(5):846-853. DOI: 10.1111/chd.12822. View

11.

Baumgartner H, De Backer J, Babu-Narayan S, Budts W, Chessa M, Diller G . 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2020; 42(6):563-645. DOI: 10.1093/eurheartj/ehaa554. View

12.

Laredo M, Waldmann V, Chaix M, Ibrahim R, Casteigt B, Dubuc M . Lead Extraction With Baffle Stenting in Adults With Transposition of the Great Arteries. JACC Clin Electrophysiol. 2019; 5(6):671-680. DOI: 10.1016/j.jacep.2019.01.023. View

13.

Poterucha J, Taggart N, Johnson J, Cannon B, Cabalka A, Hagler D . Intravascular and hybrid intraoperative stent placement for baffle obstruction in transposition of the great arteries after atrial switch. Catheter Cardiovasc Interv. 2016; 89(2):306-314. DOI: 10.1002/ccd.26783. View

14.

Daehnert I, Hennig B, Wiener M, Rotzsch C . Interventions in leaks and obstructions of the interatrial baffle late after Mustard and Senning correction for transposition of the great arteries. Catheter Cardiovasc Interv. 2005; 66(3):400-7. DOI: 10.1002/ccd.20504. View

15.

Khairy P, Landzberg M, Lambert J, ODonnell C . Long-term outcomes after the atrial switch for surgical correction of transposition: a meta-analysis comparing the Mustard and Senning procedures. Cardiol Young. 2005; 14(3):284-92. DOI: 10.1017/S1047951104003063. View

16.

Mustard W . SUCCESSFUL TWO-STAGE CORRECTION OF TRANSPOSITION OF THE GREAT VESSELS. Surgery. 1964; 55:469-72. View