„ Clinical vignette

Atrioventricular sequential pacemaker implantation in an adult patient with a Fontan circulation

Krzysztof Boczar1Andrzej Ząbek1Lidia Tomkiewicz-Pająk23Jacek Gajek4Agnieszka Sławuta5Maciej Dębski6Barbara Małecka17
1Department of Electrocardiology, John Paul II Hospital, Kraków, Poland
2Department of Cardiac and Vascular Diseases, Institute of Cardiology, Jagiellonian University Medical College, Kraków, Poland
3John Paul II Hospital, Kraków, Poland
4Department of Emergency Medical Service, Wroclaw Medical University, Wrocław, Poland
5Department of Internal and Occupational Disease, Hypertension and Clinical Oncology, Wroclaw Medical University, Wrocław, Poland
6Norfolk and Norwich University Hospital, University of East Anglia, Norwich, United Kingdom
7Institute of Cardiology, Jagiellonian University Medical College, Kraków, Poland

Correspondence to:

Krzysztof Boczar, MD, PhD,

Department of Electrocardiology,

John Paul II Hospital,

Prądnicka 80, 31–202 Kraków, Poland,

phone: +48 12 614 22 77,

e-mail: krzysiek.boczar@gmail.com

Copyright by the Author(s), 2022

DOI: 10.33963/KP.a2022.0063

Received: January 24, 2022

Accepted: March 8, 2022

Early publication date: March 8, 2022

A 29-year-old female was referred to our hospital with recurrent syncope and heart failure symptoms. She had been born with tricuspid atresia, right ventricular hypoplasia, an atrial septal defect, ventricular septal defect, pulmonary valve stenosis, right retroesophageal subclavian artery, and persistent left superior vena cava. The patient had undergone Blalock-Taussig (BT) shunt in infancy, hemi-Fontan operation at five years of age, and fenestrated Fontan completion one year later. Electrocardiographic (ECG) monitoring showed episodes of sino-atrial dissociation and chronotropic incompetence (Figure 1A). The patient was listed for dual-chamber pacemaker implantation. Pre-procedural planning included a heart catheterization with a detailed hemodynamic and angiographic evaluation (Figure 1B, Supplementary material, Figure S1) and ECG-gated cardiac contrast computed tomography (CT). For a precise evaluation of intracardiac anatomy, we created a computed tomography reconstruction and printed a three-dimensional model of the Fontan circulation, heart chambers, and coronary sinus with its tributaries (Supplementary material, Figure S2). Pacemaker implantation was carried out under light analgosedation in a hybrid operating room. Venous access was gained by puncture of the left subclavian vein. Fenestration in the Fontan baffle was cannulated with the Medtronic Attain Command™ delivery system (Medtronic, Minneapolis, MN, US), and the leads were advanced into the atrium [1]. A coronary sinus lead Biotronik Sentus OTW BP was positioned in a posterior cardiac vein using a sub-selection catheter Medtronic Attain Select™ II. Finally, the lumenless Medtronic SelectSecure™ 3830 lead was placed in the right atrium via Medtronic C315HIS Delivery Catheter.

Figure 1. A. Electrocardiographic (ECG) monitoring with episodes of sino-atrial dissociation and chronotropic incompetence. B. Fluoroscopy of a heart catheterization with detailed hemodynamic and angiographic evaluation of the Fontan circulation. The arrow indicates fenestration between the Fontan circulation and the atrium. C. The chest radiograph after the procedure, showing the correct position of both pacing leads. D. ECG registration showing dual-chamber pacing

The procedure and postoperative period were uneventful. The chest radiograph showed the correct position of both leads (Figure 1C). Pacing parameters were excellent, and appropriate pacemaker function was confirmed on ECG monitoring (Figure 1D). Echocardiography showed no intracardiac thrombi or pericardial effusion. Warfarin was commenced for thromboprophylaxis [2]. This case shows that transvenous pacemaker implantation can successfully and safely accomplish restoring atrioventricular synchrony and chronotropic competence.

Supplementary material

Supplementary material is available at https://journals.viamedica.pl/kardiologia_polska.

Article information

Acknowledgments: The authors would like to thank Mateusz Hołda MD, PhD, for preparing a 3D heart model.

Conflict of interest: None declared.

Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at kardiologiapolska@ptkardio.pl.


  1. Okólska M, Skubera M, Matusik P, et al. Chronotropic incompetence causes multiple organ complications in adults after the Fontan procedure. Kardiol Pol. 2021; 79(4): 410417, doi: 10.33963/KP.15853, indexed in Pubmed: 33687864.
  2. Umamaheshwar KL, Singh AS, Sivakumar K. Endocardial transvenous pacing in patients with surgically palliated univentricular hearts: A review on different techniques, problems and management. Indian Pacing Electrophysiol J. 2019; 19(1): 1522, doi: 10.1016/j.ipej.2018.11.013, indexed in Pubmed: 30508590.


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