Junctional Rhythm and Atrial Ectopy Presenting as Chronic Abdominal Pain in Fontan Circulation.

Authors

Laurence Campens, Michael Rahbek Schmidt, Christian Jøns

Published in

JACC. Case reports. Volume 31. Issue 45. Pages 110837. Nov 11, 2026.

Abstract

Rhythm disturbances may contribute to clinically significant hemodynamic compromise in patients with Fontan circulation, even without overt Fontan failure.
A 32-year-old woman with double-inlet left ventricle and total cavopulmonary connection presented with years of debilitating abdominal pain resulting in marked functional limitation. Extensive abdominal, hepatic, lymphatic, and cardiac investigations failed to identify a cause. Holter monitoring demonstrated predominant junctional rhythm and frequent atrial ectopy, whereas cardiopulmonary exercise testing revealed heart rate-dependent restoration of sinus rhythm with suppression of atrial ectopy. Invasive assessment showed that temporary atrial pacing suppressed ectopy and improved Fontan hemodynamics. Following AAIR pacemaker implantation, abdominal pain resolved completely and functional capacity improved.
This case illustrates that rhythm-related hemodynamic compromise may present with atypical extracardiac symptoms despite reassuring conventional investigations.
Chronic abdominal pain in patients with Fontan circulation may reflect arrhythmia-related hemodynamic compromise despite apparently stable Fontan physiology.

PMID:
43018215
Bibliographic data and abstract were imported from PubMed on 12 Nov 2026.

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