Weber, Maddison M., DO¹; Aboeata, Ahmed, MD²; Brown Ton, Corinna, MS³; Lange, Kathleen A., MD⁴

¹Creighton University, Department of Internal Medicine, CHI Bergan Mercy Hospital, Email: maddieweber@creighton.edu

²Creighton University, Department of Cardiology, CHI Bergan Mercy Hospital, Email: ahmedaboeata@creighton.edu

³Creighton University School of Medicine, Email: CorinnaBrownTon@creighton.edu

⁴Chariton Valley Medical Clinic, Medical Clinic in Centerville, Iowa.

Corresponding author: Maddison Weber, Department of Internal Medicine, CHI Bergan Mercy Hospital, 7 Shore Acres Road, Council Bluffs, IA 51501, email: maddieweber@creighton.edu.

Citation: Maddison Weber M, Ahmed Aboeata, Corinna Brown Ton, Kathleen Lange A, (2025) Management Challenges in a Young Adult with Complex Congenital Heart Disease, Single Ventricle Physiology, and Persistent Supraventricular Tachycardia: A Case Report. World J Case Rep Clin Imag. 2026 March; 5(1)1-7.

Copyrights: © Weber, Maddison M, et al., 2026, This article is licensed under the Creative Commons Attribution-Non Commercial-4.0-International-License-(CCBY-NC) (https://worldjournalofcasereports.org/blogpage/copyright-policy). Usage and distribution for commercial purposes require written permission.

Abstract:

Introduction: Congenital heart disease (CHD) encompasses a wide spectrum of structural cardiac abnormalities present at birth that require lifelong multidisciplinary management. Adults with complex CHD, particularly those with Fontan physiology, remain at risk for long-term complications including arrhythmias, heart failure, and reduced functional capacity. Case Presentation: We report the case of a 25-year-old female with complex CHD including single ventricle physiology, situs inversus, and prior extracardiac Fontan palliation who presented with recurrent supraventricular tachycardia (SVT). She had a history of chronic exertional dyspnea and multiple episodes of symptomatic tachycardia requiring emergency department evaluation. Electrocardiography during tachycardia demonstrated a regular narrow-complex rhythm consistent with SVT. Diagnostic imaging excluded acute cardiopulmonary pathology. Due to recurrent arrhythmia, the patient was admitted for monitored antiarrhythmic therapy and underwent inpatient sotalol loading with continuous telemetry monitoring. She tolerated therapy well and was discharged on sotalol with significant improvement in palpitations and no further sustained tachycardia. Clinical Discussion: Arrhythmias represent a common late complication of Fontan physiology due to atrial dilation, surgical scarring, and abnormal hemodynamics. Management may be challenging because catheter ablation can be technically difficult in patients with complex postoperative anatomy. Conclusion: This case highlights the complexity of arrhythmia management in adults with Fontan circulation and underscores the importance of individualized, multidisciplinary care in this growing patient population.

Keywords: Congenital heart disease, Fontan circulation, Supraventricular tachycardia, Adult congenital heart disease, Single ventricle physiology, Sotalol therapy.

Highlights:

1. Adults with Fontan physiology are at increased risk for supraventricular arrhythmias due to atrial dilation and surgical scarring.

2. Supraventricular tachycardia in Fontan patients can significantly impair hemodynamics because pulmonary blood flow is passive.3. Electrocardiographic evaluation during tachycardia is essential to narrow the differential diagnosis of SVT in complex congenital heart disease.

4. Catheter ablation may be technically challenging in patients with extracardiac Fontan anatomy due to limited vascular access and altered atrial geometry.

5. Pharmacologic rhythm control with sotalol can be an effective management strategy in selected patients with Fontan circulation.