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Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management

Raymond N Haddad1, Ahmed Adel Hassan2, Mahmoud Al Soufi2

  • 1Service de Cardiologie Congénitale et Pédiatrique, Centre de Référence des Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149, rue de Sèvres, Paris, 75015, France. raymondhaddad@live.com.

BMC Cardiovascular Disorders
|June 4, 2025
PubMed

Insights

This case study details a complex congenital heart defect repair in an 11-year-old boy. Interventions for plastic bronchitis and pulmonary artery stenosis led to improved hemodynamics and recovery.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Interventional Cardiology

Background:

  • Complex congenital heart disease (CHD) presents unique management challenges.
  • Surgical palliation for single-ventricle physiology requires careful long-term monitoring.
  • Complications like plastic bronchitis can arise post-Fontan procedure.

Purpose of the Study:

  • To describe the management of a rare case of complex CHD with post-Fontan complications.
  • To highlight the interventional strategies for treating plastic bronchitis and pulmonary artery stenosis.
  • To evaluate the efficacy of transjugular procedures for Fontan fenestration optimization.

Main Methods:

  • Case report of an 11-year-old boy with multiple complex cardiac anomalies.
  • Review of surgical history including Kawashima shunt and fenestrated extracardiac Fontan.
  • Interventional cardiac catheterization for management of plastic bronchitis and pulmonary artery stenosis, including stenting and atrial flow regulator placement.
  • Transjugular clot aspiration and stenting for fenestration enhancement.

Main Results:

  • Successful stenting of proximal left pulmonary artery stenosis.
  • Initial transjugular placement of an atrial flow regulator complicated by thrombosis.
  • Catheter-based clot aspiration, stenting, and ballooning significantly increased fenestration diameter.
  • Improved Fontan pressures, pulmonary artery flow, and clinical symptoms post-intervention.

Conclusions:

  • Complex CHD management requires tailored interventional approaches.
  • Transjugular interventions can effectively address Fontan-associated complications like stenosis and fenestration issues.
  • Multidisciplinary care is crucial for favorable outcomes in patients with single-ventricle physiology and post-operative complications.

Keywords:
Atrial flow regulatorCongenital heart diseaseMechanical thrombectomy, transcatheter interventionThrombosis

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