Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management
Abstract We report the case of an 11-year-old boy with situs inversus, interrupted inferior vena cava with azygos continuation, persistent left superior vena cava with absent bridging vein, congenitally corrected transposition of the great arteries, pulmonary atresia, and ventricular septal defect....
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| Language: | English |
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BMC
2025-06-01
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| Series: | BMC Cardiovascular Disorders |
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| Online Access: | https://doi.org/10.1186/s12872-025-04876-5 |
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| author | Raymond N. Haddad Ahmed Adel Hassan Mahmoud Al Soufi Mohamed Kasem |
| author_facet | Raymond N. Haddad Ahmed Adel Hassan Mahmoud Al Soufi Mohamed Kasem |
| author_sort | Raymond N. Haddad |
| collection | DOAJ |
| description | Abstract We report the case of an 11-year-old boy with situs inversus, interrupted inferior vena cava with azygos continuation, persistent left superior vena cava with absent bridging vein, congenitally corrected transposition of the great arteries, pulmonary atresia, and ventricular septal defect. He underwent a Kawashima shunt at age 1 and fenestrated extracardiac conduit Fontan completion at age 9, delayed due to loss to follow-up while living abroad. Fifteen months post-op, the patient was diagnosed with plastic bronchitis. Cardiac catheterization revealed elevated Fontan pressures and proximal left pulmonary artery stenosis, which was stented (AndraTec 38 mm Optimus-XL). Although fenestration creation was considered, the procedure was initially delayed allowing for a trial of medical management. Four months later, a 6 mm Occlutech atrial flow regulator (AFR) was placed transjugularly, but thrombosis occurred 48 h later. Transjugular catheter-based clot aspiration (Penumbra Lightning® 7) and stenting (10/20 mm Cook Formula stent) followed by sequential high-pressure ballooning increased the fenestration diameter, improving symptoms and hemodynamics. He had a favorable recovery with significant improvements and remained stable at 13 weeks of follow-up. |
| format | Article |
| id | doaj-art-bcc3322669c0487c90454cec96bb1240 |
| institution | DOAJ |
| issn | 1471-2261 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cardiovascular Disorders |
| spelling | doaj-art-bcc3322669c0487c90454cec96bb12402025-08-20T03:10:28ZengBMCBMC Cardiovascular Disorders1471-22612025-06-012511610.1186/s12872-025-04876-5Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and managementRaymond N. Haddad0Ahmed Adel Hassan1Mahmoud Al Soufi2Mohamed Kasem3Service de Cardiologie Congénitale et Pédiatrique, Centre de Référence des Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de ParisDepartment of Pediatric Cardiology, Heart Centre of Excellence, Al Jalila Children’s Speciality HospitalDepartment of Pediatric Cardiology, Heart Centre of Excellence, Al Jalila Children’s Speciality HospitalDepartment of Pediatric Cardiology, Heart Centre of Excellence, Al Jalila Children’s Speciality HospitalAbstract We report the case of an 11-year-old boy with situs inversus, interrupted inferior vena cava with azygos continuation, persistent left superior vena cava with absent bridging vein, congenitally corrected transposition of the great arteries, pulmonary atresia, and ventricular septal defect. He underwent a Kawashima shunt at age 1 and fenestrated extracardiac conduit Fontan completion at age 9, delayed due to loss to follow-up while living abroad. Fifteen months post-op, the patient was diagnosed with plastic bronchitis. Cardiac catheterization revealed elevated Fontan pressures and proximal left pulmonary artery stenosis, which was stented (AndraTec 38 mm Optimus-XL). Although fenestration creation was considered, the procedure was initially delayed allowing for a trial of medical management. Four months later, a 6 mm Occlutech atrial flow regulator (AFR) was placed transjugularly, but thrombosis occurred 48 h later. Transjugular catheter-based clot aspiration (Penumbra Lightning® 7) and stenting (10/20 mm Cook Formula stent) followed by sequential high-pressure ballooning increased the fenestration diameter, improving symptoms and hemodynamics. He had a favorable recovery with significant improvements and remained stable at 13 weeks of follow-up.https://doi.org/10.1186/s12872-025-04876-5Atrial flow regulatorCongenital heart diseaseMechanical thrombectomy, transcatheter interventionThrombosis |
| spellingShingle | Raymond N. Haddad Ahmed Adel Hassan Mahmoud Al Soufi Mohamed Kasem Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management BMC Cardiovascular Disorders Atrial flow regulator Congenital heart disease Mechanical thrombectomy, transcatheter intervention Thrombosis |
| title | Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management |
| title_full | Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management |
| title_fullStr | Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management |
| title_full_unstemmed | Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management |
| title_short | Acute thrombosis of an atrial flow regulator in a failing Fontan circulation: recanalization and management |
| title_sort | acute thrombosis of an atrial flow regulator in a failing fontan circulation recanalization and management |
| topic | Atrial flow regulator Congenital heart disease Mechanical thrombectomy, transcatheter intervention Thrombosis |
| url | https://doi.org/10.1186/s12872-025-04876-5 |
| work_keys_str_mv | AT raymondnhaddad acutethrombosisofanatrialflowregulatorinafailingfontancirculationrecanalizationandmanagement AT ahmedadelhassan acutethrombosisofanatrialflowregulatorinafailingfontancirculationrecanalizationandmanagement AT mahmoudalsoufi acutethrombosisofanatrialflowregulatorinafailingfontancirculationrecanalizationandmanagement AT mohamedkasem acutethrombosisofanatrialflowregulatorinafailingfontancirculationrecanalizationandmanagement |