Off-label use of muscular VSD device for closure of a rare congenital portosystemic shunt

Hala Mounir Agha; Shady Mashoor; Mohamed Ghobashy; Antoine AbdelMassih; Hanya Gaber; Hanaa El-Karaksy

doi:10.1186/s43044-024-00467-5

Off-label use of muscular VSD device for closure of a rare congenital portosystemic shunt

Egypt Heart J. 2024 Mar 23;76(1):34. doi: 10.1186/s43044-024-00467-5.

Authors

Hala Mounir Agha¹, Shady Mashoor², Mohamed Ghobashy², Antoine AbdelMassih³, Hanya Gaber⁴, Hanaa El-Karaksy⁵

Affiliations

¹ Pediatric Cardiology Unit, Pediatrics' Department, Faculty of Medicine, Cairo University, P.O BOX: 12411, Cairo, Egypt.
² Interventional Radiology Unit, Radiology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
³ Pediatric Cardiology Unit, Pediatrics' Department, Faculty of Medicine, Cairo University, P.O BOX: 12411, Cairo, Egypt. antoine.abdelmassih@kasralainy.edu.eg.
⁴ Faculty of Medicine, Cairo University, Cairo, Egypt.
⁵ Pediatric Hepatology Unit, Faculty of Medicine, Cairo University, Cairo, Egypt.

Abstract

Background: Congenital portosystemic shunt (CPSS) is a vascular malformation in which portal blood drains toward the systemic circulation, leading to pulmonary hypertension.

Case presentation: A 10-year-old patient was brought for evaluation because of dyspnea on exertion. Echocardiography revealed a pulmonary hypertension of 75 mmHg, and multi-slice CT angiography revealed the presence of a CPSS. Closure was finally implemented using a muscular ventricular septal defect device. Follow-up of the patient revealed a gradual decline in pulmonary hypertension.

Conclusions: CPSS is an overlooked cause of reversible pulmonary hypertension (PH). Closure of such lesions and reversal pulmonary hypertension are possible via catheterization. The preferred device type depends largely on the intervening team. Plugs are the first choice for interventional radiologists, while ventricular and atrial septal occluder devices and duct occluders are preferred by pediatric cardiologists.

Keywords: CPSS; Unexplained pulmonary hypertension; mVSD device.