Hala Mounir Agha, Shady Mashoor, Mohamed Ghobashy, Antoine AbdelMassih, Hanya Gaber, Hanaa El‑Karaksy
Cairo University.
Egypt
Egyptian Heart Journal
Egypt Heart J 2024; 76:
DOI: 10.1186/s43044-024-00467-5
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes