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

## TL;DR

A 10-year-old patient with a rare congenital portosystemic shunt leading to pulmonary hypertension was successfully treated using a muscular VSD device.

## Contribution

This case demonstrates the successful use of a muscular ventricular septal defect device for closure of a rare congenital portosystemic shunt.

## Key findings

- A muscular VSD device was used to close a CPSS in a pediatric patient.
- Pulmonary hypertension was reversed following the procedure.
- CPSS is a reversible cause of pulmonary hypertension that can be treated via catheterization.

## Abstract

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

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.

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.