Patent Foramen Ovale Closure in a Patient With Ventricular Storm and Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) has late clinical manifestations of right heart dysfunction, chamber dilation, and ventricular dysrhythmias. Elevated right-sided pressure and cardiac remodeling can result in late presentation of right to left interatrial shunting.

A 70-year-old man presented with ventricular tachycardia (VT) storm and significant hypoxemia. His dysrhythmia settled with a combination of antiarrhythmic agents and a stellate ganglion block. Given the persistence of hypoxemia, which is a potential contributing factor to VT, further echocardiographic investigation diagnosed a large patent foramen ovale (PFO). After optimization of medical therapy, right heart catheterization demonstrated normal pulmonary artery pressures, and successful PFO closure with an Amplatzer device was performed.

Development of right-to-left shunting with progression of ARVC is a rare late-stage complication, and its association with ventricular dysthymia has not been previously reported in the literature. This is the first case to our knowledge of PFO closure in ARVC for VT storm.

In advanced ARVC, right-sided chamber remodeling can result in stretching of the interatrial septum, leading to right-to-left shunting, hypoxemia, and ventricular dysrhythmia. Percutaneous PFO closure can be helpful in the management of RV failure, hypoxemia, and dysrhythmia in select patients with ARVC.