Successful Transcatheter Pulmonary Valve Replacement Via the Azygous Vein in a Patient With Interrupted Inferior Vena...

In 2021, the Food and Drug Administration approved the Harmony Transcatheter Pulmonary Valve System (Medtronic) for the treatment of pulmonary valve regurgitation in pediatric and adult populations with a native or surgically repaired right ventricular outflow tract, thus extending transcatheter pulmonary valve replacement to accommodate larger native right ventricular outflow tract anatomies.

We present the case of a 15-year-old female with a history of heterotaxy, transannular patch repair of double outlet right ventricle, bilateral superior vena cava, interrupted inferior vena cava (IVC), who presented with progressive right ventricular dilation and reduced endurance by cardiopulmonary exercise testing.

The most common way of delivering the Harmony valve is through the femoral venous approach. When the patient's anatomy precludes femoral venous access, such as vascular occlusions or an interrupted IVC, an alternative option is essential.

Deployment of the Harmony valve without a DrySeal sheath through the femoral vein to the azygous vein pathway is feasible in patients with interrupted IVC and lack of internal jugular access.