Case description:
19-year-old female with idiopathic PAH diagnosed at the age of 4 with surgical valved reversed Potts shunt (rPS), from the left pulmonary artery (LPA) to the descending aorta (DsAo), at the age of 16. In her case, the valved rPS was fashioned using a 10 mm Fusion graft (Getinge
®
, Getinge, Sweden), and a 12 mm valved Contegra conduit (Medtronic
®
, MN, USA) that was trimmed at a shorter length than the graft and positioned in the middle of it, leaving the distal edges of the graft intact at the smaller diameter. She presented in 2021 with decompensated right-sided heart failure requiring continuous oxygen supplementation and pressor and ionotropic support.
Investigation:
Echocardiography showed normal LV size and function, severe RV dilation and dysfunction with RVSP of 102 mmHg (SBP: 119 mmHg). New bidirectional flow across the valve within the rPS was noted.
Management:
Patient taken to the cardiac catheterization lab for valve implantation within the rPS. Her RA pressure was 11, RV 90/16, PA 90/45 (64) with a simultaneous aortic pressure of 90/56 (71), PCWP of 14, PA saturation of 60%, AsAo saturation of 84%, and DsAo saturation of 84%. Angiography showed bidirectional shunting and an incompetent deteriorated rPS valve. We modified the 18mm Ensemble
®
transcatheter valve delivery system to fit a 20 mm Melody
®
(Medtronic, MN, USA) valve, by removing the 18 mm balloon and using a15 mm balloon instead, maintaining the external sheath of the delivery system. Once the valve was crimped onto the balloon, we advanced it with the tip of the balloon slightly inflated over a stiff wire. After valve deployment, angiography demonstrated resolution of left to right shunting. Post valve deployment, her hemodynamics were stable as prior except for a drop in her PA saturation to 49%, and DsAo saturation to 80%.
Discussion:
rPS is designed to offload the RV in severe PAH. The bovine jugular valve in this case prevented left to right flow across rPS and thus pulmonary overcirculation, functioning as it was originally intended: a valved unidirectional rPS. This valve, while originally intended to be deployed to 18-22 mm in diameter in the pulmonary position, can be functional at diameters as narrow as 12-16 mm.
