Transcatheter treatment of ‘complex’ malfunction of tricuspid valve prosthesis

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A 45-year-old man with tricuspid valve Ebstein anomaly, submitted for surgical repair by plication of the atrialized part of the right ventricle (RV) and tricuspid valve replacement with a 31-mm biologic prosthesis (Carpentier-Edwards porcine, Baxter Healthcare Corp., Edwards Division; Santa Ana, California, USA) at the age of 14 years, was referred because of signs of increased systemic venous pressure and low output symptoms. He was in New York Heart Association III–IV functional class. Clinical examination showed low cardiac output signs as well as epatomegaly, ascites and mild pleural effusion. EKG showed sinus rhythm, heart rate of 110 bpm as well as signs of right atrial enlargement. At echocardiogram, a huge right atrial enlargement was imaged due to moderate-to-severe tricuspid valve prosthesis stenosis (peak gradient 22 mmHg, mean gradient 9 mmHg) and significant intraprosthetic and periprosthetic regurgitation at the diaphragmatic insertion of the prosthetic valvular cage (Fig. 1). Due to perceived high risk and complexity of surgical tricuspid valve revalvulation and periprosthetic plasty, interventional catheterization was planned. Patient's informed consent as well as surgeon's agreement to the procedure were obtained. Cardiac catheterization was performed in general anesthesia, under three-dimensional transesophageal (3D TOE) echocardiographic guide, from femoral vein approach using an 18-Fr Mullins sheath. (COOK MEDICAL EUROPE LTD, Limerick, IRELAND) At hemodynamic evaluation, the mean right atrial pressure was 25 mmHg, and the peak-to-peak pressure gradient across the tricuspid valve prosthesis was 10 mmHg, with normal RV (25/0–15 mmHg) and pulmonary artery pressures (25/12/18 mmHg). Right atrial angiography confirmed the chamber dilatation with significant persistence of the contrast medium into the hepatic veins. In addition, a large aneurismal sac, presumably remnant of surgical plication of the atrialized part of the RV, was imaged at the diaphragmatic boundary of the tricuspid valve prosthesis (Fig. 2). RV angiography showed a significant tricuspid valve regurgitation mainly occurring at periprosthesis level through the aneurismal sac. Thus, transcatheter revalvulation and closure of the perivalvular leak were planned. At echocardiographic evaluation, the length of the prosthetic valve was about 20 mm, and its internal diameter was about 6 mm, with a heavily calcified biologic valvular tissue. Therefore, a 22-mm Melody valve (Medtronic; Minneapolis, Minnesota, USA), which has a total length of 28 mm, was implanted using a 22-mm Ensemble Trans-catheter Delivery System (Medtronic). The valvular prosthesis was easily crossed with a hydrophilic guide wire (Zipwire, Boston Scientific; Marlborough, Massachusetts, USA) that was exchanged with an extrastiff 0.035″ guide wire (Amplatz guide wire, Cordis Corporation; Miami Lakes, Florida, USA) anchored into the distal left pulmonary artery. To make the Melody valve passage across the stenotic prosthesis easier, an initial valvuloplasty with a 12-mm balloon (Wanda, Boston Scientific) was performed. No prestenting of the landing zone was performed, relying on the protective effect of the prosthetic cage. However, after successful deployment of the valve, the mean right atrial pressure did not significantly decrease despite an almost complete relief of the transvalvular gradient, presumably due to the severe paravalvular regurgitation. Thus, the paravalvular leak was passed with a hydrophilic guide wire and successfully occluded with a 12-mm Amplatzer Vascular Plug type II (St. Jude Medical Inc.; St. Paul, Minnesota, USA) device (Fig. 3; Movie 1). At the end of the procedure, the mean right atrial pressure dropped to 12 mmHg. In-hospital postprocedural course was uneventful, and the patient was discharged on day 7. At 30-day assessment, the patient's clinical status was significantly improved with no more evidence of fluid retention and/or further signs of raised systemic venous pressure. 3D TOE imaged normal opening of the bioprosthetic valve, as confirmed by a mean transvalvular gradient of 2 mmHg, along with a residual mild-to-moderate tricuspid insufficiency, mainly at intraprosthetic level (Fig. 4; Movie 2).

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