A 54-year-old patient with 2 previous tricuspid valve (TV) replacements performed at a different institution presented with worsening symptoms of chronic congestive heart failure, including lower extremity edema. Preoperative echocardiography demonstrated severe bioprosthetic TV stenosis with a mean gradient of 10 mm Hg, trace tricuspid regurgitation, as well as P2 segment flail of the mitral valve with severe eccentric mitral regurgitation. Because of the perceived difficulty with a third tricuspid procedure, the surgical plan included initial percutaneous deployment of a 29-mm Sapien (Edwards Lifesciences, Irvine, CA) transcatheter heart valve-in-valve in the tricuspid position followed by a minimally invasive mitral valve repair through a right thoracotomy in a hybrid operating room. Intraoperative transesophageal echocardiography of the bioprosthetic TV was performed, demonstrating severely restricted leaflet motion on 2-dimensional (2D) images (Supplemental Digital Content 1, Video 1, http://links.lww.com/AA/B251), mild prosthetic tricuspid regurgitation with color flow Doppler, and lack of visible leaflets on 3-dimensional (3D) images (Supplemental Digital Content 2, Video 2, http://links.lww.com/AA/B252), with apparent heavy calcification of the prosthetic valve on 2D and 3D images (Figs. 1 and 2). Deployment of the transcatheter Sapien valve was complicated by the inability to place the guidewire in the central axis of the prosthetic valve. Failure to completely expand the Sapien valve resulted in severe paravalvular leak outside the Sapien valve but inside the previously placed prosthetic valve (Supplemental Digital Content 3, Video 3, http://links.lww.com/AA/B253). The maximal mean pressure gradient through the Sapien valve was 8 mm Hg at a heart rate of 74 bpm. Placement of an Amplatzer plug (St. Jude Medical, Saint Paul, MN) did not improve the paravalvular leak. Because of the change in the surgical plan, the surgery was cancelled and the patient was brought back to the cardiac operating room on another day. For the second operation, a right anterior thoracotomy was performed. Upon opening the right atrium, it was discovered that the valve holder of the bioprosthetic TV had been left in place from the previous surgery 3 years earlier. The presence of the valve holder explained the presenting functional TV stenosis, the impression of severe valvular thickening and calcification, as well as the subsequent failure to fully expand the Sapien valve. The latter had been deployed in the space between the holder and the annulus of the bioprosthesis resulting in severe paravalvular leak (relative to the Sapien valve). The valve holder, Sapien valve, and Amplatzer plug (Fig. 3) were removed and the TV replaced with a new bioprosthetic valve in the tricuspid position. The patient provided written consent for this publication.
The differential diagnosis for bioprosthetic valve stenosis includes pannus formation, structural valve deterioration, and, infrequently, thrombus formation.1,2 A retained valve holder causing bioprosthetic valve stenosis is a rare and likely underreported event. We failed to make the correct diagnosis of a retained valve holder because the diagnosis is unusual and no reports describing the echocardiographic characteristics of this condition have been published. We suggest adding this to the differential diagnosis for bioprosthetic valve stenosis and describe some of the echocardiographic and clinical findings (Table 1).
With 2D imaging, the echocardiographic appearance of the valve was similar to that of structural valve deterioration with severely calcified leaflets and reduced mobility. In hindsight, the appearance of “calcification” extending beyond the tricuspid annulus was an indicator of the unusual diagnosis. Structural bioprosthetic valve deterioration results in thickened leaflets with reduced mobility, but extension of calcium is uncommon beyond the bioprosthetic annulus.2 With color flow Doppler, the degree of tricuspid regurgitation was less than might be expected. This may be explained by the fact that, although the valve holder reduces the effective orifice area of the valve (and increases the mean pressure gradient), the noncalcified valve leaflets can still close (albeit closing against the valve holder). Echocardiography can detect prosthetic TV stenosis with spectral Doppler-derived parameters such as an E-wave velocity >1.7 m/s, mean gradient >6 mm Hg, or pressure half-time >230 milliseconds suggesting valve stenosis.1 Such findings should ideally have been made before leaving the operating room or before discharge from hospital on the previous occasion because American Society of Anesthesiologists/Society of Cardiovascular Anesthesiologists guidelines recommend the use of transesophageal echocardiography in all adult open heart surgeries.3
On 3D imaging, the retained valve holder prevented the display of the leaflets. However, 3D imaging did show the valve holder which appears triangular in shape (“Mercedes sign with central orifice”) and extends above the annular sewing ring. The central orifice in the en face 3D view is also an unusual finding, representing the attachment site for the valve holder handle. The retained valve holder prevented the transcatheter guidewire and balloon from being positioned centrally in the bioprosthetic valve. The severe paravalvular leak, after Sapien valve deployment, resulted from failure to fully expand the Sapien valve within the bioprosthetic annulus because of constraint by the valve holder.
Finally, a clue to the unusual diagnosis is the fact that the patient presented within 3 years of valve implantation. Although younger age at implantation is associated with a higher occurrence of structural valve deterioration requiring reoperation, such early failure of a stented bioprosthesis is infrequent, because freedom from structural valve failure is 70% to 90% at 10 years and 50% to 80% at 15 years.2,4 Specifically, the linearized incidence of structural bioprosthetic TV deterioration is 0.50% per patient-year, suggesting that valve failure from structural valve deterioration within 3 years is unlikely even in this young patient.5
A retained valve holder should be a “never event.” Adding the valve holder to the surgical count may help prevent this event from occurring. Next, the valve holder is radiolucent and is not visible on fluoroscopy. Making the valve holder radio-opaque will alert the physician, although this will most likely only occur in the postoperative period. In conclusion, the specific 3D echocardiographic characteristics, in contrast to the nonspecific 2D, color, and spectral Doppler characteristics can help to differentiate a retained valve holder from structural valve deterioration.
Clinician’s Key Teaching Points
By Kent H. Rehfeldt, MD, Kimberly Howard-Quijano, MD, and Martin J. London MD
- The differential diagnosis for bioprosthetic tricuspid valve stenosis includes pannus ingrowth, degenerative changes with calcification, or, less frequently, thrombotic occlusion. Although bioprosthetic tricuspid prosthesis degeneration occurs more quickly in younger patients, approximately 70% to 90% of patients remain free of structural failure 10 years after implantation.
- Bioprosthetic tricuspid valve stenosis is diagnosed on the basis of 2D or 3D echocardiographic findings of thickened or calcified leaflets with reduced mobility. Spectral Doppler findings include an E-wave velocity >1.7 m/s, a mean diastolic gradient >6 mm Hg, and a pressure half-time >230 milliseconds.
- In this case, a patient with a bioprosthetic tricuspid valve implanted 3 years prior presented with worsening heart failure symptoms including lower extremity edema. Echocardiography revealed prosthetic tricuspid stenosis, with a mean diastolic gradient of 10 mm Hg. Placement of a percutaneous, valve-in-valve prosthesis was complicated by the inability to position a guidewire in the center of the previously placed prosthesis, failure to completely expand the transcatheter valve, and severe paravalvular regurgitation after deployment. A subsequent open heart procedure revealed a retained tricuspid prosthesis valve holder from the previous operation.
- Although the diagnosis of a retained valve holder was not recognized by echocardiography, several unusual findings noted during the transesophageal echocardiography examination included the inability to visualize bioprosthetic leaflets, apparent calcification that extended beyond the tricuspid annulus, and a central orifice in the en face 3D view that represented the site of attachment of the valve holder handle. In addition, stenotic degeneration of a bioprosthesis 3 years after implantation is unusual, even in a younger patient.
Name: Jeremy M. Bennett, MD.
Contribution: This author drafted the manuscript.
Attestation: Jeremy M. Bennett approved the final manuscript.
Name: Robert Deegan, MBChB, BAO, PhD, FFARCSI.
Contribution: This author made critical revisions to the manuscript.
Attestation: Robert Deegan approved the final manuscript.
Name: Simon Maltais, MD, PhD.
Contribution: This author made critical revisions to the manuscript.
Attestation: Simon Maltais approved the final manuscript.
Name: Mias Pretorius, MBChB, MSCI.
Contribution: This author obtained the ultrasound images and made critical revisions to the manuscript.
Attestation: Mias Pretorius approved the final manuscript and is the archival author.
This manuscript was handled by: Martin J. London, MD.
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