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Double Valve Repair for Marfan Aortic Root Aneurysm and Bileaflet (Barlow’s) Mitral Valve

Wednesday, December 5, 2018

By

George Stavridis

Stavridis GT, Kantidakis G, Kaminiotis VV, Vassili M-I, Rankin JS. Double Valve Repair for Marfan Aortic Root Aneurysm and Bileaflet (Barlow’s) Mitral Valve. December 2018. doi:10.25373/ctsnet.7390304.

Objectives

Repair of simultaneous complex aortic and mitral valve defects in patients with Marfan syndrome can be difficult. This video illustrates a double valve repair using two remodeling annuloplasty rings, as a technical simplification for simultaneous reconstruction.

Video Summary

Risk-adjusted outcomes of multiple valve procedures may be better with repair than with prosthetic valve replacement (1, 2). The patient in this video was a 38-year-old woman with Marfan syndrome, along with aortic root aneurysm and severe aortic and mitral valve insufficiency. Echocardiography showed bileaflet mitral valve (MV) prolapse with multiple jets, consistent with a Barlow’s valve. A trileaflet aortic valve (AV) had severe insufficiency with a posterior jet. The aortic annulus and sinus measured 23 mm and 50 mm, respectively, and the sinotubular junction was 33 mm. On inspection, the MV appeared myxomatous with primarily prolapsing posterior segments. Two pairs of 2-0 polytetrafluoroethylene chords were secured in the anterior and posterior papillary muscles and then stuffed into the ventricle. After placement of a large mitral annuloplasty ring, the chords were retrieved from the left ventricle, and they were attached to the left and right prolapsing posterior leaflet segments using a weaving technique (3, 4). Each chord was tied to the proper length during final valve testing (an “adjustable” chordal replacement). A small posterior leaflet cleft also was closed, and a water test confirmed a competent mitral valve.

The trileaflet AV was inspected and the leaflets appeared normal.  A 23 Hagar dilator confirmed the echocardiographic annular measurement. Using ball leaflet sizers, a 21 mm ring was chosen for the aortic annuloplasty. The ring was implanted by first burying the three commissural posts into the subcommissural triangles using three mattress sutures. The ring was lowered below the valve, and two annular looping sutures were passed around each sinus aspect. After tying all nine sutures, good leaflet coaptation was achieved. Because the left coronary sinus was normal, only the right and noncoronary sinuses were replaced with two tongues of a remodeling graft, and the right coronary button was reimplanted. The distal aortic anastomosis was completed, and the aorta was unclamped. The transesophageal echocardiogram showed a competent MV and mild AV insufficiency, and the patient recovered uneventfully.

Conclusion

Simultaneous AV and MV annuloplasty with remodeling geometric rings simplifies simultaneous repair of Marfan aortic and mitral insufficiency. This approach could facilitate aortic valve repair in this setting.

References

  1. Rankin JS, Thourani VH, Suri RM, et al. Associations between valve repair and reduced operative mortality in 21,056 mitral/tricuspid double valve procedures. Eur J Cardiothorac Surg. 2013;44(3):472-477.
  2. Vohra HA, Whistance RN, Hechadi J, et al. Long-term outcomes of concomitant aortic and mitral valve repair. J Thorac Cardiovasc Surg. 2014;148(2):454-460.
  3. Rankin JS, Orozco RE, Rodgers TL, Alfery DD, Glower DD. “Adjustable” artificial chordal replacement for repair of mitral valve prolapse. Ann Thorac Surg. 2006;81(4):1526-1528.
  4. Rankin JS. Mitral valve repair for Barlow’s syndrome using adjustable artificial chordal replacement. CTSNet, Inc. http://www.ctsnet.org/article/mitral-valve-repair-barlow%E2%80%99s-syndrome-using-adjustable-artificial-chordal-replacement. Published June 27, 2010. Accessed July 23, 2018.

Dr Rankin is a consultant for BioStable Science and Engineering, Inc, in Austin, Texas. BioStable Science and Engineering provided support for this video.

Comments

Submitted by George Tolis, Jr. on
This is a very nicely done video. I have a couple of questions : 1) Aren't you concerned that you are leaving behind the entire left sinus of Valsalva in a patient with clear stigmata of Marfan's disease (as you stated during the introduction)? Wouldn't a David Procedure have been preferable since it would guarantee that no diseased sinus tissue is left behind? 2) You have some residual AI on your final echo which is eccentric (seems to be heading toward the septum, although it is hard to tell from the one view posted). At 6 :14 - 6:18, it seems that the right cusp is bigger than the left and the non coronary cusps, and this asymmetry may indeed be the source of the "bunching" of the right leaflet by the perfectly symmetric Rankin ring resulting in the eccentric AI with the jet toward the septum. Even though the jet may quantify as "mild" based on echo criteria, its eccentric nature is concerning when it comes to long term freedom from progression of residual AI. Wouldn't a David Procedure be preferable since it would enable you to re-implant the three commissures with more flexibility to account for the valve's built in asymmetry and not limited by the rigid 120 angle of the ring? 3) Smart move not to use felt. You or someone else will be very thankful in a few years. One way or another, this is a nice video.
Submitted by J. Rankin on
Response It is with great respect that we respond to the comments of Dr. George Tolis Jr. about our video on Marfan double valve repair. The main focus of this video was aortic and mitral valve repair, but certainly, selective sinus replacement of only the abnormal sinuses in a Marfan patient is an important topic. In patients with the more severe aortopathies, such as Ehlers-Danlos or Loeys-Dietz syndromes, our practice is to replace all 3 sinuses routinely. In Marfan or sporadic root aneurysms, however, Prof. Urbanski’s experience (J Thorac Cardiovasc Surg. 2018;155:43-51) suggests that normal sized sinuses remain stable long-term if selectively retained. More data are needed, but the practice of selective sinus replacement is becoming more common, especially in entities such as bicuspid root aneurysms with normal right-left fused sinuses, and a markedly enlarged non-coronary sinus. But, we are not fixed in this approach, and would welcome further discussion and relevant outcome data. This patient was the first case of aortic ring annuloplasty in Greece, operated almost 2 years ago. The pre-repair TEE showed a posteriorly directed AI jet, indicative of right coronary leaflet prolapse. All 3 leaflets sized to a #21 ring (so no “bunching” or leaflet asymmetry), and after annuloplasty, good central coaptation was obtained. However, if one looks carefully, mild right coronary leaflet prolapse persisted, and probably is the source for the Grade 1 residual AI. The mild prolapse produced a posteriorly directed AI jet toward the anterior mitral leaflet, as is characteristic. In retrospect, the final inspection of leaflet heights should have been more careful, and a small plication stitch should have been placed in the right coronary leaflet. Greater attention to such issues, along with liberal ultrasonic Nodular release for scarred Noduli, are now producing routine Grade 0 AI in all pathologies. However, this degree of Grade 1 AI has been stable in our series, and has been associated with uniform ventricular recovery and no symptoms. Finally, the goal with aortic ring annuloplasty (like mitral reconstruction) is one good repair operation and no more interventions involving pledgets. Thank you again for your excellent comments and kind words. George T. Stavridis MD J. Scott Rankin MD

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