Concomitant Ravitch Repair and Mitral Valve Repair in a 13-Year-Old Patient with Marfan Syndrome

This video presents a case of concomitant Ravitch repair and mitral valve repair in a thirteen-year-old patient with Marfan syndrome.

The Patient

The patient was born prematurely at twenty-five weeks. She has a known history of not only Marfan syndrome, but also pectus excavatum and mitral valve prolapse with moderate mitral regurgitation. Both her pectus deformity and mitral valve prolapse had been monitored closely for thirteen years with serial imaging. Around six months prior to intervention, the patient developed some mild dyspnea on exertion. In addition, she was entering the appropriate age range for pectus repair and her left ventricular function had become slightly depressed. After a multidisciplinary discussion, the decision was made to proceed with a combined approach.

MRI images demonstrated the severity of the patient’s pectus deformity. Her sternum was almost touching her spine, with a Haller index of 30. Her heart was fully displaced into the left chest, and she had severe compression of her IVC.

Upon transthoracic echo, the patient was noted to have a prolapsed and thickened mitral valve with moderate regurgitation. At this time her aortic root was only moderately dilated with trace aortic insufficiency.

The Surgery

The procedure began with a midline sternotomy. The perichondrium was incised and elevated off the underlying cartilage for ribs three through eight on the right and ribs two through eight on the left. The cartilages were then fully excised. Next, the perichondrium and intercostal muscles were divided along the left border of the sternum lateral to the left internal mammary artery and a retractor was placed in this space, which provided great exposure to the heart.

Once the patient was cannulated and on cardiopulmonary bypass, the heart was arrested and the left atrium was opened. Upon inspection, the mitral valve appeared to have bileaflet prolapse along with annular dilatation.

First, an annuloplasty was performed with a 32 mm Edwards Physio mitral ring, which seated nicely. While testing the valve, surgeons noted a cleft in the posterior leaflet which was leaking. This was repaired with interrupted 5-0 Prolene sutures.

After closing the left atrium and weaning from cardiopulmonary bypass, attention was turned to the intraoperative TEE, which showed significant residual mitral regurgitation secondary to persistent anterior leaflet prolapse. As a result, surgeons decided to reinitiate cardiopulmonary bypass and rearrest the heart.

The left atrium was opened back up, and the valve was tested again. At this point, surgeons thought an Alfieri stitch would be the best option for addressing the anterior leaflet prolapse. This was accomplished with 4-0 figure-eight Prolene suture between the midportion of the anterior and posterior leaflets.

The patient was once again weaned from CPB, this time with no residual mitral regurgitation and only moderate mitral stenosis with a mean gradient of 6 mmHg. The patient was then fully decannulated and achieved adequate hemostasis.

Next, the pericardium was loosely approximated prior to removing the retractor.

Attention was then turned back to the Ravitch repair. The remaining posterior perichondrium and intercostal muscles were divided in a U-shape, fully detaching the sternum from its attachments to the xiphoid process.

Next, a wedge osteotomy was created just cephalad to the insertion of the third rib. This allowed the sternum to rest in a neutral position parallel to the floor. A titanium plate was then secured to the sternum. A biobridge was then placed underneath the sternum and attached to the medial aspects of the fourth ribs bilaterally to provide additional support to the repair. Finally, the perichondrium was fully reapproximated with interrupted horizontal mattress Vicryl sutures. 

Prior to closing, skin flaps were raised bilaterally over the pectoralis muscles. This allowed for the muscle to be approximated in the midline over the Ravitch repair without any tension. Drains were placed above and below this layer to prevent any seroma formation.

This patient recovered very well after her surgery. She was discharged home on postoperative day ten without any major issues. Her most recent echo, which was about four months after surgery, again showed no significant mitral regurgitation. Her mean gradient across the valve had gone down to two.

Conclusions

There are two main takeaways from this case. First, that a Ravitch repair can safely be performed concomitantly with cardiac surgery and excellent outcomes can be achieved in both cases.
Second, this case shows that an Alfieri stitch can be useful in addressing mitral regurgitation secondary to valve prolapse with minimal mitral stenosis that often improves over time.
 

References

1. Zaki, A. L., P. R. Vargo, D. P. Schraufnagel, V. Kalahasti, S. Murthy, E. E. Roselli and D. P. Raymond (2021). "Modified Ravitch Procedure for Pectus Excavatum Combined With Complex Cardiac Surgery." Semin Thorac Cardiovasc Surg 33(4): 1146-1153.

Disclaimer

Paul Chai is a consultant for Edwards. The information and views presented on CTSNet.org represent the views of the authors and contributors of the material and not of CTSNet. Please review our full disclaimer page here.