Marfan Syndrome: Single-Stage Surgical Repair of Pectus Excavatum Combined With Mitral Valve Repair and David Procedure

A 29-year-old male with a known history of Marfan syndrome was referred to the authors’ institute for surgical assessment. Physical examination showed a severe symmetric pectus excavatum, with a Haller Index of 7.8. Preoperative workup included a transesophageal echocardiography that revealed severe mitral valve regurgitation due to bileaflet mitral valve prolapse and a 5 cm aortic root dilatation involving the sinuses of Valsalva. The aortic valve was tricuspid and no alterations in flow were observed. A single-stage surgical repair of pectus excavatum and congenital heart lesions was recommended.

Operative Steps

Preparation
The patient was placed in a decubitus supine position and the arms were tucked at the side. General anesthesia was induced and endotracheal intubation was performed with single lumen tube. Draping was prepared from sternal notch to the knees and laterally from side to side. A prophylactic dose of cefazolin was administered.

Modified Ravitch Technique
The procedure was performed through a vertical midline skin incision. Skin flaps were raised with electrocautery to the sternal notch superiorly, the anterior axillary line laterally, and the costal margin inferiorly. The pectoralis muscles were elevated off the sternum and anterior chest wall with electrocautery and were reflected laterally to expose the involved costal cartilages.

Once the costal cartilages were bilaterally exposed, the authors began the subperichondrial resection of the depressed costal cartilages following the modified Ravitch technique. An anterior longitudinal perichondrial incision was made from the costal to the sternal margin. The plane between the perichondrium and the cartilage was established with a mosquito forceps and a periosteal elevator. The cartilage was separated posteriorly by passing a Doyen. Then the cartilage was divided with a knife, grasped, and removed from the perichondrial sheath. The same technique was performed to remove all remaining abnormal costal cartilages bilaterally from the 4^th to the 7^th ribs.

Cardiac Approach and Cardiopulmonary Bypass
Cardiac lesions were approached through a median sternotomy. After the sternal spreader was inserted, the pericardium was opened in reverse T-shaped fashion. Before cannulation, 34,000 IU of heparin were given. Cardiopulmonary bypass (CPB) was initiated and adequate flows were achieved. Antegrade cardioplegia was infused. Consequently, adequate asystole was achieved throughout the whole aortic cross-clamping phase.

Mitral Valve Repair
The left atrium was transseptally approached after opening the right atrium. Inspection of the mitral valve confirmed an isolated prolapse of the P2 leaflet, with excess tissue on both leaflets. Plication of the P2 was done with several stiches of 4-0 Prolene. Additionally, a 38 mm Carpentier-Edwards Physio Annuloplasty Ring™ was inserted. Finally, the atrial approaches were closed with running sutures.

David Procedure
A valve-sparing root replacement (David procedure) was carried out using a 30 mm Gelweave Valsalva™ Conduit Graft. Both native coronary arteries were successfully reimplanted in the prosthetic graft. The aortic valve was examined confirming the normal morphology of the valve without any structural abnormality.

Completion
The patient was successfully weaned from CPB and decannulated. After exhaustive hemostasis, the pericardium was sutured in order to avoid future cardiac adhesions. Once the sternum was closed, a temporary retrosternal plate was placed at the level of the 6^th intercostal space and fixed bilaterally to the ribs, providing chest stability and keeping the sternum up. Finally, the pectoral muscles were brought in the midline, and the rectus abdominis was secured to the inferior aspect of the pectoralis muscles with interrupted sutures. Drains were placed under and below the muscular flaps. The remaining portions of the wound were closed in layers, and the skin was closed with a subcuticular stitch. Aortic cross-clamp time was 164 minutes and CPB time was 210 minutes.

Titanium Plate Removal
Five months after the surgery, the titanium plate was removal through a right periareolar incision.

Preference Card

Titanium plate: Measurements are taken preoperatively in the outpatient clinic based on the patient anatomy.

38 mm Carpentier-Edwards Physio Annuloplasty Ring™.

30 mm Gelweave Valsalva™ Conduit Graft.

Tips and Pitfalls

Advantages
Controversy still remains regarding the simultaneous or multi-staged surgical repair of pectus excavatum and congenital heart diseases in patients with Marfan syndrome. Single-stage procedures offer several benefits, since patients are undergoing only one surgical intervention. It reduces risks associated with additional anesthetic procedures, decreases the amount of surgical injuries and postoperative pain, and avoids the costs related to further hospitalizations. Additionally, single-stage surgical repair of pectus excavatum and heart lesions provides immediate advantages such as postoperative hemodynamic and respiratory improvement because of organ decompression. Patients also usually express an immediate cosmetic satisfaction, which might benefit their postoperative recovery.

Caveats
Postoperative bleeding may represent a life-threatening complication after these complex surgeries. Hence, surgeons must be extremely meticulous with hemostasis. It is advisable to start the procedure performing the cartilage resection before heparinization. Otherwise, it could be more tedious and challenging. Furthermore, resecting costal cartilages at the beginning of the surgery may reduce the cardiac displacement, improve the exposure, and facilitate the approach to the cardiovascular structures after sternotomy.

Titanium plates provide stability and firmness to the chest wall. These short bars have to be fixed properly in order to prevent migration and organ damage. Physicians must be aware that these metallic plates might impede the access to the heart in case of a postoperative emergent reentry. Titanium plates can also impair the effectiveness of chest compression if cardiopulmonary resuscitation is required, although not as much as the Nuss bars do.

References and Suggested Reading

1. Jones WG, Hoffman L, Devereux RB, Isom OW, Gold JP. Staged approach to combined repair of pectus excavatum and lesions of the heart. Ann Thorac Surg. 1994;57(1):212-214.
2. Weymann A, Ruhparwar A, Karck M. Repair of pectus excavatum and aortic valve-sparing operation: One-stage strategy. Asian Cardiovasc Thorac Ann. 2017;25(2):163.
3. Rousse N, Juthier F, Prat A, Wurtz A. Staged repair of pectus excavatum during an aortic valve-sparing operation. J Thorac Cardiovasc Surg. 2011;141(5):e28-30.
4. Raffa GM, Kowalewski M, Malvindi PG, et al. Aortic surgery in Marfan patients with severe pectus excavatum. J Cardiovasc Med (Hagerstown). 2017;18(5):305-310.
5. Stępiński P, Stankowski T, Aboul-Hassan SS, Szymańska A, Marczak J, Cichoń R. Combined mitral valve replacement associated with the Bentall procedure, diaphragmatic hernia repair and reconstruction of the pectus excavatum in a 26-year-old patient with Marfan syndrome. Kardiochir Torakochirurgia Pol. 2016;13(2):135-139.
6. Kansara B, Singh A, Girotra S, Iyer KS. Combined Bentall and modified Ravitch procedures in a patient with Marfan syndrome. J Anaesthesiol Clin Pharmacol. 2013;29(1):95-98.