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J Thorac Cardiovasc Surg 2009;137:e34-e36
© 2009 The American Association for Thoracic Surgery

Brief Communication

Simultaneous repair for aortic incompetence with annuloaortic ectasia and pectus excavatum by modified Ravitch procedure with pectus bars in an adult patient with Marfan syndrome

Yang Gi Ryu, MD, Man-Jong Baek, MD*, Hyun Koo Kim, MD, Young Ho Choi, MD, Young-Sang Sohn, MD, Hark Jei Kim, MD

Department of Thoracic and Cardiovascular Surgery, Guro Hospital, Korea University Medical Center, Seoul, Republic of Korea

Received for publication July 18, 2008; accepted for publication July 26, 2008.

* Address for reprints: Man-Jong Baek, MD, Department of Thoracic and Cardiovascular Surgery, Guro Hospital, Korea University Medical Center, Guro 2-Dong, Guro-Gu, Seoul, Republic of Korea, 152-703. (Email: mdmjbaek{at}korea.ac.kr).

Concomitant thoracic reconstruction in patients with Marfan syndrome, pectus excavatum, and associated aortic or cardiac surgery poses a major clinical challenge.

Clinical Summary

A 39-year-old man was seen with dyspnea in March 2008. He had no history of musculoskeletal disease or heart disease apart from Marfanoid features according to the Ghent criteria1Go seen on general examination: kyphoscoliosis, pectus excavatum, arachnodactyly, severe myopia, and diastolic murmur on the apex. Routine laboratory values were also unremarkable. Transthoracic and transesophageal echocardiography showed severely dilated ascending aorta, severe aortic regurgitation, moderate mitral and tricuspid regurgitation, and a small type II atrial septal defect. Computed tomographic scans showed a fusiform aneurysm of the proximal ascending aorta with a maximum diameter of 73 mm, severe pectus excavatum (pectus index of 32.6; Figure 1 ), thoracolumbar scoliosis, and dural ectasia of the lumbosacral area.


Figure 1
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  		Figure 1. Preoperative computed tomography of pectus excavatum and annuloaortic ectasia. Axial computed tomographic scan at level of aortic root shows severe depression of sternum and marked dilatation of aortic root, with severe displacement of heart into left side of chest with compression of left lung.

 
Surgery was initiated with a chevron submammary skin incision. The pectoralis muscles were detached from the sternum and retracted laterally, and the rectus abdominis muscles were mobilized inferiorly to expose the deformed costal cartilages. Segmental fractures from the second to seventh costal cartilage were performed at the costochondral junction. A transverse anterior osteotomy of the sternum was created over the level of the manubriosternal junction and fifth cartilage. Bilateral pleura below the costochondral junction were opened to obtain an optimal operative field of the heart, ascending aorta, and aortic arch. The sternum was dissected free from the intercostal neurovascular bundles bilaterally, medial to the internal thoracic arteries all the way up to the second costal cartilage level. This pedicled sternum was then wrapped with wet gauze and retracted cephalad in trapdoor fashion, resulting in perfect exposure of both the heart and the aortic root (Figure 2, A ). Under cardiopulmonary bypass, the mitral valve was replaced with a mechanical valve, the aortic root was replaced with a 25-mm valved conduit (St Jude Medical Inc, St Paul, Minn), tricuspid valvuloplasty was performed by the De Vega method, and the atrial septal defect was closed directly. After an uneventful weaning from cardiopulmonary bypass, the chest wall reconstruction was completed. Both 280-mm and 320-mm pectus bars (MX-bar system; Medix Align Technology, Seoul, Korea) were bent into a convex shape, conforming to the desired curvature of the thoracic cavity anteriorly. The bars were placed across the mediastinum under the pedicled sternum. Both ends and hinge points of the bars were firmly fixed to the ribs with pericostal steel wire sutures (Figure 2, B). The pectoralis and rectus muscles were reattached to the sternum after positioning of mediastinal and pleural drains. The patient was discharged after an uneventful recovery on postoperative day 35 with a regimen of warfarin sodium.


Figure 2
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  		Figure 2. Intraoperative findings. A, Retraction of pedicled sternum in trapdoor fashion. B, Reconstruction of thorax with pectus bars.

 
Discussion

There is a definite association of cardiovascular anomalies with thoracic skeletal deformities in patients with Marfan syndrome. The main issue in such cases is how to approach and repair both the pectus excavatum and any cardiac disease in a single stage because of the difficulties that may result from the cardiac displacement into the left thoracic cavity. Some reports discourage simultaneous repair of both lesions because of concerns regarding the potential for major complications, such as limited exposure of the heart, excessive bleeding, and increased risk of wound infection.2Go Others, however, have reported successful single-stage corrections of both lesions without any complications.3,4Go Historically, numerous modifications have been proposed for simultaneous repair of the pectus excavatum and cardiac disease, including sternal turnover or horizontal or vertical sternal splits. With regard to internal prosthetic sternal support, controversy remains among surgeons regarding its necessity and the propriety of its routine use, whether selectively or otherwise.

Notwithstanding, good sternal healing has been reported with temporary or permanent retrosternal bars, resulting in an essentially normal, cosmetically appealing restoration of the chest wall with no pectus recurrence.5Go This approach, however, may present difficulties in approach for reoperation. Although recent clinical results have been favorable for the minimally invasive repair of pectus excavatum in some older patients, the Ravitch-type procedure is still superior to the minimally invasive method because of the more complex nature and greater severity of deformity in adult patients with Marfan syndrome. Furthermore, with respect to intracardiac exposure and postoperative sternal viability, retraction of the pedicled sternum in trap door fashion as described here could safely and effectively achieve excellent cardiac and aortic root exposure for complex procedures requiring long ischemic times, because both internal thoracic arteries are well preserved.

The described modifications of the Ravitch technique are intended to achieve simultaneous repair of both pectus excavatum and cardiac lesions. We hope that they may lead to development of ever safer and more effective strategies that will allow greater reduction in morbidity and mortality in this high-risk population.

References

  1. De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet 1996;62:417-426.[Medline]
  2. Shamberger RC, Welch KJ, Castaneda AR, Keane JF, Fyler DC. Anterior chest wall deformities and congenital heart disease. J Thorac Cardiovasc Surg 1988;96:427-432.[Abstract]
  3. Pevni D, Lev-Ran O, Shapira I, Mohr R. Combined repair of pectus excavatum and coronary artery bypass grafting. Eur J Cardiothorac Surg 2000;17:495-497.[Abstract/Free Full Text]
  4. Chien HF, Chu SH. Simultaneous Bentall's procedure and sternal turnover in a patient with Marfan syndrome. J Cardiovasc Surg (Torino) 1995;36:559-562.[Medline]
  5. Kim HK, Choi YH, Shim JH, Baek MJ, Sohn YS, Kim HJ. Modified Ravitch procedure: using a pectus bar for posttraumatic pectus excavatum. Ann Thorac Surg 2007;84:647-648.[Abstract/Free Full Text]



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