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Ann Thorac Surg 1997;64:557-559
© 1997 The Society of Thoracic Surgeons


Case Reports

Simultaneous Repair of Pectus Excavatum and Congenital Heart Disease

Maryann M. DeLeon, MD, Kathy E. Magliato, MD, Patrick T. Roughneen, MD, Lynn Graham, RN, Theresa M. Kudukis, MD, Serafin Y. DeLeon, MD

Departments of Thoracic-Cardiovascular Surgery and Pediatrics, Loyola University Medical School, Maywood, Illinois

Accepted for publication March 28, 1997.


    Abstract
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Most repair of heart lesions and pectus deformity has been performed in adult patients using long incisions, sternal splits, excision of deformed cartilages, and sternal turnover operations that could result in poor cosmesis and chest growth in children because of sternal devascularization. We performed simultaneous pectus repair and atrial septal defect closure in 2 children using a short longitudinal incision and avoiding a transverse or longitudinal sternal split. After extraperichondrial excision of the deformed cartilages and mobilization of the sternum from the neurovascular bundles, a transverse wedge of sternum was removed at the level of the third cartilages, allowing cephalad retraction of the sternum and providing excellent exposure for the intracardiac operation. The cosmetic appearance remains excellent in both patients at 1 and 4 years postoperatively.


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Most reports on repair of intracardiac lesions associated with pectus excavatum have been written on adult patients with Marfan's syndrome [15]. These patients had urgent or emergency treatment of aneurysm of the ascending aorta and aortic valve insufficiency. Transverse sternal splits, mobilization or excision of deformed cartilages, and sternal turnover operations were used to achieve exposure of the heart and repair of the pectus deformity [16].

In children, the literature is scant concerning the management of congenital heart disease associated with pectus excavatum. To minimize reoperations, we have performed and now report our experience with simultaneous repair of pectus excavatum and congenital heart defects.

Over a 31/2-year period ending in September 1995, two patients underwent a simultaneous pectus repair and closure of an atrial septal defect in our institution. The ages were 6 months and 31/2 years. Both had severe pectus deformity. The 6-month-old patient was quite symptomatic with tachypnea, chest retraction, and failure to thrive.

The operation was carried out through a short skin incision ranging from 3 to 3.5 cm in length. The Ravitch technique for pectus repair was performed. The pectoralis major muscles were undermined exposing the sternum and the deformed cartilages. The perichondrium of the four lowermost and deformed cartilages was opened and the cartilages were removed. The sternum was dissected free from the intercostal neurovascular bundles medial to the internal mammary arteries to the level of third costal cartilages. The third cartilages were transected tangentially. They would later be used as struts to provide support for the anteriorly displaced sternum. A wedge of sternum was resected transversely at the level of the third cartilages. The sternum was then retracted cephalad and the atrial septal defect repair was undertaken in the usual fashion using cardiopulmonary bypass (Fig 1Go). After the intracardiac procedure, the right pleura was opened widely for chest tube drainage. At the closure, the sternum was displaced anteriorly, supported by the third cartilages, and secured in place with sutures. The rectus abdominis muscles and the most inferior neuromuscular bundles were sutured together to serve as a cradle for the sternum. The pectoralis muscles were reapproximated anterior to the sternum. When an abbreviated skin incision is used, slow retraction allows stretching of the skin without causing it to lacerate and achieves excellent exposure for the repair of both the heart defects and pectus excavatum.



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Fig 1. . Diagram showing exposure of the heart and great vessels after excision of the deformed cartilages and cephalad retraction of the sternum (inset).

 
Both patients had an uneventful postoperative course. Both patients were extubated on the day of the operation, and chest tubes were removed at 32 and 40 hours. The postoperative cosmetic appearance remains excellent at 1 and 4 years postoperatively (Fig 2Go).



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Fig 2. . (A) Preoperative picture of a 6-month-old patient with a severe pectus deformity and a large atrial septal defect. (B) Appearance of the chest 4 years later. The length of the incision is shown ( dark line).

 

    Comment
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In a pectus excavatum deformity, the displacement of the heart into the left chest and posterior concavity of the sternum makes simple midsternotomy approach unsatisfactory for concomitant repair of a cardiac lesion [1, 2, 47]. When the heart defect is repaired but the pectus deformity is not addressed, compression of the heart with severe hemodynamic compromise from the deformed sternum and cartilages has occurred after the intracardiac operation necessitating subsequent repair of the pectus excavatum [4].

The numerous modifications that have been used for the simultaneous repair of heart defects and pectus excavatum can generally be divided into two approaches. One approach is to add a longitudinal or transverse sternal split to the midsternotomy incision with mobilization or excision of the deformed cartilages to facilitate exposure for the intracardiac procedure with subsequent completion of the pectus repair [2, 5, 7]. Another approach is the sternal and cartilage "turnover operation," which would achieve the necessary exposure for the intracardiac operation and also facilitate the repair of the pectus excavatum [1, 6]. Viability of the sternum and the cartilages can be maintained by preserving the internal mammary arteries.

A variant of these approaches is to stage the repair. Because of the concerns about sternal viability, extensive dissection and prolonged operative length, Jones and colleagues [2] opted for a staged procedure. They performed a midsternotomy with mobilization of the deformed cartilages and a Bentall procedure in a 29-year-old patient with Marfan's syndrome followed by excision of the cartilages and completion of the pectus repair 3 days later. Additionally, Kalangos and colleagues [3] also reported performing a Bentall procedure in a 47-year-old patient with Marfan's syndrome through a midsternotomy. At the conclusion of cardiopulmonary bypass and after administration of protamine, excision of deformed cartilages and a Ravitch repair of the pectus deformity were performed. They reported that "despite the displacement of the heart in the left hemithorax, exposure was judged satisfactory for the cardiovascular procedures."

The problems associated with most reported procedures include technical difficulty with prolonged operative time and excessive bleeding, limited mediastinal operative exposure, multiple staged procedures, an extended incision with poor cosmesis and increased risk of devascularization of the sternum and cartilages. These problems had more significance in children because of consideration for chest wall growth and the cosmetic appearance of the incision itself [8].

In children, our approach for the simultaneous repair of a congenital heart defect and pectus excavatum proves safe and satisfying. It achieves good cosmetic results by using an abbreviated skin incision and ensures sternal viability by avoiding the addition of transverse or longitudinal sternal splits. Use of this approach allows excellent exposure and minimal bleeding. Additionally, it precludes the need for multiple procedures.


    Footnotes
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Address reprint requests to Dr Serafin Y. DeLeon, Department of Thoracic and Cardiovascular Surgery, Loyola University Medical Center, 2160 S First Ave, Maywood, IL 60153.


    References
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 Abstract
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  1. Doty DB, Hawkins JA. A turnover operation for pectus excavatum at the time of correction of intracardiac defects. J Thorac Cardiovasc Surg 1983;86:787–90.[Abstract]
  2. Jones GW, 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;57:212–4.[Abstract]
  3. Kalangos A, Delay D, Murith N, Pretre R, Bruschweiler I, Faidutti B. Correction of pectus excavatum combined with open heart surgery in a patient with Marfan's syndrome. Thorac Cardiovas Surg 1995;43:220–2.[Medline]
  4. Miller DR, Pugh DM. Repair of ascending aortic aneurysm and aortic aneurysm and aortic regurgitation complicated by acute cardiac compression by pectus excavatum in Marfan's syndrome. J Thorac Cardiovasc Surg 1970;59:673–84.[Medline]
  5. Tschirkov A, Natschev G, Mishev B, et al. An easy and safe approach for simultaneous repair of severe pectus excavatum and the underlying lesions of the heart and thoracic aorta. J Thorac Cardiovasc Surg 1989;88:305–6.
  6. Gould WL, Jett GH, Bostwick J, Jones EL, Mansour KA. Simultaneous repair of severe pectus excavatum and aortic valve replacement following previous open-heart surgery. Ann Thorac Surg 1988;45:82–4.[Abstract]
  7. Karl TR. A technique for concurrent repair of pectus excavatum and intracardiac defects. J Cardiac Surg 1988;3:487–9.[Medline]
  8. Wilson WR, Ilbawi MN, DeLeon SY, Piccione W, Tubeszewski K, Cutilleta AF. Partial median sternotomy for repair of heart defects: a cosmetic approach. Ann Thorac Surg 1992;54:892–3.[Abstract]



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This Article
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