Original Thoracic

Pectus Excavatum and Cardiac Surgery: Simultaneous Correction Advocated Joachim Schmidt1 Bassam Redwan1 Sven Martens1 Karsten Wiebe1

Volkan Koesek1

1 Department of Cardiothoracic Surgery, University Hospital of

Muenster, Muenster, Germany Thorac Cardiovasc Surg 2014;62:238–244.

Abstract

Keywords

► cardiac ► chest ► pectus excavatum

Tonny Djie-Tiong Tjan1

Address for correspondence Joachim Schmidt, MD, Department of Cardiothoracic Surgery, University Hospital of Muenster, AlbertSchweitzer Campus Building A1, Muenster 48149, Germany (e-mail: [email protected]).

Background Severe pectus excavatum may be present in combination with cardiac conditions requiring open-heart surgery. The best strategy for this situation has been debated controversially. Patients and Methods In a retrospective study, we analyzed all our patients undergoing concurrent pectus excavatum correction and open-heart surgery. Results Ten patients aged 9 to 70 years underwent a simultaneous combined surgical procedure between 2001 and 2013. Indications for cardiac surgery were various forms of congenital and acquired heart disease including coronary artery disease with internal thoracic artery grafts and ascending aortic aneurysms. A modified Ravitch procedure was performed for pectus excavatum correction (mean Haller-Index 5.0). Mean operating time was 364 (210–495) minutes and mean duration of cardiopulmonary bypass was 125 (54–222) minutes. All procedures were completed successfully. Postoperatively minor complications were observed in three patients. In-hospital and 30-day mortalities were nil. Good cosmetic and functional results were achieved in all patients. Conclusions Our data demonstrate that simultaneous pectus excavatum correction and cardiac surgery is effective and reliable. A combined approach is advocated if candidates for cardiac surgery present with significant pectus excavatum deformity.

Introduction Pectus excavatum accounts for approximately 90% of all congenital abnormalities of the chest wall and usually occurs as an isolated abnormality.1 Severe cases are generally considered candidates for surgical correction, but frequently the deformity remains untouched because most candidates for stand-alone correction have merely a cosmetic indication. However, patients with congenital or acquired heart disease requiring surgery may present with concomitant, severe pectus excavatum.2,3 In addition, in patients with cardiac disease secondary to inherited connective-tissue disorders, such as Marfan, Ehlers Danlos, or Loeys–Dietz syndromes,

received October 14, 2013 accepted November 28, 2013 published online February 7, 2014

Hermann Aebert1

pectus excavatum is a known manifestation of these diseases.2,4 Underlying cardiac disease may even be exacerbated by the chest wall abnormality. Because of a displaced sternum, especially the right ventricular filling and cardiac output during exercise can be reduced.5–7 In patients undergoing open-heart surgery, correction of a concomitant pectus deformity is often delayed for fear of major complications such as bleeding, wound healing problems, infection, and instability of the chest wall following extensive dissection of sternum, ribs, and soft tissues.8,9 The complexity of the combined procedure, extended operating times, additional median sternotomy, and adverse effects of cardiopulmonary bypass (CPB) present additional risk factors.

© 2014 Georg Thieme Verlag KG Stuttgart · New York

DOI http://dx.doi.org/ 10.1055/s-0034-1367737. ISSN 0171-6425.

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238

However, an uncorrected pectus excavatum deformity may hinder or prevent adequate exposure of cardiac structures via sternotomy.4,7,9,10 This may result in compromised or incomplete treatment of cardiac disease. Furthermore, excessive cardiac compression and displacement is known to result in postoperative hemodynamic instability and impairment of pulmonary function jeopardizing a successful outcome.9,11 Up till now, no consensus regarding the best surgical strategy has been reached. Here, we report our experience in performing simultaneous open-heart surgery and pectus excavatum correction by a modified Ravitch technique.

Patients and Methods All our patients undergoing open-heart surgery and concomitant pectus excavatum correction between 2000 and 2013 were evaluated in this retrospective analysis. Ten patients were identified. In all patients, moderate-to-severe pectus

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excavatum was present. Mean age was 43 (9–70) years. The average Haller index was 5.0 with a range from 3.4 to 7.9 (►Table 1). Nine patients had no previous chest surgery. One patient had a history of two prior failed open pectus repairs resulting in persisting severe pectus excavatum. One patient with ascending aortic aneurysm was diagnosed with Loeys– Dietz syndrome as connective tissue disorder. In the remaining patients, no hereditary diseases were diagnosed. However, genomic analyses were not performed. Indications for cardiac surgery are listed in ►Table 1. Preoperative assessment included pulmonary function, routine laboratory tests, echocardiography, cardiac catheterization, electrocardiography, chest X-ray, and computed tomography. Informed consent was obtained from all patients and/or their parents.

Surgical Technique Following a vertical skin incision and division of the fascia, soft tissue layers including the pectoralis muscles were lifted

Table 1 Patient characteristics Patient no.

Sex (M/F)

Age (y)

ASA

Heart disease

Comorbidity

Thorax deformity

Haller index

1

M

72

2

Coronary artery disease

Replacement of abdominal aortic aneurysm

Pectus excavatum

4.8

2

M

48

4

Ascending aortic aneurysm with dilatation of the aortic annulus

Atrial fibrillation, multiple sclerosis

Pectus excavatum

5.5

3

F

59

3

Mitral valve regurgitation

Atrial fibrillation

Pectus excavatum

7.9

4

M

70

4

Mitral valve regurgitation

NYHA III, atrial fibrillation

Pectus excavatum

3.4

5

F

14

4

Loeys–Dietz syndrome, ascending aortic aneurysm with dilatation of the aortic annulus

NYHA III, ulcerative colitis, spina bifida, severe scoliosis

Pectus excavatum

5.4

6

M

9

3

Atrioseptal defect



Pectus excavatum

4.9

7

M

38

4

Pulmonary and tricuspid valve regurgitation, patent foramen ovale



Pectus excavatum

3.9

8

M

51

4

Coronary artery disease

NYHA III–IV, COPD, atrial fibrillation

Pectus excavatum

4.7

9

M

38

3

Mitral valve regurgitation

Failed previous Ravitch procedure (twice)

Persistent pectus excavatum

4.8

10

M

26

3

Aortic valve stenosis

Severe scoliosis

Pectus excavatum

5.1

Abbreviation: COPD, chronic obstructive pulmonary disease. Thoracic and Cardiovascular Surgeon

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Pectus Excavatum and Cardiac Surgery

Pectus Excavatum and Cardiac Surgery

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Fig. 1 Intraoperative images of patient 8 (Haller index 4.7) showing the extent of the thoracic deformity. Special retractor allowed for sufficient elevation of the left thoracic wall.

from the costal surface of the thoracic wall to expose the deformity in full extent. The upper part of the rectus abdominis muscles and the xyphoid were divided, and the sternum was cut in the midline (►Fig. 1). If median sternotomy did not allow for adequate exposure of the heart, the sternum was split transversely for elevation of depressed sternal segments. In addition, cutting of the costal cartilages including the ribs at the funnel borders was performed to achieve sufficient exposure. The cardiac procedure was performed only after adequate exposure of the heart was established. Following protamine administration and weaning from CPB, the pectus deformity was corrected. The elevated sternum segments were cut and trimmed after complete bilateral costal separation, in most cases from the third through the seventh rib. If required, extra incisions of the tabula externa or complete cuts of the sternum were made. If the ribs had not been shortened enough previously, this was completed following elevation of the sternum. In most children and adolescents, a limited costal dissection and cutting of anterolateral rib portions was sufficient. One to two transverse stainless-steel bars were positioned onto the lateral ribs and posterior to the sternum. For adjustment of protuberant costal arches, additional diagonal bars were inserted following shortening of the respective ribs in some cases. In a minority of cases, the bars were placed anterior or through the sternum according to surgeons’ preference (►Fig. 2). The bars were fixated to the ribs with strong absorbable sutures. Drainage tubes were placed and final hemostasis was reassured. Stainless-steel wires were used for osteosynthesis of the sternum. Rib cartilages were adapted by absorbable sutures. Postoperative treatment at intensive care unit and weaning from ventilation followed the usual regimen for cardiac cases. The postoperative mobilization practice was analogous to routine pectus correction procedures.

omy (n ¼ 1). Different types of retractors, including devices to elevate the left-sided anterior hemithorax, were applied (►Fig. 1). The cardiac procedures performed are listed in detail in ►Table 2. In the case of patient 1, the sternum was elevated following sternal osteotomy, but no metal bar was needed for stabilization (►Table 2). Average operating time was 364 (210–495) minutes and average duration of CPB was 125 (54–222) minutes (►Table 2). There were no revisions for bleeding or instability. No significant respiratory problems occurred. No sternal dehiscence or wound infection was observed in our series. Postoperative complications were observed in three patients. A paralytic ileus (patient 1, ►Tables 1 and 2) on postoperative day 4 required an explorative laparotomy. On the following day, the same patient developed a secondary hematothorax necessitating insertion of a chest drain (n ¼ 1). A second

Results Correction of the thoracic wall deformity was performed for marked displacement of the heart, compression of the right ventricle (►Fig. 3), and inadequate access for the cardiac procedure. In no patient, pectus excavatum was corrected primarily for cosmetic reasons. Access to the heart was achieved by a modified median (n ¼ 9) or transverse sternotThoracic and Cardiovascular Surgeon

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Fig. 2 Pre- and postoperative X-rays of patient 3 undergoing simultaneous mitral valve replacement and pectus excavatum correction.

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240

Fig. 3 A preoperative magnetic resonance imaging of patient 8 showing the pectus excavatum deformity with heart displacement into the left hemithorax and compression of the right ventricle

patient (patient 2, ►Tables 1 and 2) developed a postoperative sero-pneumothorax on postoperative day 6, which was treated successfully with a chest tube. This patient also experienced delayed wound healing, which was effectively treated with one superficial operative revision. A third patient (patient 8, ►Tables 1 and 2) presented with transitory left heart failure following coronary artery bypass graft surgery on postoperative day 2 and was supported with an intraaortic balloon pump for 4 days. On postoperative day 8, a leftsided pleural effusion in this same patient was drained (n ¼ 1). The further course of these and the remaining patients was uneventful and the cosmetic results were judged to be good or excellent. Mean hospital stay was 20 (10–28) days. In-hospital and 30-day mortalities were nil. Postoperative follow-up at 3 and 6 months showed uncomplicated wound healing, adequate sternum stability, and good cosmetic results in all patients. Within the follow-up period, no relapse occurred. So far, the metal bars were removed in eight patients after 12 to 18 months without complications or impairment of the cosmetic results.

Discussion Pectus excavatum complicating open-heart surgery is a rare situation. Staged operations for each problem have mostly been applied in the past with the intention to minimize the operative risk. However, severe pectus excavatum significantly impairs exposure of the heart and jeopardizes the cardiac procedure as well as the postoperative function of the heart. We would like to stress that insufficient exposure, but not cosmetic aspects, is the indication for a combined procedure. Despite several reports describing complications, this strategy persisted for decades.8,9 On the contrary, simultaneous procedures are intricated by an additional median sternotomy, prolonged operation time, extensive preparation of bony and soft tissues with the risks of increased bleeding, and impaired wound healing.12 Our experience in 10 patients indicates that a combined procedure of correction of pectus excavatum and concomi-

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tant open cardiac surgery can be performed safely with adequate morbidity and no mortality. In the past, only solitary cases describing a simultaneous procedure have been published. However, the positive results from our study are supported by two recent case series published in 2008 and 2013. Okay et al reported six patients undergoing concomitant pectus excavatum correction and cardiac surgery.13 Recently, Casamassima et al have published a first series of nine patients with connective tissue disorders and coexistent cardiac disease.4 Here, pectus excavatum repair was achieved using a modified Nuss technique after completion of the cardiac procedure via a median sternotomy. Both authors regarded the simultaneous procedure to be clearly beneficial. Regarding these results and our study, the historical dread of associated complications in combined procedures does not seem to be justified. A variety of technical approaches have been proposed to overcome the problem of concomitant correction of pectus excavatum and cardiac surgery.13–18 Midsternotomy has been considered to be inappropriate for cardiac surgery in patients with pectus excavatum, mainly because of the extremely lowered and often rotated sternum in combination with an extensive displacement into the left thoracic cavity. In addition, median sternotomy in combination with CPB is associated with wound healing problems and sternal dehiscence.4,8,19 Some authors recommended a lateral thoracotomy to avoid improper access to the heart, in particular if a mitral valve procedure has to be performed.20 This seems to be an interesting alternative. However, displacement of the heart and the short distance between sternum and vertebral column still impair access to the heart. Postoperative cardiac edema may foster additional compression of the heart and deterioration of cardiac function due to the persisting chest wall deformity. Moreover, only some cardiac procedures can be performed safely via lateral approaches. Adequate exposure is the cornerstone for surgical success. The approach to the heart has to be carefully planned and the strategy individually adapted to the anatomical deformity encountered. In most cases, a simple midline sternotomy will not be sufficient. In our experience, additional splitting or resection of ribs and transversal incision of the sternum provided sufficient exposure of the heart via median sternotomy. In special cases, a transverse sternotomy may be an alternative. However, both internal thoracic arteries are severely endangered by this approach. Some authors proposed sternal turnover techniques with complete removal and insertion of the corpus sterni.16,21 However, impairment of blood supply is associated with increased risks for major complications such as sternal necrosis, osteomyelitis, and mediastinitis. Parasternal approaches13,17,18 and finally numerous variations of a median sternotomy have also been described.14,15 Midsternotomy for open-heart surgery carries a significant risk for sternal wound infections and dehiscence.22 In our study, the only patient with wound healing complication could be successfully treated with a superficial revision 8 weeks after the initial surgery. Metal bars placed into this field after extended tissue mobilization have to be considered Thoracic and Cardiovascular Surgeon

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B. Modified Ravitch procedure using one metal bar for sternum stabilization

A. Aortic valve replacement

B. Modified Ravitch procedure using one metal bar for sternum stabilization

A. Mitral valve reconstruction with ring annuloplasty

B. Modified Ravitch procedure with four metal bars

A. CABG (LIMA diagonal branch, sequential vein graft to marginal branch, and RCA)

B. Modified Ravitch procedure using three metal bars for sternum stabilization

A. Pulmonary valve replacement, tricuspid valve reconstruction, and closure of PFO

B. Modified Ravitch procedure using one metal bar for sternum stabilization

A. Closure of an ASD

B. Modified Ravitch procedure using one metal bar for sternum stabilization

A. Supracoronary replacement of ascending aorta with a valve-bearing conduit

270

330

540

303

210

338

452

398

495

58

101

150

70

54

173

151

222

158

108

Duration of CPB (min)

10

26

22

28

17

17

17

15

25

22

Length of stay (day)

Left heart failure, pleural effusion

Wound dehiscence, pneumothorax

Ileus, hematothorax

Complication

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Abbreviations: ASD, atrioseptal defect; CABG, coronary artery bypass graft surgery; LAD, left anterior descending artery; LIMA, left internal mammary artery; PDA, posterior descending artery; PFO, patent foramen ovale; RCA, right coronary artery.

10

9

8

7

6

5

B. Modified Ravitch procedure using four metal bars for sternum stabilization

A. Mitral valve replacement

B. Modified Ravitch procedure using one metal bar for sternum stabilization

A. Mitral valve replacement

B. Modified Ravitch procedure using four metal bars for sternum stabilization

A. Supracoronary replacement of ascending aorta with a valve-bearing conduit

307

Duration of surgery (min)

Pectus Excavatum and Cardiac Surgery

4

3

2

A. CABG (LIMA to LAD, sequential vein graft to diagonal branch and PDA)

1

B. Modified Ravitch procedure without metal bars

Procedures

Patient no.

Table 2 Surgical procedures

242 Schmidt et al.

as an additional risk factor exacerbating wound healing problems. In case of infection, the bars have to be removed at the costs of compromising chest wall stability, respiration, and the final cosmetic result. Because of these potential risks, we modified our strategy and do not recommend presternal positioning and implantation of more than two bars. Future studies will have to show if resorbable materials for stabilization could be of advantage in this situation. Preservation of the internal mammary arteries not only enables the surgeon to use these vessels as bypass grafts but also reduces the risk of wound healing problems. In the two patients of our series who underwent coronary artery bypass surgery with use of the left mammary artery, no postoperative wound complications were observed. Overall, an increased incidence of sternal dehiscence and infection was not observed in our patients. To minimize the potential bleeding risk, the major part of the pectus correction was performed after CPB and reversal of heparinization. In other reports, complete dissection of the thoracic wall was performed before CPB. Still, in cases of prolonged CPB time or disproportionate blood loss, the decision whether—and to which extent—to proceed with the pectus repair should be made individually after completion of the cardiac procedure and reassessment of the patient. In our series, the procedure was well tolerated by all patients with an uneventful intraoperative course. A higher risk of bleeding complications due to the extended resection of cartilages in the Ravitch technique compared with the Nuss technique was anticipated by Casamassima et al, but cannot be supported by our data. We have experienced only one minor bleeding complication, which was controlled by inserting a chest drain. The cosmetic results and the sternum stability were good or excellent in all patients at follow-up visits. In adults, the Nuss procedure results in poorer and less reliable outcomes than in juvenile and pediatric age group.23 We used conventional metal bars that may be introduced via a median incision. Casamassima et al applied longer Lorenz bars, inserted via small lateral incisions similar to a Nuss procedure. In our opinion, the type of stabilization bar does not cause a significant difference, if complete dissection of the anterior thoracic wall was achieved. A modified Nuss procedure with sparing of systematic rib dissection may be acceptable only in juvenile patients. Applying different techniques and bars, the series of Casamassima et al and our series seem to show similar cosmetic and functional outcomes. In contrast to some of the previously reported cases, our collective consists of only few juvenile patients with a connective tissue disorder. At least half of our patients presented with acquired heart disease. Forty percent of patients were older than 50 years, and 70% of patients were older than 30 years. Although a rare condition, these patients had an urgent indication for open-heart surgery and presented with an uncorrected moderate-to-severe pectus deformity. The series published from Casamassima et al presented a different cohort of mostly juvenile patients, all with connective tissue disorders. More than 70% were youn-

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ger than 30 years and almost 50% of their collective were Loeys–Dietz syndrome patients. However, they come to a similar conclusion. Individualized surgical planning is recommended for each patient with a concomitant disease, but particularly in young patients, and especially in those with a Loeys–Dietz syndrome, an early simultaneous approached should be anticipated.4 The question whether a simultaneous procedure is superior to a staged approach cannot be answered easily because of the rarity of the condition. Only a limited number of reports have been published. Our series supports the increasing evidence that simultaneous pectus excavatum correction and cardiac surgery can be performed effectively and reliably with good cosmetic and functional results.

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repair for aortic incompetence with annuloaortic ectasia and pectus excavatum by modified Ravitch procedure with pectus bars in an adult patient with Marfan syndrome. J Thorac Cardiovasc Surg 2009;137(1):e34–e36 17 Willekes CL, Backer CL, Mavroudis C. A 26-year review of pectus deformity repairs, including simultaneous intracardiac repair. Ann Thorac Surg 1999;67(2):511–518 18 Javangula KC, Batchelor TJ, Jaber O, Watterson KG, Papagiannopoulos K. Combined severe pectus excavatum correction and aortic root replacement in Marfan’s syndrome. Ann Thorac Surg 2006;81(5):1913–1915 19 Haller C, Sarai K, Siepe M, Beyersdorf F. Concomitant mitral valve replacement and re-re-repair of severe pectus deformity correction in a patient with Marfan syndrome. J Thorac Cardiovasc Surg 2010;140(5):e75–e76

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Pectus excavatum and cardiac surgery: simultaneous correction advocated.

Severe pectus excavatum may be present in combination with cardiac conditions requiring open-heart surgery. The best strategy for this situation has b...
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