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Table of Contents
CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 3  |  Page : 138-141

One-stage surgical repair of heart disease and pectus excavatum in marfan syndrome strategy about the first case performed in the UAE


1 Department of Cardiac Surgery, Claude Bernard University Lyon 1, Villeurbanne, France; Department of Cardiac Surgery, Zayed Military Hospital; Department of Cardiac Surgery, Mediclinic Hospital Airport Road, Abu Dhabi, UAE
2 Department of Cardiac Surgery, Zayed Military Hospital, Abu Dhabi, UAE
3 Department of Thoracic, Cleveland Clinic Abu Dhabi, Abu Dhabi, UAE

Date of Web Publication24-Sep-2018

Correspondence Address:
Olivier Jegaden
Department of Cardiac Surgery, Mediclinic Hospital, Airport Road, Po Box 48481, Abu Dhabi

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HMJ.HMJ_48_18

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  Abstract 


The association of pectus excavatum and cardiac anomalies in patients with Marfan syndrome is not exceptional. The surgical treatment is always challenging. The first case performed in the UAE of a complex surgery in a one-stage procedure with simultaneous complex cardiac valve repair(valve-sparing aortic root replacement associated with mitral valve repair) and pectus deformity correction is reported. An update of the surgical strategy is proposed.

Keywords: Aorta, Marfan syndrome, mitral valve regurgitation, pectus excavatum


How to cite this article:
Jegaden O, Ashafy S, Obeso A. One-stage surgical repair of heart disease and pectus excavatum in marfan syndrome strategy about the first case performed in the UAE. Hamdan Med J 2018;11:138-41

How to cite this URL:
Jegaden O, Ashafy S, Obeso A. One-stage surgical repair of heart disease and pectus excavatum in marfan syndrome strategy about the first case performed in the UAE. Hamdan Med J [serial online] 2018 [cited 2022 Aug 10];11:138-41. Available from: http://www.hamdanjournal.org/text.asp?2018/11/3/138/239179




  Introduction Top


In Marfan syndrome(MFS), the association of pectus excavatum and cardiac anomalies is not exceptional.[1] Surgical strategy has been controversial regarding the repair of chest deformity and heart disease.[2] The first case performed in the UAE of such a complex repair is reported, and an update of the surgical strategy is proposed.


  Case Report Top


A29-year-old malewith a known history of MFS was referred to our institute for surgical assessment. The patient complained about shortness of breath, palpitations as well as psychological impact owing to chest wall deformity with a severe symmetric pectus excavatum[Figure1]. Auscultation detected severe systolic mitral murmur. Echocardiography revealed a severe mitral valve regurgitation due to posterior mitral valve prolapse and a 5-cm aortic root dilation with a normal aortic valve. Pre-operative computed tomography(CT) scan confirmed the severity of the chest deformity and a critical dilation of the aortic root [Figure 2] and [Figure 3]. The patient was proposed for a one-stage combined surgical repair of pectus excavatum and valve lesions through a sternotomy approach.
Figure1: Pre-operative image of the patient showing a severe and symmetric pectus excavatum

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Figure2: Pre-operative angiographic computed tomographic scans. Transverse cross section. The heart is displaced in the left hemithorax. Right cardiac cavities are compressed. Haller index is 7.8. Aortic root is dilated

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Figure3: Pre-operative angiographic computed tomographic scan. Sagittal cross section. Severe pectus excavatum with compression of the aortic root. Three-dimensional reconstruction of the aorta showing an aortic root aneurysm>50 mm

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First, a sub-perichondrial resection of the depressed costal cartilages, following the modified Ravitch technique,[3] was performed to remove all abnormal costal cartilages bilaterally from the fourth to the seventh ribs. Then, the cardiac lesions were approached through a median sternotomy. The mitral valve was exposed first with a trans-septal approach, and a mitral valve repair was done with a simple plication on P2 and an annuloplasty using a 38-mm complete ring. Then, a valve-sparing aortic root replacement according to David Procedure was carried out using a 30-mm Gelweave Valsalva™ Conduit Graft. Finally, after the closure of the sternum, a temporary retrosternal titanium plate was placed at the level of the sixth intercostal space and fixed bilaterally to the ribs providing chest stability and keeping the sternum up. The post-operative outcome was event free.

Two months after the surgery, CT scan showed an evident improvement of the chest deformity and a remarkable decompression of the right cardiac chambers[Figure4]. Six months after the surgery, echocardiography demonstrated a good cardiac function without residual aortic or mitral regurgitation. Then, the retrosternal plate was removed under general anaesthesia from a right side skin incision with a stable and satisfactory cosmetic result[Figure5].
Figure4: Post-operative angiographic computed tomographic scans. Transverse cross section showing the corrected thoracic deformity (Haller index of 3.2) without any residual compression of the heart. The aortic root has a normal diameter according to the graft implanted

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Figure5: Post-operative image of the patient showing the corrected thoracic deformity with a satisfactory cosmetic result

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  Discussion Top


MFS is a severe, chronic, life-threatening disease with multiorgan involvement including the skeletal and cardiovascular systems. It is well known that poor life expectancy in MFS is mainly triggered by cardiovascular complications. In most cases, aortic root dilation is the predominant aortic manifestation. To avoid acute aortic syndromes, prophylactic surgery of the aortic root is recommended in patients with MFS who have a maximum ascending aortic diameter of≥50 mm or≥45 mm in case of risk factors.[4] Although composite graft replacement with an aortic valve prosthesis as Bentall procedure remains the standard, most studies have shown excellent results using valve-sparing root replacement,[5],[6] and nowadays, valve-sparing procedure is recommended in young patients.[4] The David re-implantation technique is more appropriate in MFS because the aortic annulus is more stabilised with less long-term aortic insufficiency.[7],[8]

Mitral valve dysfunction is common in MFS and mainly related to valve prolapse.[9] Interestingly, survival and risk of reoperation after mitral valve repair are similar in MFS and degenerative disease, and in MFS, survival is better after mitral valve repair than replacement.[10] The cardiac strategy developed in the present case, in respect of the guidelines, with concomitant David procedure and mitral valve repair has confirmed the good results of valve preservation techniques in MFS.[11]

Pectus excavatum is often associated with a shift of cardiac structures in the left thorax and/or their mechanical compression by the depressed chest wall.[12] During cardiac surgery, the chest deformity involves difficulty in dividing the sternum in the midline, in retraction symmetrically and in cardiac exposure;[13] moreover, when the pectus deformity is not addressed, the compression of the heart by pectus deformity may contribute to post-operative hemodynamic instability. One-stage procedure with simultaneous cardiac and pectus deformity repair has been performed successfully.[14],[15] In children, cardiac surgery with left thoracic approach and combined with a Nuss procedure was reported.[16] In adults, chest wall is less flexible and sternotomy approach is more appropriate, especially to reach the mitral valve; however, the risk of post-operative sternal dehiscence is a matter of concern when a Nuss procedure is combined with.[14] Consequently, in the present case, a modified Ravitch procedure was done first to facilitate the sternotomy and the exposure of the cardiac structures. The temporary titanium plate was useful to provide firmness to the chest wall according to the Wurtz technique.[17]


  Conclusion Top


In patients with MFS, when cardiac anomalies are associated with pectus excavatum and their correction is mandatory, single-stage repair of both lesions is currently the method of choice. As an alternative to Nuss procedure, modified Ravitch procedure and median sternotomy provide a good exposure of the cardiac lesions and make their repair easy in respect to the current guidelines. Modified Ravitch procedure also allows to correct the chest wall deformity successfully without compromising the post-operative stability of the sternum, as it has been demonstrated in the reported case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
PepeG, GiustiB, SticchiE, AbbateR, GensiniGF, NistriS, etal. Marfan syndrome: Current perspectives. Appl Clin Genet 2016;9:55-65.  Back to cited text no. 1
    
2.
HasegawaT, YamaguchiM, OhshimaY, YoshimuraN, OkaS, OotakiY, etal. Simultaneous repair of pectus excavatum and congenital heart disease over the past 30years. Eur J Cardiothorac Surg 2002;22:874-8.  Back to cited text no. 2
    
3.
RavitchMM. The operative treatment of pectus excavatum. Ann Surg 1949;129:429-44.  Back to cited text no. 3
    
4.
FalkV, BaumgartnerH, BaxJJ, De BonisM, HammC, HolmPJ, etal. 2017 ESC/EACTS guidelines for the management of valvular heart disease. Eur J Cardiothorac Surg 2017;52:616-64.  Back to cited text no. 4
    
5.
CoselliJS, VolguinaIV, LeMaireSA, SundtTM, ConnollyHM, StephensEH, etal. Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: Aprospective, multicenter, comparative study. JThorac Cardiovasc Surg 2014;147:1758-66, 1767.e1-4.  Back to cited text no. 5
    
6.
TreasureT, TakkenbergJJ, PepperJ. Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms. Heart 2014;100:1571-6.  Back to cited text no. 6
    
7.
EsakiJ, LeshnowerBG, BinongoJN, LasanajakY, McPhersonL, HalkosME, etal. The David V valve-sparing root replacement provides improved survival compared with mechanical valve-conduits in the treatment of young patients with aortic root pathology. Ann Thorac Surg 2016;102:1522-30.  Back to cited text no. 7
    
8.
PriceJ, MagruderJT, YoungA, GrimmJC, PatelND, AlejoD, etal. Long-term outcomes of aortic root operations for Marfan syndrome: Acomparison of Bentall versus aortic valve-sparing procedures. JThorac Cardiovasc Surg 2016;151:330-6.  Back to cited text no. 8
    
9.
ThacoorA. Mitral valve prolapse and Marfan syndrome. Congenit Heart Dis 2017;12:430-4.  Back to cited text no. 9
    
10.
HelderMR, SchaffHV, DearaniJA, LiZ, StulakJM, SuriRM, etal. Management of mitral regurgitation in Marfan syndrome: Outcomes of valve repair versus replacement and comparison with myxomatous mitral valve disease. JThorac Cardiovasc Surg 2014;148:1020-4.  Back to cited text no. 10
    
11.
JavadikasgariH, RoselliEE, AftabM, SuriRM, DesaiMY, KhosraviM, etal. Combined aortic root replacement and mitral valve surgery: The quest to preserve both valves. JThorac Cardiovasc Surg 2017;153:1023-300.  Back to cited text no. 11
    
12.
NussD, Kelly RE Jr., CroitoruDP, KatzME. A10-year review of a minimally invasive technique for the correction of pectus excavatum. JPediatr Surg 1998;33:545-52.  Back to cited text no. 12
    
13.
JohnsonWR, FedorD, SinghalS. Systematic review of surgical treatment techniques for adult and pediatric patients with pectus excavatum. JCardiothorac Surg 2014;9:25.  Back to cited text no. 13
    
14.
HysiI, VincentelliA, JuthierF, BenhamedL, BanfiC, RousseN, etal. Cardiac surgery and repair of pectus deformities: When and how? Int J Cardiol 2015;194:83-6.  Back to cited text no. 14
    
15.
WeymannA, RuhparwarA, KarckM. Repair of pectus excavatum and aortic valve-sparing operation: One-stage strategy. Asian Cardiovasc Thorac Ann 2017;25:163.  Back to cited text no. 15
    
16.
RoubertieF, RamananS, LavrandF, ThamboJB. Combined Nuss procedure and cardiac procedure through a left anterolateral thoracotomy. Ann Thorac Surg 2016;102:e537-e539.  Back to cited text no. 16
    
17.
BrianE, BenhamedL, WurtzA. Substernal titanium support after open pectus repair. Ann Thorac Surg 2016;101:832-3.  Back to cited text no. 17
    


    Figures

  [Figure1], [Figure 2], [Figure 3], [Figure4], [Figure5]



 

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