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Eyes wide shut - unusual two stage repair of pectus excavatum and annuloaortic ectasia in a 37 year old marfan patient: case report
© Grapow et al; licensee BioMed Central Ltd. 2011
Received: 17 November 2010
Accepted: 2 May 2011
Published: 2 May 2011
We report about a 37 year old male patient with a pectus excavatum. The patient was in NYHA functional class III. After performed computed tomography the symptoms were thought to be related to the severity of chest deformation. A Ravitch-procedure had been accomplished in a district hospital in 2009. The crack of a metal bar led to a reevaluation 2010, in which surprisingly the presence of an annuloaortic ectasia (root 73 × 74 mm) in direct neighborhood of the formerly implanted metal-bars was diagnosed. Echocardiography revealed a severe aortic valve regurgitation, the left ventricle was massively dilated presenting a reduced ejection fraction of 45%. A marfan syndrome was suspected and the patient underwent a valve sparing aortic root replacement (David procedure) in our institution with an uneventful postoperative course. A review of the literature in combination with discussion of our case suggests the application of stronger recommendations towards preoperative cardiovascular assessment in patients with pectus excavatum.
There are no guidelines concerning the clinical evaluation of patients with isolated pectus excavatum prior surgical repair, but some recommendations do exist [1, 2]. Besides radiographic evaluation using a computerized tomographic scan (CT), performance of an ECG, transthoracic or transesophageal echocardiogram, pulmonary function testing and cardio-pulmonary exercise testing are suggested. However, the extent of physical und especially image-guided examinations is generally on discretion of the physician in charge. In our patient important signs have been ignored retrospectively, which finally led to an unusual two-stage repair.
The patient was transferred to the Hospital Clinic for surgical correction of the cardiovascular pathology. After midline sternotomy the two titan bars were identified. The lower dislocated and broken bar was removed completely, the upper bar was cut and 3 cm were removed. The pericardium was totally intact after the Ravich-procedure. After opening of the pericardium the huge annuloaortic aneurysm became visible. After heparinization and installation of the extracorporal circulation with aortic cannulation of the arch and venous cannulation using a two-stage cannula placed into the right atrium cardiopulmonary bypass was started. The ascending aorta was distally crossclamped directly underneath the brachiocephalic trunc. After inspection of the aortic valve and almost complete resection of the ascending aorta, a valve sparing aortic root replacement (David procedure) using a straight 30 mm Hemashield prosthesis with lateral insertion of the coronary ostia was performed. The echocardiography showed a perfect valve function with low gradients. After weaning from bypass and decannulation protamin was substituted. The sternum was closed using the Robiscek wire reinforcement technique. Apart from a short period of atrial flutter and a spontaneously resolved paralytic ileus the patient's postoperative course was uneventful and he was discharged at day 10 postoperatively.
Historically, when underlying anatomical structures were not considerably affected by the pectus excavatum, a two-stage repair with a first intervention focused on the cardiovascular pathology followed by a second operation addressing the thoracic wall was recommended . Simultaneous repair of both lesions was discouraged because of concerns regarding the potential for major complications, such as limited exposure of the heart, excessive bleeding, and increased risk of wound infection. In the last decade reports about successful simultaneously performed single-stage repairs became evident [4–6].
Rhee and colleagues  report about children with isolated pectus excavatum without a suspected connective tissue disorder, who were referred for routine echocardiographic evaluation. Importantly they found a significantly higher prevalence of aortic root dilatation in those children compared to an age-matched control population.
In our opinion a careful cardiological assessment in patients with pectus excavatum should be obligatory. Driven by our experience and literature besides CT we strongly recommend to perform screening echocardiography, a non-invasive, safe and inexpensive method, in all patients even with isolated pectus excavatum in order to identify those patients with concomitant cardiovascular manifestations.
Written informed consent was obtained from the patient for publication of this case report and any accompanying image. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Jaroszewski D, Notrica D, McMahon L, Steidley DE, Deschamps C: Current management of pectus excavatum: a review and update of therapy and treatment recommendations. J Am Board Fam Med. 2010, 23: 230-239. 10.3122/jabfm.2010.02.090234.View ArticlePubMedGoogle Scholar
- Kelly RE: Pectus excavatum: historical background, clinical picture, preoperative evaluation and criteria for operation. Semin Pediatr Surg. 2008, 17: 181-193. 10.1053/j.sempedsurg.2008.03.002.View ArticlePubMedGoogle Scholar
- 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.PubMedGoogle Scholar
- Willekes CL, Backer CL, Mavroudis C: A 26-year review of pectus deformity repairs, including simultaneous intracardiac repair. Ann Thorac Surg. 1999, 67: 511-518. 10.1016/S0003-4975(99)00015-6.View ArticlePubMedGoogle Scholar
- Ryu YG, Baek MJ, Kim HK, Choi YH, Sohn YS, Kim HJ: 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. J Thorac Cardiovasc Surg. 2009, 137: e34-36. 10.1016/j.jtcvs.2008.07.042.View ArticlePubMedGoogle Scholar
- 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: e75-76. 10.1016/j.jtcvs.2010.06.029.View ArticlePubMedGoogle Scholar
- DePaepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE: Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet. 1996, 62: 417-426. 10.1002/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R.View ArticleGoogle Scholar
- Rhee D, Solowiejczyk D, Altmann K, Prakash A, Gersony WM, Stolar C, Kleinman C, Anyane-Yeboa K, Chung WK, Hsu D: Incidence of aortic root dilatation in pectus excavatum and its association with Marfan syndrome. Arch Pediatr Adolesc Med. 2008, 162: 882-885. 10.1001/archpedi.162.9.882.View ArticlePubMedGoogle Scholar
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