Combined Severe Pectus Excavatum Correction and Aortic Root Replacement in Marfan’s Syndrome (original) (raw)
Related papers
Aortic Surgery in a Patient with Marfan Syndrome and Pectus Excavatum
2012
Severe cardiovascular disorders, including aortic dissection and aneurismal dilation of the aorta, are the main life-threatening complications of the Marfan syndrome 1 . Approximately two thirds of individuals who have this syndrome have chest wall deformities such as pectus excavatum or pectus carinatum 2 . When a patient with pectus excavatum needs aortic surgery, the surgeon may face a major clinical challenge in choosing the optimal surgical approach 3 . We present a case of the Marfan syndrome with severe pectus excavatum who underwent aortic surgery (Iranian Heart Journal 2012; 13 (1):55 -58).
Journal of Cardiothoracic Surgery, 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.
Descending aortic replacement after Nuss for pectus excavatum in a Marfan patient-Case report
International journal of surgery case reports, 2016
The Nuss procedure for pectus excavatum (PE) repair has been successfully performed in Marfan syndrome (MFS) patients however there is concern for future risk of aortic dilation/rupture and need for emergent access with support bars in place. We present a 45 year-old male with MFS that required descending aortic replacement shortly after modified Nuss repair. The majority of MFS patients have severe PE and repair with the Nuss procedure is not uncommon. The risk for life threatening aortic dilation, dissection, or rupture in such patients is a concern when utilizing this technique. Our work has been reported in line with the CARE criteria. Nuss repair should be considered in MFS patients with technique modifications and careful consideration of future risk of aortic dilation and rupture.
Interactive cardiovascular and thoracic surgery, 2011
A 32-year-old female patient with Marfan syndrome was admitted for repair of annuloaortic ectasia and severe pectus excavatum. Because the chest cage deformity was severe, concomitant reparative surgery of the chest wall was performed. Partial median sternotomy and left second-fifth rib division was made to obtain good surgical field. The patient underwent valve-sparing aortic root remodeling successfully. After complete neutralization of heparin, additional division of the right ribs was performed and each rib and the sternum was reshaped. Pectus excavatum was completely repaired by this method. We believe this approach is efficacious for intracardiac repair with severe pectus excavatum.
The Journal of Heart and Lung Transplantation, 2009
A 24-year-old man with Marfan syndrome underwent mitral valve repair for prolapse at age 13. He sustained an acute type A aortic dissection at age 20 and underwent aortic root/ascending aortic replacement with a mechanical valved conduit. He initially did well after the latter procedure, but end-stage heart disease developed 4 years later, apparently secondary to primary cardiomyopathy. Pre-transplant evaluation revealed residual chronic dissection with aneurysmal dilatation of the distal ascending aorta, transverse arch, and descending thoracic aorta. He underwent combined orthotopic heart transplantation (OHT) and total arch replacement (stage I elephant trunk procedure). Subsequently, he underwent extent II thoracoabdominal aneurysm repair, leaving no residual aortic disease. The 2 procedures resulted in total cardioaortic replacement, thus definitively managing his cardiomyopathy and aortic disease resulting from Marfan syndrome. The operative strategies employed represent a novel approach in this clinical setting. This report emphasizes that patients with this disease should not be denied potentially life-saving OHT on the basis of concomitant aortic disease, but rather should be treated in centers offering expertise in both areas of surgical therapy.
Aortic root replacement and valvuloplasty in a woman with severe pectus excavatum
Journal of cardiovascular and thoracic research, 2018
We describe a case of a 43-year old woman with Marfan's syndrome, tricuspid valve regurgitation and severe pectus excavatum who required an aortic root replacement and valvuloplasty for an ascending aortic aneurism with aortic valve regurgitation and tricuspid valve regurgitation. There was a severe angulation of the sternum which was close to 1.5 cm to the column vertebrae. Such cases are quite rare and always a challenge for surgeons to achieve adequate exposure of the heart and prevent excessive cardiac compression. We present our access, a safe and efficient exposure of the heart in order to achieve the best outcome.
Aortic root surgery in Marfan syndrome
The Israel Medical Association journal : IMAJ, 2008
BACKGROUND As the shortcomings of the Bentall operation and its variants in the Marfan syndrome have become apparent, the recent cusp-sparing techniques (remodeling or reimplantation) bear promise of better mid-term and long-term outcomes. OBJECTIVE To examine the results of aortic root surgery in patients with Marfan syndrome. METHODS During the period March 1994 to September 2007, 220 patients underwent aortic valve-sparing surgery; 20 were Marfan patients (group 1) who were compared with another 20 Marfan patients undergoing composite aortic root replacement (group 2). Fourteen patients had aortic dissection and 26 had thoracic aortic aneurysm. There were 31 males and 9 females with a mean age of 37.9 +/- 13.8 years. In group 1, reimplantation was used in 13 patients, remodeling in 4, and aortic valve repair with sinotubular junction replacement in 3. In group 2, a mechanical valve conduit was used. Mean logistic Euroscore was 12.27 +/- 14.6% for the whole group, five of whom wer...
Aortic repair in Marfan's syndrome: prevention of mechanical leaflets damage
Cardiovascular Surgery, 2001
Aortic incompetence in Marfan's syndrome results from distortion or dilatation of the sinuses of Valsalva, annuloaortic ectasia or a combination of these problems. Valve leaflets in these patients are macroscopically normal in spite of aortic insufficiency. Replacement of the ascending aorta, root and aortic valve with a composite graft was, for a long time, the treatment of choice for Marfan patients. Valve-preserving procedures (remodeling or reimplantation) provide the advantages of avoiding the shortcomings of standard surgical techniques, and maintaining the functional integrity of the left ventricular (LV) outflow tract, aortic root and ascending aorta. We developed a modified valve-sparing reimplantation technique for avoiding leaflet damage. This was achieved by leaving a 'cushion' of aortic wall (8-10 mm) that, sewn on the Dacron graft, works as a 'damper' and prevents leaflets injury during the systolic opening of the valve. For final judgment of this operative method long-term results are necessary.