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Stent graft placement of the thoracoabdominal aorta in a patient with Marfan syndrome

2003; Elsevier BV; Volume: 125; Issue: 6 Linguagem: Inglês

10.1016/s0022-5223(02)73606-4

1097-685X

Tatjana Fleck, Doris Hutschala, H. Tschernich, Erwin Rieder, Martin Czerny, Ernst Wolner, Martin Grabenwöger,

Connective tissue disorders research

Mutations in the gene encoding fibrillin are the cause of Marfan syndrome, an autosomal dominant disorder with skeletal, ocular, and cardiovascular complications. The leading cause of premature death in patients with Marfan syndrome is progressive dilatation of the aortic root and ascending aorta, resulting in aortic regurgitation and acute aortic dissection.1Treasure T. Cardiovascular surgery for Marfan syndrome.Heart. 2000; 84: 674-678Crossref PubMed Google Scholar, 2Roseborough G.S. Williams G.M. Marfan and other connective tissue disorders conservative and surgical considerations.Semin Vasc Surg. 2000; 13: 272-282PubMed Google Scholar A 24-year-old woman with Marfan syndrome was admitted to our department because a routine computed tomographic (CT) scan revealed a Crawford type II thoracoabdominal aortic aneurysm with a maximum diameter of 5 cm. Other manifestations of Marfan syndrome exhibited by the patient included hyperextensible joints and bilateral inguinal hernia. A replacement of the thoracoabdominal aorta with deep hypothermia and circulatory arrest was subsequently performed on February 2, 1992. Visceral vessels were reimplanted by using the Carrel patch technique. The postoperative course was complicated through a reperfusion edema of the right lung requiring an upper lobe lobectomy on postoperative day 10 and a compartment syndrome at the cannulation leg for cardiopulmonary bypass. Finally, the patient recovered and could be discharged after 32 days. Microscopic histologic examination of the aortic wall revealed cystic medial necrosis. Regular follow-up every 6 months showed a regularly perfused graft with a closed entry tear. The other aortic regions were without further enlargement. Three years later, in 1995, the patient underwent emergency replacement of the ascending thoracic aorta with a 32-mm Vascutek prosthesis (Sulzer Vascutek vascular prosthesis; Sulzer Medica Ltd, Renfrewshire, Scotland) and aortic valve with a Carbomedics 25-mm mechanical prosthesis (Sulzer Medica) as a result of acute aortic dissection type A. After an uneventful postoperative course, the patient was discharged on postoperative day 11. Follow-up CT scan in March 1997 showed an aneurysm of the brachiocephalic trunk extending into the right subclavian artery, requiring replacement of the 2 arteries In April 1999, a new aneurysm in the area of the Carrel patch visceral arteries was detected. Because the patient refused to undergo another operation with deep hypothermia and circulatory arrest, we decided to implant a stent graft at the site of the new aneurysm (Figure 1, A). As preparation before stent graft implantation, bypasses from the visceral arteries to the thoracoabdominal prosthesis were constructed because the origins of the visceral arteries will be occluded by the stent graft. Therefore a 32-mm infrarenal tube graft was implanted to serve as a distal landing zone for the stent graft (Sulzer Medica). Moreover, two 6-mm Dacron prostheses (Uni Graft KDV; B. Braun GmbH, Melsungen, Germany) were implanted as bypass conduits to the renal arteries. Subsequently, 8-mm Dacron grafts were interpositioned to the splenic artery, the celiac trunk, and the superior mesenteric artery (Figure 2, B). In a second-stage procedure in June 1999, the whole thoracoabdominal aorta was stented with 2 Gore Excluder stents (40 mm in diameter and 20 cm in length; W.L. Gore & Associates, Inc, Flagstaff, Ariz) through the right femoral artery. (Figure 2, A, and Figure 1, B) Since then, no more aneurysms occurred thus far. A close follow-up with CT scans and angiography of the entire aorta and its branches is done every 6 months to attempt to recognize any changes (Figure 1, Figure 2, Figure 3). Figure 2A, Angiogram before stent graft and before interposition of Dacron grafts from the visceral arteries to the thoracoabdominal prosthesis. B, Angiogram after stent graft deployment into the thoracoabdominal aortic prosthesis and connection of the renal arteries, the splenic artery, the celiac trunk, and the superior mesenteric artery with Dacron grafts into the prosthesis.View Large Image Figure ViewerDownload (PPT)Figure 3Three-dimensional magnetic resonance angiography from the last follow-up in July 2002 (4 years after placement). The stent graft is regularly perfused, and no migration has occurred since the last follow-up examination. No visible endoleak could be detected.View Large Image Figure ViewerDownload (PPT) Several studies had been conducted to determine survival and event-free survival in patients with Marfan syndrome.3Groenik M. Lohuis T.A. Tijssen J.G. Naeff M.S. Hennekam R.C. van der Wall E.E. et al.Survival and complication free survival in Marfans syndrome implications on current guidelines.Heart. 1999; 82: 499-504PubMed Google Scholar, 4Birks E.J. Webb C. Child A. Radley-Smith R. Yacoub M.H. Early and long term results of a valve sparing operation for Marfan syndrome.Circulation. 1999; 100: 11-29Crossref Google Scholar They showed that at least 34% of patients with Marfan syndrome will have serious cardiovascular complications requiring major cardiac surgery in the first 10 years after diagnosis. The predominant cardiovascular manifestation of the Marfan syndrome affect the aortic root and ascending aorta counting for 82% overall mortality in these patients. Recent reports emphasized the importance of prophylactic replacement of the aortic root in these patients, which improves 10-year survival to 97% compared with 34% in patients who did not undergo surgical intervention.3Groenik M. Lohuis T.A. Tijssen J.G. Naeff M.S. Hennekam R.C. van der Wall E.E. et al.Survival and complication free survival in Marfans syndrome implications on current guidelines.Heart. 1999; 82: 499-504PubMed Google Scholar Recurrent evaluation of the entire aorta in short intervals remains the mainstay of follow-up investigations in patients with Marfan syndrome. However, in a few patients, such as the one presented here, aneurysms also develop in aortic branches.3Groenik M. Lohuis T.A. Tijssen J.G. Naeff M.S. Hennekam R.C. van der Wall E.E. et al.Survival and complication free survival in Marfans syndrome implications on current guidelines.Heart. 1999; 82: 499-504PubMed Google Scholar, 4Birks E.J. Webb C. Child A. Radley-Smith R. Yacoub M.H. Early and long term results of a valve sparing operation for Marfan syndrome.Circulation. 1999; 100: 11-29Crossref Google Scholar The possibility of aneurysms developing in aortic branches, as in the subclavian or carotid arteries in patients with Marfan syndrome, strongly suggests the importance of close observation not only of the aorta but also of the visceral and supra-aortic branches. Endovascular stent graft implantation emerged as an effective and valuable treatment alternative for patients with aortic diseases otherwise not amenable to conventional repair for a variety of reasons,5Dake M.D. Miller D.C. Semba C.P. Mitchell R.S. Walker P.J. Liddell R.P. Transluminal placement of endovascular stent grafts for the treatment of descending thoracic aortic aneurysms.N Engl J Med. 1994; 331: 1729-1734Crossref PubMed Scopus (1303) Google Scholar which was the driving force for us to administer this treatment modality to our patient. This case implies the stringency of regular follow-up examinations in patients with Marfan syndrome, even when replacement of the entire thoracic and abdominal aorta has already been performed. The development of aneurysms of the aortic branches is a rare manifestation in Marfan syndrome, but when it occurs, it has significant effect on quality of life and long-term survival in these patients.