Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome

Abstract

Background-We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. Methods and Results-Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63.6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. Conclusions-Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases. (Circulation. 1999;100[suppl II]:II-29-II-35.) Key Words: aneurysm Ⅲ valves Ⅲ aorta M arfan syndrome is an autosomal dominant heritable disorder of connective tissue that results in skeletal, ocular, and cardiovascular manifestations. The diagnosis, which has been the subject of several publications, 1-5 is made by clinical and genetic criteria. The prognosis in Marfan syndrome is determined mainly by pathological changes in the aortic root and ascending aorta, which frequently involve the aortic valve. Changes in the aortic valve mechanism are due largely to secondary aortic wall disease. To date, there is no agreement on the type and timing of surgical treatment of aortic valve and root disease in Marfan syndrome. Most patients undergo composite replacement of the aortic root and valve with a Bentall procedure. 6-14 However, all available valve substitutes carry a significant incidence of valve-related complications such as hemorrhage, thromboembolism, infection, and compromised hemodynamics. These complications could be particularly relevant in patients with Marfan syndrome because of the young age, possible existence of left ventricular disease, diffuse arterial pathology, tendency to arterial dissection or leakage, and occasional need for orthopedic operations. In 1979, we developed a valve-conserving operation for patients...

Análise do tratamento cirúrgico da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica

“…The fixation of the aortic ring was considered as the main reason for the lowest incidence of reoperation in patients who underwent reimplantation technique when compared to remodeling technique [12,[18][19][20][21][22]. Several other technical changes were suggested for the reconstruction process of the aortic root with valve preservation and were summarized by Miller [23] in the editorial referred above.…”

Treatment of Aortic Disease in Patients With Marfan Syndrome

“…This result was superior to Yacoub's experience, in which 17% of patients required reoperation by 10 years (17 patients at risk at 10 years). 32 In summary, the valve-sparing aortic root replacement presents patients with MFS with a reasonable alternative to CVG. Survival is excellent with either technique and complications are rare, but the long-term durability of this repair has not been established.…”

“…Recent outcome reports from both Yacoub's and David's institutions indicated that the operative mortality rate for either procedure is low, but the need to return to the operating room for bleeding was 6-fold higher after a Yacoub operation than a David procedure (18% versus 3%). 32,33 Long-term survival was excellent with either technique, especially for prophylactic aortic repairs. Theoretically, valvesparing aortic root replacement should be associated with lower morbidity and mortality when compared with CVG because of the complications associated with a mechanical valve.…”

“…Data from the Yacoub group indicated that 22% of patients had moderate AR at follow-up (median follow-up of 3 years). 32 The presence of mild AR early after surgery portended a progression to more severe AR over time. The prevalence of late AR was lower in David's series, but 25% of patients at 10 years had 3ϩ to 4ϩ AR.…”

Aortic root exoprosthetics as a variant of aortic regurgitation management

“…кардиология и сердечно-сосудистая хирургия, 3,2016 В хирургии аневризм корня аорты (АКА) и недоста-точности аортального клапана (АК) особенно актуальны-ми и востребованными являются клапансохраняющие операции, в основе которых лежат ремоделирование и ре-имплантация корня аорты, описанные М. Якубом [1,8,10] и T. Дэвидом [2][3][4]9]. Низкий процент осложнений, полученный в клиниках с большим опытом реконструк-тивной хирургии, и гемодинамические преимущества де-лают подобные вмешательства крайне привлекательны-ми.…”