Prophylactic surgery of the aortic root in Marfan Syndrome: reconsideration of the decision making process in the era of customised external aortic root support (original) (raw)
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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...
External Aortic Root Support to Prevent Aortic Dilatation in Patients With Marfan Syndrome
Journal of the American College of Cardiology, 2018
BACKGROUND Personalized external aortic root support (PEARS) was introduced in 2004 for prevention of aortic root dilatation in Marfan patients. The individual's aortic root is replicated by 3-dimensional printing. A polymer mesh sleeve is manufactured, which is implanted with the aim to support and stabilize the aortic wall. OBJECTIVES The aim of this study was to assess effectiveness of PEARS for prevention of aortic root dilatation in Marfan patients. METHODS A total of 24 consecutive Marfan patients operated 2004 to 2012 were prospectively monitored with magnetic resonance imaging. Following a pre-defined protocol, baseline and follow-up aorta measurements were made in a blinded random sequence. RESULTS The mean age of the patients was 33 AE 13.3 years (range: 16 to 58 years), and the mean aortic root diameter was 45 AE 2.8 mm (range: 41 to 52 mm). Follow-up was 6.3 AE 2.6 years. There was no increase in the aortic root and ascending aorta diameters, but there was a tendency toward reduction: annulus diameter 28.9 AE 2.3 mm to 28.5 AE 2.4 mm (change À0.39 mm, 95% confidence interval [CI]: À1.05 to 0.27 mm), sinus of Valsalva diameter 44.9 AE 2.9 mm to 44.5 AE 3.0 mm (change À0.37 mm, 95% CI: À1.23 to 0.51 mm), and ascending aorta diameter 32.4 AE 3.6 mm to 32.3 AE 3.7 mm (change À0.10 mm, 95% CI: À0.92 to 0.74 mm). In the same period, the descending aorta diameter increased from 22.9 AE 2.4 mm to 24.2 AE 3.0 mm (change 1.32 mm, 95% CI: 0.70 to 1.94 mm; p < 0.001) with a tendency toward increase in aortic arch diameter 24.1 AE 2.0 mm to 24.5 AE 2.8 mm (change 0.41 mm, 95% CI: À0.56 to 1.37 mm). CONCLUSIONS PEARS is effective in stabilizing the aortic root and preventing its dilatation. It is a viable alternative for prevention of aortic root dissection in Marfan patients.
Heart, 2014
Objective Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To preempt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is being superseded by valvesparing aortic root replacement to avoid lifelong anticoagulation. In 2004, a total root and valve-sparing procedure, personalised external aortic support, was introduced. We report here results among the first 30 recipients. Methods From cross-sectional digital images, the patient's own aorta is modelled by computer aided design and a replica is made in thermoplastic by rapid prototyping. On this, a personalised support of a macroporous polymer mesh is manufactured. The mesh is positioned around the aorta, closely applied from the aortoventricular junction to beyond the brachiocephalic artery. The operation is performed with a beating heart and usually without cardiopulmonary bypass. Results Between 2004 and 2011, 30 patients, median age 28 years (IQR 20-44) had this operation and have been prospectively followed for 1.4-8.8 years by February 2013. During a total of 133 patient-years there were no deaths or cerebrovascular, aortic or valve-related events. These early outcomes are better than published results for the more radical extirpative root replacement operations. Conclusions The aortic valve, the root architecture, and the blood/endothelia interface are conserved. The perioperative burden is less and there has been freedom from aortic and valvular events. A prospective comparative study is planned.
Revista Española de Cardiología (English Edition), 2008
Findings in 54 patients (mean age 39 years, range 18-66 years, 25% female) were analyzed. Of these patients, 21 had dissection of the ascending aorta (15 acute and 6 chronic) and 33 had aneurysm of the ascending aorta. Surgery was classified as emergency surgery in three cases, as urgent in 15, and as scheduled surgery in 36. The Bentall-De Bono procedure was performed in 39 patients, aortic valve reimplantation was carried out in nine, Cabrol's operation was performed in three, and a homograft was used in 3. The mean diameter of the ascending aorta was 66.6 mm. Overall, in-hospital mortality was 3.7% (33.3% for emergency surgery vs 2.8% for scheduled surgery; P<.001). During the mean follow-up period of 4 years (range, 2 months-14 years), 7 patients died, including four who died due to type-B aortic dissection. The actuarial survival rate at 2, 5, and 10 years was 94%, 83%, and 75%, respectively, with 88%, 67%, and 43% of patients, respectively, not requiring reoperation. Elective aortic root replacement was associated with a low risk and a good survival rate.
Surgical management of aortic root disease in Marfan syndrome: a systematic review and meta-analysis
Heart, 2011
Context Surgical treatment of aortic root aneurysm in Marfan syndrome (MFS) patients. Objective To compare results of total root replacement versus valve-sparing aortic root replacement in MFS patients. Data Sources PubMed, Embase and Cochrane library were searched from January 1966 until February 2010 looking for papers reporting on aortic root operations in MFS patients. 530 studies were retrieved. Study Selection Finally, 11 publications were enrolled. Inclusion criteria were observational studies reporting valve-related morbidity and mortality after total root replacement (TTR) and/or valve-sparing root replacement (VSRR) in patients with MFS and study size n$30, reflecting the centre's experience. Data Extraction Data obtained from papers reporting both TRR and VSRR cohorts were analysed separately. In case of multiple publications, the most recent and complete report was selected. If the total number of patient-years was not provided, we calculated it by multiplying the number of hospital survivors with the mean follow-up duration of that study. Results Overall, 1385 patients were analysed (972 patients had TTR and 413 patients had VSRR). Reintervention rate was 0.3%/year (95% CI 0.1 to 0.5) versus 1.3%/year (95% CI 0.3 to 2.2) (p¼0.02) and thromboembolic events rate was 0.7%/year (95% CI 0.5 to 0.9) versus 0.3%/year (95% CI 0.1 to 0.6) (p¼0.01) after TRR and VSRR, respectively. When composite valve-related events were compared, no difference existed between the two surgical strategies (p¼0.41). Among patients undergoing VSRR, reimplantation was associated with a reduced rate of reintervention compared with remodelling (0.7%/year vs 2.4%/year, p¼0.02).
The Journal of Thoracic and Cardiovascular Surgery, 2015
Objectives: Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysms, but the optimal procedure remains undefined. Methods: Adult patients with Marfan syndrome who had Bentall or aortic valvesparing root replacement (VSRR) procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact. Results: One hundred sixty-five adult patients with Marfan syndrome (aged > 20 years) had either VSRR (n ¼ 98; 69 reimplantation, 29 remodeling) or Bentall (n ¼ 67) procedures. Patients undergoing Bentall procedure were older (median, 37 vs 36 years; P ¼ .03), had larger median preoperative sinus diameter (5.5 cm vs 5.0 cm; P ¼ .003), more aortic dissections (25.4% vs 4.1%; P<.001), higher incidence of moderate or severe aortic insufficiency (49.3% vs 14.4%; P <.001) and more urgent or emergent operations (24.6% vs 3.3%; P <.001). There were no hospital deaths and 9 late deaths in more than 17 years of follow-up (median, 7.8 deaths). Ten-year survival was 90.5% in patients undergoing Bentall procedure and 96.3% in patients undergoing VSRR (P ¼ .10). Multivariable analysis revealed that VSRR was associated with fewer thromboembolic or hemorrhagic events (hazard ratio, 0.16; 95% confidence interval, 0.03-0.85; P ¼ .03). There was no independent difference in long-term survival, freedom from reoperation, or freedom from endocarditis between the 2 procedures. Conclusions: After prophylactic root replacement in patients with Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events.