The Marfan syndrome and the cardiovascular surgeon (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...
Comparison of aortic root replacement in patients with Marfan syndrome
European Journal of Cardio-Thoracic Surgery, 2011
Objectives: Although the aortic-valve-sparing (AVS) reimplantation technique according to David has shown favorable durability results in midterm and long-term studies, composite valve grafting (CVG) according to Bentall is still considered the standard procedure. Methods: Retrospectively, we evaluated the results of aortic root replacement of patients with Marfan syndrome (MFS) who underwent surgery between January 1995 and January 2010. MFS was diagnosed using the Ghent criteria. AVS was used in 58 patients and CVG in 30 patients with MFS. AVS was done for aortic-root aneurysm (n = 48) or aortic dissection type A (n = 10). CVG was used for aortic-root aneurysm in 14 patients or aortic dissection type A in 16 patients. The mean follow-up was 3.2 (95% CI: 2.4-4.2) years. Results: In both groups, 30-day mortality was 0%. Three patients (10.0%) in the CVG group required resternotomy for postoperative bleeding versus two patients (3.4%) in the AVS group (p = 0.3). At follow-up, mortality was 10% in the CVG group versus 3.4% in the AVS group (p = 0.3). Re-operation was required in two patients (3.4%) after AVS and in three patients after CVG (10%) (p = 0.3). Three patients (10.0%) who underwent CVG had endocarditis and two patients (6.7%) had a stroke during follow-up, whereas no endocarditis and stroke occurred after AVS. After 14 years, stratified event-free survival was better in the AVS group (event-free survival was 82.3% vs 58.6%, log-rank test p = 0.086), especially after aneurysm (p = 0.057). After 10 years, freedom from aortic regurgitation !II8 in the AVS group was 80% for aneurysm and 50% after dissection (p = 0.524). Conclusion: The reimplantation technique according to David was associated with excellent survival, good valve function and a low rate of re-operation, endocarditis, and stroke. There was a trend to better event-free survival for AVS patients making it the procedure of choice in MFS patients.
Replacement of the Aortic Root in Patients with Marfan's Syndrome
New England Journal of Medicine, 1999
These results are retrospective and of a predominantly North Americanexperience but do constitute a large number over a long period of time.Several interesting points are raised. The mortality from replacement with a homograft washigher, but this is a newer and more complex procedure and may reflect alearning curve. This group would also not require anticoagulants and 2% of late mortality was from warfarin induced hemorrhage.
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.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, 2015
Valve-sparing root replacement (VSRR) is thought to reduce the rate of thromboembolic and bleeding events compared with aortic root replacement using a mechanical aortic root replacement (MRR) with a composite graft by avoiding oral anticoagulation. But as VSRR carries a certain risk for subsequent reinterventions, decision-making in the individual patient can be challenging. Of 100 Marfan syndrome (MFS) patients who underwent 169 aortic surgeries and were followed at our institution since 1995, 59 consecutive patients without a history of dissection or prior aortic surgery underwent elective VSRR or MRR and were retrospectively analysed. VSRR was performed in 29 (David n = 24, Yacoub n = 5) and MRR in 30 patients. The mean age was 33 ± 15 years. The mean follow-up after VSRR was 6.5 ± 4 years (180 patient-years) compared with 8.8 ± 9 years (274 patient-years) after MRR. Reoperation rates after root remodelling (Yacoub) were significantly higher than after the reimplantation (David)...