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Ann Thorac Surg 2010;89:93-96. doi:10.1016/j.athoracsur.2009.09.015
© 2010 The Society of Thoracic Surgeons
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Original Articles: Adult Cardiac

Aortic Valve-Sparing in 37 Patients With Marfan Syndrome: Midterm Results With David Operation

Alberto Forteza, MDa,*, Javier De Diego, MDa, Jorge Centeno, MDa, Maria Jesus López, MDa, Enrique Pérez, MDa, Carlos Martín, MDa, Violeta Sánchez, MDb, Juan J. Rufilanchas, MDa, Jose Cortina, MDa

a Department of Cardiac Surgery, University Hospital, Madrid, Spain
b Department of Cardiology, University Hospital, Madrid, Spain

Accepted for publication September 8, 2009.

* Address correspondence to Dr Forteza, Hospital 12 de Octubre, Carretera de Córdoba s/n, Madrid, 28041, Spain (Email: aforteza.hdoc{at}salud.madrid.org).


   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 References
 
Background: We reviewed our experience with aortic valve-sparing operations in Marfan syndrome during last 5 years.

Methods: Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent valve-sparing operations. Of these, 37 (68% male) were diagnosed with Marfan syndrome, according to the Ghent diagnostic criteria. Mean age was 30 ± 10 years (range, 11 to 59 years). Moderate/severe aortic regurgitation was present in 13%, and the mean diameter of the Valsalva sinuses was 50 ± 4 mm (range, 42 to 62 mm). The David V modification was performed in the last 28 patients. Additional procedures were mitral valve repair in 6, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13. Mean follow-up was 27 ± 16 months (range, 1 to 61 months).

Results: There were no in-hospital deaths and no major adverse outcomes. One patient required implantation of a mechanical prosthesis during the same procedure because of moderate aortic regurgitation. One late death occurred. No patients required reoperation. In the last follow-up, 23 patients did not have aortic regurgitation, 12 had grade I, and 1 had grade II. No thromboembolic complications have been documented, and 97% of the patients are free from anticoagulation.

Conclusions: Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent. If long-term results are similar, this technique could be the treatment of choice for these patients.


   Introduction
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 Abstract
 Introduction
Patients and Methods
 Results
 Comment
 References
 
Marfan syndrome is the most common heritable disorder associated with connective tissue defect. Prognosis in these patients is mainly determined by aortic root abnormalities, which predispose to progressive dilation, rupture, or dissection [1]. The reduced life expectancy in Marfan patients has changed radically since the introduction of the Bentall-Bono technique in the 1960s [2], considered the standard operation for treating patients with aortic root aneurysm. Despite favorable results with this operation, new techniques have been developed to avoid complications derived from the mechanical prosthesis and long-term anticoagulation. Actually, valve-sparing operations for aortic root aneurysms are increasing in frequency, and reimplantation, first described by David [3], appears to have more durable results than other techniques [4–6].

Several modifications of this technique have been proposed to improve valve durability. In this report we describe our experience with the reimplantation technique in 37 patients with Marfan syndrome and aortic root aneurysm.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 References
 
The Ethic Committee approved this study, and all the patients signed an individual consent for retrospective analysis of their data.

Patients
Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent operations in which a valve-sparing procedure (reimplantation technique) was used. Of these, 37 patients were diagnosed as Marfan syndrome according to the Ghent diagnostic criteria [7]. This study examined the clinical and echocardiography outcomes in these patients. Clinical profile of patients is presented in Table 1, and preoperative echocardiography data is given in Table 2.


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  		Table 1 Clinical Profile
 

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  		Table 2 Preoperative Echocardiography Data
 
Surgical Technique
Moderate hypothermic (32°C) cardiopulmonary bypass and antegrade-retrograde blood potassium cardioplegic arrest were used in all cases. The aortic valve-sparing reimplantation technique with a cylindrical graft (David I) was done in the first patient, the Valsalva graft (Gelweave Valsalva; Sulzer Vascutek, Renfrewshire, Scotland) was used in 8, and the David V modification was performed in the remaining 28 patients. These techniques have been described previously [3, 4, 8].

We calculated the annulus size of the graft by pulling up and approximating the commissures to a point where the leaflets closed properly and no saline infusion was lost. We used a 34-mm woven double velour Hemashield graft (Medi-Tech, Boston Scientific Corp, Natick, MA) in 19 patients and a 32-mm graft in 9 patients, necked down to the decided annular size. At the top of the commissures, a second smaller graft (26 to 28 mm) was sewn to create graft pseudosinuses. Additional shortening of the cusp was performed in 8 patients by plicating the free margin along the nodulus Arantii.

Associated procedures were mitral valve repair in 6 patients, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13.

Follow-Up
Follow-up data were available for all patients. No patient was lost to follow-up. Echocardiography study was done in the second month and annually thereafter. Additional computed tomography or magnetic resonance imaging studies were done every 2 years. All of these patients are periodically evaluated in our Marfan Center.

Aortic regurgitation was assessed as 0, none; I, minimal; II, mild; III, moderate; IV, severe. Valve performance and outcome analysis are reported as suggested by the guidelines of the American Association for Thoracic Surgery and the Society of Thoracic Surgeons [9].

Statistical Analyses
Normally distributed continuous variables were presented as means ± standard error of the media. Categoric variables were presented as frequencies and percentages. The Kaplan-Meier method was used to calculate the actuarial probability of survival and freedom from reoperation. Statistical computations were made using SPSS 16.0 software (SPSS Inc, Chicago, IL).


   Results
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 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 References
 
There were no 30-day or in-hospital deaths and no strokes, acute myocardial infarction, or any other major adverse events. Mean bypass time was 160 ± 38 minutes (range, 110 to 300 minutes), and mean aortic cross-clamp time was 136 ± 28 minutes (range, 100 to 229 minutes). Intraoperative echocardiography showed moderate aortic regurgitation in 1 patient and a mechanical prosthesis was implanted during the same procedure. Two patients required reexploration for bleeding. Mean lengths of stay were 1.6 ± 0.8 days in the intensive care unit and 15 ± 5 days in the hospital.

Follow-up was completed at a mean of 27 ± 16 months (range, 1 to 61 months). One 55-year-old patient died 8 months after the operation of abdominal aortic aneurysm rupture. Actuarial survival for patients is presented in Figure 1. At the last follow-up visit, all patients were free from valve-related complications (reoperation, thromboembolic events or endocarditis) and were free from aortic valve regurgitation greater than grade II (Table 3). All survivors were in New York Heart Association class 0 to I, and 97% of the patients were free from long-term anticoagulant treatment.


Figure 1
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  		Fig 1. Actuarial survival is shown for patients with Marfan syndrome after aortic valve reimplantation.

 

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  		Table 3 Follow-Up Echocardiography Findings in 36 Patients
 

   Comment
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
References
 
The Center for Marfan Syndrome of the Hospital Universitario 12 de Octubre has evaluated 400 patients since 2004, and 190 fulfilled Ghent diagnostic criteria for Marfan syndrome. Dilatation of Valsalva sinuses was found in 70%, and 37 received a valve-sparing reimplantation technique within the last 5 years. This cohort is one of the most numerous to be reported. Kallembach and colleagues [10] reported 59 Marfan patients receiving a valve-sparing operation (David I reimplantation) during a 12-year period. David and colleagues [11] presented 64 Marfan patients who underwent remodeling and reimplantation during a 17-year period. Cameron and colleagues [6] reported 85 Marfan patients who had a valve-sparing procedure, of whom 44 underwent a David I reimplantation procedure in the last 7 years [6].

Historically, the Bentall-De Bono technique [2] changed the life expectancy in these patients and has been the gold standard procedure for aortic root aneurysms. Even today, it remains a safe, reproducible, and durable operation [6]. However, mechanical prosthesis complications and life-long anticoagulation in a group of very young patients have led to the development of other techniques. Valve-sparing operations, and mainly the reimplantation technique, first described by David and colleagues [3], are the preferred operations for aortic root aneurysm in Marfan patients. Volguina and colleagues [12] reported the first prospective international register study to provide comparative data on short-term clinical outcomes after aortic valve-sparing and aortic valve replacement root operations in patients with Marfan syndrome. From March 2005 to January 2008, 18 tertiary hospitals in America and Europe participated. The choice of operation was based on surgeon and patient preferences, and remarkably, 70% of the patients received a valve-sparing operation. The David V technique, used in 54% of this group, was the most common procedure.

We achieved excellent results in our 37 patients. There were no hospital deaths and no major complications. Postoperative echocardiography studies revealed normal aortic valve function in all patients. During follow-up, all patients were free from more than grade II aortic regurgitation (95% presented grade 0 to I aortic regurgitation), and no patient has required reoperation. These results are similar to the studies reported by other groups, with low perioperative mortality rates and also with outstanding outcome during follow-up [6, 7, 13].

Theoretically, creation of pseudosinuses facilitates more natural leaflet motion and reduces diastolic closing cusp stresses. These factors could enhance long-term valve durability [8]. We performed a David I reimplantation with the De Paulis Valsalva graft in 8 patients and the David V technique in the last 28 patients. We found no differences in term of proper functional reconstruction between the two techniques, but in 3 patients, the maximum commissure height exceeded the height of the Valsalva graft and the anatomic appearance was not neat.

We changed to the David V because we believe that this technique facilitates the procedure because it is technically easier to reimplant the valve inside a larger graft. As Miller [4] pointed out, this technique presents various advantages, mainly in Marfan patients: (1) using a larger graft (32 to 34 mm) facilitates exposure and eliminates buckling and crowding of the aortic root tissue, and (2) this technique gives the surgeon flexibility to readjust annulus, sinotubular junction and the height of the commissures without any additional limitations. Finally, as we pointed out previously, we found that Marfan patients have remarkably high commissures, which would exceed the height of the sinuses in the Valsalva graft. Nonetheless, other surgical groups have reported good results with the Valsalva graft or with the original David I using a cylindrical tube graft [6–8, 14]. We believe that both techniques could work equally well for non-Marfan patients.

Sizing of the graft and determining the appropriate diameters for the annulus, sinuses of Valsalva, or the sinotubular junction remains a challenge in this operation. David proposed using the height of the aortic cusps as the measurement to estimate the diameter of the graft, and gave the following formula [15]:


Formula 1

(1)
where D is graft diameter; h is average height of the leaflets, and Ao is aortic wall thickness. We used this formula in the first patients, but remarkably, the diameter was almost equal to the diameter obtained by pulling up and approximating the commissures to a point where the leaflets closed properly and no saline infusion was lost. Now we calculate the sizing of the annulus using this method. We have used a 32- to 34-mm woven double-velour Hemashield graft (Medi-Tech, Boston Scientific Corp, Natick, MA), necking down proximally with interrupted sutures to the diameter obtained by the method described previously. The pseudosinuses are created by sewing a second smaller graft to this graft, as described in the technique by Demers and Miller [16].

According to other publications, 69% of the Marfan syndrome patients evaluated in our center had aortic root dilation, the most common cardiovascular manifestation. But remarkably, the second most common cardiovascular finding by echocardiography study was patent foramen ovale, found in 61% [17]. This finding could be of interest, leading us to close the foramen ovale during the aortic root operations in an effort to prevent paradoxical embolisms in the future.

At Hospital 12 de Octubre, any Marfan patient with an aortic root aneurysm greater than 4.5 cm is evaluated as a candidate for a valve-sparing procedure. This early operation prevents an acute aortic dissection and it is justified by very low perioperative morbidity and excellent midterm results. Patients are informed about the advantages and disadvantages of the technique, mainly the possibility of valve dysfunction and the need for eventual reoperation during follow-up. Therefore, if the patient has a family history of acute aortic dissection, a progressive aortic root enlargement bigger than 2 mm/y, or a valve-sparing procedure is highly likely, the surgical intervention is advised.

Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent in our experience. We believe that if the aortic root is not excessively enlarged, the David V technique is feasible and facilitates the procedure. If long-term results are similar, this technique could replace the Bentall-Bono operation as the standard procedure in these patients.


   References
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 References
 

  1. Murdock JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome N Engl J Med 1972;286:804-808.[Medline]
  2. Bentall HH, DeBono A. A technique for complete replacement of the ascending aorta Thorax 1969;23:338-339.
  3. David TE, Feindel CM. An aortic valve sparing operation for patients with aortic valve incompetence and aneurysm of the ascending aorta J Thorac Cardiovasc Surg 1992;103:617-622.[Abstract]
  4. Miller DC. Valve sparing aortic root replacement in patients with the Marfan syndrome J Thorac Cardiovasc Surg 2003;125:773-778.[Free Full Text]
  5. David TE, Feindel CM, Webb GD, et al. Long term results of aortic valve sparing operations for aortic root aneurysm J Thoracic Cardiovasc Surg 2006;132:347-354.[Abstract/Free Full Text]
  6. Cameron DE, Alejo DE, Patel ND, et al. Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years Ann Thorac Surg 2009;87:1344-1350.[Abstract/Free Full Text]
  7. De Paepe A, Devereux RB, Dietz HC, et al. Revised diagnostic criteria for the Marfan syndrome Am J Med Genet 1996;62:417-426.[Medline]
  8. Leyh RG, Schmidtke C, Sievers HH, Yacoub MH. Opening and closing characteristics of the aortic valve after different types of valve-preserving surgery Circulation 1999;100:2153-2160.[Abstract/Free Full Text]
  9. Miller DC, Weisel RD. Guidelines for reporting morbidity and mortality after cardiac valvular operations Ann Thorac Surg 1996;62:932-935.[Abstract/Free Full Text]
  10. Kallembach K, Baraki H, Khaladj N, et al. Aortic valve-sparing operations in Marfan syndrome: what do we know after a decade? Ann Thorac Surg 2007;83:S764-S768.[Abstract/Free Full Text]
  11. David TE, Feindel CM, Webb G, et al. Aortic valve preservation in patients with aortic root aneurysm: results of the reimplantation technique Ann Thorac Surg 2007;83:S732-S735.[Abstract/Free Full Text]
  12. Volguina IV, Miller DC, LeMaire SA, et al. Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: analysis of early outcome J Thorac Cardiovasc Surg 2009;137:1124-1132.[Abstract/Free Full Text]
  13. De Olivera NC, David TE, Ivanov J, et al. Results of surgery for aortic root aneurysms in patients with Marfan syndrome J Thorac Cardiovasc Surg 2003;125:789-796.[Abstract/Free Full Text]
  14. De Paulis R, De Matteis GM, Nardi P, et al. One-year appraisal of a new aortic conduit with sinuses of Valsalva J Thorac Cardiosvasc Surg 2002;123:33-39.
  15. David TE, Feindel CM. An aortic valve sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta J Thorac Cardiovasc Surg 1992;103:617-622.[Abstract]
  16. Demers P, Miller DC. Simple modification of "T. David-V" valve-sparing aortic root replacement to create graft pseudo sinuses Ann Thorac Surg 2004;78:1479-1481.[Abstract/Free Full Text]
  17. Sanchez V, Mombiela T, Forteza A, et al. Patent foramen ovale and Marfan syndrome: a new association [abstract 2247] Circulation 2008;118:S-693.



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