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Ann Thorac Surg 2007;83:S764-S768
© 2007 The Society of Thoracic Surgeons

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Supplement

Aortic Valve–Sparing Operation in Marfan Syndrome: What Do We Know After a Decade?

Department of Thoracic and Cardiovascular Surgery, Hannover Medical School, Hannover, Germany

^_* Address correspondence to Dr Kallenbach, Department of Cardiac Surgery, University of Heidelberg, INF 110, 69120Heidelberg, Germany. (Email: klaus.kallenbach{at}med.uni-heidelberg.de).

Presented at Aortic Surgery Symposium X, New York, NY, April 27–28, 2006.

	Abstract

Top Abstract Introduction Patients and Methods Results Comment References

 
BACKGROUND: We assessed the outcome in patients with Marfan syndrome operated on exclusively with the aortic valve–sparing reimplantation technique for aortic root aneurysms during more than a decade.

METHODS: Between July 1993 and April 2005, the aortic valve–sparing reimplantation technique (David I) was used in 325 patients. In 59 patients with clinical evidence of Marfan syndrome, procedures were done for aortic root aneurysm (n = 55) or aortic dissection type A (n = 4). Their mean age was 30 ± 12 years (range, 9 to 62 years), and 37 (63%) were male. Additional procedures were arch replacement in 4 patients, coronary artery bypass grafting in 1, mitral valve surgery in 9, and closure of atrial septal defect in 3. Mean follow-up was 54 ± 37 months (range, 0 to 139 months).

RESULTS: No patient died during the first 30 days postoperatively. Mean bypass time was 163 ± 34 minutes (range, 99 to 248 minutes), and mean aortic cross clamp time was 126 ± 28 minutes (range, 78 to 202 minutes). Four patients (6.8%) required rethoracotomy for postoperative bleeding. Five late deaths (8.5%) occurred during follow-up. Reoperation of the reconstructed valve was required in 7 patients. Freedom from reoperation was 88% ± 5% at 5 years and 80% ± 9% at 10 years. Mean grade of aortic insufficiency was 1.81 preoperatively compared with 0.20 early postoperatively (p < 0.001). At last investigation, the mean grade of aortic insufficiency increased slightly to 0.22 (p = 0.16). Anticoagulation was not required in 67% of patients. One thromboembolic complication and four instances of minor bleeding were documented. All patients were in New York Heart Association functional class I (86%) or II at last contact.

CONCLUSIONS: Excellent early outcome, favorable long-term results, and acceptable durability of the reimplanted valve should encourage use of this technique in patients with Marfan syndrome.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment References

 
Although valve-sparing techniques for replacement of aortic root aneurysms have shown favorable durability in mid-term and long-term studies [1–3], use of these techniques is still being debated for treatment of aortic root aneurysms in patients with Marfan syndrome. Published data confirm fibrillin fragmentation and deficiency in the aortic cusps of patients with Marfan syndrome and significant valve deterioration in patients with advanced disease [4]. This observation has led to the hypothesis that early cusp degeneration will lead to premature failure of the reconstructed aortic valve. Conversely, aortic valve–sparing techniques with avoidance of postoperative anticoagulation are highly appealing for younger Marfan patients who may anticipate further major surgery on the downstream aorta or skeleton or who wish to become pregnant.

This study assessed outcome in patients with Marfan syndrome operated on exclusively using the aortic valve–sparing reimplantation technique for aortic root aneurysms during more than a decade at a single center to answer the question whether valve-sparing techniques are suitable for patients with Marfan syndrome.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment References

 
Patients
The Institutional Ethic Committee approved this study and waived individual consent for this retrospective analysis. From July 1993 to April 2005, 325 patients were operated on using the aortic valve–sparing reimplantation technique at Hannover Medical School. Data from 59 of these patients, who had Marfan syndrome in accordance with the Gent criteria, were analyzed retrospectively. Mean age was 30 ± 12 years (range, 9 to years), and 37 patients (63%) were male. Indications for operation were aortic root aneurysms in 55 patients (93%) and aortic dissection type A in 4 (6.7%). Comorbidities are listed in Table 1.

Surgical Technique
Standard myocardial protection techniques were used in all patients. In elective patients, standard median sternotomy and extracorporeal circulation with cannulation of the aorta and the right atrium was used. For unstable patients with acute aortic dissection type A and pericardial effusion, the left femoral artery was dissected for arterial cannulation before median sternotomy and pericardiectomy. In stable situations and more recently, the ascending aorta and the right atrium were directly cannulated for extracorporeal circulation.

In all 59 patients, we used exclusively the original aortic valve–sparing reimplantation technique as described by David and Feindel [5], also called David I. The slightly modified operative technique used for this procedure was recently described by our group [6] and was implemented in the same manner in all patients.

Follow-Up
Mean follow–up for all patients was 54 ± 37 months (minimum < 1 month; maximum, 139 months). No patient was lost to follow-up. Patients undergoing root reconstruction received either acetylsalicylic acid or Coumadin (Bayer Healthcare AG, Leverkusen, Germany) for 3 months postoperatively at the discretion of the individual surgeon. Thereafter, anticoagulation therapy was discontinued except in those patients who required antithrombotic prophylaxis for other reasons.

Before hospital discharge, transthoracic echocardiography was used for postoperative assessment of aortic valve function in all patients who underwent the operation. At 1-year to 2-year intervals thereafter, valve function was reevaluated with transthoracic color Doppler echocardiography by our cardiologist or by the referring cardiologist. Aortic regurgitation was assessed semiquantitatively as follows: 0, none; I, minimal; II, mild; III, moderate; IV, severe.

Clinical follow-up was carried out by direct patient contact or by telephone interview with the patient and the referring physician. Patients were assessed according to the New York Heart Association (NYHA) functional classification. Valve performance, complications, and outcome analysis are reported as suggested by the guidelines of the American Association for Thoracic Surgery and the Society of Thoracic Surgeons [7].

Statistical Analysis
Continuous variables are expressed as mean ± SD. All data analyses were performed with SPSS 12.0 (SPSS Inc, Chicago, IL) for Windows (Microsoft, Redmond, WA). Kaplan-Meier analysis was used for the evaluation of time-related variables. Differences in aortic insufficiency between preoperative and postoperative echocardiograms were tested for statistical significance using the Wilcoxon signed rank test for data not normally distributed. A value of p < 0.05 was considered significant.

	Results

Top Abstract Introduction Patients and Methods Results Comment References

 
Perioperative Outcome
The aortic valve was successfully preserved in all 59 patients. No patient died during the first 30 days postoperatively. Mean bypass time was 163 ± 34 minutes (range, 99 to 248) minutes, and mean aortic cross-clamp time was 126 ± 28 minutes (range, 78 to 202) minutes. When it was used, mean circulatory arrest time was 21 ± 8 minutes (range, 15 to 30 minutes). Additional procedures were arch replacement in 4 patients, coronary artery bypass grafting in 1, mitral valve reconstruction in 9, closure of atrial septal defects in 3, correction of chest wall deformities in 3, and peripheral bypass grafting in 1. Rethoracotomy for bleeding was required in 4 patients (6.8%). Mean stay in the intensive care unit was 1.5 ± 0.9 days, and the mean duration of hospitalization was 14 ± 6 days.

During the first 30 days, 2 patients (3.4%) required reoperation on the reconstructed aortic valve. A 39-year-old woman received a 24-mm prosthesis in an uneventful operation, and intraoperative TEE revealed complete aortic valve competence after reimplantation. But while hospitalized, progressive central aortic insufficiency and an atrial septal defect (ASD) developed. On postoperative day 20, she underwent composite replacement and ASD closure. Intraoperatively, valve cusps and geometry were intact. The reason for the valve failure remained unclear, but a technical problem is possible. Her further recovery was uneventful.

Another patient, a 36-year-old woman, experienced postoperative biventricular dysfunction for unknown reasons. Aortic valve incompetence and myocardial infarction caused by coronary malperfusion were ruled out. The patient underwent orthotopic heart transplantation 4 weeks after the David procedure; unfortunately, the graft failed intraoperatively. Despite implantation of a biventricular assist device, the patient died of multiorgan failure 4 weeks after transplantation.

One patient sustained a transient ischemic attack on postoperative day 2, but recovered without sequelae.

Follow-Up
Five patients (8.5%) died during follow-up. One, as mentioned, required heart transplantation owing to biventricular heart failure and died 8 weeks after primary graft failure. In 1 patient, the cause of death was rupture of the native downstream aorta 5 years after primary repair. Two other patients died 5 and 6 years postoperatively for unknown reasons; even so, because of the respective guidelines, the cause of their deaths was considered to be cardiac [7]. One patient died 4 years after having undergone mechanical aortic valve replacement as a reoperation after valve reimplantation; therefore, the cause of death not directly attributable to valve failure after reimplantation. Kaplan-Meier estimates and actuarial survival are given in Figure 1.

View larger version (12K): [in this window] [in a new window]   Fig 1. Actuarial survival for patients with Marfan syndrome after aortic valve–sparing reimplantation surgery.

A 22-year old patient required arch replacement 40 months after the David procedure. Because preoperative echocardiography showed II+ aortic insufficiency, a mechanical valve was implanted to preclude development of increasing insufficiency. Two patients underwent mechanical valve implantation after 3 and 7 years, respectively, for progressive aortic insufficiency, without an explanation of the underlying mechanism of valve deterioration. Kaplan-Meier estimates and actuarial freedom from reoperation of the reimplanted aortic valve are given in Figure 2.

View larger version (12K): [in this window] [in a new window]   Fig 2. Actuarial freedom from reoperation of the reconstructed aortic valve, regardless of underlying reason.

View larger version (23K): [in this window] [in a new window]   Fig 3. Grade of aortic valve insufficiency preoperatively (pre-OP), early postoperatively (post-OP), and at last visit of Marfan patients undergoing aortic valve reimplantation. (AI = aortic insufficiency; Pts, patients.)

	Comment

Top Abstract Introduction Patients and Methods Results Comment References

 
We achieved excellent early results with the reimplantation technique for valve-sparing replacement of the aortic root in patients with Marfan syndrome. No patients died during the first 30-days. One patient died 8 weeks after the operation, without leaving the hospital; however, the cause of death was unrelated to the reimplanted valve. This result is comparable with studies by other groups who have reported remarkably low perioperative mortality rates after valve-sparing aortic root reconstruction in Marfan patients [8, 9] and can compete with results of composite replacement of the aortic root in Marfan syndrome [10, 11].

Outcome during follow-up is favorable, too. Late mortality is comparable with other observational studies, demonstrating that patients with Marfan syndrome have a higher mortality rate than the average population [12]. All patients presented in excellent clinical condition at last contact, with almost all currently in NYHA class I, and none in NYHA class III or IV. In contrast to reports of long-term outcome after mechanical composite replacement [13], neither thromboembolic nor severe bleeding events with clinical sequelae have been reported.

One patient experienced a transient ischemic attack early postoperatively, with complete recovery. However, because we have observed such events twice in our overall cohort of patients operated on using the reimplantation technique [3], we now initiate Coumadin or aspirin therapy for 3 months postoperatively. The proposed advantage of valve-sparing techniques for aortic root replacement—avoidance of lifelong anticoagulation with all its known complications—has been confirmed by our results. This advantage is especially appealing to young Marfan patients anticipating further major operations on the downstream aorta or skeleton as well for women wishing to become pregnant.

The most important potential drawback of aortic valve–sparing surgery is reduced durability of the reconstructed valve. We report 7 patients in this series who required reoperation on the reimplanted valve. As published recently, the presence of Marfan syndrome in our overall cohort of patients operated with the David procedure represents a risk factor for reoperation on the reconstructed valve [3]. This raises the question whether we should continue to recommend reconstruction of the aortic valve in Marfan patients, given that they exhibit accelerated valvular degeneration.

Looking in more detail for underlying reasons for valve failure, the answer to this question becomes easier. One patient required heart transplantation because of biventricular heart failure early postoperatively, but the reconstructed valve was free of insufficiency. Another patient was reoperated on 20 days after reimplantation because of severe aortic insufficiency, although the initial intraoperative echocardiography demonstrated the absence of insufficiency. The reason for valve failure remained unclear because the leaflets appeared undamaged during reoperation. Technical problems as the underlying reason in both cases were discussed but not identified.

Reoperation became necessary in 2 patients operated on at the beginning of our series because of a technical problem: cusp coaptation was below the lower edge of the prosthesis. We have learned that this finding is associated with increased insufficiency of the reimplanted valve and can be avoided by adequate height of resuspension of the commissures in the Dacron (Boston Scientific Corp, Wayne, NJ) prosthesis [14]. A young patient underwent reoperation for arch replacement. Because he showed grade II aortic insufficiency and this same pattern of low cusp coaptation, the aortic valve replacement was undertaken prophylactically.

In only 2 patients was reoperation required, at 3 and 7 years, respectively, because of morphologic changes such as fibrosis or destroyed cusps after initial reimplantation. To what extent typical fibrillin fragmentation of cusps in Marfan patients is responsible for valve failure cannot be answered. In summary, only 2 patients (3.4% overall) who required reoperation would fit into the category of cusp degeneration owing to Marfan syndrome. All the other reasons for reoperation were nonspecific and could have occurred in any patient undergoing aortic valve reimplantation.

At Hannover Medical School, any Marfan patient presenting with significant aortic root aneurysm is a potential candidate for the David procedure. Early operation, if the aortic root diameter is 4 cm or greater, is justified by a very low perioperative mortality because it may prevent the catastrophe of an acute type A aortic dissection. Careful intraoperative inspection of the valve is pivotal, however. Stress fenestration or significant prolapse of cusps should warn the surgeon of morphologic changes in the texture of the valve that may negatively influence outcome after reconstruction. In this situation, composite replacement with a biologic or mechanical valve may be a better choice.

	References

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