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Ann Thorac Surg 2007;83:S740-S745
© 2007 The Society of Thoracic Surgeons

Supplement

Preservation of the Bicuspid Aortic Valve

Hans-Joachim Schäfers, MD, PhD*, Diana Aicher, MD, Frank Langer, MD, Henning F. Lausberg, MD

Department of Thoracic and Cardiovascular Surgery, University Hospitals of Saarland, Homburg/Saar, Germany

* Address correspondence to Dr Schäfers, Department of Thoracic and Cardiovascular Surgery, University Hospitals of Saarland, 66421 Homburg/Saar, Germany. (Email: h-j.schaefers{at}uniklinikum-saarland.de).

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


   Abstract
Top
 Abstract
Introduction
 Material and Methods
 Results
 Comment
 References
 
BACKGROUND: Bicuspid anatomy of the aortic valve is a common reason for aortic regurgitation and is associated with aortic dilatation in more than 50% of patients. We have observed different patterns of aortic dilatation and used different approaches preserving the valve.

METHODS: Between October 1995 and February 2006, a regurgitant bicuspid valve was repaired in 173 patients. The aorta was normal in 57 patients who underwent isolated repair. Aortic dilatation mainly above commissural level (n = 38) was treated by separate valve repair plus supracommissural aortic replacement. In 78 patients, aortic dilatation involved the root and was treated by root remodeling.

RESULTS: Hospital mortality and perioperative morbidity were low in all three groups. Myocardial ischemia was significantly shorter in repair plus aortic replacement than remodeling (p < 0.001). Freedom from aortic regurgitation II or greater at 5 years varied between 91% and 96%. Freedom from reoperation at 5 years was 97% after remodeling, but only 53% after repair plus aortic replacement (p = 0.33). Symmetric prolapse was the most frequent cause for reoperation.

CONCLUSIONS: The long-term stability of bicuspid aortic valve repair is excellent in the absence of aortic pathology. In the presence of aortic dilatation, root remodeling leads to equally stable valve durability. In patients with less pronounced root dilatation, separate valve repair plus aortic replacement may be a less complex alternative. Symmetric prolapse should be avoided if the ascending aorta is replaced.


   Introduction
Top
 Abstract
 Introduction
Material and Methods
 Results
 Comment
 References
 
A bicuspid aortic valve is a common reason for significant aortic regurgitation, particularly in the third and fourth decades of life [1]. In these young patients the choice of valve substitute is difficult. With a mechanical valve the linearized risk of thromboembolic complications and anticoagulation-related hemorrhage is low [2], but the cumulative risk may be substantial due to the long life expectancy. In addition, many young patients do not wish to be confined to coumadin-based anticoagulation for lifestyle reasons. A biologic prosthesis, however, is associated with suboptimal durability in this age group [3]. Thus, for patients with a regurgitant bicuspid aortic valve, repair rather than replacement appears as an attractive option.

Bicuspid aortic valve anatomy is also associated with aortic dilatation in more than 50% of the patients [4]. Aortic dilatation may aggravate aortic valve regurgitation, and there is increasing evidence that a diameter of more than 4.5 cm is associated with an impaired long-term prognosis [5]. In conjunction with valve reconstruction, dilatation of the ascending aorta may have to be eliminated to stabilize the repair [6].

It is as yet unclear whether dilatation in the presence of a bicuspid aortic valve involves root and ascending aorta in similar fashion. Consequently, the best surgical approach to the combination of regurgitant bicuspid aortic valve and proximal dilatation is unknown. We have seen different patterns of dilatation in these patients and decided to choose two different approaches. The results of the retrospective analysis of data collected prospectively are analyzed.


   Material and Methods
Top
 Abstract
 Introduction
 Material and Methods
Results
 Comment
 References
 
Of 500 patients undergoing reconstructive aortic valve surgery between October 1995 and February 2006, 173 were treated for regurgitant bicuspid aortic valves and are the subject of this investigation. The study was approved by the local Ethics Committee, and the committee waived the need for patient consent for this data analysis in anonymous fashion. The age of the patients ranged from 2 to 79 years (mean, 48 ± 16); 139 were male. Aortic dilatation, defined as a maximum aortic diameter of 4.5 cm or more, was present in 116 instances. In addition, the data of the 57 patients with regurgitant bicuspid aortic valves and normal aortic size are presented.

Analysis of preoperative angiograms showed three different configurations of the ascending aorta. In 57 patients, the aortic diameter was less than 4 cm, and there was defined sinotubular narrowing (Fig 1A). Aortic dilatation greater than 4.5 cm was observed in 38 patients, and dilatation was most prominent above the commissures without narrowing of the sinotubular junction (Fig 1B). In the remaining 78 patients (aortic diameter greater than 4.5 cm, and in most instances, greater than 5.5 cm), angiography showed dilatation involving the root (Fig 1C). Intraoperative measurements demonstrated sinotubular junction diameters greater than 3.2 cm in all and greater than 4 cm in many.


Figure 1
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  		Fig 1. (A) Typical aortogram with normal aorta and preserved sinotubular narrowing. (B) Aortogram in a patient with mild dilatation of the ascending aorta. There is no sinotubular narrowing. (C) Aortogram of a patient with marked dilatation involving also the aortic root.

 
The surgical strategy was chosen according to the dilating pattern. In the absence of aortic pathology, isolated valve repair was undertaken. If aortic dilatation was predominantly above the level of the root (sinotubular diameter less than 3.3 cm), valve reconstruction was performed and the ascending aorta replaced for elimination of aortic pathology and stabilization of the sinotubular junction. If dilatation involved the root (sinotubular diameter greater than 3.3 cm), root remodeling in addition to valve repair was performed [6].

Isolated valve reconstruction (n = 57) primarily consisted of correction of cusp prolapse. Relative length of the free cusp margin was assessed using radial tension on the commissures. Correction of cusp prolapse included plication of the free margin or triangular resection of the fused cusp in the presence of marked tissue redundancy (>8 mm) or dense fibrosis or calcification in the raphe. Calcification of the raphe or deficiency of cusp tissue was treated by excision and insertion of glutaraldehyde-fixed autologous pericardium. Subcommissural root plication was performed with 3-0 braided sutures and Teflon (Impra, subsidiary of L.R. Bard, Tempe, Arizona) felt.

For the combination of aortic valve repair and supracommissural aortic replacement (n = 38), identical principles were applied to the aortic valve. Subcommissural plication sutures were used in all patients. A 26-mm or 28-mm Dacron (C.R. Bard, Haverhill, Pennsylvania) graft was sutured to the sinotubular junction, shortened, and connected to the distal aorta. This approach was modified after the initial 20 patients. Since then, we have first connected a short segment of Dacron graft (3 to 4 cm) to the aortic root. The valve configuration was then inspected again, and any residual symmetric or asymmetric cusp prolapse corrected. As in all repair procedures, we have routinely assessed valve configuration by measuring height difference between free margin and insertion of the noncoronary cusp in the past 12 months [7]. To do this reproducibly, we used a calliper that allows intraoperative measurement with the cusp in a closed position (Fig 2A, B). The free margin of the noncoronary cusp was shortened to achieve a minimum effective height of 8 mm, and the other margin was shortened to identical level. This height corresponded with intraoperative and postoperative echocardiographic measurements ± 1 mm (Fig 2C). Aortic replacement was then completed if necessary with a second vascular graft.


Figure 2
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  		Fig 2. (A) Schematic drawing of the aortic root with a calliper introduced to measure the height difference between aortic insertion and free margin ("effective height"). (AN = annulus; eH = effective height; LH = leaflet height; STJ = sinotubular junction.) (B) Intraoperative photograph of bicuspid valve after root remodeling has been performed. The calliper is placed at the aortic insertion of the noncoronary cusp with its long arm, the short arm rests on the free cusp margin. The height difference can be measured in millimeters. (C) Intraoperative transesophageal echocardiogram of a patient after repair of a bicuspid aortic valve. A dotted line is drawn at the level of the aortic insertions. The height between the free cusp margin and the aortic insertion can be measured perpendicular to the insertion level.

 
In more extensive dilatation of the root (n = 78), root remodeling modified for bicuspid anatomy was used [6]. Cusp prolapse was corrected as needed [8]. During the past 12 months, cusps were repaired to achieve a height difference of at least 8 mm. Additional procedures (coronary artery bypass, arch replacement, mitral repair) were performed as dictated by the individual pathology.

Intraoperatively all patients were studied using transesophageal echocardiography. The degree of aortic regurgitation was determined primarily by the size of the regurgitant jet determined by color Doppler [9] and the downward slope of the continuous wave Doppler [10]. All patients were studied at least once before discharge, namely, between postoperative days 5 and 7. Further echocardiographic studies were performed at 3, 6, and 12 months and yearly thereafter. Follow-up was complete in all patients and ranged from 2 to 125 months (mean, 40 ± 28), for a cumulative follow-up of 520 patient-years.

Patients who underwent root remodeling were compared with separate valve reconstruction and ascending aortic replacement; the patients with isolated aortic valve repair were taken for additional comparison.

All data are presented as mean ± SD. Statistical analysis included comparison of parametric and continuous variables between the groups using one-way analysis of variance. Kaplan-Meier curves were calculated for freedom of relevant regurgitation, freedom from reoperation, and freedom from valve replacement using a commercially available software package (Prism; GraphPad, San Diego, California). The curves were compared between the groups using the Mantel-Haenszel logrank test. A p value less than 0.05 was considered as statistically significant.


   Results
Top
 Abstract
 Introduction
 Material and Methods
 Results
Comment
 References
 
The groups were not directly comparable (Table 1). Most importantly, the techniques required for valve reconstruction included significantly less frequent use of pericardial patches for cusp repair in the remodeling group (p < 0.001). Bypass and myocardial ischemic times were significantly longer (p < 0.001) with root remodeling compared with the other two groups. After eliminating patients with concomitant operations, bypass and ischemic time were similar for valve reconstruction versus valve repair plus aortic replacement; root remodeling required significantly more time (p < 0.001).


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Two patients died in hospital (early mortality, 1.1%). One patient died of noncardiac causes 7 months postoperatively; all others are alive and well. There was no atrioventricular conduction disturbance; no patient had clinical symptoms consistent with thromboembolic complications early or late postoperatively.

One patient required reoperation on the operative day for dehiscence of a suture line after isolated aortic valve repair with excision of calcium from the fused cusp; the valve was re-repaired using autologous pericardium. In 1 patient, endocarditis developed 2 months after isolated valve repair and was initially treated conservatively. He was reoperated on 9 months later for moderate aortic regurgitation and underwent valve replacement. Five additional patients were reoperated on for aortic regurgitation between 6 and 53 months postoperatively. The aortic valve was replaced in 2; it was re-repaired in the remaining 3.

Long-term results were similar in the three groups. Freedom from aortic regurgitation II or greater at 5 years was 95.5% after remodeling and 91% after valve repair plus aortic replacement versus 92.1% after isolated valve repair (Fig 3). Freedom from reoperation at 5 years was 97% after remodeling and 94.3% after valve reconstruction. After repair plus aortic replacement, it was only 53.3%. This difference was not significant (p = 0.33) owing to the low number of patients beyond 4 years of follow-up. Since several of the valves were re-repaired, freedom from valve replacement at 5 years was 98.7% after remodeling, 97.8% after reconstruction, and 80% after reconstruction plus aortic replacement (Fig 4). Reasons for reoperation were cusp suture dehiscence (n = 2), endocarditic destruction (n = 1), and symmetric cusp prolapse (n = 4).


Figure 3
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  		Fig 3. Kaplan-Meier analysis of freedom from aortic regurgitation of II or greater in the three groups: AVR+asc (dashed line) = separate aortic valve repair plus supracommissural aortic replacement; AVR (solid line) = isolated aortic valve repair. (Dotted line = remodeling.)

 

Figure 4
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  		Fig 4. Kaplan-Meier analysis of freedom from aortic valve replacement in the three groups: AVR+asc (dashed line) = separate aortic valve repair plus supracommissural aortic replacement; AVR (solid line) = isolated aortic valve repair. (Dotted line = remodeling.)

 
Careful analysis of the echocardiograms in the patients who had to be reoperated on for regurgitation with cusp prolapse showed symmetric prolapse of both cusps already early postoperatively despite acceptable valve function (remodeling, n = 2; repair plus replacement, n = 2). In these patients the degree of regurgitation increased over time. The most striking finding was an abnormal configuration of the aortic valve. Not only was cusp tissue prolapsing into the left ventricular outflow tract, but also the height difference between the coaptation point of the cusps and the aortic insertion was abnormally low, namely, 4 to 6 mm (Fig 5). This height difference ranged between 8 and 11 mm in almost all remaining patients. In 2 of these patients, the valve was replaced. In the last 2 patients, prolapse was corrected in both cusps by shortening of the free margin. The resulting height difference was 9 mm, and the 2 valves have been competent and stable since. Suture dehiscence occurred in 2 patients. In both, the torn cusp was reconstructed using autologous pericardium.


Figure 5
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  		Fig 5. Typical echocardiogram of a patient with symmetric prolapse after separate valve repair and aortic replacement. The dotted line marks the coaptation level of the free margins, the solid line, the level of the aortic insertion. The most striking finding is an abnormally low height difference of 4 mm between central cusps and aortic insertion.

 

   Comment
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
References
 
Aortic valve reconstruction appears to be particularly interesting for young patients with a malfunctioning bicuspid aortic valve who can be expected to have suboptimal durability of any biologic heart valve substitute. Reconstruction of the aortic valve may have the additional advantage of lower rates of thromboembolism and endocarditis [11]. In a large series, excellent early results could be obtained with repair [12]. Later data, however, showed a significant rate of reoperations within the first 5 years. Apart from triangular cusp resection, no specific risk factor for failure could be identified [13].

In accordance with the observations of others [14], we have previously hypothesized that progression of aortic dilatation may be an important mechanism of valve failure in the presence of bicuspid anatomy [6]. We have previously proposed root remodeling for regurgitant bicuspid aortic valves and concomitant aortic dilatation, and midterm results have been gratifying [15]. Our current data support the previous conclusions regarding the excellent 5- to 10-year stability of remodeling in the presence of bicuspid aortic valves. Our current data also show that excellent midterm results with freedom from reoperation of 94% at 8 years can be achieved by isolated aortic valve repair if the aorta is normal in size and configuration.

This raises the important question of which criteria should be used for a normal aorta, and what aorta is large enough to warrant root replacement. It may also be questioned whether root replacement as a more complex procedure is justified for all forms of aortic dilatation. Finally, some bicuspid valves may require complex repair procedures including partial cusp replacement, and omitting root remodeling would simplify the operation.

While exact dimensions were difficult to quantify exactly in all patients, we observed normal aortic configuration with preserved sinotubular narrowing in approximately 40%. Aortic dilatation was present in the majority of patients, consistent with other findings [4, 16]. Interestingly, however, we saw different dilatation patterns. In some patients, maximum dilatation was at the level of the mid ascending aorta, and in most of these, the sinotubular diameter was enlarged to less than 3.5 cm by intraoperative measurement on the flaccid aorta. In the remaining patients, there was marked dilatation involving the aortic root. We hypothesized that remodeling might not be necessary in patients with less pronounced root dilatation.

In using the less complex approach of separate repair plus supracommissural replacement for less pronounced aortic dilatation, we had hoped to provide an alternative to remodeling. While 5-year freedom from reoperation was not significantly worse than that of remodeling, the apparent difference in the Kaplan-Meier curves was a source of concern. Interestingly, there was no echocardiographic evidence of progressive root dilatation. Instead, symmetric prolapse was found as the reason for valve failure in the separate repair plus replacement group. It also occurred after root remodeling. That was confirmed by the finding that symmetric shortening of the free margin in the 2 patients who were re-repaired led to normal valve configuration and function.

The most prominent echocardiographic phenomenon indicating cusp prolapse was a decreased height difference between the central cusp margins and the aortic insertions, a finding that had previously been described in conjunction with subsequent failure of the reimplantation operation [17]. This height difference is different from the leaflet or cusp height used before, which is the largest distance from insertion line to free margin with the cusp flattened out [18]. The height difference, which we have termed "effective height," depends on the complex relationship of root and cusp dimensions and can easily be determined by echocardiography. In 40 healthy persons, we have found effective height to range between 8 and 11 mm, which correlates with old data [19].

We have routinely instituted intraoperative calliper measurement of effective height and tried to achieve a height at least 8 mm. By using the calliper for repeated intraoperative measurements, we have found that reduction of the sinotubular junction diminishes effective height both in remodeling and supracommissural aortic replacement. A reduction of sinotubular diameter by 3 to 4 mm consistently led to a height decrease of 2 to 3 mm. This change in cusp configuration can be easily corrected by shortening of the free margin. We have thus changed our repair sequence for any procedure involving reduction of sinotubular diameter. Aortic replacement is performed first with or without root replacement using a short Dacron graft. The cusps are then inspected, and prolapse is defined and corrected by measuring effective height. If necessary, a second graft is added. Using this approach, we have been able to improve the early results of aortic valve repair further and have not seen regurgitation of more than grade I in the last 30 repair procedures.

In conclusion, we have found that the results of reconstruction of bicuspid aortic valves are excellent if there is no aortic pathology. If aortic dilatation is present and treated by aortic replacement, the results of root remodeling are as good as after isolated valve repair. Separate valve repair and supracommissural ascending aortic replacement may be an alternative for some patients with less pronounced root dilatation, provided that adequate configuration of aortic cusps and root are achieved. Any operation that reduces sinotubular diameter, however, may induce symmetric cusp prolapse, which has to be corrected to avoid limiting midterm stability of the valves.


   References
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 

  1. Ward C. Clinical significance of the bicuspid aortic valve Heart 2000;83:81-85.[Free Full Text]
  2. Khan S. Long-term outcomes with mechanical and tissue valves J Heart Valve Dis 2002;11(Suppl 1):8-14.[Medline]
  3. Banbury MK, Cosgrove III DM, Lytle BW, Smedira NG, Sabik JF, Saunders CR. Long-term results of the Carpentier-Edwards pericardial aortic valve: a 12-year follow-up Ann Thorac Surg 1998;66(Suppl):73-76.[Abstract/Free Full Text]
  4. Nistri S, Sorbo, MD, Marin M, Palisi M, Scognamiglio R, Thiene G. Aortic root dilatation in young men with normally functioning bicuspid aortic valves Heart 1999;82:19-22.[Abstract/Free Full Text]
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  8. Langer F, Graeter T, Nikoloudakis N, Aicher D, Wendler O, Schäfers HJ. Valve-preserving aortic replacement: does the additional repair of leaflet prolapse adversely affect the results J Thorac Cardiovasc Surg 2001;122:270-277.[Abstract/Free Full Text]
  9. Galassi AR, Nihoyannopoulos P, Pupita G, Odwadara H, Crea F, McKenna WJ. Assessment of color flow imaging in the grading of valvular regurgitation Eur Heart J 1990;11:1101-1108.[Abstract/Free Full Text]
  10. Wilkenshoff UM, Kruck I, Gast D, Schroder R. Validity of continuous wave Doppler and colour Doppler in the assessment of aortic regurgitation Eur Heart J 1994;15:1227-1234.[Abstract/Free Full Text]
  11. Langer F, Aicher D, Kissinger A, et al. Aortic valve repair using a differentiated surgical strategy Circulation 2004;110(Suppl 1):II67-II73.[Medline]
  12. Fraser Jr CD, Wang N, Mee RB, et al. Repair of insufficient bicuspid aortic valves Ann Thorac Surg 1994;58:386-390.[Abstract]
  13. Casselman FP, Gilinov A, Akhrass R, Kasirajan V, Blackstone E, Cosgrove D. Intermediate-term durability of bicuspid aortic valve repair for prolapsing leaflet Eur J Cardiothorac Surg 1999;15:302-308.[Abstract/Free Full Text]
  14. Moidl R, Moritz A, Simon P, Kupilik N, Wolner E, Mohl W. Echocardiographic results after repair of incompetent bicuspid aortic valves Ann Thorac Surg 1995;60:669-672.[Abstract/Free Full Text]
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