Table 2:

Summary of included studies

Author, year and countryDemographicsaStudy designOutcomesaResultsa
Aicher et al. (2004), Germany [15]n = 60
Male 83.3%
Mean age 53 ± 12 years
Aortic dissection 6.7%
Retrospective cohort study comparing valve-sparing aortic root replacement in patients with TAV or BAVIn-hospital mortality
Postoperative AI grade
5-year overall survival
TE events/endocarditis during follow-up
Reoperation during follow-up
5-year mean AV gradient
5-year freedom from AI > II
5-year freedom from reoperation
0%
0.8 ± 0.7
82%
0%
1.7%
4.5 ± 2.3 mmHg
96%
98%
Aicher et al. (2007), Germany [16]n = 81
Male 85.2%
Aortic dissection 7.4%
AI > II 55.6%
Marfan 0%
Retrospective cohort study of aortic root remodelling in patients with BAV, AI and dilatation of the ascending aorta. Comparison made with patients with TAVHospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0%
94%
87%
97%
99%
Aicher et al. (2010), Germany [17]n = 205Retrospective cohort study comparing AV repair in patients with BAV and TAV, limited raw data presented for BAV5-year freedom from AI > II
10-year freedom from AI > II
86%
83%
Aicher et al. (2011), Germany [4]n = 316
Male 84.8%
Age range 3–79 years
Aortic dissection 8%
Severe AI 72.8%
Retrospective case series evaluating the effect of valve configuration on outcomes after repair of BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0.63%
92%
81%
81%
84%
Alsoufi et al. (2005), Canada [18]n = 71
Male 87.3%
Mean age 41.5 ± 13.2 years
Retrospective case series of prospectively collected data on AV repair for AI secondary to BAV, aortic remodelling/ replacement includedHospital mortality
Postoperative AI > II
8-year overall survival
Freedom from TE/haemorrhage
8-year freedom from reoperation
8-year freedom from endocarditis
8-year freedom from AI > II
0%
0%
96.7%
100%
82.3%
90%
44.2%
Ashikhmina et al. (2010), USA [19]n = 108
Male 91%
Mean age 41 years
Retrospective case series of BAV repair. Patients with valve-sparing aortic root replacements were excludedIn-hospital mortality
10-year overall survival
10-year freedom from reoperation
10-year freedom from AVR
0%
87%
64%
49%
Badiu et al. (2010), Germany [20]n = 11
Male 100%
Mean age 37 ± 15.8 years
Aortic dissection 0%
Marfan 0%
Retrospective cohort study comparing BAV and TAV repair for AIOperative mortality
5-year overall survival
5-year freedom from reoperation
5-year freedom from AI
5-year freedom from TE events
0%
100%
100%
57.1%
95.9%
Bakhtiary et al. (2009), Germany [21]n = 14
Male 92.9%
Mean age 58 ± 5 years
AI > I 78.6%
Retrospective case series of patients undergoing the modified David procedure for incompetent BAV.Endocarditis
NE
Operative death
In-hospital death
Late death
0%
0%
0%
0%
0%
Boodhwani et al. (2009), Belgium [12]n = 90

Retrospective cohort study comparing aortic valve repair for AI in BAV and TAV, limited raw data presented for BAVComparison of freedom from AI recurrence between BAV and TAVP = 0.7
Boodhwani et al. (2010), Belgium [22]n = 122
Male 92%
Mean age 44 ± 11 years
AI > II 86.1%
Retrospective case series of patients undergoing surgery for BAV in association with either AI or dilatation of the proximal aortaIn-hospital mortality
Discharge AI < II
8-year overall survival
5-year freedom from AI > II
8-year freedom from AV reoperation
8-year freedom from AVR
8-year freedom from TE and bleeding
0%
93%
97 ± 2%
94 ± 3%
83 ± 5%
90 ± 5%
96 ± 2%
Casselman et al. (1999), USA [23]n = 94
Male 93%
Mean age 38 ± 10 years
Retrospective case series of aortic valve repair in patients with BAV and AIImmediate reoperation
Immediate postoperative AI > II
7-year freedom from AV reoperation
8.5%
2.1%
84%
Davierwala et al. (2003), Canada [24]n = 44
Mean age 39.3 ± 12.1 years
Male 93.2%
Preoperative CCF 13.6%
Retrospective cohort study of prospectively collected data comparing AV repair to AVR in patients with AI caused by BAV. Procedures on the aorta were includedOperative mortality
Late death
TE/Haemorrhagic events
5-year freedom from AI > I
5-year freedom from AV reoperation
0%
0%
0%
79 ± 8%
91 ± 5%
de Kerchove et al. (2009), Belgium [25]n = 54Retrospective cohort study comparing the impact of preoperative AI on outcome after aortic valve-sparing surgery, limited raw data presented for BAV5-year freedom from AI > II
8-year freedom from AV reoperation
98 ± 2%
91 ± 9%
Delius et al. (1998), USA [26]n = 16Retrospective cohort study of aortic valve repair in patients with either subvalvular or supravalvular aortic stenosis. Comparison made between BAV and TAV10-year overall survival
5-year freedom from reoperation
5-year freedom from AVR
100%
43%
43%
Doss et al. (2010), Germany [27]n = 66
(A) n = 49; (B) n = 17
Mean age 41.2 ± 12 years
(A) 58 years; (B) 39 yrs
Male 78.8%
(A) 82.3%; (B) 77.6%
AI > II 95.4%
(A) 82.3%; (B) 100%
Retrospective cohort study of (A) patch augmentation plus reduction aortoplasty vs (B) modified David procedure in patients with BAV and AI5-year mortality

5-year reoperation

5-year endocarditis

5-year conduction disturbance/thromboembolism/AI > I

(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 0%
(B) 0%
Fraser et al. (1994), USA [28]n = 72
Male 94.4%
Mean age 39 ± 11 years
Retrospective case series of AV repair for AI secondary to BAVOperative death
Late death
3-year freedom from AV reoperation
0%
0%
89.5%
Kin et al. (2003), Japan [29]n = 19
Male 98%
Mean age 42 ± 17 years
Retrospective case series of AV repair for AI secondary to BAV, some patients underwent concomitant procedures on the aortaHospital death
Early reoperation
Reoperation at follow-up
Late death
5-year overall survival
5-year freedom from AV reoperation
5.2%
5.2%
15.8%
5.2%
90 ± 7%
76 ± 23%
Lausberg et al. (2006), Germany [30]n = 89
n = 34; (B) n = 56
Retrospective cohort study comparing (A) AV repair alone to (B) AV repair plus aortic root remodelling in patients with AI, limited data for BAV aloneFreedom from significant AI in (A)
Freedom from reoperation in (A)
(A) 89.1%
(A) 100%
Mangini et al. (2010), Italy [31]n = 31
Mean age 49.9 ± 17.3 years
Male 83.9%
AI > I 96.8%
Prospective case series of patients undergoing repair of BAV for AI30-day operative mortality
Discharge AI > I
5-year freedom from reoperation
3.2%
3.2%
96.6%
McMullan et al. (2007), Australia [32]n = 21
Median age 12.6 years
Mean follow-up 36.4 months
Retrospective cohort study of tricuspidisation with cusp extension vs Ross procedure in children with AI or AS associated with BAVEarly reoperation
Endocarditis during follow-up
AVR during follow-up
AI > 2 during follow-up
9.5%
4.8%
9.5%
19.0%
Minakata et al. (2004), USA [14]n = 54Retrospective case series of AV repair for AI, limited raw data presented for BAVReoperation during index admission
Reoperation during follow-up
5-year reoperation rate
3.7%
11.1%
9%
Moidl et al. (1995), Austria [33]n = 14
Mean age 30.9 ± 12 years
Male 92.9%
Preoperative AI grade 3.5 ± 0.1
Retrospective case series of valve-sparing correction of AI and BAVReoperation during index admission21.4%
Nash et al. (2004), USA [34]n = 77
Mean age 38 ± 10 years
Male 93%
Retrospective case series of the echocardiographic factors that predict successful AV repair in patients with BAV and AIAVR during index admission
Reoperation during index admission
Perioperative death
Thromboembolism
Endocarditis
2.6%
3.9%
0%
0%
0%
Odim et al. (2005), USA [35]n = 39Retrospective case series of AV repair with pericardial leaflet extensionEarly mortality
2-year freedom from reoperation
2.6%
70–90%
Pretre et al. (2006), Switzerland [36]n = 12
Median age 18 years
Male 75%
Retrospective case series of AV repair with tricuspidization of the BAVPostoperative morbidity
AR > I
0%
8.3%
Rao et al. (2000), Canada [37]n = 23Retrospective case series of AV repair for multiple pathologies, limited raw data presented for BAVComposite endpoint of reoperation or AINo difference between BAV and TAV (P > 0.05)
Schafers et al. (2000), Germany [38]n = 16
Age range 35–73 years
Male 75%
Aortic dissection 6.3%
Retrospective case series of AV repair and root replacement in patients with BAV, AI and aortic dilatationIn-hospital mortality
AV reoperation during follow-up
Reoperation during follow-up
0%
0%
6.3%
Schafers et al. (2007), Germany [39]n = 173
Mean age 48 ± 16 years
Male 80.3%
Retrospective cohort study comparing (A) root remodelling, (B) AV repair + supracommissural aortic replacement, (C) AV repair aloneIn-hospital mortality


TE
Endocarditis
(A) 0%
(B) 2.6%
(C) 1.8%
0%
(A) 0%
(B) 0%
(C) 1.3%
Schafers et al. (2010), Germany [40]n = 153
Mean age 51 ± 12 years
Male 86.9%
Preoperative AI grade 2.6 ± 0.8
Aortic dissection 3.9%
Retrospective case series of valve-preserving root replacement for AI and BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > I
10-year freedom from reoperation
10-year freedom from AVR
TE events
Endocarditis
10-year freedom from AV complications
0.7%
91%
90%
95%
97%
2.6%
0%
91%
Veldtman et al. (2006), USA [41]n = 21
Mean age 45 ± 12 years
Male 61.9%
Marfan 9.5%
AI > I 19.0%
Retrospective case series of aortic root repair or replacement with preservation of the BAVPerioperative death
Late death
Reoperation
0%
4.8%
9.5%
Author, year and countryDemographicsaStudy designOutcomesaResultsa
Aicher et al. (2004), Germany [15]n = 60
Male 83.3%
Mean age 53 ± 12 years
Aortic dissection 6.7%
Retrospective cohort study comparing valve-sparing aortic root replacement in patients with TAV or BAVIn-hospital mortality
Postoperative AI grade
5-year overall survival
TE events/endocarditis during follow-up
Reoperation during follow-up
5-year mean AV gradient
5-year freedom from AI > II
5-year freedom from reoperation
0%
0.8 ± 0.7
82%
0%
1.7%
4.5 ± 2.3 mmHg
96%
98%
Aicher et al. (2007), Germany [16]n = 81
Male 85.2%
Aortic dissection 7.4%
AI > II 55.6%
Marfan 0%
Retrospective cohort study of aortic root remodelling in patients with BAV, AI and dilatation of the ascending aorta. Comparison made with patients with TAVHospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0%
94%
87%
97%
99%
Aicher et al. (2010), Germany [17]n = 205Retrospective cohort study comparing AV repair in patients with BAV and TAV, limited raw data presented for BAV5-year freedom from AI > II
10-year freedom from AI > II
86%
83%
Aicher et al. (2011), Germany [4]n = 316
Male 84.8%
Age range 3–79 years
Aortic dissection 8%
Severe AI 72.8%
Retrospective case series evaluating the effect of valve configuration on outcomes after repair of BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0.63%
92%
81%
81%
84%
Alsoufi et al. (2005), Canada [18]n = 71
Male 87.3%
Mean age 41.5 ± 13.2 years
Retrospective case series of prospectively collected data on AV repair for AI secondary to BAV, aortic remodelling/ replacement includedHospital mortality
Postoperative AI > II
8-year overall survival
Freedom from TE/haemorrhage
8-year freedom from reoperation
8-year freedom from endocarditis
8-year freedom from AI > II
0%
0%
96.7%
100%
82.3%
90%
44.2%
Ashikhmina et al. (2010), USA [19]n = 108
Male 91%
Mean age 41 years
Retrospective case series of BAV repair. Patients with valve-sparing aortic root replacements were excludedIn-hospital mortality
10-year overall survival
10-year freedom from reoperation
10-year freedom from AVR
0%
87%
64%
49%
Badiu et al. (2010), Germany [20]n = 11
Male 100%
Mean age 37 ± 15.8 years
Aortic dissection 0%
Marfan 0%
Retrospective cohort study comparing BAV and TAV repair for AIOperative mortality
5-year overall survival
5-year freedom from reoperation
5-year freedom from AI
5-year freedom from TE events
0%
100%
100%
57.1%
95.9%
Bakhtiary et al. (2009), Germany [21]n = 14
Male 92.9%
Mean age 58 ± 5 years
AI > I 78.6%
Retrospective case series of patients undergoing the modified David procedure for incompetent BAV.Endocarditis
NE
Operative death
In-hospital death
Late death
0%
0%
0%
0%
0%
Boodhwani et al. (2009), Belgium [12]n = 90

Retrospective cohort study comparing aortic valve repair for AI in BAV and TAV, limited raw data presented for BAVComparison of freedom from AI recurrence between BAV and TAVP = 0.7
Boodhwani et al. (2010), Belgium [22]n = 122
Male 92%
Mean age 44 ± 11 years
AI > II 86.1%
Retrospective case series of patients undergoing surgery for BAV in association with either AI or dilatation of the proximal aortaIn-hospital mortality
Discharge AI < II
8-year overall survival
5-year freedom from AI > II
8-year freedom from AV reoperation
8-year freedom from AVR
8-year freedom from TE and bleeding
0%
93%
97 ± 2%
94 ± 3%
83 ± 5%
90 ± 5%
96 ± 2%
Casselman et al. (1999), USA [23]n = 94
Male 93%
Mean age 38 ± 10 years
Retrospective case series of aortic valve repair in patients with BAV and AIImmediate reoperation
Immediate postoperative AI > II
7-year freedom from AV reoperation
8.5%
2.1%
84%
Davierwala et al. (2003), Canada [24]n = 44
Mean age 39.3 ± 12.1 years
Male 93.2%
Preoperative CCF 13.6%
Retrospective cohort study of prospectively collected data comparing AV repair to AVR in patients with AI caused by BAV. Procedures on the aorta were includedOperative mortality
Late death
TE/Haemorrhagic events
5-year freedom from AI > I
5-year freedom from AV reoperation
0%
0%
0%
79 ± 8%
91 ± 5%
de Kerchove et al. (2009), Belgium [25]n = 54Retrospective cohort study comparing the impact of preoperative AI on outcome after aortic valve-sparing surgery, limited raw data presented for BAV5-year freedom from AI > II
8-year freedom from AV reoperation
98 ± 2%
91 ± 9%
Delius et al. (1998), USA [26]n = 16Retrospective cohort study of aortic valve repair in patients with either subvalvular or supravalvular aortic stenosis. Comparison made between BAV and TAV10-year overall survival
5-year freedom from reoperation
5-year freedom from AVR
100%
43%
43%
Doss et al. (2010), Germany [27]n = 66
(A) n = 49; (B) n = 17
Mean age 41.2 ± 12 years
(A) 58 years; (B) 39 yrs
Male 78.8%
(A) 82.3%; (B) 77.6%
AI > II 95.4%
(A) 82.3%; (B) 100%
Retrospective cohort study of (A) patch augmentation plus reduction aortoplasty vs (B) modified David procedure in patients with BAV and AI5-year mortality

5-year reoperation

5-year endocarditis

5-year conduction disturbance/thromboembolism/AI > I

(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 0%
(B) 0%
Fraser et al. (1994), USA [28]n = 72
Male 94.4%
Mean age 39 ± 11 years
Retrospective case series of AV repair for AI secondary to BAVOperative death
Late death
3-year freedom from AV reoperation
0%
0%
89.5%
Kin et al. (2003), Japan [29]n = 19
Male 98%
Mean age 42 ± 17 years
Retrospective case series of AV repair for AI secondary to BAV, some patients underwent concomitant procedures on the aortaHospital death
Early reoperation
Reoperation at follow-up
Late death
5-year overall survival
5-year freedom from AV reoperation
5.2%
5.2%
15.8%
5.2%
90 ± 7%
76 ± 23%
Lausberg et al. (2006), Germany [30]n = 89
n = 34; (B) n = 56
Retrospective cohort study comparing (A) AV repair alone to (B) AV repair plus aortic root remodelling in patients with AI, limited data for BAV aloneFreedom from significant AI in (A)
Freedom from reoperation in (A)
(A) 89.1%
(A) 100%
Mangini et al. (2010), Italy [31]n = 31
Mean age 49.9 ± 17.3 years
Male 83.9%
AI > I 96.8%
Prospective case series of patients undergoing repair of BAV for AI30-day operative mortality
Discharge AI > I
5-year freedom from reoperation
3.2%
3.2%
96.6%
McMullan et al. (2007), Australia [32]n = 21
Median age 12.6 years
Mean follow-up 36.4 months
Retrospective cohort study of tricuspidisation with cusp extension vs Ross procedure in children with AI or AS associated with BAVEarly reoperation
Endocarditis during follow-up
AVR during follow-up
AI > 2 during follow-up
9.5%
4.8%
9.5%
19.0%
Minakata et al. (2004), USA [14]n = 54Retrospective case series of AV repair for AI, limited raw data presented for BAVReoperation during index admission
Reoperation during follow-up
5-year reoperation rate
3.7%
11.1%
9%
Moidl et al. (1995), Austria [33]n = 14
Mean age 30.9 ± 12 years
Male 92.9%
Preoperative AI grade 3.5 ± 0.1
Retrospective case series of valve-sparing correction of AI and BAVReoperation during index admission21.4%
Nash et al. (2004), USA [34]n = 77
Mean age 38 ± 10 years
Male 93%
Retrospective case series of the echocardiographic factors that predict successful AV repair in patients with BAV and AIAVR during index admission
Reoperation during index admission
Perioperative death
Thromboembolism
Endocarditis
2.6%
3.9%
0%
0%
0%
Odim et al. (2005), USA [35]n = 39Retrospective case series of AV repair with pericardial leaflet extensionEarly mortality
2-year freedom from reoperation
2.6%
70–90%
Pretre et al. (2006), Switzerland [36]n = 12
Median age 18 years
Male 75%
Retrospective case series of AV repair with tricuspidization of the BAVPostoperative morbidity
AR > I
0%
8.3%
Rao et al. (2000), Canada [37]n = 23Retrospective case series of AV repair for multiple pathologies, limited raw data presented for BAVComposite endpoint of reoperation or AINo difference between BAV and TAV (P > 0.05)
Schafers et al. (2000), Germany [38]n = 16
Age range 35–73 years
Male 75%
Aortic dissection 6.3%
Retrospective case series of AV repair and root replacement in patients with BAV, AI and aortic dilatationIn-hospital mortality
AV reoperation during follow-up
Reoperation during follow-up
0%
0%
6.3%
Schafers et al. (2007), Germany [39]n = 173
Mean age 48 ± 16 years
Male 80.3%
Retrospective cohort study comparing (A) root remodelling, (B) AV repair + supracommissural aortic replacement, (C) AV repair aloneIn-hospital mortality


TE
Endocarditis
(A) 0%
(B) 2.6%
(C) 1.8%
0%
(A) 0%
(B) 0%
(C) 1.3%
Schafers et al. (2010), Germany [40]n = 153
Mean age 51 ± 12 years
Male 86.9%
Preoperative AI grade 2.6 ± 0.8
Aortic dissection 3.9%
Retrospective case series of valve-preserving root replacement for AI and BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > I
10-year freedom from reoperation
10-year freedom from AVR
TE events
Endocarditis
10-year freedom from AV complications
0.7%
91%
90%
95%
97%
2.6%
0%
91%
Veldtman et al. (2006), USA [41]n = 21
Mean age 45 ± 12 years
Male 61.9%
Marfan 9.5%
AI > I 19.0%
Retrospective case series of aortic root repair or replacement with preservation of the BAVPerioperative death
Late death
Reoperation
0%
4.8%
9.5%

aWhere studies have included both patients with BAV and TAV repair, only demographics, outcomes and results of patients with BAV are reported.

AI: aortic insufficiency; AS: aortic stenosis; AV: aortic valve; AVR: aortic valve replacement; BAV: bicuspid aortic valve; CCF: congestive cardiac failure; TAV: tricuspid aortic valve; TE: thromboembolism.

Table 2:

Summary of included studies

Author, year and countryDemographicsaStudy designOutcomesaResultsa
Aicher et al. (2004), Germany [15]n = 60
Male 83.3%
Mean age 53 ± 12 years
Aortic dissection 6.7%
Retrospective cohort study comparing valve-sparing aortic root replacement in patients with TAV or BAVIn-hospital mortality
Postoperative AI grade
5-year overall survival
TE events/endocarditis during follow-up
Reoperation during follow-up
5-year mean AV gradient
5-year freedom from AI > II
5-year freedom from reoperation
0%
0.8 ± 0.7
82%
0%
1.7%
4.5 ± 2.3 mmHg
96%
98%
Aicher et al. (2007), Germany [16]n = 81
Male 85.2%
Aortic dissection 7.4%
AI > II 55.6%
Marfan 0%
Retrospective cohort study of aortic root remodelling in patients with BAV, AI and dilatation of the ascending aorta. Comparison made with patients with TAVHospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0%
94%
87%
97%
99%
Aicher et al. (2010), Germany [17]n = 205Retrospective cohort study comparing AV repair in patients with BAV and TAV, limited raw data presented for BAV5-year freedom from AI > II
10-year freedom from AI > II
86%
83%
Aicher et al. (2011), Germany [4]n = 316
Male 84.8%
Age range 3–79 years
Aortic dissection 8%
Severe AI 72.8%
Retrospective case series evaluating the effect of valve configuration on outcomes after repair of BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0.63%
92%
81%
81%
84%
Alsoufi et al. (2005), Canada [18]n = 71
Male 87.3%
Mean age 41.5 ± 13.2 years
Retrospective case series of prospectively collected data on AV repair for AI secondary to BAV, aortic remodelling/ replacement includedHospital mortality
Postoperative AI > II
8-year overall survival
Freedom from TE/haemorrhage
8-year freedom from reoperation
8-year freedom from endocarditis
8-year freedom from AI > II
0%
0%
96.7%
100%
82.3%
90%
44.2%
Ashikhmina et al. (2010), USA [19]n = 108
Male 91%
Mean age 41 years
Retrospective case series of BAV repair. Patients with valve-sparing aortic root replacements were excludedIn-hospital mortality
10-year overall survival
10-year freedom from reoperation
10-year freedom from AVR
0%
87%
64%
49%
Badiu et al. (2010), Germany [20]n = 11
Male 100%
Mean age 37 ± 15.8 years
Aortic dissection 0%
Marfan 0%
Retrospective cohort study comparing BAV and TAV repair for AIOperative mortality
5-year overall survival
5-year freedom from reoperation
5-year freedom from AI
5-year freedom from TE events
0%
100%
100%
57.1%
95.9%
Bakhtiary et al. (2009), Germany [21]n = 14
Male 92.9%
Mean age 58 ± 5 years
AI > I 78.6%
Retrospective case series of patients undergoing the modified David procedure for incompetent BAV.Endocarditis
NE
Operative death
In-hospital death
Late death
0%
0%
0%
0%
0%
Boodhwani et al. (2009), Belgium [12]n = 90

Retrospective cohort study comparing aortic valve repair for AI in BAV and TAV, limited raw data presented for BAVComparison of freedom from AI recurrence between BAV and TAVP = 0.7
Boodhwani et al. (2010), Belgium [22]n = 122
Male 92%
Mean age 44 ± 11 years
AI > II 86.1%
Retrospective case series of patients undergoing surgery for BAV in association with either AI or dilatation of the proximal aortaIn-hospital mortality
Discharge AI < II
8-year overall survival
5-year freedom from AI > II
8-year freedom from AV reoperation
8-year freedom from AVR
8-year freedom from TE and bleeding
0%
93%
97 ± 2%
94 ± 3%
83 ± 5%
90 ± 5%
96 ± 2%
Casselman et al. (1999), USA [23]n = 94
Male 93%
Mean age 38 ± 10 years
Retrospective case series of aortic valve repair in patients with BAV and AIImmediate reoperation
Immediate postoperative AI > II
7-year freedom from AV reoperation
8.5%
2.1%
84%
Davierwala et al. (2003), Canada [24]n = 44
Mean age 39.3 ± 12.1 years
Male 93.2%
Preoperative CCF 13.6%
Retrospective cohort study of prospectively collected data comparing AV repair to AVR in patients with AI caused by BAV. Procedures on the aorta were includedOperative mortality
Late death
TE/Haemorrhagic events
5-year freedom from AI > I
5-year freedom from AV reoperation
0%
0%
0%
79 ± 8%
91 ± 5%
de Kerchove et al. (2009), Belgium [25]n = 54Retrospective cohort study comparing the impact of preoperative AI on outcome after aortic valve-sparing surgery, limited raw data presented for BAV5-year freedom from AI > II
8-year freedom from AV reoperation
98 ± 2%
91 ± 9%
Delius et al. (1998), USA [26]n = 16Retrospective cohort study of aortic valve repair in patients with either subvalvular or supravalvular aortic stenosis. Comparison made between BAV and TAV10-year overall survival
5-year freedom from reoperation
5-year freedom from AVR
100%
43%
43%
Doss et al. (2010), Germany [27]n = 66
(A) n = 49; (B) n = 17
Mean age 41.2 ± 12 years
(A) 58 years; (B) 39 yrs
Male 78.8%
(A) 82.3%; (B) 77.6%
AI > II 95.4%
(A) 82.3%; (B) 100%
Retrospective cohort study of (A) patch augmentation plus reduction aortoplasty vs (B) modified David procedure in patients with BAV and AI5-year mortality

5-year reoperation

5-year endocarditis

5-year conduction disturbance/thromboembolism/AI > I

(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 0%
(B) 0%
Fraser et al. (1994), USA [28]n = 72
Male 94.4%
Mean age 39 ± 11 years
Retrospective case series of AV repair for AI secondary to BAVOperative death
Late death
3-year freedom from AV reoperation
0%
0%
89.5%
Kin et al. (2003), Japan [29]n = 19
Male 98%
Mean age 42 ± 17 years
Retrospective case series of AV repair for AI secondary to BAV, some patients underwent concomitant procedures on the aortaHospital death
Early reoperation
Reoperation at follow-up
Late death
5-year overall survival
5-year freedom from AV reoperation
5.2%
5.2%
15.8%
5.2%
90 ± 7%
76 ± 23%
Lausberg et al. (2006), Germany [30]n = 89
n = 34; (B) n = 56
Retrospective cohort study comparing (A) AV repair alone to (B) AV repair plus aortic root remodelling in patients with AI, limited data for BAV aloneFreedom from significant AI in (A)
Freedom from reoperation in (A)
(A) 89.1%
(A) 100%
Mangini et al. (2010), Italy [31]n = 31
Mean age 49.9 ± 17.3 years
Male 83.9%
AI > I 96.8%
Prospective case series of patients undergoing repair of BAV for AI30-day operative mortality
Discharge AI > I
5-year freedom from reoperation
3.2%
3.2%
96.6%
McMullan et al. (2007), Australia [32]n = 21
Median age 12.6 years
Mean follow-up 36.4 months
Retrospective cohort study of tricuspidisation with cusp extension vs Ross procedure in children with AI or AS associated with BAVEarly reoperation
Endocarditis during follow-up
AVR during follow-up
AI > 2 during follow-up
9.5%
4.8%
9.5%
19.0%
Minakata et al. (2004), USA [14]n = 54Retrospective case series of AV repair for AI, limited raw data presented for BAVReoperation during index admission
Reoperation during follow-up
5-year reoperation rate
3.7%
11.1%
9%
Moidl et al. (1995), Austria [33]n = 14
Mean age 30.9 ± 12 years
Male 92.9%
Preoperative AI grade 3.5 ± 0.1
Retrospective case series of valve-sparing correction of AI and BAVReoperation during index admission21.4%
Nash et al. (2004), USA [34]n = 77
Mean age 38 ± 10 years
Male 93%
Retrospective case series of the echocardiographic factors that predict successful AV repair in patients with BAV and AIAVR during index admission
Reoperation during index admission
Perioperative death
Thromboembolism
Endocarditis
2.6%
3.9%
0%
0%
0%
Odim et al. (2005), USA [35]n = 39Retrospective case series of AV repair with pericardial leaflet extensionEarly mortality
2-year freedom from reoperation
2.6%
70–90%
Pretre et al. (2006), Switzerland [36]n = 12
Median age 18 years
Male 75%
Retrospective case series of AV repair with tricuspidization of the BAVPostoperative morbidity
AR > I
0%
8.3%
Rao et al. (2000), Canada [37]n = 23Retrospective case series of AV repair for multiple pathologies, limited raw data presented for BAVComposite endpoint of reoperation or AINo difference between BAV and TAV (P > 0.05)
Schafers et al. (2000), Germany [38]n = 16
Age range 35–73 years
Male 75%
Aortic dissection 6.3%
Retrospective case series of AV repair and root replacement in patients with BAV, AI and aortic dilatationIn-hospital mortality
AV reoperation during follow-up
Reoperation during follow-up
0%
0%
6.3%
Schafers et al. (2007), Germany [39]n = 173
Mean age 48 ± 16 years
Male 80.3%
Retrospective cohort study comparing (A) root remodelling, (B) AV repair + supracommissural aortic replacement, (C) AV repair aloneIn-hospital mortality


TE
Endocarditis
(A) 0%
(B) 2.6%
(C) 1.8%
0%
(A) 0%
(B) 0%
(C) 1.3%
Schafers et al. (2010), Germany [40]n = 153
Mean age 51 ± 12 years
Male 86.9%
Preoperative AI grade 2.6 ± 0.8
Aortic dissection 3.9%
Retrospective case series of valve-preserving root replacement for AI and BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > I
10-year freedom from reoperation
10-year freedom from AVR
TE events
Endocarditis
10-year freedom from AV complications
0.7%
91%
90%
95%
97%
2.6%
0%
91%
Veldtman et al. (2006), USA [41]n = 21
Mean age 45 ± 12 years
Male 61.9%
Marfan 9.5%
AI > I 19.0%
Retrospective case series of aortic root repair or replacement with preservation of the BAVPerioperative death
Late death
Reoperation
0%
4.8%
9.5%
Author, year and countryDemographicsaStudy designOutcomesaResultsa
Aicher et al. (2004), Germany [15]n = 60
Male 83.3%
Mean age 53 ± 12 years
Aortic dissection 6.7%
Retrospective cohort study comparing valve-sparing aortic root replacement in patients with TAV or BAVIn-hospital mortality
Postoperative AI grade
5-year overall survival
TE events/endocarditis during follow-up
Reoperation during follow-up
5-year mean AV gradient
5-year freedom from AI > II
5-year freedom from reoperation
0%
0.8 ± 0.7
82%
0%
1.7%
4.5 ± 2.3 mmHg
96%
98%
Aicher et al. (2007), Germany [16]n = 81
Male 85.2%
Aortic dissection 7.4%
AI > II 55.6%
Marfan 0%
Retrospective cohort study of aortic root remodelling in patients with BAV, AI and dilatation of the ascending aorta. Comparison made with patients with TAVHospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0%
94%
87%
97%
99%
Aicher et al. (2010), Germany [17]n = 205Retrospective cohort study comparing AV repair in patients with BAV and TAV, limited raw data presented for BAV5-year freedom from AI > II
10-year freedom from AI > II
86%
83%
Aicher et al. (2011), Germany [4]n = 316
Male 84.8%
Age range 3–79 years
Aortic dissection 8%
Severe AI 72.8%
Retrospective case series evaluating the effect of valve configuration on outcomes after repair of BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > II
10-year freedom from reoperation
10-year freedom from AVR
0.63%
92%
81%
81%
84%
Alsoufi et al. (2005), Canada [18]n = 71
Male 87.3%
Mean age 41.5 ± 13.2 years
Retrospective case series of prospectively collected data on AV repair for AI secondary to BAV, aortic remodelling/ replacement includedHospital mortality
Postoperative AI > II
8-year overall survival
Freedom from TE/haemorrhage
8-year freedom from reoperation
8-year freedom from endocarditis
8-year freedom from AI > II
0%
0%
96.7%
100%
82.3%
90%
44.2%
Ashikhmina et al. (2010), USA [19]n = 108
Male 91%
Mean age 41 years
Retrospective case series of BAV repair. Patients with valve-sparing aortic root replacements were excludedIn-hospital mortality
10-year overall survival
10-year freedom from reoperation
10-year freedom from AVR
0%
87%
64%
49%
Badiu et al. (2010), Germany [20]n = 11
Male 100%
Mean age 37 ± 15.8 years
Aortic dissection 0%
Marfan 0%
Retrospective cohort study comparing BAV and TAV repair for AIOperative mortality
5-year overall survival
5-year freedom from reoperation
5-year freedom from AI
5-year freedom from TE events
0%
100%
100%
57.1%
95.9%
Bakhtiary et al. (2009), Germany [21]n = 14
Male 92.9%
Mean age 58 ± 5 years
AI > I 78.6%
Retrospective case series of patients undergoing the modified David procedure for incompetent BAV.Endocarditis
NE
Operative death
In-hospital death
Late death
0%
0%
0%
0%
0%
Boodhwani et al. (2009), Belgium [12]n = 90

Retrospective cohort study comparing aortic valve repair for AI in BAV and TAV, limited raw data presented for BAVComparison of freedom from AI recurrence between BAV and TAVP = 0.7
Boodhwani et al. (2010), Belgium [22]n = 122
Male 92%
Mean age 44 ± 11 years
AI > II 86.1%
Retrospective case series of patients undergoing surgery for BAV in association with either AI or dilatation of the proximal aortaIn-hospital mortality
Discharge AI < II
8-year overall survival
5-year freedom from AI > II
8-year freedom from AV reoperation
8-year freedom from AVR
8-year freedom from TE and bleeding
0%
93%
97 ± 2%
94 ± 3%
83 ± 5%
90 ± 5%
96 ± 2%
Casselman et al. (1999), USA [23]n = 94
Male 93%
Mean age 38 ± 10 years
Retrospective case series of aortic valve repair in patients with BAV and AIImmediate reoperation
Immediate postoperative AI > II
7-year freedom from AV reoperation
8.5%
2.1%
84%
Davierwala et al. (2003), Canada [24]n = 44
Mean age 39.3 ± 12.1 years
Male 93.2%
Preoperative CCF 13.6%
Retrospective cohort study of prospectively collected data comparing AV repair to AVR in patients with AI caused by BAV. Procedures on the aorta were includedOperative mortality
Late death
TE/Haemorrhagic events
5-year freedom from AI > I
5-year freedom from AV reoperation
0%
0%
0%
79 ± 8%
91 ± 5%
de Kerchove et al. (2009), Belgium [25]n = 54Retrospective cohort study comparing the impact of preoperative AI on outcome after aortic valve-sparing surgery, limited raw data presented for BAV5-year freedom from AI > II
8-year freedom from AV reoperation
98 ± 2%
91 ± 9%
Delius et al. (1998), USA [26]n = 16Retrospective cohort study of aortic valve repair in patients with either subvalvular or supravalvular aortic stenosis. Comparison made between BAV and TAV10-year overall survival
5-year freedom from reoperation
5-year freedom from AVR
100%
43%
43%
Doss et al. (2010), Germany [27]n = 66
(A) n = 49; (B) n = 17
Mean age 41.2 ± 12 years
(A) 58 years; (B) 39 yrs
Male 78.8%
(A) 82.3%; (B) 77.6%
AI > II 95.4%
(A) 82.3%; (B) 100%
Retrospective cohort study of (A) patch augmentation plus reduction aortoplasty vs (B) modified David procedure in patients with BAV and AI5-year mortality

5-year reoperation

5-year endocarditis

5-year conduction disturbance/thromboembolism/AI > I

(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 2.0%
(B) 0%
(A) 0%
(B) 0%
Fraser et al. (1994), USA [28]n = 72
Male 94.4%
Mean age 39 ± 11 years
Retrospective case series of AV repair for AI secondary to BAVOperative death
Late death
3-year freedom from AV reoperation
0%
0%
89.5%
Kin et al. (2003), Japan [29]n = 19
Male 98%
Mean age 42 ± 17 years
Retrospective case series of AV repair for AI secondary to BAV, some patients underwent concomitant procedures on the aortaHospital death
Early reoperation
Reoperation at follow-up
Late death
5-year overall survival
5-year freedom from AV reoperation
5.2%
5.2%
15.8%
5.2%
90 ± 7%
76 ± 23%
Lausberg et al. (2006), Germany [30]n = 89
n = 34; (B) n = 56
Retrospective cohort study comparing (A) AV repair alone to (B) AV repair plus aortic root remodelling in patients with AI, limited data for BAV aloneFreedom from significant AI in (A)
Freedom from reoperation in (A)
(A) 89.1%
(A) 100%
Mangini et al. (2010), Italy [31]n = 31
Mean age 49.9 ± 17.3 years
Male 83.9%
AI > I 96.8%
Prospective case series of patients undergoing repair of BAV for AI30-day operative mortality
Discharge AI > I
5-year freedom from reoperation
3.2%
3.2%
96.6%
McMullan et al. (2007), Australia [32]n = 21
Median age 12.6 years
Mean follow-up 36.4 months
Retrospective cohort study of tricuspidisation with cusp extension vs Ross procedure in children with AI or AS associated with BAVEarly reoperation
Endocarditis during follow-up
AVR during follow-up
AI > 2 during follow-up
9.5%
4.8%
9.5%
19.0%
Minakata et al. (2004), USA [14]n = 54Retrospective case series of AV repair for AI, limited raw data presented for BAVReoperation during index admission
Reoperation during follow-up
5-year reoperation rate
3.7%
11.1%
9%
Moidl et al. (1995), Austria [33]n = 14
Mean age 30.9 ± 12 years
Male 92.9%
Preoperative AI grade 3.5 ± 0.1
Retrospective case series of valve-sparing correction of AI and BAVReoperation during index admission21.4%
Nash et al. (2004), USA [34]n = 77
Mean age 38 ± 10 years
Male 93%
Retrospective case series of the echocardiographic factors that predict successful AV repair in patients with BAV and AIAVR during index admission
Reoperation during index admission
Perioperative death
Thromboembolism
Endocarditis
2.6%
3.9%
0%
0%
0%
Odim et al. (2005), USA [35]n = 39Retrospective case series of AV repair with pericardial leaflet extensionEarly mortality
2-year freedom from reoperation
2.6%
70–90%
Pretre et al. (2006), Switzerland [36]n = 12
Median age 18 years
Male 75%
Retrospective case series of AV repair with tricuspidization of the BAVPostoperative morbidity
AR > I
0%
8.3%
Rao et al. (2000), Canada [37]n = 23Retrospective case series of AV repair for multiple pathologies, limited raw data presented for BAVComposite endpoint of reoperation or AINo difference between BAV and TAV (P > 0.05)
Schafers et al. (2000), Germany [38]n = 16
Age range 35–73 years
Male 75%
Aortic dissection 6.3%
Retrospective case series of AV repair and root replacement in patients with BAV, AI and aortic dilatationIn-hospital mortality
AV reoperation during follow-up
Reoperation during follow-up
0%
0%
6.3%
Schafers et al. (2007), Germany [39]n = 173
Mean age 48 ± 16 years
Male 80.3%
Retrospective cohort study comparing (A) root remodelling, (B) AV repair + supracommissural aortic replacement, (C) AV repair aloneIn-hospital mortality


TE
Endocarditis
(A) 0%
(B) 2.6%
(C) 1.8%
0%
(A) 0%
(B) 0%
(C) 1.3%
Schafers et al. (2010), Germany [40]n = 153
Mean age 51 ± 12 years
Male 86.9%
Preoperative AI grade 2.6 ± 0.8
Aortic dissection 3.9%
Retrospective case series of valve-preserving root replacement for AI and BAVIn-hospital mortality
10-year overall survival
10-year freedom from AI > I
10-year freedom from reoperation
10-year freedom from AVR
TE events
Endocarditis
10-year freedom from AV complications
0.7%
91%
90%
95%
97%
2.6%
0%
91%
Veldtman et al. (2006), USA [41]n = 21
Mean age 45 ± 12 years
Male 61.9%
Marfan 9.5%
AI > I 19.0%
Retrospective case series of aortic root repair or replacement with preservation of the BAVPerioperative death
Late death
Reoperation
0%
4.8%
9.5%

aWhere studies have included both patients with BAV and TAV repair, only demographics, outcomes and results of patients with BAV are reported.

AI: aortic insufficiency; AS: aortic stenosis; AV: aortic valve; AVR: aortic valve replacement; BAV: bicuspid aortic valve; CCF: congestive cardiac failure; TAV: tricuspid aortic valve; TE: thromboembolism.

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