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 DR Smruti prakash Sahu

 4th yr DNB Resident
 G. Kuppuswamy Naidu Memorial Hospital
 Prevelance 1-2 %
 Male : Female – 3:1
 Complex disease – leads to
 Severe valvular dysfunction
 Aortopathy and aortic dissection
 Infective Endocarditis
 Unequally sized cusps
 Larger leaflet is the "conjoined"
 Presence of a central ridge
 Usually in the center of the
conjoined leaflet
 Smooth cusp margins
 Excludes tricuspid valves which
fused due to inflammatory
processes (eg, rheumatic fever)
Irregularity and scarring within
the raphe
 Sporadic
 Familial  Autosomal dominant
 Turner’s syndrome- 30% BAV inheritance with variable
 Other congenital Cardiac
malformation  Shones syndrome

 Coarcation Of Aorta- 50 %  Gene association

 NOTCH 1 Gene
 AC2A gene
 Hypoplastic Left Heart Syndrome
 William Syndrome-10%
 Surgical -Sievers
Epidemiology and Natural History

 Thoracic aortic dilatation- 40%

 Bicuspid Aortic aneurysm
 Aortic root
 Tubular ascending aorta-60-70%
 Proximal aortic arch
 20-30 % BAV –develop aneurysmal enlargement of Aorta over 9-25
 84% ultimately develop aneurysm
 5% risk of developing aortic dissection
 Approximately 9% to 15% of first-order family members also have
BAV disease
 Conditions associated
 Patients with BAV disease presented at a smaller aortic diameter
(4.6 vs 4.9 cm for patients without BAV). Also, their aortas grew more
rapidly than those of patients with TAV: 1.9 mm/year compared with
1.3 mm/year(Davies and colleagues).
 Overall growth rates for the bicuspid aorta, on the order of 0.4 to
0.6mm/year, with no differences noted according to specific
pattern of leaflet fusion.
Natural history
 Survival of asymptomatic patients with BAV

Identical to expected survival of matched population Michelena et al

Medical events

Michelena et al
Surgical events
Michelena et al
The largest study (n=642) in symptomatic/asymptomatic BAV pts:
10-year 96% survival
Michelena et al
Similar to normal population
Independent predictors of primary cardiac events:
Age ≥30 yrs Michelena et al
Moderate/Severe AS
Moderate/Severe AR
BAV- Aortopathy
 Prevalence of tubular ascending aorta dilation increases with age:
 <30 yrs 30-39yrs 40-49yrs 50-60yrs >60yrs
 56% 74% 85% 91% 88%

 Genetic evidence
 Aortopathy prevalent in 1st degree relatives of BAV pts
 Aortic dimension differences in BAV of controls in spite of
haemodynamic variable adjustments
 Aortic dilation in BAVs (incl. children) without AS/AR
 Progressive aortic dilation with or without AVR
 Haemodynamic evidence
 Recent MRI studies -
 Abnormal transvalvular-flow patterns despite apparent normally functioning
 Regional increases in wall-shear stress
 Sensitivity 92%, Specificity 96%
 Findings:
 Raphe
 Systolic doming & eccentric
closure line(LAX)
 Evaluate in systole; raphe may
appear trileaflet

 Surveillance
 Class I [AHA]
 Annual aortic imaging if
 Aortic dilation >4.5 cm
 Rapid rate of change in aortic diameter
 Family history of dissection

 Screening
 First-degree family members of pts with BAV

 Scarce evidence of efficacy

 No evidence for altering natural history in BAV
 AHA recommendation
 Dilated aortic root/ascending aorta:
 ACEI/ARB & BB to reduce SBP to the lowest tolerated levels
 AS/AR:
 Treatment of systemic hypertension

 Repair of BAV
 When to consider:
 Regurgitant valves
 Pliable leaflets
 Minimal fibrosis/calcification
 No more than mild cusp thickening
 Minor fenestrations
 Effective height: Height to which central free margin of cusp rises
over the aortic insertion line of cusp
 N = 9-10mm
 Prolapse: <6-7mm
 Restore cusp integrity- Closing tears/perforations by direct suture or
autologous pericardial patching
 Line-up discloses presence of tissue redundancy

Sufficient tissue; closure of cleft

Excess tissue; triangular resection, plication

 Deficient tissue
 Overcorrecting free margin of the conjoint cusp to a length shorter
than free margin of reference cusp
 Increases systolic doming
Commissural repair

Resuspension of detached commissure Misalignment & splaying

-pledgeted sutures & plication -pledgeted oblique Cabrol-like
BAV repair with dilated annulus

Bavaria et al. STS 2013. Ann Thor Surg.

Aicher et al. CirculaGon 2011;123:178-185
Failure of BAV repair in dilated annulus irrespective of
SCA or Remodelling style root

Etz et al. Ann Thorac Surg


Etz et al. Ann Thorac

Surg 2007;84:1186-94

Survival similar to age/sex matched controls at 12 yrs