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![BAV - Management
AHA 2014: Surgical Intervention
Class 1
Diameter of the aortic sinuses or ascending aorta is greater than 5.5 cm [B]
Class 2a
Diameter of the aortic sinuses or ascending aorta is greater than 5.0 cm and a
risk factor for dissection is present (family history of aortic dissection or if the
rate of increase in diameter is 0.5 cm per year) [C]
Replacement of the ascending aorta is reasonable in patients with a bicuspid
aortic valve who are undergoing aortic valve surgery because of severe AS or
AR if the diameter of the ascending aorta is greater than 4.5 cm. [C]](https://image.slidesharecdn.com/bicuspidaorticvalve-180111215148/75/Bicuspid-aortic-valve-28-2048.jpg)
Uploaded byJyotindra Singh
Bicuspid aortic valve
This document discusses bicuspid aortic valve (BAV), including its pathogenesis, diagnosis, natural history, and management. Key points include: - BAV has a genetic component and is associated with accelerated aortic valve disease and aortopathy. - Diagnosis is typically by echocardiogram which can identify the raphe and systolic doming. MRI/CT may be needed if unclear on echo. - Complications include aortic stenosis, aortic regurgitation, endocarditis, and aortic aneurysm/dissection. Progression is often faster than tricuspid valves. - Management involves surveillance of the aorta size and valve function. Surgery is recommended
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Bicuspid aortic valve
- 1. BICUSPID AORTIC VALVE ByJyotindra Singh adult cardiothoracic
- 3. BAV - Pathogenesis Genetics •Autosomal dominant, variable penetrance • 9% prevalence in first-degree relatives • 24% if >1 person affected • Causal genes largely unknown • NOTCH1 (9q34.3)
- 13. BAV - Diagnosis TransthoracicEcho • Sensitivity 92%, Specificity 96% • Accuracy inverse to calcification • Findings: • Raphe • Systolic doming & eccentric closure line (LAX) • Evaluate in systole; raphe may appear trileaflet Diastole Systole Raphe Doming
- 17. BAV - NaturalHistory • Valvular dysfunction - AS, AR, endocarditis • Aortopathy
- 18. BAV - NaturalHistory The largest study (n=642) in symptomatic/asymptomatic BAV pts: 10-year 96% survival Similar to normal population Tzemos et al. JAMA;2008:300;11:1317-1325.
- 19. BAV - AS Surgicalseries of 932 resected aortic valves for AS: • 49% had BAV • Age at intervention • BAV: 67±11 • Tricuspid: 74±8 Roberts et al. CirculaGon. 2005;111:920-925.
- 20. BAV - AS Diseaseprogression • Similar degenerative changes as seen in tricuspid valves • Exacerbated by BAV folding/creasing/turbulent flow • Results in accelerated disease progression • Most common reason for valve replacement Roberts et al. CirculaGon. 2005;111:920-925.
- 21. BAV - AS Influenceof valve morphology • 310 patients with BAV • 202 (65%): R-L fusion, 108 (35%): R-N fusion • Follow-up 14±7 yrs • 49 (16%) had interventions • Freedom from intervention: 64% R-N vs. 91% R-L • AS more progressive in R-N pts Fernandes et al. JACC 2007:2211-4.
- 22. BAV - AR Lessfrequent occurrence than AS • Surgical series of 542 pts who underwent AVR (1991-1996): • 13% (pure AR) vs 75% (pure AS) • Mean age: • 46 yrs (AR) vs 65 yrs (AS) Low intervention rates • Olmsted county (Michelena): 47% had some degree of AR at baseline; 3% had intervention for severe AR • Toronto study (Tzemos): 21% had moderate/severe AR at baseline; 6% had intervention for symptomatic AR Mechanisms • Valve prolapse • Aortic root/annular dilatation • Endocarditis .)
- 23. BAV - Endocarditis Recentstudies suggest low incidence: • Olmsted county: • 2% per year incidence • Toronto study: • 0.3% per year incidence AHA guidelines no longer suggest bacterial endocarditis prophylaxis, except if prior history of endocarditis. Tzemos et al. JAMA;2008:300;11:1317-1325 Michelena et al CirculaGon. 2008;117:2776-2784.)
- 25. BAV - Aortopathy Patternsof Aortic Dilation Type 1: Dilation of tubular ascending aorta primarily along convexity with mild-moderate root dilation. Most common; associated with R-L cusp fusion & AS Type 2: Isolated tubular ascending aorta dilation, which may extend into the arch, with relative sparing of aortic root. Associated with R-N cusp fusion. Type 3: Root phenotype - isolated root dilation, normal tubular/arch dimensions. Rarer; associated with younger age at diagnosis; genetic.
- 26. BAV - Aortopathy Pathophysiology Haemodynamicevidence Recent MRI studies - • Abnormal transvalvular-flow patterns despite apparent normally functioning BAVs • Regional increases in wall-shear stress However, valve morphology did not predict events in population studies
- 27. BAV - Aortopathy NaturalHistory Ascending aortic aneurysm: • Rate of growth higher in persons with BAV c/f tricuspid valve • Tricuspid: 0.16 mm/yr uniformly over 6 decades • BAV: 0.20 - 1.9 mm/yr Accelerating rate of aortic dimension increase with increasing age
- 28. BAV - Management AHA2014: Surgical Intervention Class 1 Diameter of the aortic sinuses or ascending aorta is greater than 5.5 cm [B] Class 2a Diameter of the aortic sinuses or ascending aorta is greater than 5.0 cm and a risk factor for dissection is present (family history of aortic dissection or if the rate of increase in diameter is 0.5 cm per year) [C] Replacement of the ascending aorta is reasonable in patients with a bicuspid aortic valve who are undergoing aortic valve surgery because of severe AS or AR if the diameter of the ascending aorta is greater than 4.5 cm. [C]
- 29. Bicuspid Aortic Valveand Aortopathy: Diagnosis and Follow-Up Recommendations COR LOE An initial TTE is indicated in patients with a known bicuspid aortic valve to evaluate valve morphology, to measure the severity of AS and AR, and to assess the shape and diameter of the aortic sinuses and ascending aorta for prediction of clinical outcome and to determine timing of intervention I B Aortic magnetic resonance angiography or CT angiography is indicated in patients with a bicuspid aortic valve when morphology of the aortic sinuses, sinotubular junction, or ascending aorta cannot be assessed accurately or fully by echocardiography I C
- 30. Bicuspid Aortic Valveand Aortopathy: Diagnosis and Follow-Up Recommendations COR LOE Serial evaluation of the size and morphology of the aortic sinuses and ascending aorta by echocardiography, CMR, or CT angiography is recommended in patients with a bicuspid aortic valve and an aortic diameter greater than 4.0 cm, with the examination interval determined by the degree and rate of progression of aortic dilation and by family history. In patients with an aortic diameter greater than 4.5 cm, this evaluation should be performed annually I C
- 31. Bicuspid Aortic Valveand Aortopathy: Intervention Recommendations COR LOE Operative intervention to repair the aortic sinuses or replace the ascending aorta is indicated in patients with a bicuspid aortic valve if the diameter of the aortic sinuses or ascending aorta is greater than 5.5 cm I B Operative intervention to repair the aortic sinuses or replace the ascending aorta is reasonable in patients with bicuspid aortic valves if the diameter of the aortic sinuses or ascending aorta is greater than 5.0 cm and a risk factor for dissection is present (family history of aortic dissection or if the rate of increase in diameter is ≥0.5 cm per year) IIa C
- 32. Bicuspid Aortic Valveand Aortopathy: Intervention Recommendations COR LOE Replacement of the ascending aorta is reasonable in patients with a bicuspid aortic valve who are undergoing aortic valve surgery because of severe AS or AR (Sections 3.4 and 4.4) if the diameter of the ascending aorta is greater than 4.5 cm IIa C
- 33. BAV - Management Medical •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 Current trial: Atenolol and Telmisartan in BAV aortopathy - RCT
- 34. BAV - Management Repairof BAV • Attractive given young cohort of BAV pts with AI • No RCTs of repair vs replacement • When to consider: • Regurgitant valves • Pliable leaflets • Minimal fibrosis/calcification • No more than mild cusp thickening • Minor fenestrations
- 35. BAV - Management Repairof BAV - Techniques 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
- 36. BAV - Management Repairof BAV - Techniques Deficient tissue • Overcorrecting free margin of the conjoint cusp to a length shorter than free margin of reference cusp • Increases systolic doming
- 37. BAV - Management Repairof BAV - Techniques Commissural repair Resuspension of detached commissure Misalignment & splaying -pledgeted sutures & plication -pledgeted oblique Cabrol-like stitch
- 39. BAV - Management Bentall's Survivalsimilar to age/sex matched controls at 12 yrs Etz et al. Ann Thorac Surg 2007;84:1186-94
- 63. Much more tocome Are we all still awake?
- 64. "The key overall conceptualquestion in front of the cardioaortic surgical community is: Can we repair virtually every pure BAV AI presentation as we presently perform in the mitral valve repair arena?
- 65. Factors – Poorrepair Outcomes • excessive calcification of the leaflets, including the commissures or raphe; • severely enlarged annular diameters without robust subannular stabilization; • leaflet surface areas that are not adequate to provide excellent cusp coaptation (and therefore would require leaflet augmentation); • and reimplantation with very extensive cusp repair.