rare cause of severe aortic insufficiency

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Rare cause of severe aortic insufficiency Martin Penicka ECHOLAB Cardiovascular Center, OLV Ziekenhuis, Aalst, Belgium [email protected] Case of the month: Severe AI; 5 2011 dinsdag 20 september 2011

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Martin PenickaECHOLABCardiovascular Center, OLV Ziekenhuis, Aalst, Belgium

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Page 1: Rare cause of severe aortic insufficiency

Rare cause of severe aortic insufficiency

Martin PenickaECHOLAB

Cardiovascular Center, OLV Ziekenhuis, Aalst, [email protected]

Case of the month: Severe AI; 5 2011

dinsdag 20 september 2011

Page 2: Rare cause of severe aortic insufficiency

CASE description

• 51 years old sportsman referred for AI• 1995 murmur• 1999 moderate AI• 2006 severe AI

• Clinical examination: length 1.86 m, weight 98 kg

• No symptoms (validated with exercise test, regular sport)

• No coronary artery disease at coronary angiography

• No significant commorbidities

Case of the month: Severe AI; 5 2011

dinsdag 20 september 2011

Page 3: Rare cause of severe aortic insufficiency

Echo-Doppler examination: 3D echo jet3D color Doppler showssevere AI (grade 3+/4)

Case of the month: Severe AI; 5 2011

3D reconstruction of vena contractaconfirmed severe AI with an effective regurgitant orifice of 34 mm²

ERO 34 mm²

dinsdag 20 september 2011

Page 4: Rare cause of severe aortic insufficiency

Echo-Doppler examination: 3D echo jet3D color Doppler showssevere AI (grade 3+/4)

Case of the month: Severe AI; 5 2011

3D reconstruction of vena contractaconfirmed severe AI with an effective regurgitant orifice of 34 mm²

ERO 34 mm²

dinsdag 20 september 2011

Page 5: Rare cause of severe aortic insufficiency

Etiology of AI: guadricuspid aortic valveCase of the month: Severe AI; 5 2011

dinsdag 20 september 2011

Page 6: Rare cause of severe aortic insufficiency

Etiology of AI: guadricuspid aortic valveCase of the month: Severe AI; 5 2011

dinsdag 20 september 2011

Page 7: Rare cause of severe aortic insufficiency

Quadricuspid aortic valveCase of the month: Severe AI; 5 2011

• The aortic valve may be unicuspid, bicuspid, tricuspid, or quadricuspid

• The quadricuspid aortic valve (QAV) is the rarest from congenital abnormalities of the aortic valve cusps

• Incidence at autopsy 0.008%• Incidence during AVR 1%

• First description in 1862, since then only 190 cases in the literature

• If dysfunctional the dysfunction is usually pure AI• Aortic stenosis with a QAV is exceedingly rare

Hurwitz, Am J Cardiol 1973Roberts, Am J Cardiol 2007Timperly, Clinic Cardiol 2002 Tutarel, J Heart Valve Dis 2004

dinsdag 20 september 2011

Page 8: Rare cause of severe aortic insufficiency

Is this patient with severe AI indicated for aortic valve surgery ?

Summary of the case• No exercise limitation or symptoms (validation with exercise test)• No coronary artery disease or significant disease of other valves• Normal LVEF (LVEF = 65%)• No LV dilatation (LVEDd = 48 mm, LVESd = 24 mm)• No aortic root (36 mm) or aorta ascedens (37 mm) dilatation

At this moment, no indication for aortic valve surgery (see next slide)

Follow-up (Cardiology, Echo, Exercise test) recommendations:•1 x year if EF, LV and aortic diameters are stable

•1 x 6 months in case of deterioration or when close to the threshold for surgery

Case of the month: Severe AI; 5 2011

dinsdag 20 september 2011

Page 9: Rare cause of severe aortic insufficiency

Guidelines: indication for surgeryA) Severe aortic regurgitation1. Symptomatic patients IB2. Asymptomatic patients (validation using exercise test) a) LVEF ≤ 50% IB b) LVEDd > 70 mm or LVESd > 50 mm (LVESd index > 25 mm/m²) IIaC3. Cardiac surgery for other reason (CABG, other valve, asc aorta) IC

B) Whatever the severity of AI in pts with significant aortic root / aorta disease: Marfan’s syndrome: Ao ≥ 45 mm (≥ 23 cm/m²) IC Bicuspid AV: Ao ≥ 50 mm (≥ 25 cm/m²) IIaC Other patients: Ao ≥ 55 mm (≥ 28 cm/m²) IIaC

C) AVR may be considered in patients with moderate AI while undergoing CABG or other valve surgery IIbC

Case of the month: Severe AI; 5 2011

ESC guidelines, Eur Heart J 2007ACC/AHA guidelines, Circulation 2008

dinsdag 20 september 2011