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Case of the month: Severe AI; 5 2011




           Rare cause of severe aortic
                  insufficiency

                                Martin Penicka
                                    ECHOLAB
               Cardiovascular Center, OLV Ziekenhuis, Aalst, Belgium
                           martin.penicka@olvz-aalst.be




dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011



                            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




dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011



   Echo-Doppler examination: 3D echo jet
                                       3D reconstruction of vena contracta
      3D color Doppler shows
                                       confirmed severe AI with an effective
      severe AI (grade 3+/4)
                                       regurgitant orifice of 34 mm²




                                                                ERO 34 mm²




dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011



   Echo-Doppler examination: 3D echo jet
                                       3D reconstruction of vena contracta
      3D color Doppler shows
                                       confirmed severe AI with an effective
      severe AI (grade 3+/4)
                                       regurgitant orifice of 34 mm²




                                                                ERO 34 mm²




dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011


 Etiology of AI: guadricuspid aortic valve




dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011


 Etiology of AI: guadricuspid aortic valve




dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011


                      Quadricuspid aortic valve
• 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 1973
                                                  Roberts, Am J Cardiol 2007
                                                  Timperly, Clinic Cardiol 2002
                                                  Tutarel, J Heart Valve Dis 2004
dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011



     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


dinsdag 20 september 2011
Case of the month: Severe AI; 5 2011


     Guidelines: indication for surgery
 A) Severe aortic regurgitation
 1. Symptomatic patients                                    IB
 2. Asymptomatic patients (validation using exercise test)
        a) LVEF ≤ 50%                                       IB
        b) LVEDd > 70 mm or
           LVESd > 50 mm (LVESd index > 25 mm/m²)           IIaC
 3. 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

                                             ESC guidelines, Eur Heart J 2007
                                             ACC/AHA guidelines, Circulation 2008
dinsdag 20 september 2011

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Rare cause of severe aortic insufficiency

  • 1. Case of the month: Severe AI; 5 2011 Rare cause of severe aortic insufficiency Martin Penicka ECHOLAB Cardiovascular Center, OLV Ziekenhuis, Aalst, Belgium martin.penicka@olvz-aalst.be dinsdag 20 september 2011
  • 2. Case of the month: Severe AI; 5 2011 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 dinsdag 20 september 2011
  • 3. Case of the month: Severe AI; 5 2011 Echo-Doppler examination: 3D echo jet 3D reconstruction of vena contracta 3D color Doppler shows confirmed severe AI with an effective severe AI (grade 3+/4) regurgitant orifice of 34 mm² ERO 34 mm² dinsdag 20 september 2011
  • 4. Case of the month: Severe AI; 5 2011 Echo-Doppler examination: 3D echo jet 3D reconstruction of vena contracta 3D color Doppler shows confirmed severe AI with an effective severe AI (grade 3+/4) regurgitant orifice of 34 mm² ERO 34 mm² dinsdag 20 september 2011
  • 5. Case of the month: Severe AI; 5 2011 Etiology of AI: guadricuspid aortic valve dinsdag 20 september 2011
  • 6. Case of the month: Severe AI; 5 2011 Etiology of AI: guadricuspid aortic valve dinsdag 20 september 2011
  • 7. Case of the month: Severe AI; 5 2011 Quadricuspid aortic valve • 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 1973 Roberts, Am J Cardiol 2007 Timperly, Clinic Cardiol 2002 Tutarel, J Heart Valve Dis 2004 dinsdag 20 september 2011
  • 8. Case of the month: Severe AI; 5 2011 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 dinsdag 20 september 2011
  • 9. Case of the month: Severe AI; 5 2011 Guidelines: indication for surgery A) Severe aortic regurgitation 1. Symptomatic patients IB 2. Asymptomatic patients (validation using exercise test) a) LVEF ≤ 50% IB b) LVEDd > 70 mm or LVESd > 50 mm (LVESd index > 25 mm/m²) IIaC 3. 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 ESC guidelines, Eur Heart J 2007 ACC/AHA guidelines, Circulation 2008 dinsdag 20 september 2011