MioAtlas
G1: Left-sided valves

Aortic stenosis in bicuspid aortic valve

Aortic valve stenosis developing on a congenital bicuspid aortic valve due to chronic abnormal hemodynamic shear stress precipitating premature calcification and stiffness (typically 1–2 decades earlier than in the tricuspid form).

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Definition

Aortic valve stenosis developing on a congenital bicuspid aortic valve due to chronic abnormal hemodynamic shear stress precipitating premature calcification and stiffness (typically 1–2 decades earlier than in the tricuspid form), frequently associated with ascending aortic or root dilation (bicuspid aortopathy) [V1] [V4] [G1-02].

Severity criteria

  • Mild (L): Peak transaortic velocity (Vmax) 2.6–2.9 m/s; Mean transaortic gradient < 20 mmHg; Aortic valve area (AVA) > 1.5 cm²; Velocity ratio (Vratio) > 0.50 [V1] [V4].
  • Moderate (M): Vmax 3.0–3.9 m/s; Mean gradient 20–39 mmHg; AVA 1.0–1.5 cm²; Vratio 0.25–0.50 [V1] [V4].
  • Severe (G): Vmax ≥ 4.0 m/s; Mean gradient ≥ 40 mmHg; AVA < 1.0 cm² (or indexed AVA < 0.6 cm²/m²); Vratio < 0.25 [V1] [V4].

Findings by layer

Hemodynamics

  • Aortic mean gradient: L < 20 mmHg, M 20–39 mmHg, G ≥ 40 mmHg [V1] [V4].
  • Peak aortic velocity: L 2.6–2.9 m/s, M 3.0–3.9 m/s, G ≥ 4.0 m/s [V1] [V4].
  • Systemic arterial pressure: Narrow pulse pressure with slow upstroke in severe stages [B4] [V1].

Rhythm

  • Normal sinus rhythm [V1].

ECG

  • Concentric left ventricular hypertrophy due to pressure overload (left ventricular hypertrophy voltage criteria, tall R waves in V5-V6, ST depression and asymmetric T-wave inversion in lateral leads) [E1] [V1].

Heart sounds and murmurs

  • Aortic ejection click: Sharp high-frequency early systolic click audible at apex and right/left sternal border, characteristic of mild and moderate forms where cusps retain elasticity and doming mobility; tends to diminish or disappear in severe forms as cusps become severely calcified and immobile [G1-02] [G1-16] [V4].
  • Aortic systolic murmur: Diamond-shaped ejection mesosystolic murmur (crescendo-decrescendo) auscultated with maximum intensity at the 2nd right intercostal space (aortic area), radiating bilaterally to carotids [G1-14] [V4].
  • Second heart sound (S2): Aortic component (A2) decreased in intensity and delayed, producing paradoxical splitting of S2 in severe stages [G1-11] [V4].
  • Fourth heart sound (S4): Frequent due to vigorous atrial contraction against a stiff, hypertrophied LV [G1-13].

Arterial pulse

  • In severe forms: Slow-rising pulse with decreased amplitude and delayed peak (pulsus parvus et tardus / anacrotic pulse) [B4] [V1].

Jugular venous pulse

  • Generally normal; prominent 'a' wave if septal hypertrophy is present, reducing right ventricular compliance (Bernheim effect) [B3].

Maneuvers

  • Valsalva maneuver (strain phase): Markedly decreases systolic murmur intensity [B1].
  • Squatting / Standing: Squatting increases the murmur by increasing preload; standing attenuates it [B1].
  • Isometric handgrip: Decreases or maintains aortic murmur intensity due to increased systemic afterload [B1].

Simulator severity grades

Simulator severity grades: Mild, Moderate, Severe
Baseline rhythm: Normal Sinus Rhythm

Also known as: Bicuspid aortic valve stenosis · Stenotic bicuspid aortic valve · BAV stenosis · Congenital bicuspid aortic stenosis · BAV

Other pathologies in this group

Sources

  1. [B1] Bedside diagnosis of systolic murmurs . N Engl J Med (1988) . DOI: 10.1056/NEJM198806163182404 PubMed: 2897627
  2. [B3] The jugular venous pressure revisited . Cleve Clin J Med (2013) . DOI: 10.3949/ccjm.80a.13039 PubMed: 24085809
  3. [B4] The Carotid Pulse . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250154
  4. [E1] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part V: electrocardiogram changes associated with cardiac chamber hypertrophy: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191097 PubMed: 19228820
  5. [G1-02] International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes . J Thorac Cardiovasc Surg (2021) . DOI: 10.1016/j.jtcvs.2021.06.019 PubMed: 34304896
  6. [G1-11] The Second Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250183
  7. [G1-13] The Fourth Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250185
  8. [G1-14] Systolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250186
  9. [G1-16] Ejection Clicks . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250188
  10. [V1] 2025 ESC/EACTS Guidelines for the management of valvular heart disease . Eur Heart J (2025) . DOI: 10.1093/eurheartj/ehaf194 PubMed: 40878295
  11. [V4] Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography . Eur Heart J Cardiovasc Imaging (2017) . DOI: 10.1093/ehjci/jew335 PubMed: 28363204