MioAtlas
G2: Right-sided valves

Valvular pulmonic stenosis (doming)

Congenital stenosis of the pulmonary valve characterized by commissural fusion and systolic dome-like (doming) excursion of thin pliable cusps.

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Definition

Congenital right ventricular outflow tract obstruction at the pulmonary valve level due to leaflet thickening, commissural fusion, or cusp dysplasia, causing dome-like systolic opening (doming), generating an accelerated turbulent jet into the pulmonary artery and systolic pressure overload on the right ventricle [C4] [C5] [G1-01] [G23-04].

Severity criteria

  • Mild (L): Peak transpulmonary velocity (Vmax) < 3.0 m/s; Peak transvalvular gradient < 36 mmHg [C5] [G1-01].
  • Moderate (M): Vmax 3.0–4.0 m/s; Peak gradient 36–64 mmHg [C5] [G1-01].
  • Severe (G): Vmax > 4.0 m/s; Peak gradient > 64 mmHg (or mean gradient > 35–40 mmHg) [C5] [G1-01] [C4].

Findings by layer

Hemodynamics

  • Accelerated transpulmonary peak flow velocity: L < 3.0 m/s, M 3.0–4.0 m/s, G > 4.0 m/s [C5] [G1-01].
  • Markedly elevated RV-PA systolic pressure gradient with right ventricular systolic pressures that can equal or exceed systemic arterial pressure in severe forms [C5] [G1-01].
  • Distal pulmonary artery pressure normal or low [C5].

Rhythm

  • Normal sinus rhythm [C5].

ECG

  • Severe concentric right ventricular hypertrophy: tall R waves in V1 (> 7 mm), R/S ratio > 1 in V1, ST segment depression and asymmetric T wave inversion in right precordial leads V1–V3 (RV systolic overload pattern) [E1] [C5].
  • Right axis deviation of the QRS (right axis deviation > +90° to +110°) and peaked P waves of right atrial enlargement (P pulmonale) [E1].

Heart sounds and murmurs

  • Systolic ejection murmur: Harsh, raspy mid-systolic or mid-to-late systolic ejection murmur (diamond crescendo-decrescendo), auscultated with maximum intensity at the 2nd left intercostal space (pulmonic area), radiating to the left clavicle and interscapular region. Murmur duration prolongs and peaks later with increasing severity [G1-14] [G23-04].
  • Pulmonic ejection click: High-frequency valve opening click audible in pulmonic area immediately after S1, characterized by its unique acoustic property of decreasing or disappearing during inspiration (inspiratory rise in RV end-diastolic pressure passively opens the leaflets prior to ventricular systole) [G1-16] [G23-04] [G23-05].
  • Second heart sound (S2): Widely split S2 (S2 split wide), with markedly delayed and soft/muffled pulmonic component (P2) (P2 soft) due to prolonged right ventricular ejection time and stiff leaflets [G1-11] [G23-04].
  • Right-sided fourth heart sound (S4): Frequent in severe forms due to forceful right atrial contraction against a stiff, hypertrophied right ventricle [G1-13].

Arterial pulse

  • Normal arterial pulse, or small-volume pulse (parvus) in extreme stenosis with reduced right-sided forward output [B4].

Jugular venous pulse

  • Giant 'a' wave (giant a wave) very prominent in severe forms reflecting forceful right atrial contraction against non-compliant hypertrophied RV [B3].

Maneuvers

  • Deep inspiration: Attenuates or abolishes the pulmonic ejection click (characteristic differential feature distinguishing it from aortic clicks) [G23-04] [G23-05].
  • Valsalva maneuver: Decreases murmur intensity during strain phase and shows rapid recovery (in 1–2 beats) upon release of effort [B1].

Simulator severity grades

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

Also known as: Pulmonary valve stenosis · Congenital pulmonary stenosis · Valvular pulmonic stenosis · Pulmonic stenosis · PS

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. [C4] 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines . Circulation (2025) . DOI: 10.1161/CIR.0000000000001402 PubMed: 41411375
  5. [C5] 2020 ESC Guidelines for the management of adult congenital heart disease . Eur Heart J (2020) . DOI: 10.1093/eurheartj/ehaa554 PubMed: 32860028
  6. [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
  7. [G1-01] Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice . J Am Soc Echocardiogr (2009) . DOI: 10.1016/j.echo.2008.11.029 PubMed: 19130998
  8. [G1-11] The Second Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250183
  9. [G1-13] The Fourth Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250185
  10. [G1-14] Systolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250186
  11. [G1-16] Ejection Clicks . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250188
  12. [G23-04] Auscultatory and phonocardiographic signs of pulmonary stenosis . Br Heart J (1957) . DOI: 10.1136/hrt.19.3.303 PubMed: 13446314
  13. [G23-05] Recommendations for evaluation of prosthetic valves with echocardiography and doppler ultrasound: a report From the American Society of Echocardiography's Guidelines and Standards Committee and the Task Force on Prosthetic Valves, developed in conjunction with the American College of Cardiology Cardiovascular Imaging Committee, Cardiac Imaging Committee of the American Heart Association, the European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography and the Canadian Society of Echocardiography, endorsed by the American College of Cardiology Foundation, American Heart Association, European Association of Echocardiography, a registered branch of the European Society of Cardiology, the Japanese Society of Echocardiography, and Canadian Society of Echocardiography . J Am Soc Echocardiogr (2009) . DOI: 10.1016/j.echo.2009.07.013 PubMed: 19733789