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Pulmonary Valve Disease (Stenosis & Regurgitation)

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Pulmonary Valve Disease (Stenosis & Regurgitation)

Also called

narrowing & Leaky Pulmonary Valve

ICD-10

I37.0 + I37.1

Specialty

Cardiology

Onset

chronic

Reviewed

June 2026

On This Page

Overview

Pulmonary valve disease affects the pulmonary valve, which is located between the right ventricle (RV) and the pulmonary artery. It includes:

  • Pulmonary Stenosis (PS): Narrowing of the pulmonary valve that obstructs blood flow from the right ventricle to the pulmonary artery during systole. It is usually congenital.
  • Pulmonary Regurgitation (PR): Failure of the pulmonary valve to close properly, causing backflow of blood from the pulmonary artery into the right ventricle during diastole.

Etiology & Risk Factors

1. Pulmonary Stenosis (PS)

Most Common Cause

  • Congenital pulmonary valve stenosis (most cases)

Other Causes (Rare)

  • Rheumatic fever
  • Carcinoid heart disease

2. Pulmonary Regurgitation (PR)

Common Causes

  • Pulmonary hypertension (most common cause of significant PR)
  • Pulmonary valve damage after balloon valvuloplasty or valve surgery
  • Congenital heart disease
  • Infective endocarditis
  • Carcinoid heart disease

General Risk Factors (Pulmonary Valve Disease)

  • Congenital heart disease
  • Noonan syndrome (associated with pulmonary stenosis)
  • Rheumatic fever
  • Carcinoid syndrome/heart disease
  • Pulmonary hypertension
  • Previous pulmonary valve intervention or surgery
  • Infective endocarditis

Pathophysiology

1. Pulmonary Stenosis (PS)

  • Narrowing of the pulmonary valve obstructs blood flow from the right ventricle (RV) to the pulmonary artery during systole.
  • This causes:
    • Right ventricular outflow obstruction
    • Pressure overload of the RV
    • Right ventricular hypertrophy (RVH)
    • If severe, right-sided heart failure

Simple Flow:
Pulmonary valve narrowing → RV outflow obstruction → RV pressure overload → RV hypertrophy → Right-sided heart failure

2. Pulmonary Regurgitation (PR)

  • Pulmonary valve fails to close properly, allowing blood to flow back from the pulmonary artery into the RV during diastole.
  • This causes:
    • Right ventricular volume overload
    • Progressive RV dilation
    • Eventually right-sided heart failure if severe

Simple Flow:
Pulmonary valve incompetence → Blood leaks back into RV → RV volume overload → RV dilation → Right-sided heart failure

Clinical Presentation

  1. Pulmonary Stenosis (PS)

    Symptoms

    • May be asymptomatic if mild.
    • Exercise intolerance
    • Symptoms of heart failure in more severe disease.

    Physical Examination

    • Crescendo–decrescendo systolic murmur heard best at the 2nd left intercostal space (left upper sternal border).
    • Wide splitting of S₂.

    Pulmonary Regurgitation (PR)

    Symptoms

    • Often asymptomatic if mild.
    • If severe:
      • Fatigue
      • Exercise intolerance
      • Symptoms of right-sided heart failure

    Physical Examination

    • Early diastolic decrescendo murmur heard best at the 2nd left intercostal space.
    • Graham Steell murmur may be present.

History Taking

  • Symptoms

    • “Do you get short of breath during exercise or daily activities?”
    • “Do you feel tired more easily than before?”
    • “Do you have chest pain?”
    • “Do you feel your heart racing or skipping beats (palpitations)?”
    • “Have you ever fainted or felt dizzy?”

    Symptoms of Right-Sided Heart Failure

    • “Do you have swelling in your legs or ankles?”
    • “Have you noticed abdominal swelling or weight gain due to fluid?”

    Risk Factors

    • “Were you born with a heart defect?”
    • “Do you have Noonan syndrome?”
    • “Have you had rheumatic fever?”
    • “Have you ever had infective endocarditis?”
    • “Have you had previous pulmonary valve surgery or balloon valvuloplasty?”
    • “Have you been told you have pulmonary hypertension?”

Pulmonary Stenosis Vs Pulmonary Regurgitation

Pulmonary Stenosis vs Pulmonary Regurgitation

BOTH AFFECT THE PULMONARY VALVE BUT WITH OPPOSITE FLOW PROBLEMS Pulmonary stenosis (PS) obstructs RV outflow → RV pressure overload & hypertrophy. Pulmonary regurgitation (PR) allows backflow into the RV during diastole → RV volume overload & dilation. PS is usually congenital; mild PR is a common, often benign incidental finding.
Pulmonary Stenosis (PS)
MechanismObstruction to RV outflow across the pulmonary valve, mainly affecting systolic ejection.
Most common causeCongenital (most common cause overall, often isolated or part of syndromes e.g. Noonan, tetralogy of Fallot). Rare acquired causes: rheumatic, carcinoid syndrome.
Haemodynamic effectRV pressure overload → concentric RV hypertrophy → eventual RV failure if severe/untreated.
VS
Pulmonary Regurgitation (PR)
MechanismBackflow of blood from pulmonary artery into RV during diastole due to valve incompetence.
Most common causePulmonary hypertension (functional annular dilation) or iatrogenic (post-repair of tetralogy of Fallot/pulmonary valvotomy). Mild physiological PR is common and benign.
Haemodynamic effectRV volume overload → progressive RV dilation & dysfunction if chronic/severe.
Feature Pulmonary Stenosis Pulmonary Regurgitation
Murmur Systolic ejection murmur, crescendo-decrescendo, upper left sternal border, radiates to back; widely split S2 with soft P2 Early diastolic decrescendo murmur (Graham Steell if 2° to pulmonary hypertension), upper left sternal border
JVP findings Prominent "a" wave if RV hypertrophy reduces compliance Usually unremarkable unless severe RV failure develops; may see elevated JVP late
Pulse/other signs RV heave; ejection click that decreases with inspiration (unusual right-sided finding) RV heave if RV dilated; signs of underlying pulmonary hypertension
Key echo finding Increased systolic gradient across pulmonary valve (peak velocity/gradient); doming valve; post-stenotic PA dilation Color Doppler diastolic regurgitant jet; PR jet width/duration; RV dilation; paradoxical septal motion if severe
Right heart chamber effect RV hypertrophy predominates (pressure overload); RV size may be normal early RV dilation predominates (volume overload); progressive RV dysfunction if chronic severe
Common associations Congenital heart disease, Noonan syndrome, tetralogy of Fallot, carcinoid (rare) Pulmonary hypertension, post-TOF repair, post-balloon valvotomy, endocarditis (rare), carcinoid
Definitive treatment Balloon valvuloplasty (first-line for valvular PS); surgical valvotomy/replacement if unsuitable Treat underlying cause; pulmonary valve replacement (surgical or transcatheter) if severe with RV dilation/dysfunction or symptoms
Pulmonary Stenosis — Clinical Picture
Symptoms — often asymptomatic if mild; exertional dyspnea, fatigue, syncope, chest pain if severe
Signs — RV heave, palpable thrill at upper left sternal border, cyanosis if associated shunt (e.g. ToF)
Severity marker — peak gradient ≥64 mmHg or peak velocity >4 m/s = severe
ECG — right axis deviation, RV hypertrophy, P pulmonale if severe
Pulmonary Regurgitation — Clinical Picture
Symptoms — usually asymptomatic for years; exertional dyspnea, fatigue, palpitations (arrhythmia) once RV fails
Signs — RV heave, signs of right heart failure (oedema, raised JVP) in advanced disease
Severity markers — PR jet occupying >50% of diastole, dense/steep deceleration, significant RV dilation
Key context — common late complication after tetralogy of Fallot repair; monitored closely with serial echo/cardiac MRI for timing of valve replacement

Investigations

  • Investigations (Ix) – Pulmonary Valve Disease (PS & PR)

    1. Transthoracic Echocardiography (TTE) – Gold Standard

    • First-line and confirmatory test
    • Assesses:
      • Valve structure
      • Severity of pulmonary stenosis (PS) or pulmonary regurgitation (PR)
      • Right ventricular function
      • Pressure gradient across the pulmonary valve (for PS)

    2. ECG

    • May be normal in mild PS.
    • May show right ventricular hypertrophy (RVH) in severe disease.

    3. Chest X-ray

    • May show pulmonary artery dilatation.

    4. Cardiac Catheterization

    • Performed in selected patients, especially before valve repair when indicated.

    High-Yield Point

    • TTE is the gold-standard investigation for pulmonary valve disease.

Diagnosis

  • Diagnostic Approach

    • Suspect pulmonary valve disease based on:
      • Symptoms
      • Characteristic heart murmur on examination
    • Confirm the diagnosis with transthoracic echocardiography (TTE).

    Gold-Standard Test

    • Transthoracic echocardiography (TTE)
      • Confirms the diagnosis.
      • Assesses the severity of pulmonary stenosis and valve function.

    Supportive Investigations

    • ECG
      • May be normal in mild pulmonary stenosis.
      • May show right ventricular hypertrophy (RVH) in severe disease.
    • Chest X-ray
      • May show pulmonary artery dilatation.
    • Cardiac catheterization
      • Considered before repair in selected patients.
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Management

1. Pulmonary Stenosis (PS)

Mild Disease

  • Regular follow-up with clinical assessment and echocardiography.

Symptomatic Moderate-to-Severe PS

  • Balloon pulmonary valvuloplasty (preferred treatment).
  • Pulmonary valve replacement if balloon valvuloplasty is not suitable or unsuccessful.

Critical Pulmonary Stenosis in Neonates 🚨

  • Maintain the patent ductus arteriosus (PDA) with prostaglandin E₁ (alprostadil) until definitive treatment can be performed.

2. Pulmonary Regurgitation (PR)

  • Treat the underlying cause (e.g., pulmonary hypertension).
  • Monitor with regular echocardiography.
  • Pulmonary valve replacement may be required in severe symptomatic disease or when there is significant right ventricular dysfunction.

Complications

    • Right ventricular hypertrophy (RVH) (especially in pulmonary stenosis)
    • Right ventricular dilation (especially in pulmonary regurgitation)
    • Right-sided heart failure
    • Exercise intolerance
    • Progressive valve dysfunction if left untreated

Prognosis

  • Mild pulmonary valve disease generally has a good prognosis and often requires only regular follow-up.
  • Prognosis worsens with severe disease, development of right ventricular dysfunction, or heart failure.
  • Early intervention (e.g., balloon valvuloplasty for pulmonary stenosis or valve replacement when indicated) improves outcomes.

Key Points / Clinical Pearls

    • Pulmonary stenosis (PS) = narrowed pulmonary valve → right ventricular pressure overload.
    • Pulmonary regurgitation (PR) = leaky pulmonary valve → right ventricular volume overload.
    • Most PS cases are congenital.
    • Gold-standard investigation: Transthoracic echocardiography (TTE).
    • PS murmur: Crescendo–decrescendo systolic murmur at the 2nd left intercostal space.
    • PR murmur: Early diastolic decrescendo (Graham Steell) murmur.
    • Preferred treatment for symptomatic moderate-to-severe PS: Balloon pulmonary valvuloplasty.
    • Both conditions can eventually lead to right-sided heart failure if severe or untreated.