Makindo Medical Notes

Related Subjects: |Pulmonary Stenosis |Pulmonary Regurgitation |Pulmonary Hypertension |Pulmonary Embolism

🫀 About

• Pulmonary regurgitation (PR) is usually well-tolerated and often asymptomatic, but in the presence of pulmonary hypertension it can cause right ventricular (RV) overload → RV failure.
• Most commonly due to dilation of the pulmonary annulus, preventing proper valve closure.

⚠️ Aetiology

• Commonest cause: Pulmonary hypertension → annular dilation.
• Congenital/Acquired:
  • Post-surgical repair of Tetralogy of Fallot (TOF).
  • Carcinoid syndrome → serotonin damage to right-sided valves.
  • Rheumatic disease (rare pulmonary valve involvement).
  • Infective endocarditis.
  • Syphilis (very rare).

🔬 Pathophysiology

• Backflow during diastole → RV volume overload.
• Leads to RV hypertrophy, dilatation, arrhythmia risk, and eventually right heart failure.
• Worsens if a right-to-left shunt is present (e.g. PFO/ASD).

🩺 Clinical Features

• Often asymptomatic if mild/moderate.
• Severe PR:
  • Fatigue, exertional dyspnoea, presyncope/syncope.
  • Right HF: peripheral oedema, ascites, hepatomegaly.
• Examination:
  • Early diastolic murmur at 2nd LICS, ↑ with inspiration (Carvallo’s sign).
  • Prominent P2 if pulmonary hypertension present.
  • Elevated JVP, RV heave, hepatomegaly in advanced disease.

🔍 Investigations

• ECG: RVH, RAD, ± RBBB.
• CXR: Post-stenotic PA dilatation, enlarged RV, prominent pulmonary arteries.
• Echocardiogram (TTE/TOE): Diagnostic, assess severity & RV size/function.
• Cardiac MRI: Gold standard for quantifying RV volumes and regurgitant fraction (especially in post-TOF repair patients).
• Bloods: Rule out infection, systemic disease (CRP, FBC, syphilis serology if indicated).

💊 Management

• Medical:
  • Observation if mild and asymptomatic.
  • Treat pulmonary hypertension (oxygen, diuretics, vasodilators).
  • Monitor RV function regularly with echo/MRI.
• Interventional:
  • Percutaneous pulmonary valve replacement (PPVR) – increasingly first choice in post-TOF repair patients.
  • Surgical valve replacement – mechanical, bioprosthetic, or homograft in severe disease or unsuitable anatomy for PPVR.

📊 Prognosis & Follow-Up

• Mild PR = benign, requires periodic echo (e.g. every 2-3 yrs).
• Moderate/severe PR with RV dilation → yearly review and earlier intervention if RV end-diastolic volume ↑ significantly.
• Post-TOF patients: lifelong follow-up in ACHD (Adult Congenital Heart Disease) centres.
• Untreated severe PR → arrhythmias, progressive RV failure, poor outcomes.

📚 References

• European Heart Journal: Valve Disease Guidelines
• AHA Guidelines – Pulmonary Valve Disease

📝 Exam tip: An early diastolic murmur at the pulmonary area that gets louder with inspiration = pulmonary regurgitation. Distinguishes it from aortic regurgitation, which is heard at LLSB and does not vary with inspiration.

Cases — Pulmonary Regurgitation (PR)

• Case 1 — Post-Surgical PR after TOF Repair 🩺: A 19-year-old man, previously operated on for Tetralogy of Fallot, presents with progressive exertional dyspnoea. Exam: early diastolic decrescendo murmur at the left upper sternal edge, RV heave. Echo: severe pulmonary regurgitation with RV dilatation. Diagnosis: Severe pulmonary regurgitation post-TOF repair. Management: Regular echo monitoring; pulmonary valve replacement considered if RV function deteriorates.
• Case 2 — Pulmonary Hypertension-Related PR 💨: A 52-year-old woman with longstanding idiopathic pulmonary arterial hypertension presents with fatigue and ankle swelling. Exam: Graham Steell murmur (high-pitched early diastolic murmur at left upper sternal border), elevated JVP, peripheral oedema. Diagnosis: Pulmonary regurgitation secondary to pulmonary hypertension. Management: Treat underlying PAH (endothelin antagonists, PDE5 inhibitors); diuretics for RV failure; valve replacement rarely needed unless severe and symptomatic.
• Case 3 — Congenital PR (Isolated) 👶: A 12-year-old boy with an incidental murmur found on routine exam. Asymptomatic, normal growth. Echo: isolated mild pulmonary regurgitation with no RV dilatation. Diagnosis: Congenital mild pulmonary regurgitation. Management: No treatment required; benign; periodic follow-up with echo.

Teaching Commentary 🧠

Pulmonary regurgitation is usually secondary (post-surgical, pulmonary hypertension), but can rarely be primary congenital. - Signs: Early diastolic murmur (best at left upper sternal edge), RV heave, wide split S2. - Causes: Post-TOF repair, pulmonary hypertension (Graham Steell murmur), carcinoid heart disease, infective endocarditis, congenital absence of valve. - Management: Usually supportive and directed at underlying cause. Valve replacement (surgical or percutaneous) is considered if severe PR causes RV dilatation/dysfunction.