Makindo Medical Notes

⚠️ Pulmonary hypertension (PH) is a progressive condition of raised pulmonary artery pressures leading to right heart strain and failure. Early recognition is crucial as treatment can slow progression but rarely reverses disease.

📖 About

• Defined as mean pulmonary artery pressure (mPAP) > 25 mmHg at rest, > 30 mmHg with exercise.
• 🟣 Primary Pulmonary Hypertension (PPH) = no identifiable cause.
• Rare disease: ~2 cases per million per year.

⚙️ Aetiology & Pathophysiology

• ⬆️ Pulmonary vascular resistance → sustained pressure overload on right ventricle.
• Flow equal in systemic & pulmonary circuits (no shunts), but pulmonary bed is normally much larger.
• Imbalance: vasoconstriction, endothelial dysfunction, fibrosis & thrombosis → progressive narrowing.

🔗 Associations

• 💊 Appetite suppressants (e.g. fenfluramine).
• 🔥 Vasculitis affecting pulmonary vessels.
• 🤲 Connective tissue diseases (esp. systemic sclerosis).

🧬 Genetics

• Familial PH: autosomal dominant, often BMPR2 or ALK1 mutations.

📂 Classification

• 🟣 Pulmonary arterial hypertension (PAH).
• 🔵 Pulmonary venous hypertension (often left heart disease).
• 🟤 PH with respiratory disease or hypoxia (e.g. COPD, OSA, ILD).
• 🟠 PH due to chronic thromboembolic disease (CTEPH).
• 🟢 PH due to miscellaneous pulmonary vascular disorders.

🩺 Clinical

• Most common in 👩 women aged 20–30 years.
• Symptoms: progressive exertional breathlessness, fatigue.
• Signs: loud P2 🔊, RV heave, right-sided S3.
• JVP: prominent "a" & "v" waves; TR murmur (pansystolic).
• Pulmonary flow murmur may be heard.

🔍 Investigations

• 🧪 Bloods: FBC → polycythaemia; U&E for renal function.
• 📈 ECG: RVH, RBBB, P pulmonale.
• 📸 CXR: Enlarged pulmonary arteries, peripheral pruning.
• 🫀 Echo: RV dilatation, TR, pressure estimates.
• CT-PA: Rule out chronic thromboembolic disease.
• Pathology: Plexiform lesions, in situ thrombosis.

💊 Management

• Oxygen for hypoxia, diuretics for oedema, anticoagulation, digoxin (supportive).
• 🟢 Calcium channel blockers (high-dose nifedipine/amlodipine) if vasoreactive subset.
• 🟣 Prostacyclin analogues (Iloprost infusion/nebulised) for advanced disease (NYHA III/IV).
• 🔴 Endothelin receptor antagonists (e.g. Bosentan).
• 🟡 PDE-5 inhibitors (Sildenafil) → nitric oxide–like vasodilation.
• ⚪ Atrial septostomy (palliative, under evaluation).
• 🫁 Lung / heart-lung transplantation for end-stage cases.
• 👩‍🍼 Contraception strongly advised – pregnancy is contraindicated.
• Advice: avoid high altitude ✈️, restrict strenuous exercise.
• 🩸 Venesection if severe polycythaemia (symptomatic hyperviscosity).

📚 References

• ESC/ERS Guidelines on Pulmonary Hypertension
• NICE CKS: Pulmonary Hypertension