Pulmonary hypertension
المؤلف:
James Carton
المصدر:
Oxford Handbook of Clinical Pathology 2024
الجزء والصفحة:
3rd edition , p63
2025-01-18
762
Definition
• A mean pulmonary artery pressure >25 mmHg at rest or >30 mmHg during exercise.
Subtypes
• Secondary pulmonary hypertension is a complication of chronic lung or cardiac disease.
• Primary pulmonary hypertension occurs in the absence of chronic lung or heart disease.
Epidemiology
• Secondary pulmonary hypertension is quite common.
• Primary pulmonary hypertension is rare.
Aetiology
• Common causes of secondary hypertension include COPD, interstitial lung disease, left ventricular failure (LVF), and chronic pulmonary thromboemboli.
• Primary pulmonary hypertension may be idiopathic or associated with certain drugs, HIV infection, collagen vascular disease, and congenital systemic-to-pulmonary shunts.
Pathogenesis
• Chronic hypoxia and obliterative pulmonary fibrosis both lead to the development of raised pressure in the pulmonary arterial circulation.
Presentation
• Secondary pulmonary hypertension causes worsening of the symptoms of the pre- existing condition with increasing breathlessness.
• Primary pulmonary hypertension presents with exertional dyspnoea and fatigue. Dizziness and syncope are also common.
Macroscopy
• the presence of atherosclerosis in large pulmonary arteries is a clue to underlying pulmonary hypertension.
• Right ventricular hypertrophy may also be present.
Histopathology
• Muscular hypertrophy and intimal proliferation of small pulmonary arteries and muscularization of pulmonary arterioles.
• Severe cases show plexiform lesions, characterized by a proliferation of slit- like vascular spaces from the arterial wall.
• Very severe cases may display fibrinoid necrosis of the arterial wall.
Prognosis
• Secondary pulmonary hypertension generally implies significant underlying cardiac or lung disease with poor prognosis.
• Prognosis of primary pulmonary hypertension is also very poor with 5- year survival rates of only 25– 50%.
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