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Pathology - Pulmonary hypertension
Definition: A mean pulmonary artery pressure above 25 mmHg at rest or 30 mmHg during exertion.
Categories Secondary pulmonary hypertension is a consequence of chronic pulmonary or cardiac conditions. Primary pulmonary hypertension manifests in the absence of chronic pulmonary or cardiac conditions.
Epidemiology
• Secondary pulmonary hypertension is prevalent. Primary pulmonary hypertension is uncommon.
Aetiology
Common etiologies of secondary hypertension encompass COPD, interstitial lung disease, left ventricular failure, and chronic pulmonary thromboemboli. Primary pulmonary hypertension may be idiopathic or linked to specific medications, HIV infection, collagen vascular disorders, and congenital systemic-to-pulmonary shunts.
Pathogenesis: Chronic hypoxia and obliterative pulmonary fibrosis contribute to elevated pressure in the pulmonary arterial circulation.
Presentation • Secondary pulmonary hypertension exacerbates the symptoms of the pre-existing illness, resulting in increased breathlessness. • Primary pulmonary hypertension manifests as exertional dyspnoea and exhaustion. Dizziness and syncope are prevalent as well.
Macroscopy • The identification of atherosclerosis in major pulmonary arteries indicates potential underlying pulmonary hypertension. • Right ventricular hypertrophy may also be observed.
Histopathology: All cases have muscular hypertrophy and intimal proliferation of tiny pulmonary arteries, together with muscularization of pulmonary arterioles. • Severe instances have plexiform lesions, marked by the proliferation of slit-like vascular gaps originating from the arterial wall. • Extremely severe cases may present with fibrinoid necrosis of the arterial wall.
Prognosis • Secondary pulmonary hypertension typically indicates substantial cardiac or pulmonary pathology with a bad prognosis. The prognosis for primary pulmonary hypertension is bleak, with 5-year survival rates ranging from 25% to 50%.
Definition: A mean pulmonary artery pressure above 25 mmHg at rest or 30 mmHg during exertion.
Categories Secondary pulmonary hypertension is a consequence of chronic pulmonary or cardiac conditions. Primary pulmonary hypertension manifests in the absence of chronic pulmonary or cardiac conditions.
Epidemiology
• Secondary pulmonary hypertension is prevalent. Primary pulmonary hypertension is uncommon.
Aetiology
Common etiologies of secondary hypertension encompass COPD, interstitial lung disease, left ventricular failure, and chronic pulmonary thromboemboli. Primary pulmonary hypertension may be idiopathic or linked to specific medications, HIV infection, collagen vascular disorders, and congenital systemic-to-pulmonary shunts.
Pathogenesis: Chronic hypoxia and obliterative pulmonary fibrosis contribute to elevated pressure in the pulmonary arterial circulation.
Presentation • Secondary pulmonary hypertension exacerbates the symptoms of the pre-existing illness, resulting in increased breathlessness. • Primary pulmonary hypertension manifests as exertional dyspnoea and exhaustion. Dizziness and syncope are prevalent as well.
Macroscopy • The identification of atherosclerosis in major pulmonary arteries indicates potential underlying pulmonary hypertension. • Right ventricular hypertrophy may also be observed.
Histopathology: All cases have muscular hypertrophy and intimal proliferation of tiny pulmonary arteries, together with muscularization of pulmonary arterioles. • Severe instances have plexiform lesions, marked by the proliferation of slit-like vascular gaps originating from the arterial wall. • Extremely severe cases may present with fibrinoid necrosis of the arterial wall.
Prognosis • Secondary pulmonary hypertension typically indicates substantial cardiac or pulmonary pathology with a bad prognosis. The prognosis for primary pulmonary hypertension is bleak, with 5-year survival rates ranging from 25% to 50%.
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