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%.

مواضيع ذات صلة

Acute circulatory failure (cardiogenic shock)

Pulmonary thromboembolism

Giant cell (temporal) arteritis

ابيضاض الدم النقوي المزمن Chronic myelocytic leukaemias

Abdominal aortic aneurysm

Infective endocarditis

تليف النقيMyelofibrosis

فقر الدم اللا مصنع ( اللا تنسجي ) Aplastic anemia

Septic shock

Distributive shock

Cardiogenic shock

Hypovolemic shock