Pulmonary Hypertension

Pulmonary Hypertension is a mean pulmonary artery pressure more than 25 mmHg, with a pulmonary capillary or left atrial pressure less than 15 mmHg.

Causes:

Pulmonary arterial hypertension

• Sporadic
• Familial
• Related to:
• Collagen vascular disease
• HIV infection
• Drugs and toxins - fenfluramine
• Systemic to pulmonary shunts - ASD, PDA, Portal Hypertension

Pulmonary Venous Hypertension

• Left sided heart disease such as MS, LVF, AS

Pulmonary Hypertension associated with hypoxaemia

• Sleep disordered breathing
• Long term exposure to high altitude
• COPD
• Interstitial lung disease

Pulmonary Hypertension due to chronic thrombotic disease

• Pulmonary embolism

Miscellaneous

• Sarcoidosis
• Compression of pulmonary vessels such as tumour

Treatment:
General:

• Anticoagulation - all patients with pulmonary hypertension are at risk of venous thromboembolism and intrapulmonary thrombus because of sluggish pulmonary blood blow, dilated heart chambers and venous stasis.
• Long term oxygen therapy
• Immunization with annual influenza and pneumococcal vaccine.

Vasodilator therapy

• Calcium channel blocker - nifedipine and amlodipine
• Prostaglandin analogues - ilioprost
• Endothelin receptor antagonists - bosenten
• Phosphodiesterase inhibitors - sildenafil