1ry pulmonary htn

Question 1

Primary pulmonary hypertension= define

Answer 1

The classification of pulmonary hypertension is currently changing with the term idiopathic pulmonary arterial hypertension (IPAH) becoming more widely used

• Primary pulmonary hypertension (PPH, now IPAH)
• pulmonary arterial pressure > 25 mmHg at rest, > 30mmHg with exercise
• PPH is diagnosed when no underlying cause can be found
• around 10% of cases are familial: autosomal dominant
• endothelin thought to play a key role in pathogenesis
• associated with HIV, cocaine and anorexigens (e.g. fenfluramine)

Question 2

Features

Answer 2

• more common in females, typically presents at 20-40 years old
• progressive SOB
• cyanosis
• right ventricular heave, loud P2, raised JVP with prominent ‘a’ waves, tricuspid regurgitation

Question 3

Investigation

Answer 3

echocardiography

Question 4

Management

Answer 4

• diuretics if right heart failure
• anticoagulation
• vasodilator therapy:
  
  • calcium channel blocker,
  • IV prostaglandins,
  • bosentan: endothelin-1 receptor antagonist
• heart-lung transplant