Pulmonary Hypertension Flashcards
What is Pulmonary Hypertension?
High pressure in the pulmonary circuit
What is normal mean arterial pressure (in pulmonary circuit)?
10 mmHg
What is the mean arterial pressure in pulmonary hypertension?
> 25 mmHg
What three features characterize Pulmonary Hypertension/
- Atherosclerosis of the pulmonary trunk
- Smooth muscle hypertrophy of pulmonary arteries
- Intimal fibrosis
What is seen in severe, long-standing Primary pulmonary hypertension?
Plexiform lesions
What are Plexiform lesions?
Tuft/group of capillaries from long standing pulmonary HTN
What two things does Pulmonary Hypertension lead to?
Right ventricular hypertrophy with eventual cor pulmonale.
What does Pulmonary Hypertension usually present with?
Exertional dyspnea or Right-sided heart failure
How is Pulmonary Hypertension sub classified?
It is sub classified into primary or secondary based on etiology.
What group is primary pulmonary hypertension classically seen in?
Young adult females
What is the etiology behind primary pulmonary hypertension?
Unknown
What are some familial forms of Primary Pulmonary Hypertension related to?
Inactivating mutations of BMPR2 (leading to plexiform lesions)
What does a mutation in BMPR2 lead to?
Proliferation of vascular smooth muscle –> thickening of vessel wall –> plexiform lesions –> pulmonary hypertension
What causes Secondary Pulmonary Hypertension?
- Hypoxemia (e.g. COPD and interstitial lung disease)
- Increased volume in pulmonary circuit (e.g. congenital heart disease)
- May also arise with recurrent pulmonary embolism
What are the four consequences of Pulmonary Embolism (PE)?
- Most PE asymptomatic
- -> lung has dual circulation
- -> most clots are very small - Pulmonary Infarction
- Sudden death (saddle PE)
- Chronic longstanding PE that gets reorganized
- -> vascular wall thickens
- -> Pulmonary HTN
