Who classically gets 1a pulm HTN?
young adult females
What is the normal pulmonary circuit pressure?
How does pulm HTN present?
- exertional dyspnea
- RH failure
What can cause increased volume in the pulmonary circuit?
congenital heart disease (Eisenmenger's Syndrome)
Thickening of the vascular smooth muscle cells of the lungs leads to _____.
What causes 2a pulm HTN?
- increased pulm circuit volume
- recurrent PE
What can PE cause?
- nothing- asymptomatic
- pulm infarction
- sudden death (saddle emboli)
- reorganization --> pulm HTN
Pulmonary HTN is defined as pressure higher than ____.
Pulm HTN leads to _____ and _____.
RV hypertrophy; cor pulmonale
This is a group of capillaries swirled together- a complication of long standing pulm HTN.
a plexiform lesion
What is cor pulmonale?
What causes 1a pulm HTN?
unknown etiology, but familial forms due to mutation in BMPR2
What is a plexiform lesion?
a group of capillaries swirled together- a complication of long standing pulm HTN
What are the characteristics of pulmonary HTN?
- atherosclerosis of the pulm trunk
- smooth muscle hypertrophy of pulm a.'s
- intimal fibrosis
- plexiform lesions
What does a BMPR2 mutation cause?
1a pulm HTN via vascular smooth muscle proliferation