Pathoma: Pulmonary HTN

Question 1

Who classically gets 1a pulm HTN?

Answer 1

young adult females

Question 2

How does pulm HTN present?

Answer 2

• exertional dyspnea
• RH failure

Question 3

What can cause increased volume in the pulmonary circuit?

Answer 3

congenital heart disease (Eisenmenger’s Syndrome)

Question 4

Thickening of the vascular smooth muscle cells of the lungs leads to _____.

Answer 4

pulm HTN

Question 5

What can PE cause?

Answer 5

1. nothing- asymptomatic
2. pulm infarction
3. sudden death (saddle emboli)
4. reorganization –> pulm HTN

Question 6

What causes 2a pulm HTN?

Answer 6

1. hypoxemia
2. increased pulm circuit volume
3. recurrent PE

Question 7

Pulmonary HTN is defined as pressure higher than ____.

Answer 7

25mmHg

Question 8

Pulm HTN leads to _____ and _____.

Answer 8

RV hypertrophy; cor pulmonale

Question 9

This is a group of capillaries swirled together- a complication of long standing pulm HTN.

Answer 9

a plexiform lesion

Question 10

What is cor pulmonale?

Answer 10

RH failure

Question 11

What causes 1a pulm HTN?

Answer 11

unknown etiology, but familial forms due to mutation in BMPR2

Question 12

What is a plexiform lesion?

Answer 12

a group of capillaries swirled together- a complication of long standing pulm HTN

Question 13

What is the normal pulmonary circuit pressure?

Answer 13

10mmHg

Question 14

What are the characteristics of pulmonary HTN?

Answer 14

• atherosclerosis of the pulm trunk
• smooth muscle hypertrophy of pulm a.’s
• intimal fibrosis
• plexiform lesions

Question 15

What does a BMPR2 mutation cause?

Answer 15

1a pulm HTN via vascular smooth muscle proliferation