PULMONARY HYPERTENSION

Question 1

A spectrum of diseases
involving the pulmonary
vasculature that leads to
restriction of blood flow
from the right ventricle

Defined as an elevation
in pulmonary arterial
pressures

Answer 1

pulmonary hypertension

Question 2

pulmonary artery pressure, MPAP for PH diagnosis?

Answer 2

MPAP > 20 mm Hg, PASP > 35 mm Hg

Question 3

PH occurs how?

Answer 3

pathology of the pulmonary artery itself or physiologic/obstructive diseases

Question 4

what group PAH is this?

also called Pulmonary Arterial
Hypertension (PAH)

Increased production of vasoconstrictor compounds (endothelin, thromboxane)

Reduced production of vasodilator compounds (prostacyclin, nitric oxide)

Answer 4

Group 1

Question 5

causes of PAH?

Answer 5

idiopathic
systemic 
-systemic sclerosis
-RA
-SLE
-Raynaud disease
-MCTD

Question 6

what group PAH is this?

Elevation of LA or LV
filling pressures leads
to back-pressure on the pulmonary circulation

Answer 6

group 2, often due to heart failure (mitral and aortic diseases, stenosis and regurgitation)

Question 7

what group PAH is this?

hypoxic pulmonary vasoconstriction

Answer 7

group 3

Question 8

AKA: Pulmonary heart disease

RV failure in the context of chronic lung disease

Triggered by the presence of
pulmonary hypertension

Answer 8

cor pulmonale, COPD is the most common cause

other causes: pulmonary fibrosis, kyphoscoliosis, OSA

Question 9

What clues are given by the following symptoms:

Exertional dyspnea and fatigue with orthopnea, dyspnea on exertion (DOE),
paroxysmal nocturnal dyspnea (PND)

Answer 9

acute left heart failure

Question 10

What clues are given by the following symptoms:

Exertional dyspnea and fatigue with chest pain

Answer 10

 Angina
 Myocardial infarction
 (Pulmonary embolism)
 (Pneumonia)

Question 11

What clues are given by the following symptoms:

Exertional dyspnea and productive cough

Answer 11

 Bronchitis or pneumonia

 Asthma or COPD

Question 12

What clues are given by the following symptoms:

Dyspnea with pleuritic chest pain and hemoptysis

Answer 12

 Pulmonary embolism

Question 13

What clues are given by the following symptoms:

Exertional dyspnea with wheezing and
sputum production

Answer 13

 Asthma or COPD

 Bronchitis or pneumonia

Question 14

gold standard in diagnosing PAH?

Answer 14

right heart catherization for group 1 PAH

• note that Low DLCO is a common finding
• sleep related breathing disorder
• polysomnography

Question 15

how to treat group 2/3 breathing disorders?

Answer 15

exercise
smoking cessation
oxygen
diuretics
assess for OSA