Pulmonary Circulation

Question 1

Increase in pulmonary vasculature resistance that leads to right ventricular hypertrophy causing right sided heart failure

Answer 1

Pulmonary hypertension

Question 2

When the pulmonary arterial pressure rises to a level inappropriate for a given cardiac output

Answer 2

Pulmonary hypertension

Question 3

Which type of pulmonary hypertension is rare and often has a fatal outcome?

Answer 3

Primary idiopathic pulmonary hypertension

Question 4

What defective gene causes primary idiopathic pulmonary hypertension?

Answer 4

BMPR2 gene: Normally inhibits pulmonary vessel smooth muscle growth and vasoconstriction

Question 5

Who is primarily affected by primary idiopathic pulmonary hypertension? When is the mean age of dx?

Answer 5

80% middle-aged or young women

50y.o

Question 6

What is the most common cause of secondary pulmonary hypertension?

Answer 6

COPD

Question 7

Other than COPD, what can cause secondary pulmonary hypertension?

Answer 7

Sleep apnea, PE, cardiac, metabolic, systemic disease

Question 8

What are the 4 types of pulmonary hypertension?

Answer 8

I. Pulmonary artery hypertension
II. Pulmonary hypertension associated with left heart disease
III. Pulmonary HTN due to hypoxemic lung dz
IV: Chronic thromboembolic

Question 9

What is the most important and potent stimulus of pulmonary arterial vasoconstriction?

Answer 9

Hypoxia

Followed by acidosis and veno-occlusive conditions

Question 10

Sxs: dyspnea, chest pain, weakness, fatigue, cyanosis, syncope, edema

Answer 10

Pulmonary hypertension

Question 11

Accentuated increase in S2 (fixed or paradoxically split S2)
Signs of R-sided heart failure
Increased JVP
Peripheral edema
Ascites
Systolic ejection click
Pulmonary regurgitation
RV heave

Answer 11

Pulmonary hypertension physical exam findings

Question 12

What should you see on a CXR of someone with pulmonary hypertension?

Answer 12

Enlarged pulm. arteries
Insterstitial/alveolar edema
Signs of heart failure

Question 13

What would you expect to see on a ECG of someone with pulmonary hypertension?

Answer 13

Cor pulmonale**
Right ventricular hypertrophy, RAE, Reactive airway disease, Right bundle branch block
R—>L shunt

Question 14

What is the definitive diagnosis of pulmonary hypertension?

Answer 14

Rights sided catheterization

Question 15

With right sided catheterization, what results are used to diagnose pulmonary hypertension?

Answer 15

Pulmonary arterial pressure >25mmHg at rest

or >35mmHg at exercise

Question 16

What results from a CBC would support a diagnosis of pulmonary hypertension?

Answer 16

Polycythemia with increased hematocrit

Question 17

What are the two principle management treatments for pulmonary hypertension?

Answer 17

Vasodilators

Oxygen therapy/anticoagulation

Question 18

What is the first line treatment for primary pulmonary hypertension

Answer 18

Calcium channel blockers (if sensitive)

Question 19

If pulmonary HTN is not sensitive to CCB, what is the next step in treatment?

Answer 19

Prostacyclines

Question 20

If pulmonary HTN is not sensitive to prostacyclines, what is the next step in treatment?

Answer 20

Endothelial receptor antagonists

Question 21

What is usually needed for pulmonary hypertension?

Answer 21

Heart lung transplant

Question 22

Right heart failure resulting form primary lung disease or pulmonary hypertension. Increased pressures and work result in hypertrophy and eventual failure of the right ventricle.

Answer 22

Cor Pulmonale

Question 23

Venous congestion
Peripheral edema
SOB
Productive cough that becomes worse during periods of heart failure

Answer 23

Cor pulmonale

Question 24

Plethora (redness), cyanosis, and warm moist skin result from ______ ______ and desaturation of arterial blood that accompanies chronic lung disease in cor pulmonale

Answer 24

Compensatory polycythemia

Question 25

With cor pulmonale, drowsiness and altered mental status result due the retention of?

Answer 25

CO2

Question 26

What does the management of cor pulmonale focus on?

Answer 26

Treatment of lung disease and heart failure

Question 27

Chronic lung disease causes hypoxemia, which can result in pulmonary hypertension and polycythemia. What can you use to combat the hypoxemia that leads to these disorders with cor pulmonale?

Answer 27

Low-flow oxygen

Question 28

An alteration in the structure and function of the right ventricle caused by a primary disorder of the respiratory system.

Answer 28

Cor pulmonale

Question 29

_________ _________ is the common link between lung dysfunction and the heart in cor pulmonale.

Answer 29

Pulmonary hypertension

Question 30

What two diseases most often result in ACUTE cor pulmonale?

Answer 30

ARDS and PE

Question 31

Which disease most commonly results in cor pulmonale?

Answer 31

COPD

Question 32

What are other important disorders to consider before diagnosing someone with cor pulmonale?

Answer 32

Disorders that cause right sided ventricular failure other than cor pulmonale (right ventricular infarction, congestive heart failure, R side failure due to genetic diseases)

Ventricular septal defect

Question 33

What is the main principle in obtaining lab work to diagnose cor pulmonale?

Answer 33

To define the underlying cause as well as evaluate possible complications

Question 34

Why should you get an H+H when looking to diagnose cor pulmonale?

Answer 34

To check for polycythemia (can increase pulmonary arterial pressure bc of increased viscosity)

Question 35

Why would you want to get an ANA when looking to diagnose cor pulmonale?

Answer 35

To check for scleroderma and other collagen vascular diseases

Question 36

What is typically used for treatment for chronic cor pulmonale?

Answer 36

Diuretics

Calcium channel blockers

Question 37

What would you expect to see on CXR in a patient with cor pulmonale?

Answer 37

RV and proximal pulmonary artery enlargement with distal arterial attenuation (reduction of density)

Question 38

What is the best way to diagnose cor pulmonale?

Answer 38

Echocardiography: Evalues LV and RV function; echocardiography can assess RV systolic pressure but is often technically limited by the lung disorder