Pulmonary HTN & Cor Pulmonale

Question 1

Define pulm HTN (PAH)

Answer 1

• increased pulm a. pressure –> RV hypertrophy –> R side heart failure

Question 2

What does the right ventricle best respond to?

Answer 2

• volume changes/expansion

Question 3

Define Group 1 PAH

Answer 3

• increased pulm a. pressure with normal capillary wedge pressure

Question 4

Define Group 2 PAH

Answer 4

• increased pulm a. pressure & pulm capillary wedge pressure

Question 5

What is also seen in Group 2 PAH?

Answer 5

• left sided atrial or ventricular heart disease

- left sided valvular disease

Question 6

Define Group 3 PAH?

Answer 6

• chronic hypoxia with mild increase of pulm a. pressure

Question 7

Define Group 4 PAH?

Answer 7

• elevated pulm a. pressure with documented pulm a. obstruction for > 3mo

Question 8

Define Group 5 PAH?

Answer 8

• elevated pulm a. pressure in association with systemic disease & unclear mechanism

Question 9

What are the symptoms of PAH?

Answer 9

• dyspnea w/ exertion
• chest pain
• fatigue
• lethargy
• weakness
• syncope

Question 10

What labs should be drawn for PAH and what does it show?

Answer 10

• CBC: polycythemia 2ndary to chronic hypoxia

Question 11

What diagnostic studies should be ordered for PAH?

Answer 11

• CXR
• EKG
• Echo

Question 12

What will a CXR show in PAH?

Answer 12

• enlarged pulmonary a.

Question 13

What will an EKG show in PAH?

Answer 13

• RVH
• right atrial hypertrophy
• RV strain

Question 14

What will an Echo show in PAH?

Answer 14

• ejection fraction

Question 15

What is the 1 definitive diagnostic test for PAH?

Answer 15

• right heart cath

Question 16

What is the treatment for Group 1 PAH?

Answer 16

• Ca+2 channel blockers
• endothelial receptor antagonists
• phosphodiesterase-5 inhibitors
• prostacyclines
• lung transplant

Question 17

What is included in the group of prostacyclines used to treat Group 1 PAH?

Answer 17

• iloprost
• epopprostenol
• preprostinil

Question 18

What is the treatment for Group 2-5 PAH?

Answer 18

• tx underlying dz

Question 19

What is the f/u for PAH?

Answer 19

• q 3mo w/ labs prior to appt

Question 20

Define cor pulmonale

Answer 20

• alteration in the structure & function of the RV caused by a primary disorder of the pulmonary system

Question 21

What causes cor pulmonale?

Answer 21

• MC: PAH

Question 22

What are the symptoms of chronic cor pulmonale related to?

Answer 22

• underlying dz

Question 23

What are the S&S of cor pulmonale?

Answer 23

• dyspnea
• chronic productive cough
• wheezing
• fatigue/weakness
• orthopnea & PND
• LE edema

Question 24

What is seen on PE in cor pulmonale?

Answer 24

• increased JVP
• V wave in JVP
• tachypnea
• RV palpable heave
• decreased ejection fraction
• hepatomegaly
• LE edema
• cyanosis & clubbing

Question 25

What is the gold standard for diagnosing cor pulmonale?

Answer 25

- right heart cath

Question 26

What are the other diagnostic tests for cor pulmonale?

Answer 26

- echo *intitial test* - CXR - PFTs - spirometry & lung volumes - ABG - CT - V/Q scan

Question 27

What will be drawn for labs and what will be seen in cor pulmonale?

Answer 27

- CBC: polycythemia d/t hypoxia | - SaO2 less than or equal to 85%

Question 28

What will be seen on PFTs in cor pulmonale?

Answer 28

- frequently abnormal - MC: impaired diffusion capacity - restrictive ventilatory defect

Question 29

What is the treatment for cor pulmonale?

Answer 29

- tx underlying dz | - improve O2 & RV function by increasing RV contractility & decreasing pulmonary vasoconstriction

Question 30

How is cor pulmonale managed?

Answer 30

- salt restriction - smoking cessation - vasodilators - pt educations

Question 31

What are complications of cor pulmonale?

Answer 31

- syncope - hypoxia - pedal edema - passive hepatic congestion - death