Pulmonary HTN

Question 1

WHO grouping (5) of Pulm HTN

Answer 1

• Pulmonary arterial HTN
• Left heart disease
• Lung disease / hypoxia (COPD, ILD)
• Chronic thromboemboli (PE)
• Misc (sarcoidosis)

Question 2

The mean pulmonary artery pressure must be >___mmHg at rest to dx pulm HTN.

(ON EXAM)**

Answer 2

>25mmHg

Question 3

Which world health group?

• Pulm Arterial HTN secondary to various disorders
• Diseases that localize directly to the pulm arteries leading to structural changes, smooth muscle hypertrophy, & endothelial dysfunction

Answer 3

Group 1

Question 4

Which World Health group?

• Schistosomiasis
• Drugs / toxins
• HIV

Answer 4

Group 1

Question 5

Which drugs will DEFINITELY cause pulmonary HTN?

Answer 5

Appetite suppressants (aminorex, fenfluramine, dexfenfluramine)

Question 6

Which drugs/toxins will POSSIBLY be risk factors for pulm arterial HTN? (PAH)

Answer 6

• amphetamines
• L-tryptophan
• meth
• cocaine
• St. John’s Wort

Question 7

What drug is:

• associated w/ development of persistent pulm HTN of the newborn when taken by pregnant mothers?
• associated w/ poor prognosis in those established w/ PAH?

Answer 7

SSRI

Question 8

Which group?

• Pulmonary venous HTN secondary to left heart disease
• Often referred to as “pulmonary venous HTN” or “post capillary pulm HTN”

Answer 8

Group 2

Question 9

Which group?

• Pulmonary HTN secondary to lung disease or hypoxemia
• Caused by advanced obstructive and restrictive lung disease (COPD, ILD, fibrosis, bronchiectasis)

Answer 9

Group 3

Question 10

• What is shown here?
• Which group?

Answer 10

• PE (bilateral) probably from DVT
• group 4 (clotting/emboli/thrombo)

Question 11

Which group?

• Pulm HTN secondary to chronic thromboembolic occlusion of proximal and distal pulm arteries
• This classification no longer includes pts w/ non-thrombotic occlusion, such as tumors/foreign objects

Answer 11

Group 4

Question 12

Group 5 is pulm arterial HTN secondary to what 4 things?

Answer 12

• Hematologic disorders
• Metabolic disorders
• Systemic disorders (sarcoidosis)
• Misc (tumor embolization***)

Question 13

• Which organization classified severity of pulm HTN?
• What 2 things is severity based on?

Answer 13

• NYHA, and modified by WHO
• Sxs & functional status

Question 14

Which class of NYHA severity?

• without limitation of physical activity
• no dyspnea, fatigue, CP, or near syncope w/ exertion

Answer 14

Class 1

Question 15

Which class of NYHA severity?

• Slight limitation of physical activity
• no sxs at rest, but ordinary physical activity causes dyspnea, fatigue, CP, or near syncope

Answer 15

Class 2

Question 16

Which class of NYHA severity?

• “Marked” limitation of physical activity
• no sxs at rest, but LESS THAN ORDINARY activity causes sxs
• Pts usually present for tx with this class!

Answer 16

Class 3

Question 17

Which class of NYHA severity?

• Inability to perform any physical activity w/o sxs
• Evidence of right heart failure
• Dyspnea / Fatigue at rest and worsening of sxs w/ any activity

Answer 17

Class 4

Question 18

What is the #1 symptom of Pulm HTN?

Answer 18

Dyspnea on Exertion (DOE)

Question 19

• What murmur is heard w/ Pulm HTN?
• Where?
• Louder with what?

Answer 19

• Tricuspid regurg (holosystolic)
• heard along left parasternal line
• Louder w/ inspiration = Carvallo’s Sign

Question 20

ALL patients w/ Pulm HTN should be screened for what 2 things?

Answer 20

• HIV
• Collagen vascular disease

Question 21

Pts w/ idiopathic PAH often have normal PaO2 at rest, but show evidence of _____ with a ______ in PaCO2.

Answer 21

• Hyperventilation
• Decrease in PaCO2

Question 22

W/ echocardiography, Doppler flow can estimate what?

Answer 22

Right Ventricular Systolic Pressure (RVSP)

Question 23

***What is the gold standard test to dx PAH?**

Answer 23

Right sided cardiac catheterization

(RHC = right heart cath)

Question 24

Cardiac catheterization is helpful in differentiating _____ from ______ by assessment of drop in pressure across the pulmonary circulation also known as __________. ****

Answer 24

• Pulmonary arterial hypertension
• Pulmonary venous hypertension
• Transpulmonary gradient

Question 25

Are FVC and TLC on PFT testing elevated, low, or normal for a pt w/ pulm HTN?

Answer 25

Both normal

Question 26

What test differenitiates chronic thromboembolic pulmonary HTN from idiopathic pulmonary arterial HTN?

Answer 26

V/Q lung scan

(V=ventilation Q=perfusion)

Question 27

What is the most definitive diagnostic procedure for defining the distribution and extent of disease in chronic thromboembolic pulmonary HTN?

Answer 27

Pulmonary Angiography

Question 28

What diagnostic procedure is preferred for pts w/ renal failure bc the dye of angiography/CT will damage their kidneys?

Answer 28

Ventilation Quotient (V/Q) Scan

Question 29

What is first line therapy for Group 1?

Answer 29

Oral CCB such as Diltiazem or Nifedipine

Question 30

Group 1 tx***

• Oral CCB (Diltiazem or Nifedipine) should only be given to pts w/ ____ _____ _____ ______ bc they are harmful otherwise.
• (ON EXAM)

Answer 30

Positive Acute Vasodilator Response

Question 31

What do you need to treat for Group 2 pts?

Answer 31

Left Heart Failure

Question 32

Tx for Group 3 pts w/ hypoxemia

Answer 32

Supplemental Oxygen

Question 33

Tx for group 4 (3 things)

Answer 33

• Anticoagulants
• Thromboendarterectomy (special/rare surgery)
• Riociguat (DON’T GIVE TO PREGNANT WOMEN)

Question 34

Tx for group 5

Answer 34

Tx underlying etiology

Question 35

Which medication for PAH have a short medication half life requiring a reliable continuous infusion, difficulty in titration, and high cost of therapy?

(DO NOT suddently stop this med. Must titer or pt will die!)

(ON EXAM)

Answer 35

Endothelin Receptor Antagonist

Prostacyclins (Epoprostenol)