03c: Pulm HT

Question 1

Which pressure is measured to define Pulm HT?

Answer 1

Pulm artery P (over 25 mmHg at rest)

Question 2

Group 1 PAH, aka (X), includes which causes of PAH?

Answer 2

X = pulm arterial HT

1. Idiopathic (sporadic or heritable) PAH
2. PAH due to disease that targets small pulm arterioles

Question 3

Group 2 PH includes which causes of Pulm HT?

Answer 3

“Pulm venous HT”

PH due to:

1. LV dysfunction
2. L side valve disease

Question 4

Group 3 PH includes which causes of Pulm HT?

Answer 4

Pulm HT associated with disorders of respiratory system or hypoxemia

Question 5

Group 4 PH includes which causes of Pulm HT?

Answer 5

Pulm HT caused by chronic thromboembolic disease

Question 6

Group 5 PH includes which causes of Pulm HT?

Answer 6

Pulm HT from misc causes

Question 7

T/F: PAH (pulm arterial hypertension) is used exclusively for Group 1 classification.

Answer 7

True

Question 8

COPD patient presents with Pulm HT. The PH would fall into which group?

Answer 8

3

Question 9

Sarcoidosis patient presents with Pulm HT. The PH would fall into which group?

Answer 9

5 (sarcoid granulomas likely serve to extrinsically compress vasculature)

Question 10

List some of the many causes of PAH.

Answer 10

1. Idiopathic or Heritable
2. CVD
3. HIV or drug-induced
4. Portal HT
5. Shunts

Question 11

List some of the many causes of PH.

Answer 11

1. COPD
2. Obstructive sleep apnea
3. PE
4. Chronic thromboembolitic disease
5. L heart disease

Question 12

Patient with abnormal BMPR2 (bone morphogenetic protein receptor type II) is at high risk for:

Answer 12

PAH (heritable)

Question 13

Patient comes in for yearly physical and confides in you that she takes anorexigens (appetite suppressants). You must warn her that she’s at risk for:

Answer 13

Pulmonary arterial HT

Question 14

(PH/PAH) occurs in about (X) fraction of HIV patients.

Answer 14

PAH;

X = 1/200

Question 15

T/F: Cocaine/amphetamine use increases risk of developing PAH by 3 fold.

Answer 15

True

Question 16

(PH/PAH) in diabetic patient commonly due to (X), which puts them in Group (1/2/3/4/5).

Answer 16

PH;
X = LV dysfunction
2

Question 17

Endothelial dysfunction that leads to PH includes (increase/decrease) NO, prostacyclin, thromboxane, and endothelin-1 production.

Answer 17

Decrease: NO, prostacyclin
Increase: thromboxane, endothelin-1

Question 18

List the possible etiologies/causes for PAH

Answer 18

1. Vasomediator imbalance
2. Ion channel defect (impaired K channel)
3. Inflammation
4. Dysregulated angiogenesis

Question 19

Most patients with early PH present with (X) symptom(s). What is the rationale behind this?

Answer 19

Dyspnea on exertion, lethargy and fatigue;

Inability to increase CO

Question 20

As PHT progresses, (R/L) heart failure develops and which additional symptoms would patient present with?

Answer 20

RV;

1. Exertional chest pain and syncope
2. Peripheral edema

Question 21

HIV Patient presents with exertional dyspnea, cough and lethargy. You hear splitting of the (first/second) heart sound and are immediately worried he has (X).

Answer 21

Second;

X = Pulmonary hypertension

Question 22

Aside from BMPR2, genetic abnormalities in which proteins can predispose one to (X) disease?

Answer 22

X = PAH

1. Activin receptor-like kinase I
2. Serotonin receptors and transporters

Question 23

What would CXR look like in PHT? List the things you would look for.

Answer 23

Normal mainly

1. Pulm a (may be enlarged)
2. RV/RA (may be enlarged)

Question 24

List the things you’d look for on Echo when suspecting PHT. Star the MAIN finding you’re interested in.

Answer 24

1. RA/RV size/thickness
2. Mean Pulm a pressure*
3. LV, EF%

Question 25

(X) test for PHT has high false positive rate (30-40%) and MUST be confirmed with (Y) test.

Answer 25

X = Cardiac echo
Y = R heart cath

Question 26

Which PFT abnormalities are caused by PHT?

Answer 26

Decreased DLCO

Question 27

Prognosis of PH is worse in (younger/older) patient population.

Answer 27

Worse if age under 14 OR over 65

Question 28

Patient presents with symptoms of PHT. You would order a V/Q scan to evaluate him for (X) disease. Depending on the result, what additional test would you order?

Answer 28

X = chronic thromboembolic disease

Normal: no additional tests needed (accurately excludes this disease)
Abnormal: MUST confirm/define with pulmonary angiography

Question 29

List some blood tests performed in diagnostic evaluation of PHT.

Answer 29

1. HIV serology
2. Liver function tests (for portal HT)
3. Titers for CVDs (Antinuclear Ab, RF, Antineutrophil cytoplasmic Ab)

Question 30

(X) test is absolutely essential for confirming diagnosis of PH.

Answer 30

X = R heart cath

Question 31

Your 36 y.o. patient has PHT. What is the first crucial piece of therapeutic advice you give her?

Answer 31

DO NOT GET PREGNANT!!!

Question 32

List medical therapy/advice (not including drugs) for PHT.

Answer 32

1. Supplemental O2
2. Aerobic activity (as tolerated)
3. Avoid altitude

Question 33

List the three classes of agents that have been approved for treatment of PAH.

Answer 33

1. Prostacyclins
2. Endothelin receptor antagonists
3. PDE inhibitors (increase NO)