Pulmonary HTN

Question 1

Therapies (medications) used in treatment of patients with pulmonary arterial hypertension

Answer 1

Endothelin receptor antagonists

PDE-5 inhibitors (sildenafil, tadalafil)

Soluble guanylate cyclase (works on same pathway as PDE-5)

Prostacyclin analogs

Question 2

The three principle mediators of pulmonary arterial hypertension

Answer 2

Endothelin

Prostacyclin

Nitric oxide

Question 3

In pulmonary arterial hypertension what are the common changes to pulmonary vasculature?

Answer 3

Proliferation

Inflammation

Fibrosis

Hypertrophy

Question 4

Which tests should be negative to rule out WHO 4 in a workup for pulmonary arterial hypertension?

Answer 4

V/Q scan and/or CT if PE suspected

Question 5

After ruling out WHO 2, WHO 3, and WHO 4 in a patient with suspected pulmonary arterial hypertension, what is the next step?

Answer 5

Right heart catheterization

Obtain wedge pressure

Possible NO challenge

Question 6

Risk factors for pulmonary arterial hypertension

Answer 6

SCLERODERMA (or other connective tissue disorders)

AMPHETAMINE/METHAMPHETAMINE USE

HIV patients

Schistosomiasis

Liver disease, portal hypertension

Congenital/valve heart defects

Question 7

What might show on an EKG in a patient with pulmonary arterial hypertension?

Answer 7

Right ventricular hypertrophy/strain

Right atrial enlargement

Question 8

When assessing a patient for pulmonary arterial hypertension, what other condition can be ruled out with a negative V/Q scan?

Answer 8

CTEPH

Chronic thromboembolic pulmonary hypertension

Question 9

In a patient with scleroderma you may see normal PFTs and decreased … which would be suggestive of pulmonary arterial hypertension

Answer 9

Decreased DLCO

Diminished capacity for gas exchange

Question 10

Which three specialties work together in the treatment of pulmonary arterial hypertension?

Answer 10

Pulmonologists

Cardiologists

Rheumatologists

Question 11

Questions to ask when obtaining a history on patients with suspected pulmonary arterial hypertension

Answer 11

Have you ever smoked?

Do you have sleep apnea?

Question 12

First thing to look at and what to look for in diagnosis of pulmonary arterial hypertension

Answer 12

Look at echo

Look for left sided enlargement of heart. If normal, exclude WHO 2.

If right sided heart enlargement this is suggestive of PAH

Question 13

Three treatments used in general care (not therapy) of patients with pulmonary arterial hypertension

Answer 13

Anticoagulation

O2 (good vasodilator)

Fluid/volume control (diuretics, lower sodium intake)

Question 14

True of false. Pulmonary hypertension is a primary vascular issue

Answer 14

False

Pulmonary ARTERIAL hypertension is a primary vascular issue

Pulmonary hypertension could be a secondary result of many conditions

Question 15

Is a patient with class 1 or class 4 pulmonary arterial hypertension worse off?

Answer 15

Class 4 (symptoms at rest, the worst one)

(Class one is asymptomatic)

Question 16

What kind of stress test is used in the assessment of pulmonary arterial hypertension?

Answer 16

6 minute walk test

Question 17

Normal pulmonary blood pressure

Answer 17

30/12 ish

Question 18

MPAP and PAWP thresholds for PAHTN diagnosis

Answer 18

mPAP 25+ mmHg

PAWP 15 or less mmHg

(also PVR >3 wood units)

Question 19

If a patient being worked up for pulmonary arterial hypertension states that their symptoms came on suddenly, which test should be done and what would it rule out?

Answer 19

CT scan

To rule out pulmonary embolism

Question 20

Physical exam findings in pulmonary arterial hypertension

Answer 20

RHF symptoms

Leg edema, JVP, ascites