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Dr. Bullan PAH Respi Exam 2

Total questions: 71

Worksheet time: 7hrs 48mins

Name
Class
Date
1.

Which of the following factors are considered to be classified as Group I (PAH)? Select all that apply

a)

Congenital Heart Disease

b)

Idiopathic or Heritable

c)

Drugs induced and Portal Hypertension

d)

HIV

e)

Connective tissue disease

2.

 

Which of the following factors are considered to be classified as Group II (Left-Sided Heart Disease)? Select all that apply

a)

Valvular heart disease

b)

Heart failure with preserved/reduced LVEF

c)

Congenital/acquired cardiovascular conditions leading to post-capillary PH

d)

HIV

e)

Drug induced

3.

Which of the following factors are considered to be classified as Group III (Lung Disease/Hypoxia)? Select all that apply

a)

Hypoxia

b)

COPD and interstitial lung disease

c)

Sleep disorders

d)

exposure to high altitude

e)

Connective tissue disease

4.

Which of the following factors are considered to be classified as Group IV (Chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions)? Select all that apply

a)

CTEPH

b)

Pulmonary artery obstructions

c)

Obstructive disorders (angiosarcoma , intravascular tumors, congenital pulmonary arteries stenosis, parasites (hydatidosis)

d)

COPD

e)

Hypoxia

5.

Which of the following factors are considered to be classified as Group V (Unclear/multifactorial mechanisms)? Select all that apply

a)

Hematological disorders

b)

Tumoral

c)

Metabolic disorders

d)

Systemic disorders

e)

Complex congenital heart disease

6.

Which of the following is TRUE about the Pathophysiology of Group 1?

a)

Progressive vasoconstriction– left ventricular hypertrophy

b)

Progressive vasoconstriction– right ventricular hypertrophy

c)

Progressive vasodilation– right ventricular atrophy

d)

Intermittent vasoconstriction– left ventricular hypertrophy

7.

Which of the following drugs induce or cause PAH? Select all that apply

a)

Anorexigens

b)

aminorex

c)

fenfluramine

d)

benfluorex

e)

dexfenfluramine

8.

Which of the following are the symptoms for patients with PAH? Select all that apply

a)

Weakness AND Lower extremity edema

b)

Exertional dyspnea AND Complaints of general exertion intolerance

c)

Fatigue

d)

Exertional chest pain AND Syncope

e)

Dyspnea at rest as the disease progresses

9.

Which of the following diagnostic tests is NON INVASIVE to asses the need for oxygen therapy, patient's heart rate and oxygen level measured by "pulse oximeter" are recorded at the beginning of test and again at the end of 6 minutes?

a)

Echocardiogram (echo)

b)

Six Minute Walk (6MW)

c)

Pulmonary function tests (PFTs)

d)

Spirometry

e)

Diffusion capacity

10.

Which of the following diagnostics tests is a Non-invasive ultrasound test of the heart size and function + interaction of different heart chambers and valves.

a)

Echocardiogram (echo)

b)

Six Minute Walk (6MW)

c)

Spirometry

d)

Pulmonary function tests (PFTs)

e)

Lung volumes

11.

Which of the following diagnostic tests measure the lung ability in total to breathe in and out, and may reflect how "restricted" a patient's breathing is?

a)

Lung Volumes

b)

Spirometry

c)

Pulmonary function tests (PFTs)

d)

Diffusion capacity (DLCO)

e)

Echocardiogram (echo)

12.

Which of the following diagnostic test measures how fast a patient can breathe out?

a)

Spirometry

b)

Lung volumes

c)

Diffusion capacity

d)

Pulmonary function tests (PFTs)

e)

Six Minute Walk (6MW)

13.

Which of the following diagnostic tests reflects the extent of the damage to the blood vessels in the lungs?

a)

Lung Volumes

b)

Diffusion capacity (DLCO)

c)

Spirometry

d)

Pulmonary function tests (PFTs)

e)

Echocardiogram (echo)

14.

Which of the following is the gold standard for diagnosis of PAH used to assess pulmonary vasoreactivity prior to initiating therapy (INVASIVE)?

a)

Right Heart Catheterization

b)

Spirometry

c)

Lung volumes

d)

Diffusion capacity (DLCO)

e)

Pulmonary function tests (PFTs)

15.

Which of the following testing would I perform to check if patient qualifies for a CCB (Calcium Channel Bocker) in group 1 and group 2?

a)

Right Heart Catheterization

b)

Vasoreactivity testing

c)

Spirometry

d)

Lung Volumes

e)

Pulmonary function tests (PFTs)

16.

Which of the following CCB are the recommended ones? Select all that apply

a)

Diltiazem

b)

Amlodipine

c)

Nifedipine

d)

Verapamil

17.

Know this!

a)

Yes

b)

No

18.

Which of the following statements is TRUE about when do we use Calcium Channel Blockers?

a)

We want to use CCB when patient is Group 1 (PAH) and Class 2 with a positive vasoreactivity testing.

b)

We want to use CCB when patient is Group 2 (PAH) and Class 3 with a negative vasoreactivity testing.

c)

We want to use CCB when patient is Group 3 (PAH) and Class 3 with a positive vasoreactivity testing.

d)

We want to use CCB when patient is Group 3 (PAH) and Class 4 with a positive vasoreactivity testing.

19.

A 32 year old woman presents to your office with a PMHx of congenital heart disease and diabetes. She complains of mild limitation of activity, lower extremity edema, and fatigue. Regular physical activity causes increased dyspnea, chest pain and fatigue. Vasoreactivity testing is negative.

What group of PH does this patient have?

a)

Group 1

b)

Group 2

c)

Group 3

d)

Group 4

e)

Group 5

20.

A 32 year old woman presents to your office with a PMHx of congenital heart disease and diabetes. She complains of mild limitation of activity, lower extremity edema, and fatigue. Regular physical activity causes increased dyspnea, chest pain and fatigue. Vasoreactivity testing is negative.

What WHO functional Classification does this patient fall under?

a)

Class 1

b)

Class 2

c)

Class 3

d)

Class 4

21.

A 32 year old woman presents to your office with a PMHx of congenital heart disease and diabetes. She complains of mild limitation of activity, lower extremity edema, and fatigue. Regular physical activity causes increased dyspnea, chest pain and fatigue. Vasoreactivity testing is negative.

Is this patient a candidate for CCBs?

a)

Yes, she can use Amlodipine, Nifedipine or Diltiazem

b)

Yes, she can use Verapamil

c)

No, this patient is not a candidate for CCB because she has a negative testing result.

22.

Which of the following are considered supportive measures/therapy? Select all that apply

a)

Oxygen

b)

Loops diuretics

c)

Digoxin

d)

Warfarin

e)

Cilostazol

23.

Which of the following is the anticoagulant of choice as supportive therapy?

a)

Rivaroxaban

b)

Apixaban

c)

Warfarin

d)

Dabigatran

e)

Edoxaban

24.

Which of the following is considered the drug of choice to manage thrombosis risk associated with disease to normalize the ratio to 1.5-2.5?

a)

Edoxaban

b)

Warfarin

c)

Apixaban

d)

Rivaroxaban

25.

Which of the Following is TRUE regarding Digoxin used as supportive therapy? Select all that apply

a)

monitor potassium levels and renal function

b)

Increase cardiac output for patients with right ventricular heart failure

26.

Which of the following do we use to manage volume overload and volume depletion must be monitored (dehydration)?

a)

oxygen

b)

diuretics

c)

digoxin

d)

warfarin

27.

Which of the following can be used in some patients who PaO2 is low (less than 60 mmHg) and PAH patients with resting, exercise-induced, or nocturnal hypoxemia?

a)

Oxygen

b)

Digoxin

c)

Diuretics

d)

Warfarin

28.

What is the goal oxygen saturation?

a)

greater than 60%

b)

greater than 90%

c)

less than 90%

d)

less than 75%

29.

Know this acronym!

a)

Yes

b)

No

30.

Which of the following are TRUE regarding Dihydropyridine CCBs use/dosing? Select all that apply

a)

CCB therapy can be initiated as an outpatient with either long-acting nifedipine (30 mg/day) or diltiazem (120 mg/day), which is then increased to the maximal tolerated dose over days to weeks

b)

Nifedipine

Long-acting

(30 mg/day)

c)

Diltiazem ER

(120 mg/day)

d)

Reassessed after three to six months of treatment

31.

When should be reassessed a patient who initiates an outpatient tx of CCB increased to the maximal tolerated dose?

a)

In a year of treatment

b)

three to six months of treatment

c)

Within 1 week of treatment

d)

In 12 months of treatment

32.

Which of the following are the ADRS for Amlodipine? Select all that apply

a)

Gingival hyperplasia

b)

Hyperthyroidism

33.

Which of the following are TRUE regarding Amlodipine? Select all that apply

a)

ADRS: Peripheral edema, Gingival hyperplasia, Flushing, Headache

b)

Preferred in patients with bradycardia (HR<60BPM)

c)

Monitor: Heart rate and blood pressure

d)

Starting: 2.5mg PO daily- titrate weekly

Maintenance: 10-30mg PO daily

e)

ADRs: Sinus bradycardia, AV block, Maculopapular rash, SJS/TEN, Peripheral edema, Bradycardia, Hypotension

34.

Which of the following drugs is preferred in patient with Tachycardia and must be avoided in heart failure patients ?

a)

Amlodipine

b)

Diltiazem

c)

Nifedipine

35.

Which of the following are the ADRs for Diltiazem and monitor liver function test? Select all that apply

a)

Sinus bradycardia, Bradycardia, Hypotension

b)

Maculopapular rash

c)

SJS/TEN

d)

AV block

e)

Peripheral edema

36.

Which of the following drugs are preferred in patients with bradycardia (HR<60BPM)? Select all that apply

a)

Verapamil

b)

Amlodipine

c)

Diltiazem

d)

Nifedipine

37.

Which of the following are the ADRs for Nifedipine which is preferred for Bradychardia? Select all that apply

a)

asthenia (weakness)

b)

Peripheral edema

c)

flushing

38.

Which of the following drugs I have to monitor symptoms of heart failure and asthenia (Weakness)?

a)

Nifedipine

b)

Amlodipine

c)

Diltiazem

d)

Verapamil

39.

What is one contraindication of Revatio (Sildenafil) due to increased risk of hypotension?

a)

Nitrates and Riociguat

b)

Bosentan

40.

When using Revatio 20mg three times a day (Sildenafil) we must be cautious of which of the following?

a)

Amlodipine, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.

b)

Bosentan, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.

c)

Diltiazem, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.

d)

Nifedipine, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.

41.

Which of the following is a potent CYP3A4 inducer that can decrease Sildenafil concentrations by 50%?

a)

Riociguat

b)

Amlodipine

c)

Bosentan

d)

Diltiazem

e)

Nifedipine

42.

Which of the following are the ADRs for Sildenafil (Revatio)? Select all that apply

a)

epistaxis

b)

dyspepsia

c)

systemic hypotension

43.

Which of the following are TRUE about Tadalafil (Adcirca)? Select all that apply

a)

40 mg orally once daily

with or without food

b)

20 mg orally three times daily

c)

Contraindicated with nitrates and Riociguat, and be cautious with Bosentan because it decreases Tadalafil concentration by 50%

d)

ADRs: vision changes, dyspepsia, headache, myalgia, and flushing

44.

What BLACK BOX WARNING is associated with the Tracleer Access Program? Select all that apply

a)

Risk of hepatotoxicity

b)

Renal toxicity

c)

Risk of embryo-fetal toxicity

d)

Helps to promote fertilization in women

e)

Risk of hematologic disorders

45.

Which of the following are ADRs for Bosentan (Tracleer) ? Select all that apply

a)

teratogenic

b)

liver toxicity

c)

anemia

d)

edema

e)

renal disorders

46.

What is the starting/maintenance dose for Bosentan (Tracleer) under the REMS program in which prescribers, patients and pharmacies must enroll?

a)

start 22.5 mg orally twice daily and maintain 25 mg orally twice daily

b)

start 62.5 mg orally twice daily and maintain 125 mg orally twice daily

c)

start 12.5 mg orally twice daily and maintain 25 mg orally twice daily

d)

start 75 mg orally twice daily and maintain 150 mg orally twice daily

47.

Which of the following is the MOA for Ambrisentan (Letairis) ?

a)

Dual ETA and ETB receptor antagonist that improves exercise capacity, functional class, hemodynamics, echocardiographic and Doppler variables, and time to clinical worsening

b)

Selective ETA receptor antagonist that improves exercise capacity and hemodynamics and delays clinical worsening in PAH

c)

Dual, nonselective (ETA and ETB) antagonists

48.

Which of the following Endothelin Receptor Antagonists it distributed in specialty pharmacies and women who enrolls in the program must be tested child bearing age and monthly during treatment?

a)

Bosenton (Tracleer)

b)

Ambrisentan (Letairis)

c)

Macitentan (Opsumit)

49.

Which of the following are TRUE regarding Ambrisentan (Letairis)? Select all that apply

a)

5 mg orally daily and titrate to 10 mg orally daily

b)

REMS: Letairis Education and Access Program limiting to specialty pharmacies only

c)

ADRs: teratogenic, peripheral edema, nasal congestion, flushing, anemia, and palpitations

d)

Woman of child bearing age tested before, and monthly during treatment

e)

Risk of Hepatoxicity

50.

Which of the following Endothelin Receptor Antagonistsis dual, nonselective (ETA and ETB) antagonist dosed 10mg by mouth daily (NO TRITRATION)?

a)

Macitentan (Opsumit)

b)

Ambrisentan (Letairis)

c)

Bosentan (Tracleer)

51.

Which of the following are TRUE regarding Macitentan (Opsumit)? Select all that apply

a)

dual, nonselective (ETA and ETB) antagonist

b)

10mg by mouth daily (no titration)

c)

ADRs: nasopharyngitis, headache, anemia, edema

d)

Tissue selectivity and increased lipophilicity relative to others

e)

Opsumit Risk Evaluation and Mitigation Strategy Program states: Program is for women only

Embryo-fetal toxicity

Monthly pregnancy tests

52.

Which of the following are Prostacyclin Analogs? Select all that apply

a)

Treprostinil

b)

Epoprostenol

c)

Iloprost

d)

Macitentan

e)

Ambrisentan

53.

Which of the following drug classes acts on prostacyclin receptors to cause vasodilation, inhibition of platelet aggregation, and has antiproliferative properties?

a)

Endothelin Receptor Antagonists

b)

Prostacyclins- Analogs

c)

Prostacyclin IP Receptor Agonist

d)

Guanylate Cyclase Stimulator

54.

Which of the following Prostacyclins-Treprostinil is formulated as an IV/SC dosage form?

a)

Remodulin

b)

Tyvaso

c)

Orenitram

55.

Which of the following Prostacyclins-Treprostinil is formulated as an Inhaled dosage form?

a)

Remodulin

b)

Tyvaso

c)

Orenitram

56.

Which of the following Prostacyclins-Treprostinil is formulated as an Oral dosage form and CANNOT be stopped abruptly?

a)

Remodulin

b)

Tyvaso

c)

Orenitram

57.

Which of the following are the ADRs for Prostacyclins-Treprostinil (Remodulin, Tyvaso, Orenitram) ? Select all that apply

a)

flushing, hypotension

b)

cough (inhalation dosage form = Tyvaso)

c)

nausea, vomiting, diarrhea

d)

jaw pain

e)

risk of bloodstream infection

58.

Which of the following are TRUE regarding Epoprostenol? Select all that apply

a)

Brand: Flolan, Veletri

b)

Administered continuously via infusion pump through central venous catheter

c)

Short half life so requires a backup medication cassette and pump to avoid interruption in therapy, which can lead to rebound PH

d)

ADRs: jaw pain, diarrhea, flushing, and arthralgias

e)

This is the only prostacyclin that IMPROVED SURVIVAL

59.

Which of the following are the ADRs for Iloprost (Ventavis)? Select all that apply

a)

cough

b)

throat irritation

c)

jaw pain

60.

Which of the following is the drug class for Selexipag (UPTRAVI)?

a)

Prostacyclin Analogs

b)

Prostacyclin IP Receptor Agonist

c)

Endothelin Receptor Antagonists

d)

Guanylate Cyclase Stimulator

61.

Which of the following are true regarding Selexipag (Uptravi)?

a)

Treatment interruptions greater than 3 days require re-titration

b)

ADRs: flushing, headache, diarrhea, nausea, jaw pain, hyperthyroidism and myalgias

c)

Starting: 200 mcg orally twice daily with max dose 1,600 mcg orally twice daily

d)

Agonizing the prostacyclin IP receptor coupled with Gs protein, leading to increased cyclic adenosine monophosphate and relaxation of vascular smooth muscle

e)

CI: PDE5 inhibitors and nitrates

62.

Which of the following drugs requires re-titration if treatment interruptions greater than 3 days?

a)

Iloprost (Ventavis)

b)

Selexipag (Uptravi)

c)

Epoprostenol (Flolan, Veletri)

d)

Treprostinil (Remodulin, Tyvaso, Orenitram)

e)

Riociguat (Adempas)

63.

Which of the following drugs acts in synergy with Nitric Oxide and directly stimulates sGC independent of Nitric Oxide availability?

a)

Epoprostenol

b)

Riociguat

c)

Iloprost

d)

Uptravi

e)

Remodulin

64.

Which of the following drugs has similar clinical effects as PDE5 inhibitors contraindicated with PDE5 inhibitors and nitrates?

a)

Selexipag (Uptravi)

b)

Riociguat (Adempas)

c)

Iloprost (Ventavis)

d)

Epoprostenol (Flolan, Veletri)

e)

Teprostinil (Remodulin, Tyvaso, Orenitram)

65.

Which of the following is the drug class for Riociguat (Adempas)?

a)

Prostacyclin IP Receptor Agonist

b)

Guanylate Cyclase Stimulator

c)

Prostacyclin Analogs

d)

Endothelin Receptor Antagonists

66.

Which of the following are the ADRSs for Riociguat (Adempas)? Select all that apply

a)

GERD

b)

major bleeding

67.

A 67 year old man present to your hospital complaining of fatigue and is unable to perform any physical activity at rest. Dyspnea is also present at rest, and symptoms are increased by almost any physical activity. Patient has a past medical history of Pulmonary Hypertension group 1. Patient had negative vasoreactive testing. In the past he had success with Revatio but his symptoms are worsening.

What WHO Functional Classification does this patient fall under?

a)

Class 2

b)

Class 4

c)

Class 2

d)

Class 3

68.

A 67 year old man present to your hospital complaining of fatigue and is unable to perform any physical activity at rest. Dyspnea is also present at rest, and symptoms are increased by almost any physical activity. Patient has a past medical history of Pulmonary Hypertension group 1. Patient had negative vasoreactive testing. In the past he had success with Revatio but his symptoms are worsening.

What drug/drugs would you recommend putting him on?

a)

Nifedipine

b)

Epoprostenol IV

c)

Diltiazem

d)

Digoxin

e)

Oxygen

69.

A 67 year old man present to your hospital complaining of fatigue and is unable to perform any physical activity at rest. Dyspnea is also present at rest, and symptoms are increased by almost any physical activity. Patient has a past medical history of Pulmonary Hypertension group 1. Patient had negative vasoreactive testing. In the past he had success with Revatio but his symptoms are worsening.

What would you do for this patient if he continued to fail therapies? Select all that apply

a)

Palliative care

b)

Consider lung transplant

c)

Balloon Septostomy

d)

Riociguat

e)

Teprostinil IV

70.

Know this group in specific.

a)

Yes

b)

No

71.

Supportive therapy is important for symptom management.

a)

True

b)

False