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WorksheetsDr. Bullan PAH Respi Exam 2
Total questions: 71
Worksheet time: 7hrs 48mins
Which of the following factors are considered to be classified as Group I (PAH)? Select all that apply
Congenital Heart Disease
Idiopathic or Heritable
Drugs induced and Portal Hypertension
HIV
Connective tissue disease
Which of the following factors are considered to be classified as Group II (Left-Sided Heart Disease)? Select all that apply
Valvular heart disease
Heart failure with preserved/reduced LVEF
Congenital/acquired cardiovascular conditions leading to post-capillary PH
HIV
Drug induced
Which of the following factors are considered to be classified as Group III (Lung Disease/Hypoxia)? Select all that apply
Hypoxia
COPD and interstitial lung disease
Sleep disorders
exposure to high altitude
Connective tissue disease
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
CTEPH
Pulmonary artery obstructions
Obstructive disorders (angiosarcoma , intravascular tumors, congenital pulmonary arteries stenosis, parasites (hydatidosis)
COPD
Hypoxia
Which of the following factors are considered to be classified as Group V (Unclear/multifactorial mechanisms)? Select all that apply
Hematological disorders
Tumoral
Metabolic disorders
Systemic disorders
Complex congenital heart disease
Which of the following is TRUE about the Pathophysiology of Group 1?
Progressive vasoconstriction– left ventricular hypertrophy
Progressive vasoconstriction– right ventricular hypertrophy
Progressive vasodilation– right ventricular atrophy
Intermittent vasoconstriction– left ventricular hypertrophy
Which of the following drugs induce or cause PAH? Select all that apply
Anorexigens
aminorex
fenfluramine
benfluorex
dexfenfluramine
Which of the following are the symptoms for patients with PAH? Select all that apply
Weakness AND Lower extremity edema
Exertional dyspnea AND Complaints of general exertion intolerance
Fatigue
Exertional chest pain AND Syncope
Dyspnea at rest as the disease progresses
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?
Echocardiogram (echo)
Six Minute Walk (6MW)
Pulmonary function tests (PFTs)
Spirometry
Diffusion capacity
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.
Echocardiogram (echo)
Six Minute Walk (6MW)
Spirometry
Pulmonary function tests (PFTs)
Lung volumes
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?
Lung Volumes
Spirometry
Pulmonary function tests (PFTs)
Diffusion capacity (DLCO)
Echocardiogram (echo)
Which of the following diagnostic test measures how fast a patient can breathe out?
Spirometry
Lung volumes
Diffusion capacity
Pulmonary function tests (PFTs)
Six Minute Walk (6MW)
Which of the following diagnostic tests reflects the extent of the damage to the blood vessels in the lungs?
Lung Volumes
Diffusion capacity (DLCO)
Spirometry
Pulmonary function tests (PFTs)
Echocardiogram (echo)
Which of the following is the gold standard for diagnosis of PAH used to assess pulmonary vasoreactivity prior to initiating therapy (INVASIVE)?
Right Heart Catheterization
Spirometry
Lung volumes
Diffusion capacity (DLCO)
Pulmonary function tests (PFTs)
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?
Right Heart Catheterization
Vasoreactivity testing
Spirometry
Lung Volumes
Pulmonary function tests (PFTs)
Which of the following CCB are the recommended ones? Select all that apply
Diltiazem
Amlodipine
Nifedipine
Verapamil
Know this!
Yes
No
Which of the following statements is TRUE about when do we use Calcium Channel Blockers?
We want to use CCB when patient is Group 1 (PAH) and Class 2 with a positive vasoreactivity testing.
We want to use CCB when patient is Group 2 (PAH) and Class 3 with a negative vasoreactivity testing.
We want to use CCB when patient is Group 3 (PAH) and Class 3 with a positive vasoreactivity testing.
We want to use CCB when patient is Group 3 (PAH) and Class 4 with a positive vasoreactivity testing.
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?
Group 1
Group 2
Group 3
Group 4
Group 5
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?
Class 1
Class 2
Class 3
Class 4
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?
Yes, she can use Amlodipine, Nifedipine or Diltiazem
Yes, she can use Verapamil
No, this patient is not a candidate for CCB because she has a negative testing result.
Which of the following are considered supportive measures/therapy? Select all that apply
Oxygen
Loops diuretics
Digoxin
Warfarin
Cilostazol
Which of the following is the anticoagulant of choice as supportive therapy?
Rivaroxaban
Apixaban
Warfarin
Dabigatran
Edoxaban
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?
Edoxaban
Warfarin
Apixaban
Rivaroxaban
Which of the Following is TRUE regarding Digoxin used as supportive therapy? Select all that apply
monitor potassium levels and renal function
Increase cardiac output for patients with right ventricular heart failure
Which of the following do we use to manage volume overload and volume depletion must be monitored (dehydration)?
oxygen
diuretics
digoxin
warfarin
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?
Oxygen
Digoxin
Diuretics
Warfarin
What is the goal oxygen saturation?
greater than 60%
greater than 90%
less than 90%
less than 75%
Know this acronym!
Yes
No
Which of the following are TRUE regarding Dihydropyridine CCBs use/dosing? Select all that apply
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
Nifedipine
Long-acting
(30 mg/day)
Diltiazem ER
(120 mg/day)
Reassessed after three to six months of treatment
When should be reassessed a patient who initiates an outpatient tx of CCB increased to the maximal tolerated dose?
In a year of treatment
three to six months of treatment
Within 1 week of treatment
In 12 months of treatment
Which of the following are the ADRS for Amlodipine? Select all that apply
Gingival hyperplasia
Hyperthyroidism
Which of the following are TRUE regarding Amlodipine? Select all that apply
ADRS: Peripheral edema, Gingival hyperplasia, Flushing, Headache
Preferred in patients with bradycardia (HR<60BPM)
Monitor: Heart rate and blood pressure
Starting: 2.5mg PO daily- titrate weekly
Maintenance: 10-30mg PO daily
ADRs: Sinus bradycardia, AV block, Maculopapular rash, SJS/TEN, Peripheral edema, Bradycardia, Hypotension
Which of the following drugs is preferred in patient with Tachycardia and must be avoided in heart failure patients ?
Amlodipine
Diltiazem
Nifedipine
Which of the following are the ADRs for Diltiazem and monitor liver function test? Select all that apply
Sinus bradycardia, Bradycardia, Hypotension
Maculopapular rash
SJS/TEN
AV block
Peripheral edema
Which of the following drugs are preferred in patients with bradycardia (HR<60BPM)? Select all that apply
Verapamil
Amlodipine
Diltiazem
Nifedipine
Which of the following are the ADRs for Nifedipine which is preferred for Bradychardia? Select all that apply
asthenia (weakness)
Peripheral edema
flushing
Which of the following drugs I have to monitor symptoms of heart failure and asthenia (Weakness)?
Nifedipine
Amlodipine
Diltiazem
Verapamil
What is one contraindication of Revatio (Sildenafil) due to increased risk of hypotension?
Nitrates and Riociguat
Bosentan
When using Revatio 20mg three times a day (Sildenafil) we must be cautious of which of the following?
Amlodipine, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.
Bosentan, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.
Diltiazem, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.
Nifedipine, a potent CYP3A4 inducer can decrease Sildenafil concentrations by 50%.
Which of the following is a potent CYP3A4 inducer that can decrease Sildenafil concentrations by 50%?
Riociguat
Amlodipine
Bosentan
Diltiazem
Nifedipine
Which of the following are the ADRs for Sildenafil (Revatio)? Select all that apply
epistaxis
dyspepsia
systemic hypotension
Which of the following are TRUE about Tadalafil (Adcirca)? Select all that apply
40 mg orally once daily
with or without food
20 mg orally three times daily
Contraindicated with nitrates and Riociguat, and be cautious with Bosentan because it decreases Tadalafil concentration by 50%
ADRs: vision changes, dyspepsia, headache, myalgia, and flushing
What BLACK BOX WARNING is associated with the Tracleer Access Program? Select all that apply
Risk of hepatotoxicity
Renal toxicity
Risk of embryo-fetal toxicity
Helps to promote fertilization in women
Risk of hematologic disorders
Which of the following are ADRs for Bosentan (Tracleer) ? Select all that apply
teratogenic
liver toxicity
anemia
edema
renal disorders
What is the starting/maintenance dose for Bosentan (Tracleer) under the REMS program in which prescribers, patients and pharmacies must enroll?
start 22.5 mg orally twice daily and maintain 25 mg orally twice daily
start 62.5 mg orally twice daily and maintain 125 mg orally twice daily
start 12.5 mg orally twice daily and maintain 25 mg orally twice daily
start 75 mg orally twice daily and maintain 150 mg orally twice daily
Which of the following is the MOA for Ambrisentan (Letairis) ?
Dual ETA and ETB receptor antagonist that improves exercise capacity, functional class, hemodynamics, echocardiographic and Doppler variables, and time to clinical worsening
Selective ETA receptor antagonist that improves exercise capacity and hemodynamics and delays clinical worsening in PAH
Dual, nonselective (ETA and ETB) antagonists
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?
Bosenton (Tracleer)
Ambrisentan (Letairis)
Macitentan (Opsumit)
Which of the following are TRUE regarding Ambrisentan (Letairis)? Select all that apply
5 mg orally daily and titrate to 10 mg orally daily
REMS: Letairis Education and Access Program limiting to specialty pharmacies only
ADRs: teratogenic, peripheral edema, nasal congestion, flushing, anemia, and palpitations
Woman of child bearing age tested before, and monthly during treatment
Risk of Hepatoxicity
Which of the following Endothelin Receptor Antagonistsis dual, nonselective (ETA and ETB) antagonist dosed 10mg by mouth daily (NO TRITRATION)?
Macitentan (Opsumit)
Ambrisentan (Letairis)
Bosentan (Tracleer)
Which of the following are TRUE regarding Macitentan (Opsumit)? Select all that apply
dual, nonselective (ETA and ETB) antagonist
10mg by mouth daily (no titration)
ADRs: nasopharyngitis, headache, anemia, edema
Tissue selectivity and increased lipophilicity relative to others
Opsumit Risk Evaluation and Mitigation Strategy Program states: Program is for women only
Embryo-fetal toxicity
Monthly pregnancy tests
Which of the following are Prostacyclin Analogs? Select all that apply
Treprostinil
Epoprostenol
Iloprost
Macitentan
Ambrisentan
Which of the following drug classes acts on prostacyclin receptors to cause vasodilation, inhibition of platelet aggregation, and has antiproliferative properties?
Endothelin Receptor Antagonists
Prostacyclins- Analogs
Prostacyclin IP Receptor Agonist
Guanylate Cyclase Stimulator
Which of the following Prostacyclins-Treprostinil is formulated as an IV/SC dosage form?
Remodulin
Tyvaso
Orenitram
Which of the following Prostacyclins-Treprostinil is formulated as an Inhaled dosage form?
Remodulin
Tyvaso
Orenitram
Which of the following Prostacyclins-Treprostinil is formulated as an Oral dosage form and CANNOT be stopped abruptly?
Remodulin
Tyvaso
Orenitram
Which of the following are the ADRs for Prostacyclins-Treprostinil (Remodulin, Tyvaso, Orenitram) ? Select all that apply
flushing, hypotension
cough (inhalation dosage form = Tyvaso)
nausea, vomiting, diarrhea
jaw pain
risk of bloodstream infection
Which of the following are TRUE regarding Epoprostenol? Select all that apply
Brand: Flolan, Veletri
Administered continuously via infusion pump through central venous catheter
Short half life so requires a backup medication cassette and pump to avoid interruption in therapy, which can lead to rebound PH
ADRs: jaw pain, diarrhea, flushing, and arthralgias
This is the only prostacyclin that IMPROVED SURVIVAL
Which of the following are the ADRs for Iloprost (Ventavis)? Select all that apply
cough
throat irritation
jaw pain
Which of the following is the drug class for Selexipag (UPTRAVI)?
Prostacyclin Analogs
Prostacyclin IP Receptor Agonist
Endothelin Receptor Antagonists
Guanylate Cyclase Stimulator
Which of the following are true regarding Selexipag (Uptravi)?
Treatment interruptions greater than 3 days require re-titration
ADRs: flushing, headache, diarrhea, nausea, jaw pain, hyperthyroidism and myalgias
○Starting: 200 mcg orally twice daily with max dose 1,600 mcg orally twice daily
Agonizing the prostacyclin IP receptor coupled with Gs protein, leading to increased cyclic adenosine monophosphate and relaxation of vascular smooth muscle
CI: PDE5 inhibitors and nitrates
Which of the following drugs requires re-titration if treatment interruptions greater than 3 days?
Iloprost (Ventavis)
Selexipag (Uptravi)
Epoprostenol (Flolan, Veletri)
Treprostinil (Remodulin, Tyvaso, Orenitram)
Riociguat (Adempas)
Which of the following drugs acts in synergy with Nitric Oxide and directly stimulates sGC independent of Nitric Oxide availability?
Epoprostenol
Riociguat
Iloprost
Uptravi
Remodulin
Which of the following drugs has similar clinical effects as PDE5 inhibitors contraindicated with PDE5 inhibitors and nitrates?
Selexipag (Uptravi)
Riociguat (Adempas)
Iloprost (Ventavis)
Epoprostenol (Flolan, Veletri)
Teprostinil (Remodulin, Tyvaso, Orenitram)
Which of the following is the drug class for Riociguat (Adempas)?
Prostacyclin IP Receptor Agonist
Guanylate Cyclase Stimulator
Prostacyclin Analogs
Endothelin Receptor Antagonists
Which of the following are the ADRSs for Riociguat (Adempas)? Select all that apply
GERD
major bleeding
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?
Class 2
Class 4
Class 2
Class 3
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?
Nifedipine
Epoprostenol IV
Diltiazem
Digoxin
Oxygen
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
Palliative care
Consider lung transplant
Balloon Septostomy
Riociguat
Teprostinil IV
Know this group in specific.
Yes
No
Supportive therapy is important for symptom management.
True
False
