Pulmonary Vascular Disease Flashcards

1
Q

Which of these is a Endothelin receptor antagonist?

A) Ambrisentan
B) Tadalafil
C) Riociguat
D) Treprostinil
E) Selexipag

A

Ambrisentan

Other Endothelin receptor antagonist include: Bosentan, and Macitentan

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2
Q

What is the most common side effect of Abrisentan?

A) Abnormal LFT’s
B) Mood changes
C) Low Hb
D) Peripheral Odema
E) None of the above

A

Peripheral Oedema

“An increased incidence of peripheral oedema was reported with ambrisentan use, while there was no increased incidence of abnormal liver function.” - 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

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3
Q

Selexipag is what type of medication?

A) Oral Prostacyclin analogue
B) Nebulised Prostacyclin analogue
C) IV Prostacyclin analogue
D) SC Prostacyclin analogues
E) Oral Prostacyclin receptor agonist

A

Oral Prostacyclin receptor agonist

Selexipag is an orally available, selective, prostacyclin receptor agonist that is chemically distinct from prostacyclin, with different pharmacology.

Side effects: headache, diarrhoea, nausea, and jaw pain.

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

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4
Q

Which gene is most commonly found in cases of heritable pulmonary arterial hypertension?

A) BMPR2
B) EIF2AK4
C) GDF2
D) Sox17
E) KCNK3

A

BMPR2

“In the evolving list of genes known to be associated with PAH, experience is largely restricted to BMPR2 mutation carriers who carry a lifetime risk of developing PAH of ∼20%, with penetrance higher in female carriers (42%) compared with male carriers (14%).” - 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

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5
Q

What is the mode of inheritance of hereditary haemorrhagic telangiectasia (HHT)?

A) Autosomal Dominant
B) Autosomal Recessive
C) X-Linked Dominant
D) X- Linked Recessive
E) Mitochondrial

A

Autosomal Dominant

HHT is inherited as an autosomal dominant trait and most
commonly results from a pathogenic sequence variant in ENG encoding endoglin (HHT type 1/ HHT1), ACVRL1 encoding ALK-1 (HHT type 2/ HHT2) or SMAD4.

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6
Q

Which of these symptoms is classically described in hepatopulmonary syndrome (HPS)?

A) Orthopnea
B) Platypnoea
C) Bendopnoea
D) Palpitations
E) Angina

A

Platypnoea

Platypnoea and orthodeoxia, the increase in dyspnoea or deoxygenation while in the standing position, are classically described in HPS.

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7
Q

Which of these tests is commonly used to diagnose intravascular vasodilatation in HPS?

A) ECG
B) TTE
C) Contrast enhanced TTE
D) Myocardial perfusion scan
E) CPET

A

Contrast enhanced TTE (Bubble echo)

This opacifies the left atrium four or more beats after the initial appearance of contrast in the right atrium in patients with HPS, whereas those with a cardiac right-to-left shunt (i.e. atrial septal defect) will show opacification of the left atrium within the first three beats

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8
Q

Which of these Pulmonary Hypertension medications is teratogenic?

A) Calcium channel blockers
B) Endothelin receptor antagonists
C) Phosphodiesterase 5 inhibitors
D) Prostacyclin analogues

A

Endothelin receptor antagonists

Endothelial receptor antagonists have teratogenic effects and should not be used during pregnancy

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9
Q

Which of these is not used as vasoreactivity testing in PAH?

A) Inhaled Nitric Oxide
B) Inhaled Iloprost
C) IV Adenosine
D) IV Epoprostenol

A

IV Adenosine

Inhaled NO or inhaled iloprost, are the recommended test compounds. There is similar evidence for intravenous (i.v.) epoprostenol, but due to incremental dose increases and repetitive measurements, testing takes much longer and is therefore less feasible. Adenosine i.v. is no longer recommended due to frequent side effects.

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10
Q

Which of these is not an absolute contraindication for systemic thrombolysis in acute PE?

A)History of haemorrhagic stroke or stroke of unknown origin
B) Ischaemic stroke in previous 6 months
C) Central nervous system neoplasm
D) Major trauma, surgery, or head injury in previous 3 weeks
E) Non-compressible puncture sites

A

Non-compressible puncture sites

Absolute:

History of haemorrhagic stroke or stroke of unknown origin

Ischaemic stroke in previous 6 months

Central nervous system neoplasm

Major trauma, surgery, or head injury in previous 3 weeks

Bleeding diathesis

Active bleeding

Relative:

Transient ischaemic attack in previous 6 months

Oral anticoagulation

Pregnancy or first post-partum week

Non-compressible puncture sites

Traumatic resuscitation

Refractory hypertension (systolic BP >180 mmHg)

Advanced liver disease

Infective endocarditis

Active peptic ulcer

  • 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism
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11
Q

You review a 33 year old pregnant female in SDEC with a high suspicion of PE. Her CXR shows a patch of opacification on the right lower. She has no calf swelling or tenderness.

What is the most appropriate next investigation?

A) B/L leg dopplers
B) V/Q scan
C) CTPA
D) Treat as PE for 6 months only. No Investigations needed.
E) Echocardiogram

A

CTPA

When the chest X-ray is abnormal and there is a clinical suspicion of PE, CTPA should be performed in preference to a V/Q scan.

  • Thromboembolic Disease in Pregnancy and the Puerperium: Acute Management Green-top Guideline No. 37b April 2015
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12
Q

You diagnose a patient with PE. Her blood pressure is 120/80. Her saturations are 94% on 35% FiO2. Troponin is raised. CTPA shows bilateral PE with features of right heart strain?

What is her severity classification of PE?

A) High risk PE
B) Intermediate High risk PE
C) Intermediate Low risk PE
D) Low Risk PE
E) Submassive PE

A

Intermediate High risk PE

Intermediate-risk group who display evidence of both RV dysfunction (on echocardiography or CTPA) and elevated cardiac biomarker levels in the circulation (particularly a positive cardiac troponin test) are classified into the intermediate−high-risk category

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13
Q

You have seen a patient in PE follow up clinic. He was diagnosed as a unprovoked PE 3 months ago. He has no identifiable risk factors.

What is his risk of VTE recurrence?

A) <3%
B) 3-8%
C) 8-10%
D) 10-15%
E) >15%

A

3-8%

Low risk <3% - Major transient or reversible factors with >10 fold risk of VTE

Intermediate risk 3-8% - transient or reversible factors with <10 fold risk of VTE (first VTE only), Non malignant persistent risk factors, No identifiable risk factor.

High risk >8% - Active cancer, Antiphospholipid syndrome, and previous VTE

  • 2019 ESC Guidelines for the diagnosis and management of acute pulmonary embolism
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14
Q

Which of these is a positive vasoreactivity test in PAH?

A) Reduction in mPAP by ≤10 mmHg to reach an absolute value ≤40 mmHg, with increased or unchanged CO
B) Reduction in mPAP by ≥10 mmHg to reach an absolute value ≤40 mmHg, with increased or unchanged CO
C) Reduction in mPAP by ≥10 mmHg to reach an absolute value ≥40 mmHg, with increased or unchanged CO
D) Reduction in mPAP by ≥10 mmHg to reach an absolute value ≥40 mmHg, with decreased CO
E) Reduction in mPAP by ≤10 mmHg to reach an absolute value ≤40 mmHg, with decreased CO

A

Reduction in mPAP by ≥10 mmHg to reach an absolute value ≤40 mmHg, with increased or unchanged CO

  • 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
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15
Q

You see patient in SDEC with a confirmed PE. You would like to start him on apixaban

At what CrCl/min does BNF say apixaban should be avoided?

A) <30ml/min
B) <25ml/min
C) <20ml/min
D) <15ml/min
E) <10ml/min

A

<15ml/min

Manufacturer advises avoid if creatinine clearance less than 15 mL/minute—no information available. [online BNF accessed 03/02/25]

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16
Q

Which of these 2 drugs is a definite association with developing Pulmonary HTN as per 2022 ESC/ERS guideline?

A) Amiodarone
B) Bosentan
C) Fenfluramine
D) Nitrofurantoin
E) Toxic Rapeseed Oil

A

Fenfluramine & Toxic Rapeseed Oil

Definite Association:

Aminorex
Benfluorex
Dasatinib
Dexfenfluramine
Fenfluramine
Methamphetamines
Toxic rapeseed oil