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Medical Studies 1 > 9.6 Pulmonary Hypertension > Flashcards

9.6 Pulmonary Hypertension Flashcards

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USMLE® Step 1 flashcards
1
Q

True or false: pulmonary hypertension is a disease

A
  • False
  • It is not a disease, it is a haemodynamic measurement
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2
Q

Pulmonary hypertension vs pulmonary artery hypertension

A

pulmonary arterial hypertension is a disease group within the wider haemodyanmic range of pulmonary hypertension

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3
Q

How do you calculate mPAP (mean Pulmonary Artery Pressure)?`

A

1/3 sPAP + 2/3 dPAP

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4
Q

What is the mPAP cutoff for pulmonary hypertension? Why is/isn’t this sufficient for a diagnosis?

A
  • Cutoff is 20mmHg
  • This is not sufficient for a diagnosis, since a wide range of other factors can influence mPAP
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5
Q

List some factors other than pulmonary hypertension that can increase mPAP

A
  • Hyperviscosity
  • Increased CO
  • Left to right cardiac shunts
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6
Q

Other than evidence of mPAP >= 20, what else is needed to diagnose pulmonary arterial hypertension?

A

Evidence of increased pulmonary vascular resistance

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7
Q

What level of pulmonary vascular resistance is required to diagnose pulmonary arterial hypertension? Is this likely to be right 100% of the time?

A

Cutoff is >=3 Wood Units (WU)

In reality, it should probably be lower

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8
Q

What does pulmonary artery wedge pressure (PAWP) inform us about

A
  • It provides an approximation of left arterial pressure, and thus left ventricular preload
  • We can use it to assess for left-heart-related implications of pulmonary hypertension
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9
Q

List two kinds of pulmonary hypertension

A
  • Pre-capillary (PAH)
  • Isolated post-capillary (IpcPH)
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10
Q

Describe the mPAP, PAWP, and PVR in Pulmonary Arterial Hypertension

A

mPAP:> 20mmHg
PAWP: <=15mmHg (not left-heart involved, so not elevated)
PVR: >3 WU

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11
Q

Describe the mPAP, PAWP, and PVR in Isolated, post-capillary Pulmonary Hypertension (IpcPH)

A

mPAP: >20mmHg
PAWP: > 15mmHg (left-heart involvement)
PVR: <3 WU

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12
Q

Describe the changes that occur to pulmonary arteries in Pulmonary Arterial Hypertension

A
  • Intimal fibrosis
  • Endothelial proliferation
  • SMC hyperplasia and hypertrophy
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13
Q

Describe the changes in cardiac output, pulmonary vascular resistance, and pulmonary arterial pressure that occur during the worsening of PAH

A
  • PVR increases, and PAP increases to prevent CO from falling too much (check with Ohm’s Law)
  • Eventually, heart begins to fail, and PAP decreases, causing CO to decrease
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14
Q

What is the most common cause of pulmonary artery hypertension? What are some examples of this?

A
  • Left heart disease
  • HFrEF/HFpEf
  • Valvular disease
  • Congenital abnormalities
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15
Q

Other than left-sided heart disease, list some less common causes of pulmonary hypertension

A
  • Lung disease/hypoxia
  • Pulmonary artery obstructions (e.g. clot)
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16
Q

List some causes of pulmonary arterial hypertension

A
  • Idiopathic
  • Heritable
  • Drug/toxin-associated
  • Connective tissue disease
  • Calcium channel blockers
17
Q

Presenting symptoms of pulmonary hypertension

A
  • Dyspnoea (usually during exercise)
  • Fatigue
  • Exercise intolerance
  • Syncope on exertion
  • Anginal chest pain
18
Q

What symptoms of disease progression may be seen in pulmonary hypertension?

A

Right heart failure: Raised JVP, and peripheral oedema

19
Q

Describe the physical exam results of a patient with pulmonary hypertension

A

Typically normal; maybe 2nd pulmonary heart sound accentuated or RV heave in late-stage

20
Q

Blood-based investigations for patient with suspected pulmonary hypertension

A
  • FBC (anaemia)
  • EUC (renal hypertension)
  • LFTs (Right heart failure)
  • NT-proBNP/BNP (Right heart failure)
  • Autoimmune screen (connective tissue disorder)
21
Q

What ECG signs might indicate pulmonary hypertension?

A
  • Right ventricular hypertrophy
  • Right axis deviation
22
Q

What is the main screening tool for pulmonary hypertension? How does it work?

A

Echocardiography. By passing a doppler catheter through the tricuspid valve, you can estimate right ventricular systolic pressure

23
Q

Does a negative echocardiogram definitively exclude pulmonary hypertension?

A

No

24
Q

In the context of pulmonary hypertension, what are ventilation perfusion scans good at detecting?

A

They are good at detecting chronic thromboembolic pulmonary hypertension

25
Q

Left heart disease is associated with pulmonary hypertension. What are some risk factors of left heart disease

A
  • Systemic hypertension
  • Diabetes
  • Obesity
  • Older age
  • Atrial fibrillation
26
Q

CT chests are used to screen for pulmonary hypertension. What are some features that indicate this diagnosis on CT?

A
  • RV dilation
  • RA dilation
  • HRCT signs of lung disease
27
Q

What is the purpose of pulmonary function tests in screening for pulmonary hypertension

A

To assess for any other lung disease that may be comorbid/contributing to lung disease

28
Q

Describe gold-standard diagnosis of pulmonary hypertension

A
  • Right-heart catheterisation
  • Measures RA pressure, RV pressure, pulmonary arterial pressure, PAWP, and cardiac output
29
Q

What values can we calculate as a result of right-heart catheterisation?

A
  • Transpulmonary gradient (difference between mPAP and PAWP)
  • Pulmonary vascular resistance (TPG/CO)
  • Cardiac index (CO/Body surface area)
30
Q

Describe the four functional classes of pulmonary hypertension

A

FC I: Asymptomatic
FC II: Limited to moderate/high exertion
FC III: Limited day-to-day
FC IV: Bed-ridden

31
Q

What are the three drug pathways for pulmonary hypertension treatment?

A
  • Endothelin pathway (constrictor)
  • Nitric oxide pathway (dilator)
  • Prostacyclin pathway (dilator)
32
Q

List three endothelin receptor antagonists (hint: what is the suffix?)

A
  • Bosentan
  • Ambrisentan
  • Macitentan
33
Q

List three drugs that act on the nitric oxide pathway to treat pulmonary hypertension

A
  • Sildenafil
  • Tadalafil
  • Riociguat
34
Q

A patient with pulmonary hypertensionf is responsive to inhaled vasodilators. What drug should we give them>

A

Try them on calcium channel blockers

35
Q

During management of pulmonary hypertension, patients are started on two drugs. If they still remain at high risk, what other treatments may be considered?

A
  • Additional IV drugs
  • Lung transplant if all else fails
36
Q

Some patients may not be suited to IV pulmonary hypertension therapy. Why?

A
  • Don’t want IV therapy
  • Elderly
  • IV drug user (don’t want cannula)
37
Q
A

Medical Studies 1 (90 decks)

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