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BLOCK 9; Week 4-M > Basic Science of PAH > Flashcards

Basic Science of PAH Flashcards

1
Q

What are the different types of PH?

A

Pulmonary Arterial HTN–rare progressive severe syndrome, low prognosis for survival
left sided congenital heart disease
COPD & lung disease
thromboembolic PH-patients need anticoagulant therapy

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2
Q

What are the subclassifications of PAH, the rare & severe syndrome?

A
  1. idiopathic PAH
  2. heritable/familial PAH
  3. Drug or Toxin induced PAH
  4. Atypical
  5. Persistent PAH of newborn
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3
Q

What falls into the category of atypical PH?

A

HIV infection, portal hypertension, congenital heart diseases, connective tissue disease, Schistosomiasis chronic hemolytic anemia

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4
Q

What are some mutations that can contribute to heritable/familial PAH?

A
  1. Type 2 Bone Morphogenetic Receptor (BMPR2)
  2. Mutation of Activin A receptor type II-like kinase-1 (ACVRL1, also known as ALK1)
  3. 5-HT Transporter Polymorphisms
  4. microRNA controlling gene expression
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5
Q

T/F Patients with PH often need anticoagulant therapy.

A

True. Can promote thromboembolism b/c of the remodeling of the pulmonary arteries.

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6
Q

How does ASD lead to PH?

A

atrial septal defect
get shunting of blood from left–>right.
right heart gets overwhelmed, get a ton of blood in the pulmonary arteries
get HTN & remodeling of these arteries.

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7
Q

What is an example of a drug/toxin that induced pulmonary arterial hypertension?

A

anorexigens…drugs that people were taking for a period of time to kill the appetite & lose weight.
1/1000 were affected.

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8
Q

What is a new treatment for persistent PH of a newborn, a form of severe PAH?

A

inhaled NO

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9
Q

Type 2 bone morphogenic receptor is a part of what family of receptors?

A

transforming growth factor beta family of receptors

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10
Q

What happens with the BMPR2 mutation?

A

if this is normally stimulated it decreases proliferation & enhances apoptosis.
When it is downregulated in idiopathic or heritable PAH….it can’t decrease these things…get thicker arteries & hypertension.

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11
Q

What is alteration in microRNA expression a deal with PH?

A

this controls expression of proteins, binds mRNA.

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12
Q

How does the regulation of ventilation/perfusion work in the lungs?

A
  1. when an alveolus is open & filled with oxgyen…more blood will be directed there.
  2. when an alveolus is more closed or holding less oxygen–>the capillaries will constrict & redirect blood flow elsewhere.
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13
Q

What is hypoxic pulmonary vasoconstriction? When does this occur?

A

this when blood vessels will constrict in the absence of oxygen
sorta like what happens in skeletal muscle
happens in the lung at high altitudes

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14
Q

What are the 3 mechanisms proposed for hypoxic pulmonary vasoconstriction?

A
  1. Redox hypothesis
  2. ROS hypothesis
  3. Energy state/AMPK hypothesis
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15
Q

Describe the redox hypothesis for HPV.

A

hypoxia affects mitochondria & you get activation of NADPH oxidase
ROS decreases, more reduced redox state
inhibition of K+ channels
membrane depolarization, opening of Ca++ channels.
More calcium–>vasoconstriction

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16
Q

Describe the ROS hypothesis.

A

inhibition of mitochondrial oxidative phosphorylation
ROS increases
get more release of calcium from SR
increase in calcium & vasoconstriction

17
Q

Describe the Energy state/ampk hypothesis.

A

inhibition of mitochondrial oxidative phosphorylation due to the hypoxia
increased activation of AMPK
increased release of Ca++ from SR via RyR

18
Q

What are the factors contributing to the elevated Pulmonary Arterial Pressure in PAH patients?

A
  1. vasoconstriction
  2. remodeling–luminal obstruction
  3. propensity of thrombosis
    Note: can see pulmonary arteries inflated on chest xray often
19
Q

What is required for diagnosis of PAH?

A

catheterization to see right heart & pulmonary arteries–assess pulmonary artery pressure:
>25 mmHg at rest
capillary wedge pressure: 3 wood units

20
Q

What is the equation for peripheral vascular resistance?

A

PVR=(Mean PAP-PA Wedge Pressure)/CO

21
Q

Is there a less invasive method for diagnosing PAH?

A

they are moving towards only requiring echocardiography as it is less invasive, but still required catheterization

22
Q

Is there a PAP during exercise required for diagnosis?

A

no, b/c even tho RV will contract vigorously during exercise the pulmonary arteries may dilate as a compensation
may or may not go up.
technically: >30 mmHg during exercise

23
Q

What is involved in capillary wedge pressure? What must it be for a diagnosis of PAH?

A

cardiac catheter is used & a balloon is inflated in a pulmonary vessel to block the flow & measure residual flow.
**need a low CWP b/c you need to exclude left heart disease induced PH

24
Q

How do you measure cardiac output?

A

via doppler flow

25
Q

What are the most important current therapies for PAH?

A
  • prostanoids
  • endothelin receptor blockers
  • phosphodiesterase inhibitors
26
Q

Other therapies for PAH?

A

L-type Ca++ channel blockers

drugs targeting NO & cGMP signaling pathways

27
Q

What is an endothelin receptor blocker? What does it do?

A

Ex: Bosentan
improves QOL & survival
nonselective ET1A & ET1B type receptor
**helps with remodeling of pulmonary vasculature & diameter of the arterial wall

28
Q

What is an example of a phosphodiesterase inhibitor?

A

sildenafil

29
Q

How do endothelin receptor blockers work in the pathway?

A

Block ET1A & ET1B receptors on smooth muscle cells.
These receptors when activated cause vasoconstriction.
BLock this–>treat PAH

30
Q

How do prostanoids work in the pathway to treat PAH?

A

Prostacyclin triggers receptor on smooth muscle cell
adenylate cyclase activated & you get more cAMP.
protein kinase is activated & vasodilation is promoted.

31
Q

How do phosphodiesterase inhibitors work to cause vasodilation in PAH?

A

PDE5 inhibitors stop the breakdown of cGMP.

This promotes thea ctivation of protein kinase A & vasodilation.

32
Q

What is used to induce PAH in a rat?

A

monocrotaline-single subcu injection

33
Q

What is the way you can get plexiform lesions in a mouse?

A

only way–>induce chronic hypoxia + sugen (VEGF inhibitor)

34
Q

How do you induce chronic hypoxia in a rat or mouse?

A

10% O2 for the mouse for 2-4 weeks.

BLOCK 9; Week 4-M (8 decks)

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