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Pulmonary Week 3 > ICL 3.2: Pulmonary Hypertension > Flashcards

ICL 3.2: Pulmonary Hypertension Flashcards

1
Q

what is group 1 HTN?

A

pulmonary arterial hypertension

can be hereditary or acquired

the LEAST common

ex. scleroderma

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2
Q

what is group 2 HTN?

A

PH-LHD = left heart failure!

can be because of preserved EF, decreased EF, or congenital heart disease

14% of pulmonary HTN cases but for USMLE this is the most common cause of pulmonary hypertension

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3
Q

what is group 3 HTN?

A

chronic hypoxia; cor pulmonale

caused by OSA or COPD

85% of pulmonary HTN cases

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4
Q

what is group 4 HTN?

A

CTEPH = chronic thromboembolism pulmonary hypertension

presence of multiple chronic or organized occlusive thrombi or emboli still present in the elastic pulmonary arteries - main, lobar, segmental or sub-segmental - after at least three months of effective anti-coagulation

PAH-like arteriole muscularization

.14% of pulmonary HTN is caused by this

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5
Q

what is group 5 HTN?

A

caused by sarcoidosis

.14% of pulmonary HTN is caused by this

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6
Q

which groups are the 2 main causes of pulmonary HTN?

A
  1. group 2 = left heart disease, HFpEF, HFrEF, valvular disease –> most common
  2. group 3 = hypoxemic lung disease, obstructive lung disease, restrictive lung disease, OSA
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7
Q

what are the causes of group 1 pulmonary HTN?

A

pulmonary arterial hypertension

  1. idiopathic
  2. heritable
  3. drug and toxin induced

associated with connective tissue disease (scleroderma), HIV, portal hypertension, congenital heart disease, schistosomiasis

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8
Q

what are the causes of group 2 pulmonary HTN?

A

pulmonary hypertension due to left-sided heart disease

  1. left ventricular-systolic dysfunction
  2. left ventricular diastolic dysfunction
  3. valvular heart disease
  4. specific congenital abnormalities
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9
Q

what are the causes of group 3 pulmonary HTN?

A

pulmonary hypertension due to lung disease or hypoxia

  1. COPD
  2. interstitial lung disease
  3. mixed restrictive or obstructive lung disease
  4. alveolar hypoventilation disorders
  5. chronic exposure to high altitude
  6. developmental lung diseases
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10
Q

what are the causes of group 4 pulmonary HTN?

A

chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions

  1. chronic thromboembolic pulmonary hypertension
  2. angiosarcoma
  3. intravascular tumors
  4. arteritis
  5. congenital stenoses
  6. parasites
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11
Q

what are the causes of group 5 pulmonary HTN?

A

pulmonary hypertension with multifactorial mechanisms

  1. hematological disorders (sickle cell)
  2. systemic disorders ( sarcoidosis, Langerhans cell granulomatosis)
  3. metabolic disorders (Gaucher’s disease)
  4. renal disease
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12
Q

what is pulmonary hypertension?

A

the MEAN pulmonary artery pressure is 25+ mmHg

you need both the systolic and diastolic pressure to calculate this

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13
Q

what is the pulmonary artery pressure with group 2 pulmonary HTN?

A

mPAP > 25 mmHg

PCWP > 15 mmHg = post-capillary hypertension

this is PH due to left heart disease

this is the only type of PH with increased PCWP

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14
Q

what is the pulmonary artery pressure with group 1,3,4,5 pulmonary HTN?

A

mPAP > 25 mmHg

PCWP < 15 mmHg = pre-capillary pulmonary hypertension

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15
Q

how do you figure out what group of PH a patient has?

A

first use non-invasive echocardiogram to diagnose + clinical signs –> can tell you if it’s groups 2 or 3

then to asses for group 4 CTEPH, use non-invasive V/Q scan to diagnose

finally, to asses for groups 1 and 5, you need an invasive right heart catheterization and pulmonary angiogram to diagnose

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16
Q

the two most common WHO groups of pulmonary hypertension account for what fraction of pulmonary hypertension?

A

99.6%

groups 2 and 3

17
Q

what are the symptoms of pulmonary hypertension?

A
  1. dyspnea on exertion is the most common (make them go on a 6 minute walk)
  2. fatigue
  3. chest pain/discomfort
  4. leg swelling
  5. syncope
  6. dizziness
  7. cough is less common
18
Q

what do you see when you’re doing a PE of pulmonary hypertension?

A
  1. lungs are clear – you’re compensating with CHF so there won’t be rales because the extra fluid is backing up in the legs, ascites, etc.
  2. cor pulmonale signs = hepatomegaly, ascites, JV, leg edema
  3. evidence of connective tissue disease if group 1
19
Q

what are the methods you can use to asses for pulmonary hypertension?

A
  1. non-invasive chest CT with contract dye
  2. non-invasive: echocardiogram with doppler
  3. invasive right heart/pulmonary artery catheter
20
Q

what do you use a chest CT to asses for with pulmonary HTN?

A
  1. assess LV/RV volumes
    RV/LV > 1.2 abnormal
  2. assess PA diameter

PA/Aorta normally is 0.9 so > 1 abnormal

PA diameter
Men: up to 29 mm
Women up to 27 mm

21
Q

what do you use a echocardiogram with doppler to asses for with pulmonary HTN?

A

good screening tool but NOT diagnostic; you need to do right heart catheter to confirm pulmonary HTN on its own

  1. assess LV/RV volumes
  2. measure PA systolic pressure
  3. LVEF
  4. RV wall thickness
  5. elevated tricuspid regurgitation jet

NO measures to calculate pulmonary vascular resistance (PVR)

22
Q

what do you use a right heart/pulmonary artery catheter to asses for with pulmonary HTN?

A

this is the accepted meta to diagnose pulmonary hypertension!

  1. PA mean pressure (systolic/diastolic and pressures)
  2. PCWP measurement
  3. cardiac output measurement by thermodilution
  4. transpulmonary gradient (TPG) as mean PAP-PCWP, and PVR as TPG/Cardiac output
  5. pulmonary vascular resistance (PVR), and Cardiac output/index
  6. pulmonary vasoreactivity: only for IPAH, HPAP, or DT PAH
23
Q

which of the below parameters measured by echocardiogram is a measure of pulmonary hypertension?

A. LV/RV volumes

B. tricuspid valve regurgitant jet

C. left ventricular ejection fraction

D. RV wall thickness

A

B. tricuspid valve regurgitant jet

the others are supportive but the tricuspid valve regurgitant tells you the severity of the pulmonary HTN

24
Q

how do you treat group 3 HTN?

A

treat the underlying disease!!

whether that be COPD, pulmonary fibrosis, interstitial lung disease or sleep disordered breathing

25
Q

why do we treat people with COPD with oxygen?

A

COPD patients with resting hypoxia, giving oxygen reduces mortality by:

  1. reduces hypoxic vasoconstriction and therefore reduces pulmonary HTN
  2. reduces polycythemia (polycythemia causes increased resistance due to blood thickening)
26
Q

how severe is the pulmonary HTN associated with sleep disordered breathing?

A

with OSA alone, you will have mild PH typically

with OSA and OHS, severe pulmonary HTN may be observed

nocturnal hypoxia has its own risk factors

27
Q

how does chronic exposure to high altitudes cause pulmonary HTN?

A

altitude decreases the inspired PO2 because of a decrease in barometric pressure

mild pulmonary HTN occurs at rest and may increase substantially with exercise

severe pulmonary HTN may occur with high altitude pulmonary edema, subacute mountain sickness and chronic mountain sickness

28
Q

how do you treat pulmonary HTN from chronic exposure to high altitudes?

A

this is NOT immediately reversed by breathing in oxygen because it’s chronic

descent from high altitude is primary treatment

can also give alpha adrenergic blockade, salmeterol B agonist or CPAP

29
Q

how do you manage group 4 pulmonary HTN?

A
  1. echo shows signs of TV dysfunction then give anticoagulation to try and breakdown clots
  2. do V/Q scan to see if there’s defects which would suggest CTEPH
  3. do right heart catheterization to confirm pulmonary HTN to diagnose CTEPH
  4. lifelong anticoagulation to prevent further clots from developing
  5. assess for surgery to see if you can remove the clots

if you can’t operate, give phosphodiesterase

30
Q

how do you treat someone with CTEPH?

A

if someone with CTEPH isn’t a candidate for surgery, give soluble guanylate cyclase stimulators = riociguat

this is only give to patients who are deemed not to be good candidates for embolectomy or balloon pulmonary angioplasty (BPA) or for patients with residual pulmonary hypertension after embolectomy/BPA.

31
Q

what are some of the genes associated with group 1 pulmonary HTN?

A
  1. IPAH and HPAH
    BMPR2* is AD; F > M

other mutations include ALK-1, KCNK3, endoglin, SMAD9, cave-in-1

  1. hereditary hemorrhagic telangiectasia (HTT) and PAH

Alk-1 mutation

  1. group 1 heritable PVOD/PCH

EIF2AK4 mutation

32
Q

what do you do for a group 1 pulmonary HTN workup?

A

you find pulmonary HTN that’s confirmed by the right heart cath so now you do acute vasoreactivity testing for groups 1 and 5 pulmonary HTN

give NO or IV epoprostenol and if you see reactivity, treat with oral CCB and continue with that if it keeps working –> a positive test is if the mPAP decreases by at least 10 mmHg to below 40 mmHg after vasodilator is given

if they don’t respond to CCB then you can give the following to WHO group 1, but no other WHO group:
1. prostanoids

  1. endothelial receptor antagonists (ERAs)
  2. phosphodiesterase-5 inhibitors
33
Q

vasoreactivity test response to medical therapy should be tested in which WHO groups?

A

groups 1 and 5

do NOT test in people with HF

34
Q

what medication can you give to WHO group 4?

A

soluble guanylate cyclase stimulator Adempas = riociguat

35
Q

what are the class I-IV functional classifications of pulmonary HTN?

A

class I: ordinary physical activity does not cause undue dyspnea, fatigue, chest pain or near syncope

class II: comfortable at rest, ordinary physical activity causes undue dyspnea, fatigue, chest pain or near syncope

class III: marked limitation of physical activity. comfortable at rest. less than ordinary activity causes undue dyspnea, fatigue, chest pain or near syncope

class IV: unable to perform any physical activity without symptoms. patients manifest signs of right heart failure. dyspnea and/or fatigue may be present at rest. discomfort is increased with any physical activity

Pulmonary Week 3 (8 decks)

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