#22 - Pulmonary arterial hypertension Flashcards

1
Q

What lab is required for diagnosis of pulmonary arterial hypertension (PAH)

A

right heart catheterization

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2
Q

Define pulmonary hypertension (PH), and pulmonary arterial hypertension (PAH)

A

increase in resting mean pulmonary arterial pressure >25 mmHg by right heart catheterization

For pulmonary arterial hypertension (PAH) the definition includes the above and the following:
normal pulmonary capillary wedge or left atrial pressure (

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3
Q

Symptoms of pulmonary arterial hypertension (PAH)

A
  • unexplained dyspnea
  • fatigue/ exercise intolerance
  • sometimes syncope
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4
Q

Physical exam signs in the early stage of pulmonary arterial hypertension (PAH)

A
  • accentuated P2

- RV heave (left parasternal lift)

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5
Q

Diagnosis of pulmonary arter

A

a

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6
Q

What are the 5 classifications of pulmonary hypertension

A

1 - pulmonary arterial hypertension (PAH)

NOTE: many patients may fall into 2 or 3 groups

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7
Q

Most common cause of pulmonary hypertension

A

Pulmonary venous hypertension due to left heart disease (constitutes 65% of pulmonary hypertension)

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8
Q

What is chronic thromboembolic pulmonary hypertension

A

occurs in 2-4% of patients after a pulmonary embolism.

if you have pulmonary hypertension 6 months after a PE, you are diagnosed.

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9
Q

Treatment for chronic thromboembolic pulmonary hypertension

A

surgical - remove the thrombus in the lungs - only a few centers do this, but it can dramatically improve the condition..

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10
Q

3 mechanisms that contribute to pulmonary arterial hypertension (PAH)

A
  • vasoconstriction (increased endothelin, decreased prostacyclin, decreased NO) - THERAPY TARGETS THIS CATEGORY
  • smooth muscle proliferation
  • thrombosis
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11
Q

Physical exam signs in the advanced stage of pulmonary arterial hypertension (PAH)

A

lots of heart sounds.

  • diastolic murmur of pulmonary regurgitation
  • holosystolic mumur of tricuspid valve (right AV valve)
  • right ventricular gallop
  • elevated JVD
  • pulsatile liver
  • peripheral edema/ascites
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12
Q

CXR findings in pulmonary arterial hypertension (PAH)

A

prominent vasculature in the hylum

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13
Q

Classic EKG findings in pulmonary arterial hypertension (PAH)

A

RV hypertrophy,
right atrial enlargement,
right axis deviation

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14
Q

Best test for initial evaluation of PAH: EKG, CXR, or echo?

A

echocardiogram (but it is far from perfect)

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15
Q

Echocardiogram: utility and limitations in diagnosis of PAH

A
  • provides estimate of pulmonary arterial systolic pressure.
  • tells you about the function/morphology of right heart
  • identifies conditions that contribute to PH (valvular disease, LV systolic dysfunction, impaired diastolic function, R to L shunt.

However, it is not very accurate in estimation of pulmonary systolic pressure - only within 10mmHg 50% of the time. It commonly overestimates AND underestimates it.
It is commonly used as a screening test for PAH.

This is why right heart cath remains the gold standard and is needed for diagnosis.

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16
Q

Utility of right heart cardiac catheterization for diagnosis of pulmonary arterial hypertension (PAH)

A
  • differentiates between arterial and venous hypertension
  • needed for diagnosis
  • determines etiology (assesses L to R shunt, L heart disease, etc)
  • establishes the severity and prognosis
  • evaluate vasoreactivity (evaluate response to drugs while catheter is in place)
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17
Q

You’ve made a diagnosis of pulmonary arterial hypertension (PAH). What is the NEXT step?

A
  • figure out where it is coming from.
  • test serology (ANA, HIV, Liver function tests)
  • sleep study (group 3)
  • pulmonary function tests, chest CT, arterial blood gas (group 3)
18
Q

What predicts poor prognosis / survival in PAH patients?

A
  • poor exercise endurance (6 minute walk test
  • syncope (represents advanced disease)
  • signs of R heart failure (pericardial effusion, high R atrial pressure, high BNP, etc.)
19
Q

PAH is a relatively benign condition, without treatment.

A

False. Deadly without treatment. Treatment improves survival though

20
Q

Goals of therapy for PAH

A
  • improve hemodynamics
  • improve exercise capacity/ quality of life
  • improve survival
21
Q

Treatment for PAH

A
  • supportive therapy (oxygen, diuretics, anticoagulants)
  • Ca channel blockers (if vasoreactivity in r heart cath - this is very good prognosis!)
  • PAH specific therapies
22
Q

lifestyle changes in PAH

A
  • avoid overexertion
  • avoid pregnancy during therapy
  • avoid high altitude
  • low sodium diet, fluid restriction
23
Q

who does PAH commonly occur in ?

A

Young women

24
Q

What happens to the Right ventricle systolic pressure in PAH?

A

it goes way up (has to overcome pulm hypertension)

-normally, it is

25
Q

Definition of apnea

A

air flow stops for > 10 seconds

26
Q

hypopnea

A

reduction in airflow for 10s

27
Q

obstructive sleep apnea: Mild vs. severe

A

mild = 5-15 episodes of apnea or hypopnea/ hour

seveere >30 apnea or hypopnea / hour

28
Q

Symptoms of obstructive sleep apnea (OSA)

A

snoring, gasping, choking
(gasping and choking 80% specific for sleep apnea!)

  • excessive daytime sleepiness, unrefreshing sleep
  • hypertension
29
Q

Exam for obstructive sleep apnea (OSA)

A
  • increased weight
  • high BP
  • obstructed nasal patency
  • large tongue, large tonsils, or large soft palate / uvula
  • small jaw
30
Q

findings in a polysomnogram in obstructive sleep apnea (OSA)

A

-complete blockage of airflow DESPITE efforts to breathe!! (thoracic effort will be present)

31
Q

treatment of choice for obstructive sleep apnea (OSA)

A

nasal continuous positive airway pressure - CPAP

-functions as an airsplit, effective in 90%, but low compliance

32
Q

T/F treatment for sleep apnea helps hypertension

A

True.

33
Q

lifestyle changes for obstructive sleep apnea (OSA)

A
  • avoid alcohol (airway narrowing/ increased apnea duration)
  • avoid sleep deprivation
  • lose weight
  • avoid supine position
34
Q

Curative therapy for obstructive sleep apnea (OSA)

A

tracheostomy. Also, other surgeries correcting defects in nasal septum/oropharynx

35
Q

Central apnea findings on polysomnogram

A

air flow stops >10s WITHOUT respiratory effort

36
Q

T/F About 50% of CHF patients have sleep disordered breathing. Most have obstructive sleep apnea.

A

FALSE. mainly central sleep apnea!

37
Q

Treatment of central sleep apnea

A

oxygen and CPAP

38
Q

cause of .obstructive sleep apnea (OSA) in children aged 2-7

A

lymphoid hyperplasia

Older children can have OSA due to obesity

39
Q

symptoms of OSA in children aged 2-7

A

similar to adults but can include aggressiveness, attention span problems, etc

40
Q

Treatment of obstructive sleep apnea in children 2-7

A

tonsillectomy / adenoidectomy

41
Q

4 preventative measures for preventing SIDS

A
  • sleep on back (NOT ON FRONT)
  • no smoke exposure
  • avoid overheating
  • no pillows/soft bedding