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pulmonary hypertension Flashcards

1
Q

what is the definition of pulmonary hypertension?

A

mean pulmonary arterial pressure 25 or more mmHg as assessed by right heart catheterisation (RHC).

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2
Q

what is the normal values for mean pulmonary arterial pressure?

A

144 plus or minus 3 mmHg

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3
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4
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5
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6
Q

what are the 5 internationally agreed categories for patients with pulmonary hypertension?

A

1) pulmonary arterial hypertension
2) PH due to left heart disease
3) PH due to lung disease/hypoxia
4) chronic thromboembolic pulmonary hypertension (CTEPH) and other pulmonary artery obstructions
5) pulmonary hypertension with unclear/multifactorial mechanisms

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6
Q

what are the subcauses of type 1-pulmonary arterial hypertension?

A

-idiopathic pulmonary arterial hypertension (IPAH_-most important cause
-familial PAH
-drug and toxin induced PAH
-associated with:
connective tissue disease
HIV
portal hypertension
CHD
schistosomiasis

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6
Q

what are the subcauses of type 3-pulmonary hypertension due to lung disease/hypoxia?

A

COPD
ILD
other lung diseases with mixed obstructive/restrictive
sleep disordered breathing eg OSA
chronic exposure to high altitudes

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7
Q

what percent of people with systemic sclerosis get pulmonary hypertension?

A

15%

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8
Q

what percent of people with portal hypertension get pulmonary hypertension?

A

5%

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9
Q

what is an example of CHD causing pulmonary hypertension and what happens during it?

A

eisenmonger’s -chronic L–>R chunt that causes RVH and eventual reversal of the shunt.

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10
Q

what happens in idiopathic pulmonary arterial hypertension (IPAH)?

A

no specific cause (but genetic links) damage to capillary endothelium regrowth and modelling causes idiopathic pulmonary arterial hypertension

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11
Q

who does IPAH usually affect and which drugs can induce it?

A

young women
SSRIs, st john’s wort, pergolide (parkinson’s drug), amphetamines, cocaine, appeitite suppressives (fenfluramine, aminorex)

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12
Q

what is the prognosis for IPAH?

A

untreated survival =2.8 years
drug treatments used to extend survival -under specialist

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13
Q

what are the risk factors for PH?

A
  • Pre-existing diseases: lung disease, left heart disease, congenital heart disease, connective tissue disorders, obesity and sleep apnoea, liver disease
    -prolonged exposure to high altitude
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14
Q

what are the symptoms of pulmonary hypertension?

A

SOB, initially while exercising and eventually while at rest
fatigue
dizziness/syncope
chest pressure or pain
haemopysis if CTEPH
cyanosis
palpitations
oedema in ankles, legs, and eventually ascites
sx of associated conditions

15
Q

what percent of acute PEs don’t clear and how can we identify these?

A

3%
post severe PE, echo needs to be done at 6 months to ensure CTEPH isn’t occuring

16
Q

what are the signs of PH?

A

raised JVP
right ventricular heave
tricuspid regurg-systolic murmur
hepatomegaly
ascites, oedema
cyanosis
signs of associated conditions

17
Q

what are the most useful investigations in ?PH and ?PAH?

A

?PH -TTE
?PAH -right heart catheterisation

18
Q

bedside tests for ?PH?

A

ECG -looking for evidence of RV strain -RVH, RBBB, RAD, arrhythmias eg AF, aflutter.
normal ECG doesn’t exclude PH
ABG -for evidence of resp disease

19
Q

blood tests for pulmonary hypertension?

A

looking for causes:
cardiac-nt-pro-BNP
connective tissue disease -autoantibody screen (dsDNA, ENAs, ANA, RF)
HIV test
LFTs

20
Q

beyond tests for pulmonary hypertension?

A

CXR -signs of resp disease, LV dysfunction, PAH
TTE -first initial inv. TOE -if needed more detail
CTPA/VQ -for ?CTEPH
PFTs -for ?resp disease
right heart catheter (RHC) -actual diagnostic test
six minute walk test -info on severity, prognosis, improvement
CT thorax -more info
USS liver -more info on liver disease

21
Q

what happens in RHC?

A

right heart catheter inserted into jugular vein and passed into right heart and pulmonary arterial tree.
pressure measurements taken at RA, RV, main pulmonary, and wedge pressure (in capillaries -wedge it into capillaries till it doesn’t go any further)

22
Q

what is the significance of wedge pressure results?

A

high pre-capillary wedge pressure -pathology before capillaries-eg most causes eg resp disease, PAH
high post capillary wedge pressure -pathology (high BP) coming from after capillaries -ie originating from left heart eg LV failure, valve disease

23
Q

when in PH do you refer for specialist services?

A

PAH
severe PH

24
Q

what is the conservative management for PH?

A

exercise within symptom limits
pulmonary rehab
flu and pneumococcal vaccines
contraceptive advice -pregnancy is associated with 30-50% mortality in PAH patients
psychosocial support

25
Q

what is the medical management for PAH?

A

all under specialist, consider:
-specific drugs which vasodilate and lower pulmonary pressure -CCBs, bosentan, PDE inhibitors eg sildenafil, prostanoids, prostacyclin analogues eg iloprost.
-diuretics for sx benefits in RHF
-LTOT if resp failure
-digoxin -improves cardiac output acutely
-oral anticoagulation

26
Q

what is the management for PH caused by lung disease?

A

treat lung disease
specialist drugs for PH actually harmful when due to lung disease

27
Q

what is a potential treatment for some cases of PH to consider after medical management?

A

cardiopulmonary transplantation

28
Q

what is the treatment for CTEPH?

A

lifelong anticoagulation
some specific PH drugs
rule out thrombophilia
consider surgery -pulmonary endarterectomy or balloon dilation. v rare and difficult-only done in Cambridge.

29
Q

what are the complications of PH?

A

relating to worsening right heart failure -peripheral oedema, severe exertional dyspnoea
pneumonia
sudden cardiac death, inc during childbirth.

30
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A
31
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32
Q

what is the basic treatment strategy of PH?

A

treat underlying cause! treat each specific cause differently
refer to specialist if needed

32
Q
A

resp (6 decks)

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