Pulmonary hypertension Flashcards
are there any gene mutations that are associated with familial PAH?
The BMPR2 mutation is detected in 55% of families
26% of sporadic cases have the same thing
It is an autosomal dominant condition with incomplete penetrance
this protein is called BMP, and it inhibits vascular smooth muscle cell growth
what is the normal mean pulmonary artery pressure?
what is the diagnostic range for PH?
normal range is 8 - 20
PH is now defined as mPAP > 25mmHg
to define pulmonary arterial pressure, we need to exclude left atrial hypertension. Therefore, we need to do a wedge pressure and assess. The PCWP should be < 15mmHg
does anything happen in normal people with inc. age wrt mPAP?
if checked when people are exercising, we will see an increase. mPAP is significantly higher in normal older age groups
the upper limit of normal in young people is 25-30
in people > 50 years, it is around 45 mmHg
what is the best screening test for patients with SOB without cardio-pulmonary disease?
a Doppler echo is useful screening tool when looking to exclude PH
Does pulmonary hypertension have any impact during pregnancy?
This is one of the MOST high risk diseases for preggos
greater than 30% mortality!
(develop RHF, are completely decompensated and unable to fix)
What are some of the supportive medications that we use in PH?
?immunisations?
?anticoag?
Immunisations with pneumovax and influenza should be done for all.
The inflammation associated with pneumonia can push up pressures, and lead to RHF/decompensation
anti-coagulation is recommended for any without contraindications
What is the difference between PH and PAH?
PAH is the primary process in the vessels.
Pulm HTN includes all patients with elevated pulm pressures, and can be those with elevated due to RHF, vasoocclusive disease, etc
what is the first line treatment for PAH?
how do you determine if it is appropriate for a patient?
calcium channel blockers - diltiazem and nifedipine (note: one is non-dihydro and one is dihydro)
we determine if people will respond to these medications by doing a RIGHT HEART CATH
during the cath we complete a vasoreactivity study to determine if a patient’s mPAP will drop to near-normal levels.
this is important because, if you respond to CCB, then it is awesome for your long term survival. We need to select out these patients, because it stops their disease progression
what does endothelin do in a normal person?
1 vasoconstriction - direct or via faciliation of other vasoconstrictor systems
- fibrosis - leads to fibroblast proliferation
- proliferation - vascular smooth muscle
- hypertrophy
- inflammation
- inc vascular permeability
- PMN activation
- cytokine production
what do the endothelin receptors do?
endothelin A receptors - lead to vasoconstriction and proli. It also causes heart Hypertrophy
the endothelin B lead to NO and prostaglandin release (this is a positive thing - but all the rest are not
What drives the changes in the pulmonary vasculature in chronic lung disease?
is there any difference with diastolic function in this condition?
In any person, there is a drive from the body to have pulmonary arteriolar vasoconstriction if there is hypoxia.
in chronic disease like COPD, this goes for a long time and causes remodelling
it is a reasonably slow progression (as opposed to primary disease)
PH in COPD seems to be associated with RV diastolic dysfunction, which is not the case with PAH. The reason is not clear, but there is lots of evidence that RV filling pressures are elevated. This leads to fluid retention/oedema (blue bloater)
are there any treatments that we can do for chronic thromboembolic pulmonary hypertension?
pulmonary end-arterectomy is the procedure.
- reasonably high mortality (5-10%) but also very good response for those that get it
