00:02
What's the clinical presentation of somebody
with primary pulmonary hypertension?
Well, the clinical presentation is dependent on
the cause, so for example, somebody with primary
pulmonary hypertension will tend to be a younger
woman, whereas multiple PEs could be any age
and sex and they could have history of previous
PEs and known risk factors or may not, or
there could be a family history of hypercoagulability.
The clinical effects though are similar, which
is progressive dyspnoea over weeks or months.
So the patient will say “I'm breathless
on exertion, I can't walk as far as I used
to walk and that ability to walk is getting
worse over time”. And that's a very similar
presentation to patients with COPD, interstitial
lung disease, and cardiac failure. Then maybe,
if somebody is having multiple PEs, occasional
episode of pleuritic chest pain or haemoptysis
that might suggest it's a PE but actually
in most patients, it's just breathlessness,
which is the main symptom.
01:00
When you examine somebody with pulmonary hypertension
or chronic PEs, they could be cyanosed, but
there aren't many signs that you can pick
up when you listen to the lungs, in fact,
usually there's nothing. They may have signs
of pulmonary hypertension and they may have
signs of decompensated right heart failure
with a raised JVP, ankle oedema, etc. but
only when the disease is really quite marked.
So as I said, if you got clinical signs of
pulmonary hypertension there's a raised JVP,
a loud pulmonary component of the second heart
sound, a right ventricular heave, a right
ventricular third and fourth heart sound.
01:38
But these are actually very difficult clinical
signs to be sure about, and a raised JVP could
occur for other reasons as well. Very severe
pulmonary hypertension will lead
to tricuspid regurgitation, as dilatation
in the right ventricle will make the valve
area larger, and basically pull the valve leaves
apart, and that will occur with pansystolic
murmur at the left sternal edge, V waves in the
JVP, which are a very obvious one and occasionally
pulsatile hepatomegaly. As I mentioned before,
if you have bad right-sided heart failure
the patient has peripheral oedema, could have
ascites, could have pleural effusions.
02:17
The problem about all those clinical signs
is that they are very nonspecific and difficult,
so you need to think about pulmonary hypertension
or chronic PEs as a cause in patients presenting
with what could be right-sided heart failure
by itself.
02:34
Confirming the presence of pulmonary hypertension,
again is not straightforward, the chest X
ray could show oligaemic lung fields, that
means the blood vessels are less distinct
than normal but that’s a very subtle sign.
Occasionally you get an X ray like we have
here as an example where the pulmonary arteries
are very large indeed, and that's much more
obvious that the patient will have pulmonary
hypertension. But unfortunately, that's not
that common. A CT scan may show enlarged
pulmonary arteries
and it’s a more sensitive way of identifying
those, but again it’s not a particularly
good way of identifying the presence of pulmonary
hypertension.
03:10
VQ scan may be abnormal if they have multiple
PEs due to the mismatched perfusion ventilation
defects we discussed before, but importantly
patients with pulmonary hypertension will
have right heart problems and therefore the
tests that you really need to use to identify
pulmonary hypertension are looking at the right
heart. So an ECG shows right heart strain,
T wave inversions V1-V3 right axis deviation.
An echocardiogram will show right ventricular
hypertrophy and dilatation, and if there's
a little bit tricuspid regurgitation, they
can measure the pulmonary artery pressure
and actually give you a record of what the
pressure is and therefore confirm the presence
of pulmonary hypertension or not.
03:58
Lung function tests as we discussed earlier,
the volumes in spirometry do not change in
patients with pulmonary hypertension by itself,
but the retransfer factor is reduced due to
the decreased blood flow to the lung. So a
transfer factor along with the echocardiogram
are the very useful tests for identifying
patients with pulmonary hypertension.
04:20
And pulmonary invasive angiography is reserved for
those cases with clear-cut pulmonary hypertension
or younger patients where it's not clear what's
going on, and you really need to identify
the disease. It's also used as a way of identifying
causes of pulmonary hypertension and their
response to treatment, but it's a very specialized
test, that's used by specialized centers in
general. If you identify somebody
who has pulmonary
hypertension, you're then next to identify,
why they have pulmonary hypertension. Now
the common causes are chronic PEs, lung disease,
and cardiac disease, so the test that you
need to identify those are CT pulmonary angiogram
or a VQ scan for the PEs, an echocardiogram,
which as well as identifying the presence
of right ventricular hypertrophy, and pulmonary
hypertension can also look for left-sided
disease that might be the cause of that pulmonary
hypertension. And lung function tests, which
again the transfer factor will reflect the
pulmonary hypertension, but if that patient has
significant lung disease, COPD, interstitial
lung disease or even chest wall restriction,
then the lung volumes in spirometry will
be abnormal. I mentioned already that invasive
pulmonary angiography can give very distinctive
appearances which will suggest chronic-embolic
disease or primary pulmonary hypertension
and that's used by specialized centers to
make the diagnosis of the cause. And there
are various blood tests that could be used
to identify patients with autoimmune diseases,
HIV and sickle-cell disease, schistosomiasis
etc.
05:56
We actually frequently use sleep studies as
well because obstructive sleep apnea, obesity
hyperventilation, nocturnal hypoxia due to
chronic lung disease are frequently common
causes of pulmonary for hypertension.