00:01
A 22-year-old graduate student is seen in clinic
because of increasing leg edema that has become refractory to diuretics.
00:07
She was given a diuretics by her primary care physician
and it was thought just to be some, perhaps some edema from her menstrual cycle.
00:17
She also has noted though, much more worrisome, increasing dyspnea on exertion
and fatigue and previously, she was healthy.
00:25
It's interesting that her mother has been diagnosed with systemic lupus erythematosus
so often, this is found in families where there's a lot of autoimmune diseases.
00:36
Physical exam shows blood pressure's pretty normal for young female, 104/78,
heart beat a little bit elevated at 86, and the peripheral oxygen saturation is abnormal,
88% on room air suggesting lung disease.
00:50
And her jugular venous pulse is also elevated, should be under 10
and particularly way under 10 for such a young woman.
00:58
The lungs are clear and the cardiac exam shows a loud pulmonic component
to the second heart sound but no murmurs.
01:06
So, let's think about that. Here's normal, lub-dub, lub-dudub, lub-dudub, lub-dub.
01:12
And the secondary component, second splitting of the second heart sound is the pulmonic component,
lub-dudub, lub-dudub, lub-dudub. There you can see it's accentuated.
01:22
You can hear it at the apex where you normally don't hear the splitting.
01:26
That tells you that P2, the pulmonic component is increased.
01:30
That suggest pulmonary hypertension and she has 2-3+ peripheral edema.
01:34
So the critical factors here in the history of course, dyspnea,
unusual in a young woman like this. Hypoxemia suggesting lung disease.
01:42
Elevated jugular venous pressure suggesting a right heart failure.
01:47
The loud P2 suggesting pulmonary hypertension and the peripheral edema suggesting some right ventricular failure.
01:55
And here's her ECG. It shows right ventricular hypertrophy.
02:00
Again, notice the large R wave in V1. You're supposed to have only a small R wave in V1.
02:05
And there's right axis deviation so again, we are dealing with somebody with cor pulmonale.
02:13
That is with a right ventricle that's been over strained, likely pulmonary hypertension.
02:19
The chest X-ray shows right ventricular dilatation and an echocardiogram
shows reduced right ventricular systolic function and the pulmonary artery systolic pressure is estimated at a frightening 96 mmHg.
02:33
In other words, the same level as her arterial blood pressure
so she has arterial blood pressure in her pulmonary circuit.
02:42
Very severe pulmonary hypertension, she has a CT pulmonary angiogram and there's no pulmonary emboli
This could one of the causes of pulmonary hypertension.
02:51
So the diagnosis is idiopathic meaning unknown cause of pulmonary hypertension with RV failure.
02:58
It's thought to be, in some form, genetics and might be related to the fact that her mother had lupus erythematosus.
03:07
It's another form of cor pulmonale, the first form we saw earlier
was due to cigarette smoking and damage to the lungs.
03:15
This one is due to damage to the very small blood vessels in the lung.
03:19
It's also called primary pulmonary hypertension or pre-capillary idiopathic pulmonary hypertension.
03:26
The little pulmonary capillaries are obliterated.
03:29
There's severe vasoconstriction in the lung
and there's also dysfunction of the pulmonary endothelium that is the lining of the small blood vessels.
03:38
And pulmonary angiography is these patients demonstrates a lung vasculature
that looks like a pruned tree where the leaves have been cut off.
03:46
This used to be untreatable but these days we have some vasodilating drugs that do help.
03:53
Endothelin blockers, prostacyclin, phosphodiesterase-type inhibitors,
that's like sildenafil, Viagra that also dilates the blood vessels in the lung,
and there's a whole bunch of others that are currently being investigated.
04:07
The major treatment if she doesn't respond to medicines would be lung or heart/lung transplantation.
04:13
And then of course in the future, we're expecting new therapies for this condition all the time.
04:20
The prognosis for this condition was formerly very poor.
04:24
It's much improved with the new drugs and with the possibility for heart or heart/lung transplantation.