Celeste,
The pulmonary pressures can be higher than systemic pressures. That is
a grave finding.
Joey's pressures were measured at 110 per cath on his original
diagnostic cath. This was higher than systemic for a young child, young
children's blood pressures run lower than ours and his systemic
pressure was around 90/40.
We never repeated the cath because all indications from his echos were
that his pressures continue to climb. Having very high pulmonary
pressures made him a great risk for anesthesia, and I did not want to
expose him to that risk if it was not going to change our course of
treatment. Although Dr. Doyle did give me estimates of his pressures, I
think the current thinking is to just say that the pressures are
greater than systemic, because it has been found that echocardiograms
can overestimate the pressures. The only way to tell what the pressures
really are is to measure them by cardiac cath. And that can pose a risk
to the patient, like I explained to Pam. If the pulmonary pressures are
really very high and the person has a reaction to anesthesia on the
table, any medication you give to raise the blood pressure will also
raise the pulmonary pressures and is likely to be ineffective, which
can basically result in sudden death.
Joey did not have a heart defect or a shunt. He had normal physiology
with PPH. Having pressures in excess of systemic puts a lot of stress
on the right side of the heart, which must work very hard to get blood
to the lungs. When he was sick or his heart was otherwise stressed he
could drop his sats very fast, because his heart just couldn't work
hard enough to get blood into his lungs. He was subject to sudden grave
episodes of decompensation, but otherwise had normal sats.
Noel has eisenmengers physiology, which I think means that the
pressures in her lungs are high enough to where they are causing the
blood to flow backwards through the shunt. Which means that her blood
does not get oxygenated, and she has very low sats. On the other hand,
because she can shunt backwards when the lung pressures get very high,
it puts her less of a risk from physiology for a pulmonary bleed
(platelets are another issue), because there is a way to release some
of that pressure on the lungs.
I am not sure which is better. It is like picking your poison(which we
don't get to pick, anyway). PH is a rotten condition.
I am sure you are probably wondering how long things can go on this
way, and that is anyone's guess. Not all PH is the same. Joey's PH
progressed very rapidly, much more so than the norm. One time, when
Joey was in the hospital with a g.i. bleed, Mr. Blunt-talking doctor
was on staff and basically told me they were worried about Joey dying
while he was in the hospital. He told me in very technical terms, but
it still made me cry all night. Joey was four, but he was very bright.
He told me the next day not to worry about what that doctor had told me
because he was not a real doctor, anyway. (This was a remark that
amused many people when they heard it, because this man was one of the
top experts in the country on pediatric arrhythmias, but he had failed
to impress Joey.)
"I'm not going to die, I am going to grow to be a bigger boy," he told
me. Dr. Doyle came by later and we discussed DNR orders. Although we
signed the papers, Joey lived for another six months and continued to
be astoundingly active. When he died Dr. Doyle was present for the
autopsy. Joey's pathology report surprised him. Some of the
pathological changes in Joey's lungs were consistent with pulmonary
hypertension that would normally have been present for at least six to
eight years. Nobody knows how you get to that stage when you are not
yet five years old.
Hope this helps your understanding and does not sound too grim. I am
very sorry for all that you are going through, and you are constantly
in my prayers.
Jennifer