“Our family is suffering terribly”: A Tragic Tale Of Post Pump Chorea by Gary Grist RN CCP Emeritus
“Posted by CCF MD on November 26, 1997 at 12:01:09: In Reply to: postpump chorea posted by CBaeta on October 26, 1997 at 17:06:52: My 7 year old son underwent his second open heart, with cpb and hypothermia, on July 28th of this year. He went into the surgery a very neurologically healthy and bright little boy. He has been diagnosed with Postpump Chorea. We have a host of excellent doctors but no one knows how this happens or what to do now except wait. Our family is suffering terribly. My son now has dystonia, hypotonia, occular [sic] motor atresia, speech problems, academic problems, personality changes, is very emotional, and ADHD. He had a normal EEG and MRI two weeks post op. Would we expect these to change? What can we do? What is his long term prognosis? I have read everything I could find on this subject but can not [sic] figure out why this happens only in the very young, usually infants yet occurred in my seven year old. Does this happen in adults? Why or why not? I would love to hear from anyone with any interest or knowledge on this subject.” http://www.medhelp.org/posts/Neurology/postpump-ch
One can search the internet for “post pump chorea” to find letters like the one above from affected family members seeking advice and recourse. I picked this letter because it best expresses a family’s anguish. I think the author may even be a doctor (MD). It was posted in 1997, but letters posted as late as 2015 can be found.
Post pump chorea is a serious cardiopulmonary bypass complication that occurs unexpectedly, usually within two weeks of surgery. Most suffers are children. Choreoathetoid (chorea) movements are common. Severe cases can develop dystonia, hypotonia, aphasia, learning difficulties, emotional lability, personality changes and attention-deficit/hyperactivity disorder. Symptoms may gradually disappear over time or never resolve. The outcome can be devastating to a patient and heart breaking to a guilt-ridden perfusionist who has no idea why things went so badly days after an uneventful bypass procedure. Post pump chorea is thought to be a slowly developing brain injury not detectable with EEG or MRI and somehow caused by the heart/lung pump or how the pump is used.
Back in the 1970s and 80s when I used bubble oxygenators, the process utilized a special high oxygen and high carbon dioxide (hyperoxia/pH-stat) gas bubbled through the blood in a vertical column; much like the air pump makes bubbles in a fish tank. As crude as that may seem, I had no cases of post pump chorea during that time. Then in the late 1980s, when I began using membrane oxygenators I jumped on board the trend of the times and began using normal oxygen and normal carbon dioxide (normoxia/alpha-stat) gas and no bubbles. The consensus of the perfusion community then was that this was “better” for the patient. My old surgeon used to say “The enemy of “Good” is “Better”. He was older and wiser and I was younger and foolish. And I was looking for “Better” because that’s what all the other perfusionists were doing. So I switched with the surgeons’ acquiescence. They were old bubbler-trained surgeons and trusted the fizzes, foams, froths, gurgles and effervesces that they could see and not the membranes which they couldn’t.
About the same time as my switch to membrane oxygenators, I noticed an uptick in certain problems, including chorea. I was guilt-ridden because I was the one who wanted to change things. But my surgeons never pointed any fingers and assured me that they had faith in me. Other programs noticed an increase in chorea during this time period as well (1). Back then I did not correlate these problems to cooling the patient too rapidly (membrane oxygenators had very efficient heat exchangers) and using normal oxygen and carbon dioxide levels.
As time passed, I realized that my pediatric patients tended to do better overall on the membrane oxygenators (shorter hospital length of stay and better survival and no chorea) when I used slower cooling and hyperoxia/pH-stat control. The brain uses more dissolved oxygen than other organs when it is cold. And under these conditions oxygen is released better from the red blood cells when the carbon dioxide is high (2). So this strategy may protect the brain better from whatever damage causes post pump chorea. The majority of adults, usually being less cool, tend to do better with normal oxygen and carbon dioxide levels. But that is by no means etched in stone. It varies from patient to patient and I don’t claim to have solved the post pump chorea problem
Unfortunately, I don’t believe that there have been any new answers for ‘CCF MD’ since the 1997 letter was written over 20 years ago (3).
Below is the link to a short YouTube video showing post-pump chorea.