August 23rd, 2009 by scanman in Better Health Network, True Stories
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…in four parts, from Paul Levy’s blog.
It is, says Paul, “From a friend of a friend,” and it starts thus…
My son is sleeping right now…had a rough weekend – his blood pressure dropped, his blood count was decreasing, and he had chest and neck pain. The clinical team adjusted his meds, gave him a unit of blood, and are now trying to figure out what to do next. He is scared and worried and wants so desperately to be “normal” again. He is scheduled for leg surgery this afternoon and then we wait to see what the next steps will be.
While I have a few quiet moments, I thought I’d document the story of how he made it this far….it is a story of extraordinary luck and a fair amount of clinical heroism.
My son was born 17 years ago with transposition of the great arteries (his heart had over-rotated and was pumping in a way that didn’t allow oxygenated blood to move from the lungs to the body and back again) so he had a 9 hour operation at a week old to reconstruct his heart.
…read the rest of part one
…and parts two, three & four.
Here, in my opinion, is the best passage from the entire saga…
My son is receiving absolute top-notch care from the only place in the area that could have saved him, but was by luck, not by any “consumerism” on our part – we didn’t Google “teenage arterial switch survivor with heart attack” or pull up HealthGrades to find the best hospital or doctors to treat him….we have benefited from the kindness and skill of a community of health care providers affiliated with a hospital that was uniquely situated to help him, but the only choice we had in this was what hospital to drive him to.
…
In part 2, we learn the reason for the young man’s sudden collapse…
We learned much later that the problem that caused the heart attack was due to his reconstructive surgery when he was a baby…as he grew and became more active, one of the reimplanted coronary arteries became pinched between the rebuilt pulmonary artery and the aorta….this was an inevitable result of the surgery that saved his life 17 years ago and would have happened at some point – while swimming, riding his bike, walking in the neighborhood, playing lacrosse, or running by himself in the neighborhood as he trained for cross country….so the fourth link – he happened to have his attack while at a school with trainers equipped with an AED, with coaches and parents and teammates right there ready and able to help him. He wasn’t alone….and he was in the best possible place to have his attack (even though he complicated things a bit by having it in the woods and falling down a steep bank)
…
Congenital cardiovascular abnormalities, especially anomalous coronary arteries, are amongst the commonest of causes of sudden cardiac death in athletes.1 Ramona had posted about a young man who collapsed and died during the Little Rock Marathon in 2008. That unfortunate young athlete had a rare disease of the coronary arteries.
Coronary artery anomalies constitute 1–3% of all congenital malformations of the heart. In approximately 0.46–1% of the normal population, anomalies of the coronary arteries are found incidentally during catheter angiography or autopsy. The etiology of coronary artery anomalies is still uncertain. Maternal transmission of some types has been suggested, particularly when only a single coronary artery is involved. Familial clustering is also reported for one of the most common anomalies, in which the left circumflex coronary artery (CX) originates from the right sinus of Valsalva. Anomalies of the coronary arteries may also be associated with Klinefelter’s syndrome and trisomy 18 (i.e., Edwards syndrome). Cardiac causes for early and sudden infant death include anomalies of the coronary arteries; the Bland-White-Garland-Syndrome may be one relevant cause. Anomalies of the coronary arteries found in children may be associated with other congenital anomalies of the heart like Fallot’s syndrome, transposition of the great arteries, Taussig-Bing heart (double-outlet right ventricle), or common arterial trunk.2
Normal Coronary Arterial Anatomy
Common variants are anomalies with origin from the contralateral side of the aortic bulb. These include an origin of the LMA or the LAD from the RSV or the proximal RCA and an origin of the RCA from the LSV or the LMA. There are four possible pathways for these aberrant vessels to cross over to their regular peripheral locations: (1) “anterior course” ventral to the pulmonary trunk or the right ventricular outflow tract, (2) “interarterial course” between the pulmonary artery and aorta, (3) “septal course” through the interventricular septum, and (4)”retro-aortic course”. Clinically, course anomalies of the coronary arteries are subdivided into “malignant” and “non-malignant” forms. Malignant forms are associated with an increased risk of myocardial ischemia or sudden death and mostly show a course between the pulmonary artery and aorta (i.e., “interarterial”). The most common case is an origin of the RCA from the LSV that courses between the aortic bulb and the pulmonary artery. Anomalies of the LMA or the LAD arising from the RSV with a similar course are associated with higher cardiac risk, too. It is suggested that myocardial ischemia and sudden death result from transient occlusion of the aberrant coronary artery, due to an increase of blood flow through the aorta and pulmonary artery during exercise or stress. The reason is either a kink at the sharp leftward or rightward bend at the vessel’s ostium or a pinch-cock mechanism between the aorta and pulmonary artery. Up to 30% of such patients are at risk for sudden death.2
…
The young man in this story probably had something like this after the surgical correction (Arterial Switch Operation) for TGA…
“Malignant” course of LAD
…a classical malignant course of the LAD between the Aorta and Pulmonary artery.
…
References:
- Sudden Death in Young Athletes: Screening for the Needle in a Haystack – Free full text article in American Family Physician.
- Text about congenital coronary artery anomalies and the two figures are from this textbook – Multi-slice and Dual-source CT in Cardiac Imaging.
Start Slide Show with PicLens Lite 
*This blog post was originally published at scan man's notes*
July 30th, 2009 by Medgadget in Better Health Network, News
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The scans presented here are of a ten year-old German girl who was discovered to be missing the right hemisphere of her brain. Incredibly, she is perfectly normal, except for a history of seizures and a slight weakness on her left side. Attending school with others of her age, it is reported that she is able to study and play sports, just like other kids around her. Of course, the mystery is how is this all possible? To answer the question, University of Glasgow scientists used an fMRI to see where the left eye’s vision is processed. Turns out that the brain’s visual area responsible for the right eye offered up some space for the left.
Normally, the left and right fields of vision are processed and mapped by opposite sides of the brain, but scans on the German girl showed that retinal nerve fibres that should go to the right hemisphere of the brain diverted to the left.
Further, the researchers found that within the visual cortex of the left hemisphere, which creates an internal map of the right field of vision, ‘islands’ had been formed within it to specifically deal with, and map out, the left visual field in the absence of the right hemisphere.
Dr Lars Muckli of the Centre for Cognitive Neuroimaging in the Department of Psychology, who led the study, said: “This study has revealed the surprising flexibility of the brain when it comes to self-organising mechanisms for forming visual maps.
“The brain has amazing plasticity but we were quite astonished to see just how well the single hemisphere of the brain in this girl has adapted to compensate for the missing half.
“Despite lacking one hemisphere, the girl has normal psychological function and is perfectly capable of living a normal and fulfilling life. She is witty, charming and intelligent.”
The girl’s underdeveloped brain was discovered when, aged three, she underwent an MRI scan after suffering seizures of brief involuntary twitching on her left side.
The scientists believe the right hemisphere of the girl’s brain stopped developing early in the womb and that when the developing optic nerves reached the optic chiasma, the chemical cues that would normally guide the left eye nasal retinal nerve to the right hemisphere were no longer present and so the nerve was drawn to the left.
This implies that there are no molecular repressors to prevent nasal retinal nerve fibres from entering the same hemisphere.
Dr Muckli added: “If we could understand the powerful algorithms the brain uses to rewire itself and extract those algorithms together with the general algorithms that the brain uses to process information, they could be applied to computers and could result in a huge advance in artificial intelligence.”
Press release: Scientists reveal secret of girl with ‘all seeing eye’…
*This blog post was originally published at Medgadget*
July 26th, 2009 by scanman in Better Health Network, True Stories
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via The Trial of a WhiteCoat – Part 14.
The radiologist that read the film had a habit of going to the surgeons the following day and asking them what they had found. He would open up a blank report so that it looked as if it was dictated at the time of the exam, but would then hold the reports as “preliminary” and finalize them after dictating in the results of the surgeries. That way it looked like he had picked up on all these small findings before anyone else knew about them. He was a decent radiologist, so no one seemed to mind that he was adding all these findings after the fact. Now it burned me.
I’m offended.
No.
That’s too light.
I’m pissed off as hell.
I believe the Americans call this kind of thing “Monday morning quarterbacking.”
Whatever you might call it, this is cheating in my book.
I don’t know why they let that radiologist get away with this kind of behaviour.
Moreover, I can’t believe that anyone would take the man’s reports seriously, leave alone the surgeons that he got information from. If by chance I was a surgeon in that hospital, I would intentionally throw him red herrings.
In case you haven’t been following Whitecoat’s account of his malpractice case, see previous posts of his epic saga here. Far better than reading any crime/legal thriller, cheap or otherwise. John Grisham could take lessons from Whitecoat.
*This blog post was originally published at scan man's notes*
July 21st, 2009 by scanman in Better Health Network
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A small mental exercise for medical bloggers.
See the following three portable (bedside) chest radiographs that were taken in an ICU setting. They are in sequence.
See if you can guess the story that they tell.
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*This blog post was originally published at scan man's notes*
July 20th, 2009 by Kenneth Trofatter, M.D., Ph.D. in Better Health Network, Health Tips
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I received the two comments below from readers and use this opportunity of their tragic experiences to revisit a concern that I raised about two years ago regarding methotrexate therapy for the presumptive diagnosis of ectopic pregnancy….
Melissa O. said…
I was told I had an ectopic pregnancy and was advised I was in need of a Methotrexate shot. I got it. One week later my hormone level was continuing to rise. Low and behold 4 days later my ultrasound showed I was carrying twins. The Dr.’s had presumed ectopic too early. Getting the shot caused me to loose Twin A and to give birth to a very much underweight 28 weeker. This experience has changed my life forever. My son fought to survive…he continues to today now 13 months old. I would hope anyone who is told they have an ectopic pregnancy would be cautious when it comes to this shot. Yes I agree it helps if your life is in danger due to an ectopic pregnancy. Just take time to ensure there is no doubt that’s what it is. My Dr couldn’t see the baby so assumed ectopic, however carrying twins like I was you’re not able to see as early as a single pregnancy. My son is paying everyday because of my mistake and doing as one Dr. said make sure you have more than one confirmation, it could cost you a perfectly healthy baby in the end.
Fri Jun 19, 05:45:00 PM 2009
Anonymous said…
Hi can someone help me? My husband and I were trying for a baby and I fell pregnant (good news). I started having a few brown spotting and slight cramping which I was advised by my GP to go to the hospital for a scan. Whilst there I had many tests and the doctors thought it might be ectopic and said he was going to keep me in for a few days to monitor my blood levels. I had a scan but being only five weeks it was hard to say. I was referred to another doctor on the ward and he told me it was ectopic. I trusted his knowledge and he said he needed to give me methotrexate now as it was Friday so the pharmacy would be shut. I was shocked but agreed of course. 3 days later I was told the baby is still alive and is in my womb. My blood levels increased after 3 days and then decreased from 7000 to 6000 on the 7 days. How long will it take to lose my baby as it’s hard to know its alive?
Fri Jul 03, 11:15:00 AM 2009
Ever since methotrexate became popular for treating ectopic pregnancies, I have seen the unfortunate scenario reported by our readers above played out time and time again. Methotrexate (MTX) is an analog of folic acid. It binds tightly to an enzyme called dihydrofolate reductase and when it does so, interferes with the production of tetrahydrofolates. In the end, this interferes with the normal production and repair of DNA by limiting the production of a key nucleotide, thymidine. Other metabolic effects are also known, but the take home message is that MTX can result in lethal damage to cells that are replicating, particularly those that are replicating rapidly, like certain cancer cells.
Because of its documented efficacy in the treatment of malignant trophoblastic cells (choriocarcinoma), MTX has been employed in recent years as an alternative to surgical therapy in selected cases of ectopic pregnancy (Lipscomb, et al. NEJM 2000;343:1325-29). Ectopic pregnancies, by definition, implant ‘outside the uterus’ with more than 95% occurring in the fallopian tubes and about 2.5% in the cornua of the uterus (where the fallopian tubes enter the uterus). For that reason, they are frequently referred to as ‘tubal pregnancies,’ although they can also occur in the cervix, ovary and intra-abdominally. The fallopian tubes cannot restrict the growth of invasive placental tissues, as can the endometrium, and they certainly cannot accommodate a growing embryo beyond a certain point before they rupture and hemorrhage. Indeed, ectopic pregnancies can be quite deadly if not treated appropriately. They are still a major cause of maternal mortality, accounting for 10-15% of all maternal deaths, and they are the leading cause of death in pregnant women in the first trimester. A ruptured ectopic pregnancy is a true medical emergency.
Because of the rising incidence of ectopic pregnancy, the risk (maternal and medical-legal) of not identifying and treating an ectopic pregnancy in a timely fashion, and the widespread acceptance and success of MTX therapy as an alternative to surgical management of an ectopic pregnancy if caught early enough, there has been a coincident increase in the inadvertent use of MTX in unrecognized early intrauterine pregnancies. The usual scenario is one in which the pregnancy is not quite as far along as anticipated and the patient happens to present with complaints of abdominal pain or some spotting and no clear intrauterine pregnancy is identified by ultrasound. The ‘absence’ of an intrauterine pregnancy can be misdiagnosed because the pregnancy really is too early, but in at least one of the scenarios above was more likely the result of the inexperience of the individual(s) performing the ultrasound study.
This situation can be especially confusing if the pregnancy hormone levels (hCG) appear to be low for the expected gestational age based on last menstrual period (as is often seen in women who ovulate later, and hence conceive later, in their cycles) or if a woman has a tender adnexal mass because a hemorrhagic corpus luteum (intraovarian bleeding at the site from which the egg was ‘hatched’) or torsion of an adnexal mass (rare this early in pregnancy) which might be very difficult to differentiate from an ectopic pregnancy.
Since MTX is a category X drug, known to be teratogenic in humans, it is important to ascertain the presence of an ectopic pregnancy rather than simply to use it empirically. Unfortunately, its inadvertent use with an intrauterine pregnancy is most likely to occur during the time of neural tube and very early cardiac development, both of which rely on folate-dependent pathways. Various algorithms are in place that employ ultrasound imaging, quantitative hCG levels, and progesterone levels to differentiate abnormal from potentially normal pregnancies and these protocols can be useful in minimizing the chance of the inadvertent use of MTX and also in directing its use when appropriate for the management of an ectopic pregnancy. Perhaps the greatest risk of ectopic pregnancy is not suspecting that one could be present. Patients who are adequately counseled and followed closely are much less likely to end up in emergency situations.
To our readers above, I am SO SORRY for both of you. This is a failing of the medical system and is a growing concern of mine due to the ready accessibility and simplicity of use of methotrexate (and also another drug, misoprostol, that is used in the ‘medical evacuation’ of the uterus when an inevitable miscarriage is suspected).
My feeling is that it should never be used in an asymptomatic or minimally symptomatic patient until either an ectopic pregnancy is seen, no intrauterine pregnancy is documented (by a competent sonographer) at hCG levels where an intrauterine pregnancy should readily be visible, the patient has significant ‘risk factors’ for an ectopic pregnancy (e.g., previous ectopic, known history of pelvic inflammatory disease or tubal reconstructive surgery) or when there are well-documented abnormalities in the rise of hCG that are highly suggestive of an ectopic pregnancy. My heart goes out to both of you.
Kind regards,
Dr T
This post, Accidental Abortion: Use Of Methotrexate For Misdiagnosed Ectopic Pregnancies, was originally published on
Healthine.com by Kenneth Trofatter, M.D., Ph.D..
