Brief case presentation...

This is an interesting patient that I was lucky to see through to a diagnosis.

A few weeks ago a 6 year old girl came in breathing 60 times/minute, lethargic, and cyanotic with a heart rate of 130. History was limited. She's normally healthy and hasn't had problems according to her parent. Further physical exam demonstrated slow capillary refill, digital clubbing, and crackles in her lungs. No heart murmurs noted but she did have what I think would best be called a fierce right ventricular heave.

We treated for pneumonia with oxygen, ampicilin and gentamycin. Likely unnecessary, but she did get better. Once her respiratory rate returned ot normal a continuous "machinery-like" murmur was noted below the left clavicle. Still no cardiac murmur. We referred her to the clinic folks who have funding for fixing things like congenital heart problems in kids.

I did some reading, and her history sounded like a Tetralogy of Fallot, although on exam she lacked a harsh murmur from the pulmonary stenosis or a murmur from a septal defect. I decided it must just be a patent PDA that has lead to heart failure.

Today there was a rare visit to Mae Sot Hospital by a team of pediatric cardiologists. Normally they have to ship the kids to Chiang Mai for evaluation, but this makes it easier. The whole trip was interesting...taking a dozen refugees and their kids to a modern hospital. They looked so out of place. We took the stairs to the fifth floor either because the elevator would have been scary for them or because they didn't want to break up the group. They squatted in the hall while we waited to be seen. One kid puked on the fancy floor and I was happy to be able to say "it's not a big deal" in Burmese to the somewhat panicked mom. Another 37 y/o woman, 8 months pregnant, carried her chunky 2 year-old up all five flights, smiling the whole way. These people are tough.

They x-rayed the girl and found a classic boot-shaped heart shadow. Then an echo was done. The diagnosis was Tetralogy of Fallot. It turns out that (I think) from birth she has had a severely stenosed pulmonic valve and so most of the flow to her lungs has been through the PDA. I didn't see anything about a septal defect. The end result is no murmur other than the PDA (ie her pulmonic has too little flow to cause an appreciable noise).

So, TOF it is, although not the classic presentation. As great as Nelson's is, it lead me somewhat astray. She'll be going to Chiang Mai on the next trip to be evaluated by surgeons/interventional cardiologists. Because of the severity, I'm not sure if they'll have a great repair, but she'll have a chance.

This would have been MUCH easier if it had been diagnosed at birth. She was likely (as are most of these kids) born at home though. I may start doing newborn rounds every morning though because I'm not sure if the clinic is sensitive enough to pick up all these things. We'll see. It will likely be good for me and the kids.

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