Morning Rounds 2

The patient is a 6 year-old male brought in by his grandmother for respiratory distress. Grandmother notes that he has been cyanotic since around 6 months of age, with repeated bouts of similar respiratory distress, but was seen only by traditional tribal medicine practitioners in the Northern Region of Ghana. He was never seen in a hospital before now. She brought him in because his distress is worse than it had been in the past.

She states that he never actually stops breathing, just that he seems to work very hard breathing. She has also witnessed “collapsing” episodes since age 1, and notes that he squats when he’s tired or exerted. She denies noticing any recent cough, wheezing, fever, or drooling. Denies any gastrointestinal symptoms like diarrhea or vomiting. Denies recent illnesses. Denies asthma or known allergies.

The patient was born via NSVD, with a “good” weight and nothing abnormal, but he has had poor weight gain his whole life despite a full diet. He lives at home with the grandmother. He started sitting/walking/talking/socializing at appropriate ages. Both parents died in their thirties of unknown causes. He has no siblings. He has never received any vaccinations. No surgical or medication history.

On exam, the patient appears acutely ill and in severe respiratory distress on O2. He is pale, cyanotic and prefers not to speak.
Vital signs: RR 28/min HR 98/min Sp02 95% on O2
He is normocephalic, no thyromegaly or lymphadenopathy. + conjunctival injection, + nasal flaring, + white scalloped areas on tongue, dentition intact, no swelling of the tongue; His lungs are clear bilaterally; a holosystolic murmur is loudest over the right subclavicular region and the left sternal border, with a gurgling quality, no sternal heave, no chest abnormalities on inspection. Abdomen is soft and nontender; Fingers and toes are clubbed and cyanotic; no skin rashes or lesions noted.

OK for you detectives…..what is it?




Based on all of this, I had a pretty clear idea of what was going on. The squatting spells really nailed the diagnosis of Tetralogy of Fallot for me. Nonmedical readers: ToF is a heart defect patients are born with where the heart vessels to the lungs and to the body don’t form correctly. Causes de-oxygenated (blue) blood to go out to the body. This leads to lots of complications…..Not good.

One of the most interesting things to review was his chest X-ray. I was looking at it thinking, “Hmm, big heart! Looks like an egg? No…more like a little boot. Aha! Boot-shaped heart!” Very fun when textbook descriptions jump out at you…..

I was still interested in the labs though, and will include them for you interested readers:

HgB 21.2 wow! Quite a compensatory mechanism here…..
Plt 159
WBC 5.1
ESR 2
Na+ 145 *
K+ 5.3 *
BUN 12.9
Cr 0.7


An echo confirmed the diagnosis, showing an overriding aorta and turbulent pulmonary artery flow. The boy was started on propanolol, antibiotics, and fluids, and referred for urgent cardiac surgery. Now I just hope he can afford it…..


(Pictured: a pilfered Google image of a bootshaped heart. My patient's heart was bigger than this, but it gives you the idea....)