A month ago, a paediatrician friend from
Alibag called me about a month-old baby with an incidental heart murmur in an
otherwise normal baby. He also thought that the pulse oximetry reading was low
but not very sure as baby was moving a lot. The baby's parents made the long
journey to our hospital, and after a quick exam, the baby's oxygen saturation
was a worryingly low 65%.
The echocardiogram showed a congenital heart
disease common for a cardiologist: Tetralogy of Fallot. This condition
is a combination of a large hole in the heart and a critical narrowing of the
blood vessel leading to the lungs. Because very little impure blood reaches the
lungs, the blood can't get enough oxygen, which results in low saturation and a
baby who is constantly irritable.
When I sat down with the parents, they were
still completely clueless about what was happening inside their child's heart.
I asked them what problems they'd noticed. The mother immediately replied,
"Constipation, doctor." Her father-in-law was quick to add, "Gas
and tummy aches, doctor."
I was losing patience. I asked them to just
describe what happened and to not tell me what they thought was wrong. The
mother then described how every time the baby strained to pass a stool, she
cried a lot, "suffocated," got breathless, and almost passed out. I
asked if this happened every time she passed a stool. "No," they
said, "just sometimes, a couple of times a day." They had even tried
various oral syrups to treat what they thought was constipation.
What they were describing wasn't constipation,
but a cyanotic spell. A Spell as the name indicates is a dangerous
symptoms and every spell is a threat to the baby's life. The triad of cyanotic
spell include shallow but fast breathing, deep cyanosis and certain degree of
unconsciousness! Baby was passing stools
as she lost control of her senses (Like what happens during a convulsion) I
asked them why they hadn't noticed the baby was blue. The father-in-law
interjected, "Blue? What blue? She's been dusky coloured since
birth!"
I explained what was happening and that we
needed to perform an emergency procedure. The parents, who thought their child
just had constipation, couldn't accept the need for an emergency cardiac
intervention. I gave them time to speak with their paediatrician and decide.
Thankfully, they gave their consent, and we
performed the procedure within a few hours. It was a straightforward procedure;
we placed a 6mm stent across the narrow artery, which immediately increased
blood flow to the lungs. The baby's oxygen saturation shot up to 96% on the
table, and within hours, she was playful and active. The family was ecstatic
and went home the next day.
It's been a month since the procedure, but
I've had a tough time explaining that the baby will need a corrective
open-heart surgery in another 6-8 months. The parents ask why, as the baby has
no symptoms now. The parents are convinced that the heart disease is cured
because the baby's colour is pink now and the "constipation" was
gone.
I've warned them that she may again develop
low saturation as she grows and may again come back with
"constipation."
I've told them to enjoy the baby growing
without symptoms for the next few months and that they can probably even
celebrate her first birthday before the definitive surgery.
P.S. A simple, modern procedure, where we placed a stent
through a small needle hole in a vein, has made a huge difference. Just a few
years ago, this would have required a major operation to open the baby's chest,
which meant a longer stay in the hospital and more risks. This new, less
invasive method gets the baby better faster and makes their final, more
permanent heart surgery safer down the road.
Dr. Srinivas L, Chief & Senior