Heart Diagnosis, Explained

Two weeks after Kiran’s first anatomy ultrasound, he had a second, more extensive ultrasound. I was by myself that day, because so many had reassured it was nothing to worry about.

It was the day I first heard the words “Tetralogy of Fallot” … only I didn’t retain those words. Best I could say was it was something that sounded French. I was in such shock.

Thankfully, they didn’t make us wait long. We had an appointment the very next day with a wonderful specialist who explained the diagnosis to us and had encouraging words about the survivability of the condition.

Later in the pregnancy – and confirmed after birth – two more heart diagnoses were added, just to make things interesting: pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs).

Kiran’s full heart diagnoses, then, is made up of three separate conditions: Tetralogy of Fallot, Pulmonary Atresia, and MAPCAs.

Tetralogy of Fallot alone has four defects associated with it:

  1. Ventricular Septal Defect (VSD) – a hole between ventricles (the pumping chambers) of the heart. This causes oxygenated and unoxygenated blood to mix.
  2. The aorta, which is usually above the left ventricle, is to the right and directly above the VSD. This means both oxygenated and unoxygenated blood are going out to the body.
  3. Thickening of the right ventricle wall. The right ventricle is working hard to pump blood out to the lungs through smaller arteries. What happens when you work a muscle? It gets bigger.
  4. Pulmonary valve defect – In Kiran’s case, pulmonary atresia so more severe – His pulmonary valve didn’t form at all. Typically, this means blood would have no pathway to get to the lungs.

That’s where the MAPCAs come in. They provided a different pathway to get blood from the heart to the lungs to be oxygenated. This is what allowed him to remain surgery-free until he was 10 1/2 months old.

Both Tetralogy of Fallot and Pulmonary Atresia are listed on the CDC’s list of critical congenital heart defects, meaning heart defects that require surgery or other procedures in the first year of life. But it was his MAPCAs that took us all the way to California for a specialty surgeon.

But that’s another story.


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