Heterotaxy and the heart

Clara has heterotaxy – a condition which means ‘different arrangement’. It commonly involves the heart and other organs, especially the stomach, intestines, spleen (often missing or in smaller pieces – Clara the latter), and liver.

In Clara’s case, we knew at our 20 week scan that her stomach was on the wrong side (the right side, rather than the left). We didn’t know anything else was to come. We saw a heart specialist first of all after that discovery – at the time I didn’t know why. It turns out, almost all forms of heterotaxy involve heart defects. It also regularly displays intestinal malrotation, and polysplenia (multiple small spleen). If only I’d known that sooner. They told me not to Google it. I wish I had done. I’d have known what to look out for. Like the night she had her first bilious vomit. I could have shouted – and maybe saved more of Clara’s gut.

Heterotaxy, congenital cardiovascular malformation and gastrointestinal abnormalities are strongly associated. [Prenatal diagnosis of left isomerism with normal heart: a case report]

Clara has a ‘left atrial isomerism’ heart defect – she has two left atriums rather than a left and a right. Usually this is associated with other structural defects such as holes, slow paces, and problems with the electrical systems.

Most patients with left atrial isomerism will require surgical procedures or catheterization to repair holes in the heart or other problems. Some patients will require implantable pacemakers or defibrillators to control abnormal heart rhythm. In rare cases, patients with left atrial isomerism won’t require any treatment, though they will need to visit a pediatric cardiologist regularly for checkups. [The Children’s Hospital of Philadelphia].

Clara had an ECG and an echo in March when she was a few months old, and her results showed her heart function was normal. Whilst we know she has left atrial isomerism, including some unusual plumbing of her vessels returning blood back into her heart, her heart pace and blood flow are normal.

Our medical miracle continues to work her very own Clara magic. Her ultrasound today revealed all is still functioning well and as expected – brilliant. We’re waiting on the results of today’s ECG, but also expecting no change. Clara doesn’t require any treatment – she’ll be checked over by cardiology again in a year.

How she does this, picking the rare conditions but always the best outcome, I’ll never know.

Also miraculously, Clara took a ten minute nap during the course of her ECG, falling asleep the minute we walked into the department and waking up when we left.

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