Acronyms, Acronyms, Acronyms... An Update On X Man and Mum

Acronyms, Acronyms, Acronyms... An Update On X Man and Mum

HLHS, CHD, DORV, VSD, SVC, GDM... OMG WTF???

In my original post The X Man - Our Journey With HLHS (Hypoplastic Left Heart Syndrome) I introduced us all to our road ahead with our little X Man and his Congenital Heart Defect known as Hypoplastic Left Heart Syndrome. Today we had our next appointment with our Cardiologist (Mum & I thought he was our surgeon, but we found out today he is our cardiologist and that we would meet the surgeon(s) just prior to surgery).

The scan today showed that the left side of the heart is still only around a quarter the size of the right.  This makes it more likely that we will go full term and have no improvement in the left sides size. There have been a couple of very rare cases where the left side of the heart has miraculously grown to a size of 70% that of the right but as the size difference is so dramatic in X Man this looks unlikely in his case.

What we did find out today was what makes X Mans case of HLHS different from "classic" HLHS, which enables us to have the operations here in Brisbane rather than having to travel to Sydney or Melbourne.  In Classic HLHS (Hypoplastic Left Heart Syndrome) there is no "outflow" from the left side of the heart, rather there is "backflow".  In X Man, there is outflow due to a condition known as DORV (Double Outlet Right Ventrical).  Because of this X Man has a variation of HLHS known as Hypoplastic Left Heart Spectrum.

Double Outlet Right Ventricle with Subaortic VSD

In double outlet right ventricle, something goes wrong during the formation of the heart in the womb, and both great arteries (the pulmonary artery and the aorta) are attached to the right ventricle. No arteries, or only a part of the aorta, are attached to the left ventricle.

We also found out today that X Man has a hole in the heart, or in medical mumbo jumbo, a VSD (Ventricular Septal Defect).  This is a hole in the septum, the wall that divides the right and left ventricle. The VSD is actually helpful — it allows X Man to live. Without the VSD, blood that traveled to the lungs and back to the left side of the heart would have no place to go, since the aorta isn’t attached to the left ventricle, and no oxygenated blood would reach the body.

There are a number of types of VSD's, X Man has what is known as Subaortic VSD.  The VSD allows oxygenated blood flowing into the left side of the heart to cross over to the right ventricle, and be pumped out to the body through the aorta.

So, all up X Man has Double Outlet Right Ventricle (DORV) spectrum, subaortic VSD. small left ventrical, mitral valve, aortic valve, hypoplastic aortic arch/aortic coartation, bilateral SVCs (right SVC to right atrium, left SVC to large coronary sinus). This diagnosis provides a positive outlook toward the doctors being able to perform the Norwood Procedure after birth.

An Update on Mum

While Mum was pregnant with Mr Two, Mum was diagnosed with Gestational Diabetes. Through careful diet management Mum was able to control the diabetes.  Last Friday Mum went and had the test done for Gestational Diabetes.  Mum has been careful with her diet from the day we found out we were pregnant, but alas, the results came back today and it is confirmed that Mum has Gestational Diabetes again.  Mum is confident that with continued control of diet that she will be able to manage the Diabetes just like she did with Mr Two.

The blood test also showed that Mum's iron levels were low, which could very well explain why Mum had been feeling off for the past few weeks.  Mum now has to take iron tablets to increase her iron stores to ensure there is enough iron not only for X Man but for Mum as well.  We have been told that Mum can expect to start feeling better as soon as a week from now. Fingers crossed.

Contortionist

We were given a copy of a 4D image the radiologist took whilst performing the scan today of our little contortionist.  Seriously little man, I know it is cramped in there, but how do you get yourself into this position?

X Man

X Man

Upcoming Appointments

We have a couple of weeks off now before we have a full day at the hospital with numerous appointments.  We meet the Diabetic Physician, we then meet the Neonatologist and have a tour of the nursery, and then we are going to meet up with a lady from HeartKids QLD to touch base.

Within the following month we will have another regular checkup at The Mater before another appointment with the Cardiologist at the start of July.  That appointment will probably be the final scan before X Man is born as by that stage the heart will be fully developed and the extent of the deformation can then be assessed ready to prepare for surgery.