Technical (medical) cardiologist report

OK so for non-medical folks - Ollie has holes in his heart and his one valve leaks a lot.  They could repair it completely but that will be very risky and might make him worse in the long run.  So they will put in a shunt to help the right side of his heart and repair the valve so that it doesn't leak so much and the pressure in the heart is less.  That makes the heart last longer and its work easier. What exactly must be done is not 100% clear at the moment because Ollie is a borderline case.  The specialists will discuss this tomorrow and then let us know, but it might also be a decision that is taken in theatre.  The operation will happen either on Thursday or Friday, depending on bed availability.  If you have some medical knowledge or are interested, a more technical explanation (with my limited cardiology knowledge) follows.  

Ollie was born with coarctation of the aorta and a complex defect call unbalanced atrioventricular canal (uAVC) or atrioventricular septal defect (uAVSD).  The coarctation (narrowing of the aorta) was fixed at 10 days old and the other defects not.  The fix was done very well and is still in perfect condition.  The uAVC defect is composed of a common AV valve (and valvular deformities) and unequal ventricular sizes.  In Ollie's case, the left ventricle is small and he has a cleft in the mitral valve leading to significant regurgitation.  Ollie had a pulmonary band placed at 3 weeks of age to prevent flooding of the lungs from the heart which occurs via the overload of the right heart through the left to right shunting.

Baby's with Down's syndrome are sometimes born with this defect but in their cases, the ventricular sizes are similar.  In such cases, repair is straightforward via a surgical procedure called a biventricular repair (BVR).  If the left ventricle is very small or even absent, then a procedure called a Fontan operation is performed.  This consists of 3 staged procedures that convert the heart to a single ventricle.  Fontan operations were first performed successfully in the 1980's and the majority of these patients are now entering their 40's.  Before 1980, these kids typically survived for 1-3 weeks post birth.  When the left ventricle is small or borderline, the decision is more difficult - if a BVR is attempted and the left ventricle can't sustain systemic circulation, then the patient can end up with poor exercise tolerance, persistent tachycardia and subaortic stenosis.  A good BVR is however associated with cure and normal life expectancy.  A Fontan operation is associated with life expectancy of 40 years and counting.  Ultrasound can only tell so much.  Ultimately the surgeon has to take the decision in situ, with the heart and defects visualized.  

Today the cardiologist confirmed (we knew this from previous scans both in SA and the UK) that Ollie is just such a borderline case.  In all likelihood, they will perform a Glenn shunt and a mitral valve repair.  That won't make him a Fontan or a BVR, which leaves options open for the future.  But it will improve his oxygen saturation and protect his right heart from overload and dilatation from the extra work it helps the left ventricle do through the septal defects.  

Most importantly - we are well and we are LOVED!