Hypoplastic Left Heart Syndrome (HLHS)

My daughter Krystall was diagnosed with Hypoplastic Left Heart Syndrome (HLHS) at my 20 week scan.  Hypoplastic left heart syndrome is a complex and life limiting Congenital Heart Defect.  Meaning the child is born with the condition.  The left side of the heart, valves, arteries, etc do not grow correctly in the womb.  Hypoplastic Left Heart Syndrome is a rare condition that occurs in around 1 in 5000 babies and accounts for 1% of all Congenital Heart Disorders.

The heart is made up of 4 chambers.  The right Atrium (blood collecting chamber), right Ventricle (blood pumping chamber for the lungs).  Left Atrium (blood collecting chamber), left Ventricle (pumping chamber that pumps oxygenated blood to the body).  The right side of the heart does the work for both sides

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There are 3 options if diagnosed before birth:

Option 1: Termination. 
Option 2: Take baby home to pass away naturally.
Option 3: 3 stage surgery.  Surgery only prolongs the life of a Hypoplastic Left Heart Syndrome sufferer. It is not a cure.

The first stage surgery, (Norwood Procedure) is performed a few days after birth.  The second stage surgery (Bi-directional Glenn Shunt) is performed between 3 months and 9 months old.  The third stage surgery is (Fontan) performed at school age.  There is also possibility for transplant at teenage / adult years.

Krystall is on medications. These are: Frusemide (a loop diuretic) to help her to urinate to stop water build-up.  Loop diuretics act in the kidney to remove excess water from the blood, by causing an increase in the removal of salts such as potassium and sodium.  Spironolactone ("potassium sparing" diuretic ) Spironolactone inhibits the action of aldosterone causing the kidneys to excrete salt and fluid while retaining potassium.  Too much water build-up will put strain on the heart and possibly cause heart failure.  Captopril an angiotensin-converting-enzyme inhibitor (ACE inhibitor) to her make heart function stronger.  Heart failure is not where the heart stops.  It is where the hearts muscle is not pumping as well as it should.

A child with Hypoplastic Left Heart Syndrome will develop their skills at roughly the same age as other children.  They will be able to ride bikes, play football (not competitively), run and walk.  Although they will get out of breathe quicker and need to rest more frequently.  Krystall will have to attend follow up outpatient appointments all her life.