Diagnosis

Double inlet left ventricle

As expected, today's cardiologist visit gave us many of the answers we have been waiting for, while at the same time confirming for us that the future for Brent and our family will look very different than what we could have envisioned.

The official diagnosis for Brent's heart defect is "Double inlet left ventricle" and it is very, very rare.  It only occurs in 5-10 out of every 100,000 live births.  Below is a brief technical explanation of the condition.

In the normal heart, the right and left lower chambers (ventricles) receive blood from the right and left upper chambers (atria). Oxygen-poor blood returning from the body flows to the right atrium and right ventricle. The right ventricle then pumps blood to the pulmonary artery, which carries blood to the lungs to pick up oxygen.  The blood, now oxygen rich, returns to the left atrium and left ventricle. The aorta then carries oxygen-rich blood to the rest of the body from the left ventricle.  However, in those with DILV, only the left lower heart chamber is developed, and both upper chambers carry blood into this ventricle. This means that oxygen-rich blood mixes with oxygen-poor blood. The mixture is then pumped into both the body and the lungs.

Brent's version of DILV also has a transposition of the great vessels in which the aorta arises from the small right ventrical, and the pulmonary artery arises from the left ventricle.  (basically the two ventricals are flip flopped).  

Additionally, he has:

• Ventricular septal defect
• His aorta is anterior and leftward
• L looped ventricles

In short, Brent will be faced with at least two, and potentially more open heart surgeries by the time he is 3 years old.  The 1st could come within the first 10 days of him being born.  The 2nd would be around 4-6 months old, and the 3rd would be around 2-4 years of age.

After that, the long term prognosis is unknown.  If the surgeries go well and he doesn't have any other complications, he should be able to live a relatively normal life.  The questions will be how long the heart will hold out, will he need additional heart surgeries in the future, possibly a heart transplant at some point, etc.  And of course, multiple open heart surgeries on infants and toddlers is a very scary prospect for us.  Still, if I had been born with this condition, there wouldn't have been any treatment options.  So, I am extremely grateful to live in a time where medical technology is going to give my son a fighting chance at life.  And of course, we are still praying for healing, guidance as we make decisions on treatment options, hospital selections, and much, much more.

At lunch today, I kept thinking about the odds.  5-10 out of 100,000!!!  I was telling Grace and kept thinking to myself - "how in the world did this happen?"  One day I hope to get a similar question from a curious, handsome little boy.  And when I get that question, I plan on telling him that it means he has a "very, very special heart made only for very special little boys".

David

P.S. - Specific Prayer Requests At This Time:

• Pray Brent's Aorta continues to grow and develop between now and the birth
• Pray that his ventricular septal defect does not narrow anymore
• If these first 2 things happen, he may not need his first open heart surgery until 4-6 months old instead of within the first 10 days.
• Pray for guidance in setting up the right hospitals and surgeons for his care
• Pray for strength and peace for the family and continued health for Grace throughout the remainder of the pregnancy