Photographs by Laurie @ Horizons Photography

June 5, 2013

technical not emotional

I have have been getting a lot of questions about Joshua's particular diagnosis. So I thought I would post today a little bit about his CHD. As I mentioned in yesterday's post, the term CHD, or Congenital Heart Defect is a blanket term for a great number of diagnosis' and I prefer to keep it that way, however in an effort to help people understand I will tell you more about Josh's form of CHD.

Pulmonary atresia (PA)
No pulmonary valve exists, so blood can't flow from the right ventricle into the pulmonary artery and on to the lungs. The right ventricle acts as a blind pouch that may stay small and not well developed. The tricuspid valve is often poorly developed, too. An opening in the atrial septum lets blood exit the right atrium, so venous (bluish) blood mixes with the oxygen-rich (red) blood in the left atrium. The left ventricle pumps this mixture of blood into the aorta and out to the body. The only source of lung blood flow is the patent ductus arteriosus (PDA), an open passageway between the pulmonary artery and the aorta. If the PDA narrows or closes, the lung blood flow is reduced to critically low levels. This can cause very severe cyanosis. Early treatment often includes using a drug to keep the PDA from closing. A surgeon can create a shunt between the aorta and the pulmonary artery to help increase blood flow to the lungs. A more complete repair depends on the size of the pulmonary artery and right ventricle. If they are very small, it may not be possible to correct the defect with surgery. In cases where the pulmonary artery and right ventricle are a more normal size, open-heart surgery may produce a good improvement in how the heart works. If the right ventricle stays too small to be a good pumping chamber, the surgeon can compensate by connecting the right atrium directly to the pulmonary artery. The atrial defect also can be closed to relieve the cyanosis.
Pulmonary atresia occurs in about one out of every 10,000 live births. 

Joshua was also born with an ASD, which was closed when he was six months old. He has had two balloon catheterizatons, one device implanted through a catheter and a catheterization to run tests (that was the more recent one). All in all there have been four. The first two didn't work, so he underwent his first open heart surgery when he was six months where they cut muscle from his right ventricle to try and make more space for the blood to flow, at that time they also cut a small flap around the ASD so that his oxygen levels wouldn't be so 'up & down'; however, by cutting that flap they caused the hole to become to large and Josh nearly lost his life. He was put on life support and almost rushed into more surgery before they decided that they would attempt a repair (using an occluder) in the cath lab. It worked. When he was two he again went into heart failure  and underwent surgery number 2, where he was given a pulmonary valve and where they attempted to fix his tricuspid valve. Less than a year after that (10.5 months to be exact) Josh was once more in heart failure and it was discovered that he was rejecting the pulmonary valve. They went in again, replaced the valve and again tried repairing the tricuspid valve using human tissue.

All these attempts have failed so far, he is now rejecting the pulmonary valve again and this time they will not try to fix the tricuspid valve but rather just give him a new one.

There you go. All the scientific stuff with none of the emotion ;)


The Stepiens are praying. #redforjosh