Dr. Parra- this is a long one

March 25, 2010- Our fist visit to Vanderbilt Children's Hospital for the first (of many) echocardiograms. We met Gabe's cardiologist, Dr. Parra. Jeff, Mama and I liked him very much despite the bad news he gave us. At least now we have a name for all that ails my little Gabe. He has been diagnosed with Hypoplastic Right Heart Syndrome which, in his particular case, consists of Tricuspid Artresia, Pulmonary Artresia, and d-Transposition of the Great Arteries.
Defects vary from individual to individual and although, one may be diagnosed with Hypoplastic Right Heart syndrome, their specific defect could be completely different than what Gabe has.

Let me try to explain these conditions as best and as briefly as possible:

Tricuspid Artresia- there is no tricuspid valve so blood can't flow normally from the right atrium to the right ventricle. As a result the right ventricle is small and not fully developed.

Pulmonary Artresia- No pumonary valve exists. Blood can't flow from the right ventricle into the pulmonary artery and on to the lungs.

d-Transposition of the Great Arteries- The pulmonary artery and the aorta (the major blood vessels leaving the heart) are switched so that the aorta arises from the right side of the heart and recieves blue blood (Unoxygenated blood) which is sent right back out to the body without becoming oxygen rich. The pulmonary artery arises from the left side of the heart, recieves red blood and sends it back to the lungs again. The result is that babies with this condition often appear very blue and have low oxygen levels in the bloodstream.

Dr. Parra basically explained that I would now be delivering at Vanderbilt (originally planned to deliver at Baptist) so that Gabe will have access to immediate cardiac care. Upon birth, he will immediately be taken to the NICU, where they will stabilize him and give him medication that will keep his patent ductus arteriosis (PDA) open (this valve closes upon birth to begin breathing oxygen) so that he will be able to continue breathing as if he were in the womb. They will continue with this medication until his first surgery within the first week of his life outside the womb.

Surgeries:

Gabe will have a series of 3 surgeries (all open heart). The first, as mentioned, will take place within his first week of life. It is called the Blalock Taussig Shunt. They will connect the aorta to the brach from the pulmonary artery with a shunt or tube.

The second, will likely occur at around 3 months of age. It is called a Bidirectional Glenn Shunt. In this surgery the main pulmonary artery is tied off and cut away. The superior vena cava is connected with the right pulmonary branch artery. The head, neck and upper body blue blood is sent to the lungs without going through the heart. The hole between the two top chambers is enlarged. The original shunt is removed.

The Third (and hopefully final) surgery will be around Gabe's 3rd birthday. It is called the Fontan Procedure. The the blue blood from the lower part of the body is directed to the lungs. This is done using the inferior vena cava, the right atrial wall and artificial material (gortex). A tube is fashioned through the top right part of the heart and connected to the right pulmonary artery branch. This rerouting allows the blue blood to enter the lungs without being pumped by the heart the heart remains available to receive the red blood from the lungs and then pump it to the body.

He will have a heart catheterization before the 2nd and 3rd surgeries to make sure that his lungs can handle the extra blood flow.

All in all, it was a good visit. Jeff and I felt better afterwards. It helped us to see what to expect and it was nice to have answers to all our questions. It was a lot to absorb and I was exhausted emotionally and physically after it was all over. We are very blessed to live in Nashville and have the resources that Vanderbilt offers so readily available. So many families have to uproot their lives and families to seek the treatment required by a state of the art facility. My prayers are with them as well as my dear boy tonight.