We talked to Dr. McKenzie just now. Here is a rough outline
- How did it go | In general, he said the classic repair that they chose to do was tough – both technically, and also in regards to decision-making. The way Reiss’ heart sits made it hard to see and operate inside the heart. Overall, Dr. M was happy with how it went, other than the conduction issues mentioned below.
- Pacemaker | He is worried about the conduction system within her heart, which resides in between the two VSDs he closed. As of right now, she is on a temporary pacemaker. They will pause the pacemaker routinely until the conduction system starts back up as it should. If it doesn’t conduct by day 7, then they will take her back into the OR to place a permanent pacemaker. As of right now, her heart rate is about 120-130 beats/minute. While this is where it was pre-surgery, a normal heart rate for her age/size is 90-100.
- Ventricular Septal Defects (VSD) | After closing the large VSD and filling her heart back up with blood, they noticed a return from the left ventricle. They looked at the flow with the help of an echo and found a smaller, remote VSD which they had to patch. We asked if there is a probability for these to move in any way. Dr. M said that was very unlikely
- Pulmonary Arteries | The PA branch was small going to the lungs, with one size smaller than the other. According to Dr. M, the Fontan (the bypass option STL wanted to do) would not have been possible (Yeah for making the right decision to come to Houston!). They widened the pulmonary arteries and were happy with the results.
- The Glenn | They took down the Glenn shunt, which was redirecting the superior vena cava (SVC) to the pulmonary artery, and placed the SVC back to a position of feeding blood to the right atrium (RA). Dr. M said we would need to keep an eye on this new SVC/RA connection because it is not a natural connection.
- Why they chose the classic repair | Dr. M thought he could have built a tunnel to connect the right ventricle to the aorta. It would have been long, though. What mostly swayed him was the positioning of Reiss’ heart, her sternum, and where the conduit to the lungs would have to be in this type of repair. Reiss’ heart faces more towards the right (versus the left) because of her inverted ventricles. That, coupled with the fact that her heart also sits flush with her breast bone makes placement of the conduit, and the changing of the conduit in subsequent surgeries, nearly impossible.
- Collaterals | These collaterals have been the issue with hem0ptysis (coughing blood) for Reiss since November 2014. According to Dr M, she had many collaterals, which were the source of most of the blood flow to the lungs. He was unable to cauterize or baffle any of these collaterals, but feels like a follow-up cath procedure to close the vessels is unnecessary. Over time, these collaterals will shrink down significantly
- Oxygen Saturations | He predicts her saturations to be 100 after this surgery, leaving the likelihood for hemoptysis low.
- Probability of a heart transplant | Dr. M thought that this procedure would carry her well into adulthood and that predicting the future in regards to a heart transplant was very hard to do.
- Conduit | Dr. M placed a man-made, Hancock conduit with a porcine valve. Because they want to control the pressures between the right and left ventricle, this conduit, which is the new pulmonary artery carrying blood from the morphological left ventricle (see side note below) to the lungs, should always be a bit narrow (known as pulmonary stenosis). This will not grow with Reiss. He couldn’t tell us if and/or when the conduit would fail. If it does, that is another open heart surgery.
- Tricuspid Valve | Reiss’ tricuspid valve is what separates the left atrium from the morphological right ventricle. Once they got in there, they saw some leakage in that area and found a cleft in one of the leaflets. They repaired the cleft and there is no longer any leakage. They do not anticipate any issues with this repair. He mentioned that it could leak in the future if the right ventricle becomes overworked. If that happened, it could enlarge, which would pull on the tricuspid valve, causing it to fail/leak.
Side note: Reiss has inverted ventricles. So the morphological left ventricle actually sits on the right side, and the morphological right ventricle on the left.
They should have Reiss ready for us in the CICU soon. We are ready to be by her bedside.