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Blake is full of energy, full of life, and always busy exploring the world around him! He's mischievous, extremely bright, loving, and sweet. On the outside, Blake appears to be just like any ordinary eight-year-old boy. Inside, however, is a very different story. Blake has congenital heart defects. These defects are extremely complex and all depend on each other to keep Blake alive.  

Blake's story starts with his nine-month well child checkup in January of 2011. Blake's doctor heard an unusually loud heart murmur and ordered an echocardiogram. This echo discovered two of his defects - pulmonary atresia and a VSD. His third defect and other diagnosis, MAPCAs and pulmonary stenosis, would soon be found. After much research and several consultations, we chose the Mayo Clinic as Blake's primary cardiac clinic.

So what is pulmonary atresia with a VSD, MAPCAs, and pulmonary stenosis? Pulmonary atresia means that Blake is missing the pulmonary valve and the trunk of his pulmonary artery. It started to develop in utero, but never finished. The VSD means he has a hole between his ventricles. The pulmonary artery delivers blood to the lungs, but because Blake's is missing, his lungs get mixed blood by taking a different path. MAPCAs are collateral arteries that help with blood delivery to the lungs. Blake's MAPCAs were connected to his aorta, not his pulmonary system. And pulmonary stenosis refers to narrowed pulmonary arteries that branch out to the lungs. 

In March of 2011, just a week before his first birthday, Blake underwent his first open heart surgery. During this surgery, the largest MAPCAs were rerouted from his aorta back to his pulmonary system. He also received an artificial pulmonary artery and valve. Surgery was successful and after just one week in the hospital, Blake was discharged the day before his birthday. 

In July of 2011, an attempted dilation procedure was aborted after an aneurysm formed in Blake's left pulmonary artery. Blake was admitted to the PICU and monitored for three days. By the grace of God, Blake recovered and was discharged. 

At the end of October 2011, Blake underwent his second open-heart surgery. Unfortunately, this surgery was not without complications. When a stent was deployed to open the left pulmonary artery, a dissection occurred causing bleeding in Blake's lung. The bleeding was nearly uncontrollable and essentially partially collapsed his left lung. Then the first attempt at extubation failed when Blake's throat swelled and almost completely closed. And finally a severe intestinal infection called C. diff required almost two additional weeks in the hospital for treatment. Despite the complications, surgery was considered a success. The stent accomplished more than it was originally intended to. The aneurysm from July was repaired, as was an additional aneurysm that had formed on the pulmonary trunk. And the valve was replaced. Through nearly three weeks in the hospital, Blake's heart remained strong and unaffected negatively by the complications. 

In February of 2012 another attempt at dilation was again aborted. Blake was then monitored every six months with echocardiograms, chest x-rays, blood work on occasion, a CT here or there, and a heart catherization from time to time until it could be determined that he’d need his next cardiac procedure. 

In July of 2016, Blake underwent sinus surgery due to narrowed nasal passages causing chronic sinus infections.

Just a month after sinus surgery, Blake’s next major open heart surgery was performed in August 2016, nearly five years after his last major cardiac intervention. It was this set of procedures that would be the most complex and life changing for Blake. In 21 hours in the OR on day one, Blake was on bypass for 13 hours. The majority of his pulmonary stenosis on the left side was repaired with dead tissue patches. Most of the VSD was closed, the stent was partially cut out and opened up, the pulmonary trunk was replaced with an upgraded size, the pulmonary valve was replaced, and a PFO (hole between atriums) was discovered and closed as well. Over five days, Blake endured 40 hours of surgery, but woke up a new boy. He acquired foot drop from his time in the OR to add to his cardiac recovery, but nearly three weeks after arriving for surgery, Blake was discharged to go home.  Three months after surgery a heart catherization revealed surgery was a success, though there was still more work to be done. Blake was allowed to continue to recover from what was a grueling surgery and also recover from his foot drop before going in for his next procedure.

In June of 2017, Blake underwent his first thoracotomy heart surgery. This surgery was significantly shorter than his last, performed on his left pulmonary artery branches instead of his heart, and required no bypass. An incision was made on Blake’s left side between his ribs for access. As many stenotic pulmonary arteries as could be reached beyond his stent were repaired with dead tissue patches. Blake did so well with this procedure he was extubated in the OR, spent only a couple of hours in cardiac ICU, and stayed just five days in the hospital. Blake dealt with a lot more pain from this incision due to all of the muscle involved. He also had side effects from pain meds. But his recovery was much faster than an open-heart surgery and bounced back within a couple of weeks.

In October of 2017, Blake went through his first successful angioplasty. Two branches of his left pulmonary arteries were dilated beyond patching areas. They remained open without the assistance of a stent. Blake spent a night or two in PICU for observation before being released.

In April of 2018, Blake went through his fifth heart surgery, a thoracotomy on his right side. Unlike 2017’s procedure, bypass was used for a few hours. Three main pulmonary artery branches on the right side were successfully repaired with dead tissue patches. Blake’s stay in cardiac ICU was two days long and he spent five days in step down due to pain management issues. His incision and chest tube sites were painful enough that he went through a minor procedure to place a nerve block via catheter. The only other complication from this surgery was that Blake’s right lung re-perfused. This means it acquired an injury, whether from being “man-handled” or because it was unable to handle the larger volume of blood flow once the arteries were patched. Blake was able to take his nerve block home after discharge, as well as requiring oxygen supplementation overnight for his first few days home. Once the nerve block was removed, Blake was pain free. Oxygen was removed once we were assured his lung was improving and it continues to improve daily.

A perfusion test was performed a few days after Blake’s right thoracotomy. This test revealed that Blake’s right lung does about 80% of his breathing, which means his left lung only does about 20%. Healthy sets of lungs have a 55:45 ratio. Cardiology was expecting this vast difference, as Blake’s right lung has always been more dominant. But this test also revealed that Blake’s lower left lobe of his lung is barely receiving any blood flow at all. At some point the dilation from October 2017 must have collapsed. This will be the targeted area of the next intervention.

A heart catherization procedure will be planned for August of 2018, possibly to deploy a stent in the artery feeding the lower lobe of Blake’s left lung.

From there on out, heart catherizations will be used for touching up areas of stenosis. Routine echocardiograms, x-rays, and other tests will be used to monitor Blake. No other major open-heart procedures will be planned until his valve starts to fail, which likely won’t be for another ten or more years! When this occurs, the rest of Blake’s VSD will be closed, possibly more pulmonary patching will be done on the left side, and his conduit may be upgraded once more.  Should the dead tissue patches fail to allow his own tissue to continue to grow and expand as they normally would do, it would again require more patching procedures. The hope is that that time won’t ever come. Routine cardiac maintenance will always be a part of Blake’s normal life. And his prognosis is great because of these medical interventions.

Blake’s cardiac defects do not define who he is; rather they are just one part of Blake’s normal life.