Marin (Double-outlet Right Ventricle, Ventricular Septal Defect) - Louisiana Pediatric Cardiology Foundation

Marin (Double-outlet Right Ventricle, Ventricular Septal Defect)

On October 11, 2007, our daughter Marin Avery Varnado was born at Baton Rouge General Hospital. She was a healthy 8 lb. 11.6 oz, and 21 ¼ inches long. She was the happy ending to a wonderful pregnancy. Everything looked great, and she scored a 9/10 on both of her APGAR tests. Our first night in the hospital with her was great…she slept well, ate well, and was the perfect baby.

The next day, the hospital pediatrician took Marin to the nursery for a well baby checkup. She came back about an hour later, and told us that they had heard a heart murmur and wanted to do an echocardiogram to make sure everything was ok with her heart. She told us not to worry though; plenty of babies have murmurs and are perfectly healthy. A few minutes later, we met the man that would become our guide through the next several months of our lives, Dr. Michael Crapanzano. Dr. Crapanzano came in to do the echocardiogram on Marin. Jacob and I waited silently as we watched the images on the monitor, and prayed that there was nothing wrong. Unfortunately, there was. Dr. Crapanzano told us that Marin had two Congenital Heart Defects that would require open-heart surgery in her first year of life. We were devastated, and in disbelief.

Marin had two defects; Double-Outlet Right Ventricle (DORV), and a large hole in her heart, or Ventricular Septal Defect (VSD). The combination of her defects was a blessing in disguise; because she was born with the VSD, blood was able to pool in this hole and go where it needed to go in her body. Without the VSD, she would have been blue and rushed immediately to emergency surgery. But she was very healthy and we were able to take her home, thinking that we would have the next six to twelve months to decide when she would have her surgery. We had already decided she would have her surgery at Boston Children’s Hospital, because they are the top hospital in the nation for pediatric cardiothoracic surgery.

After being home for a week, I received a call from Dr. Crapanzano’s PA, Lisa, that our surgeon in Boston wanted to do the surgery the next week. So that week was spent planning our trip, and trying to prepare ourselves for what lay ahead.

We arrived in Boston on October 29, and checked into the hospital the following morning to meet the team. It was a long and stressful day of lots of pre-op testing and signing consent forms. Our families arrived that afternoon to be with us for Marin’s surgery the next morning.

At 8:30 a.m. on Halloween morning, Marin was taken to surgery. Dr. John Mayer would be performing the surgery. What we thought would be a fairly straightforward repair turned into an 11-hour ordeal with two trips to the operating room. The surgery did not go well, and Marin came out of surgery on ECMO (extra-corporeal membrane oxygenation) and was bleeding severely. She received ten units of blood the night of her surgery, but the bleeding didn’t improve the next day. So Dr. Mayer told us that they were going to do a heart catheterization to look for the source of the bleeding. After the heart cath, he thought he knew the source of the bleeding, and took her back to the OR for another 6-hour surgery.

Throughout the next few days, the bleeding finally slowed and then stopped. Marin was kept on ECMO for seven days, and her chest was left open for 13 days. Two weeks later, she was finally taken off of the ventilator and was able to breathe on her own. Though her heart conduction had miraculously returned on its own two weeks after surgery, her rhythm was not stable, and the team decided she would need a pacemaker. So on December 5, Marin went back to the operating room a fourth time to have a pacemaker put in. That surgery went very well, and she recovered quickly. The next few weeks in Boston were spent working on getting her to eat again by mouth, which we were having a lot of trouble with.

Finally, on December 14, 2007 after nearly 8 weeks in Boston, we were flown home by med-evac to Our Lady of the Lake Hospital in Baton Rouge to continue working on her feeds. After several unsuccessful weeks of attempts at bottle and breastfeeding, the decision was made to put in a more permanent feeding tube. On January 8, 2008, Marin went to the operating room a fifth time to have a gastrostomy tube placed in her stomach. The surgery, done by Dr. Faith Hansbrough, went beautifully, and we were finally able to go home for good on January 13.

Unfomarin-1rtunately that wasn’t the end of our “bad luck.”  We began to notice recurrent fevers and couldn’t find a cause.  On a trip to the ER for high fever, I noticed that her heart rate on the monitor was between 30-40 beats per minute, much lower than her pacemaker was set.  She was immediately taken to the ICU to wait for the pacemaker rep to come interrogate the pacemaker the next morning.  It was reset, but looked as though it was malfunctioning and needed to be replaced ASAP.  We were quickly put on a flight to Boston.  We learned later that week that the pacemaker had been infected with a staph infection, and would need to be removed and replaced.  This required opening her chest and sternum to remove the wires, placing temporary wires for a week while the infection cleared, and then another surgery a week later to place the new pacemaker in her shoulder.  She also had a heart cath that week.  By the grace of God, everything went well through those surgeries, and we were there exactly a month.

The last of her surgeries was in April 2009 to remove a fistula that had developed where her gtube had been removed and to put tubes in her ears.

She has been great ever since, minus a week in the hospital in January in 2010 for RSV.  We also recently learned that Marin is completely dependent on the pacemaker, and no longer has an underlying heart rate.  She will always need pacemaker checkups and replacements.

The first year of Marin’s life was a trying time for all of us, but with a lot of prayer and support from family and friends, we were finally able to come home and be “normal” parents. Our lives have been so blessed by our special little girl, and this has brought us so much closer together and made us appreciate every day with her. I truly believe that God gives these special children to special families that He knows can handle it with His help. As tough as these past years have been, they have strengthened our faith in God and our love for each other. We wouldn’t trade it for anything.