Gabriella’s Story

Jun 23, 2010

CHD Awareness, Children w/ CHD(s), My Story

Gabriella is our Heart Baby. She was born 3 ½ years ago with TAPVR, a condition in which the pulmonary veins instead of attaching from her lungs back into her heart, attached to her liver. She was misdiagnosed at birth, and not diagnosed until 3 weeks of age. We almost lost Gabriella because of this misdiagnosis…her oxygen sats were only 30-40% when she was finally admitted to the PICU (located 2 hours from our home) and she crashed and had to be intubated just 7 short hours after arriving at the hospital. She had emergency surgery within 24 hours of being diagnosed, which lasted about 11 hours. When she was finally brought to the ICU after surgery, her heart was too weak to beat on it’s own and so Gabriella remained on ECMO (a bypass machine) for a couple days while her heart recovered. She spent 6 weeks in the hospital recovering from surgery. She suffered from severe Pulmonary Hypertension post-surgery, in addition to developing Pneumonia and a Chylothorax. When we finally took her home we were told she would need no further surgeries and we looked forward to a “normal life”. But God had other plans.
Gabriella was home for only 6 weeks after discharge, and at her first outpatient check-up was found to have Post Repair Pulmonary Vein Stenosis. The Pulmonary Veins that had been attached to her heart had closed off from scar tissue in the veins and she had no blood flow to her left lung. Another complete repair was done, and due to inflammation found in her heart, Gabriella was given a 50% chance of continuing to have her veins close off and for it to be eventually fatal. We were told to “hope for the best but prepare for the worst”. Again post-op, Gabriella developed Pulmonary Hypertension, Pneumonia and a Chylothorax. She was discharged after 3 weeks in the hospital this time, and a residual ASD (hole in her heart) was left to protect against future Pulmonary Hypertension.

Gabriella’s second repair was a miracle and her pulmonary veins remained open and flowing! She did well in the coming years, despite oxygen saturations that were always in the low 90′s or high 80′s. By age 2, she was seen on Echo to have a severe narrowing of her Superior Vena Cava that needed to be fixed. When a pre-op cath was done, it was found that the patch from the previous repairs had given way. In addition to the severe SVC stenosis Gabriella also had a large ASD, blood being rerouted out of the heart that should be going in through the Azygos Vein, and blood was flowing into both Atriums at the same time. Although Gabriella’s heart was “a mess” as the surgeon called it, she was showing no symptoms other than the slightly low oxygen levels due to the “rerouting” her heart had done on it’s own. A repair was done in April 2009, and was successful and Gabriella now has normal oxygen levels.

Unfortunately, because of having so many surgeries in the area where the sinus node is, Gabriella’s sinus node (the heart’s natural pacemaker) was damaged and her heart rate continued to slow. Her heart and liver were enlarging and her pulmonary pressures were increasing. Gabriella had a pacemaker implanted in Dec. of 2009. Because of her size, she had to have another full sternotomy and the pacemaker was implanted near her stomach. Today, Gabriella is the happiest child you’d ever know. She has boundless energy and surprisingly, except for getting shots, she seems to enjoy going to the Hospital and for Doctor Visits. She climbs on her Cardiologists lap and asks her to read her a story and always charms treats or some toys out of the nurses. This is not the path I expected to take when she was born, yet it has now consumed me and I am working on starting a CHD support group in my local community. You can follow our adventures over at www.reachingmylimit.com .