Saving Little Hearts

Our journey with congenital heart defects began on January 28, 2004, when an ultrasound indicated our daughter had an enlarged left ventricle. We were referred to the hospital for a fetal echocardiogram within a few days and received the diagnosis of severe critical aortic stenosis with fibroelastosis secondary to the stenosis. We scheduled a prenatal echocardiogram with Dr. Carlen Gomez at the University of Michigan’s Congenital Heart Center in Ann Arbor. She gave us the option of trying an experimental procedure in Boston to open our daughter’s valve while still in the womb in hopes of preventing HLHS. Angelina’s dilated left ventricle was not contracting well and had much scar tissue due to the pressure from the very narrow valve with very little blood flow. The aortic valve was successfully ballooned at the end of February in Boston at just 23 weeks gestation. We were the 19^th ones to have the procedure done in Boston. We were hopeful at first that she would not develop HLHS; however, despite better blow flow, her left ventricle did not respond as we had hoped.

After birth, Angelina developed sepsis (a blood infection thought to be due to a line infection) and her surgery was delayed until she was 18 days old. She had some trouble after the Norwood initially with lower saturation levels, water retention, a partially collapsed lung, and difficulty remaining extubated due to mucus. Angelina came full circle and started having saturation levels in the 90s. She was beginning to eat from a bottle. When we had everything ready to go home the next day, Angelina developed a mysterious stridor(whistling sound when she breathed) and chest retractions. She had many signs of congestive heart failure. Trachea or vocal cord conditions had to be ruled out by the ENT department as well. There were theories of the shunt being too large from her Norwood, possible aspiration while feeding, and/or a combination of factors. Angelina ultimately ended up being placed on the ventilator again, in the intensive care for a couple of days, and then was fine when she extubated! She was discharged at 2 months old. She stayed home until her hemi-fontan surgery at 5 ½ months old.

We have been fortunate that Angelina has been relatively healthy. She was in for one week recovering from her hemi fontan surgery with only one scare involving a partially collapsed lung and mucus once again. She is walking, talking, and acting like other children her age. Her favorite sayings to her 4 ½ year old brother, Nickolas, are “No, mine,” and “My show.” It is truly amazing how far she has come! Angelina has brought great joy to our home, allowing us to not take for granted the little things in life. We anticipate her cath on May 22, 2006 and her Fontan on June 26, 2006 after we celebrate her second birthday.