At my 20-week anatomy scan with my first child, we learned we would be welcoming a little girl with a combination of defects called Pentalogy of Cantrell; a 5.5 in 1 million chance. The most concerning aspect of this diagnosis was that her heart had developed entirely outside of her chest, just where her sternum should have been, completely exposed to amniotic fluid. Despite a 10% chance of even surviving to viability, we decided she would determine her own fate. At 36 weeks, Kieran Jane was delivered via EXIT procedure and determined stable enough for surgery to reduce her heart into her chest. The following two weeks were a whirlwind. We got to hold her for the first time at a week old. At 12 days old, we nearly lost her when her pulmonary artery was poked by a suture and she almost bled out. At 7 weeks, she had a Tracheostomy procedure. At 2 she was able to come off trach and vent support completely. At 4 ½ she had OHS to correct her DORV and place gortex to create a sternum to protect her heart. She will need surgery to close her abdominal omphalocele, but for now, she can grow and be a typical girl. She will be 6 in March! She loves Kindergarten, gymnastics, arts and crafts, music, and Disney princesses.

Back to Heart Warrior Stories >