We are just at the beginning of this journey with HLHS. We found out at the 20 week ultrasound that Evynn had a very complicated heart. Her post-Norwood discharge papers describe her as having dextrocardia with mirror image atrial arrangement, hypoplastic left ventricle with mitral atresia, aortic atresia with hypoplastic ascending aorta, atrial/ventricular septal defect, left superior vena cava to leftward right atrium, and aberrant left subclavian artery. What I understand from all of this, is that her heart is on the wrong side, mirror image and missing it's pump.
She was delivered at Mt. Sinai Hospital (connect underground to SickKids Hospital in Toronto) at the end of May 2011, and was taken to SickKids CCCU within an hour of her birth. There she was cared for by the most amazing team. Her Norwood surgery was performed when she was 6 days old. We were told her surgery was even more complex because of the arrangement of her heart. Although we had been prepared for a 6-8 week hospital stay, she was discharged when she was 17 days old. Evynn nurses and takes a bottle, and despite slow weight gain, she is doing amazing.
We have been told her next surgery will be sometime in Novemeber 2011, and so her journey continues...
Evynn's jouney has continued... On October 13th, she was admitted for a diagnostic cardiac catheterization for pre bidirectional cavopulmonary connection study (in & out on the same day). We thought she was just having test to get ready for her stage 2 Glen, a month away. The catheterization noted she had severe atrioventricular valve regurgitation, she was placed on a milrinone infusion & surgery was scheduled a few days later.
On October 18th, Evynn underwent a bidirectional cavopulmonary connection of the left superior vena cava to pulmonary artery, atrioventricular valve repair, division of the BT shunt, bilateral pulmonary artery plasties, atrial reduction, and azygous divison. Her recovery was rough, she came out of surgery with her chest closed but it had to be re-opened 2 days later because her heart was in a JET rhythm requiring multiple loading doses of amiodarone, continuous infusion, and an external pacemaker. It took 7 days to close her chest... she was so swollen that in one 24 hr period she diuresed over a litre of fluid.
Evynn is still in the cardiac ward at SickKids... Her valve still has moderate regurgitation and now she has moderately reduced ventricular function. She came home for 2 days because she was stable, even orally feeding 85mls in 10mins. She was re-admitted because she turned grey, was lethargic, stopped feeding, had increased work of breathing. Her doctors are still trying to find medications that work for her. Her blood pressure is no longer tolerating carvedilol & enalapril, they tried a small dose of captapril but that dropped her pressure to 42/p. So... we wait to see what she will tolerate. The team of cardiologist and surgeons have now decided not to wait any longer and are going ahead with putting Evynn on the heart transplant list.
Her journey will now take a different path...