Wren, born 2006
I was 16 weeks pregnant with Wren when I had an amnio. I was 30 years old and had 3 prior miscarriages and we had been told the baby had a slightly enlarged nuchal fold measurement at the first trimester screening. We were so relieved when everything came back normal - I finally started enjoying my pregnancy. At the time of the amnio I had a level two ultrasound and they asked us to return at 21 weeks because "they couldn't get a good view of the heart".
I returned, not suspecting anything but a fun look at our baby. But at that 21 week appointment the radiologist told us our baby had a serious left heart abnormality - the left ventricle was too small and they suspected HLHS. We should consult a pediatric cardiologist to discuss our options.
I went home and Googled HLHS and was in shock and grief. Thankfully I found www.littlehearts.org which gave me hope that our baby could survive with good medical care. I also attended a support group meeting through our Children's Hospital; CHD survivors once again gave me hope.
Our first PC appointment two weeks later brought more confusion. Wren's heart was asymmetrical but his left ventricle was not as small, typical in HLHS. We were told he had a borderline left heart with a probable coarctation. He would need surgery shortly after birth but despite six or more echocardiograms and consultations with a surgeon at Seattle Children's Hospital, no one was sure what surgery would be required or what his prognosis was.
Wren was born one week early. He was a healthy 8lb 6oz and had good apgars. He was taken immediately to the NICU where he was stabilized on prostaglandin while the cardiologists tried to decide what to do. Six days of winter storm and an ambulance transport later, Wren faced his surgery. We signed consent forms for the Norwood procedure and a COA repair and the surgeons were left with the decision when they could see what was going on. We were warmed that his situation was very serious and that it was not impossible to predict whether his left heart was capable or not.
During surgery the team decided that they would try the COA repair on its own and see whether his heart could cope. After a nerve-wracking week, Wren was doing very well and they considered the procedure a success. He went home at 3 weeks old on a feeding tube and baby aspirin.
At his 6 week follow up, the echo showed significant turbulence in the arch, recoarctation at the site of the clamp and aortic stenosis. He underwent a cath lab procedure which opened the aortic valve, reduced the coarctation and reduced his left heart pressures significantly. Since this procedure, Wren still has residual coarctation, some turbulence in this aortic arch and subaortic stenosis.
He shows few clinical signs of these conditions and is a fat and happy baby, in the 90th percentile on all stats and has met all his milestones so far. While we are prepared for Wren to need future cath procedures if the AS continues, our PC has said that they are very pleased with his progress and will be conservative in recommending surgery for the LVOT obstruction. We feel very blessed to have Wren in our lives and to have come to this point from such a dire prenatal diagnosis.
Just before his second birthday, Wren had OHS to address subaortic and aortic stenosis as well as some narrowing above the valve. Twelve months later, Wren's aortic stenosis is moderate and we are hoping to avoid a valve replacement until he is older. Dr. Hanley feels that by age 7 an adult size valve could be used. Wren is now 3 and is like any other little boy. He is on no medications and meets all his milestones.
Story by Wren's mother, Shannon - Washington