Elisabeth was diagnosed with a single umbilical artery (SUA) at 20 weeks of gestation, a condition often associated with renal abnormalities. We got a weekly non-stress test and all sorts of other prenatal testing (as an older mom), but she passed everything with flying colors, and when she was born in June she checked out just fine and we took her home and enjoyed her. Her big brothers adored playing with her and showing her off, and her grandparents enjoyed their first and only granddaughter. She was a good sleeper, and didn't seem to eat very much, but otherwise seemed a normal, happy, bright-eyed baby. She really fussed when lying on her stomach, so I didn't push it, considering that we could get around to it later.
I was mildly bothered that she wasn't rolling over by her four- month-old checkup. The doctor, however, seemed more concerned with her slow growth--she weighed less than 11 pounds--then found a heart murmur. We were told to take her to see a cardiologist, within the week--"And if you can't get an appointment that soon, have them call me and I'll get it for you." That set off our internal alarms, but after some quick research on heart murmurs we decided that worrying wouldn't do us any good. We didn't even tell our sons that she was going in for a checkup.
At the cardiologist's, they did an EKG, an echo, then said they'd like further tests. At this point it was clear something was wrong, but not what. I asked when to bring her back, and they said, "We'd like to do the tests now, in the hospital across the street." Well, okay. "We'd like to admit her." Oh? "Overnight." Oh. "To the ICU." OHHHHH.
An angiogram the next day (Friday) confirmed that Elisabeth had no detectable left coronary artery --her heart muscle was starved for oxygen, and the left ventricle in particular was damaged, plus her liver was enlarged. The cute little grunts she made while lying down were evidence of fluid beginning to collect in her lungs. We were incredibly lucky to be at the Children's Memorial Hospital in Chicago, where the heart surgeons were excellent and had a wide experience, but even they were baffled by her case. We were told that they wanted to study and think about possible options over the weekend, and we spent the time anxiously cramming information on heart anatomy and function, and on her problems in particular. On Monday the doctors would ask us to help them decide the best course of treatment.
This was absolutely terrifying. Though they kept reassuring us that the doctors were the best we could hope for--and we believed them--Elisabeth's condition was so rare that even they weren't sure what to do. Only two cases in the past 25 years had been like hers, and even those had different details. Oh, if only they could find the coronary artery! Otherwise, we were told, a transplant would probably be our best option. It seemed hard to believe, when just a couple of days before she'd been happy and seemingly healthy, at home.
The actual decision point seemed rather calm and anticlimactic. Exploratory surgery would at least tell us what we were dealing with. We didn't want to just take her home and watch her struggle when there was a chance of helping her. So surgery was scheduled for Wednesday. We spent Tuesday playing with her, taking pictures, and having her big brothers visit. And we prayed constantly.
Wednesday came, and we were told that they would report to us what they found after opening her up--ten or fifteen minutes of looking would do it. After over an hour, we looked at each other and kept repeating, "No news is good--it means they can do something, and they're doing it." Finally we learned that miraculously, her left coronary artery had been found, attached way up on the pulmonary artery--she indeed had ALCAPA, not nearly so rare as an atretic artery, and treatable. The surgeons hadn't wanted to be forced to do a bypass, since her arteries were so small (0.5 millimeters), so had kept on looking. They were able to reimplant the artery on the aorta where it belonged, although rather high up, since it was so long. She also had an ASD which was patched. (In all the fuss and fury over the missing LCA, we kept forgetting about the hole--but it was a good thing she'd had it, since it kept her blood mixing and slightly more oxygenated than it would have been, pre-surgery.) And best of all, she managed to get off the heart-lung machine on her own, without spending time on an ECMO.
It took her an agonizing two more weeks to get off the ventilator, and a total of 36 days in the hospital, before she came home, just 5 days before Christmas. Elisabeth had one major scary episode of a soaring heart rate and dropping blood pressure in the ICU, and a couple of fevers along the way, but luckily no MI's or other major setbacks. Her echos kept improving, and the week she came home her mitral valve regurgitation (a common complication with ALCAPA) was upgraded to "mild to moderate".
While anxious to have her home, we were worried that she still seemed irritable and ill--she had a fever which delayed her homecoming, and cried instead of smiling widely at everyone. Once home, she screamed and refused to eat for two days, until we put her on ibuprofen. Over the phone with several doctors, over those two days, they decided she had acid reflux as a result of the surgery, and prescribed Zantac syrup. It did the trick, and Elisabeth started eating and smiling again on Christmas Eve.
Since then she's passed a hearing exam and her mitral regurgitation has been upgraded to merely mild. Elisabeth is also beginning to grow-- three months after surgery, she's gained a pound and a half, and is beginning to sit up on her own, roll over, and has two teeth. She still gets captopril, digoxin, aspirin, and Lasix. along with the Zantac, and will be monitored for years, to see how much heart function is eventually restored. But we are thankful that Elisabeth was able to receive the only surgery which could possibly give her a chance to heal and grow up.
This was written at two in the morning, so may not be very detailed or clear. We're just coming to grips with how rare ALCAPA is, and would like to hear from others (we know of one other case here). But we also appreciate the chance to share and learn from other families dealing with all kinds of CHD. Thank you for sharing your stories and concerns here on this website.
December 2003 Update
At 17 months, Elisabeth is doing very well. She began walking at 13 months and spent the summer perfecting her running skills. She passed another hearing test in July and is beginning to use real English words just now (as opposed to pointing and making noise, which works pretty well, and her one intelligible word, "Uh-oh.")
Lissa had some problems with slow weight gain all last spring and summer which had us visiting a nutritionist and increasing her formula to 30 cal/oz., plus monthly weight checks. Her last check, in September, had her at 16 lbs, 5 oz., and since she has maintained the same growth curve she is now at six-month weight and cardiology checks. She now has nearly all her teeth and eats regular table food spiked liberally with polycose and butter. Lissa is also off the Lasix (as of March 2003) and the digoxin (October 2003) and has had her daily captopril dose lowered to only twice a day.
In other words, we have a happy, busy, bustling, normal toddler. We are constantly grateful for her ability to swing on the furniture, empty a cabinet, and shriek all over the house. Her heart function is not yet quite normal, but we have every hope that it will be in a few years, and in the meantime it supplies her with enough energy to keep ahead of all of us. We are blessed that she has come so far in just one year.
