Anna discovered her daughter’s heart defects antenatally. Her daughter, Gabriella, had an incredibly tough start to life, spending the first six weeks of her life in the hospital, overcoming numerous hurdles. Here, Anna shares their incredibly emotional journey:
(The photos in this story were taken by and belong to the author, depicting their family’s personal journey.)
It was during our 20-week scan that we first learned something was not quite right with our baby’s heart. The sonographer didn’t elaborate more than “sometimes when we see something unexpected or don’t see something we wanted to see, we will ask for a second opinion”. She then gave us an appointment letter to come back and see a consultant in two weeks. Our minds went foggy; we didn’t know what to say, how to feel, or what to do.
When we arrived home, we checked our maternity notes online, to see if any notes from the scan had been published already. However, it took a day or two for them to be uploaded. Upon checking these notes, we saw the sonographer had written ‘aortic arch-not examined’.
After two days of crying and constant googling about what could go wrong with the aortic arch and how serious the condition might be, we called the hospital asking if we could see a consultant at an earlier date – the two-week wait seemed impossible!
Following this call, we were offered an emergency appointment to see the consultant that week. At this appointment, the consultant performed another scan, in which he saw a hole in our baby’s heart. The consultant also noted that the aortic arch seemed to be particularly narrow. His original thought was it may be tetralogy of Fallot (ToF), although this was quickly disregarded a few days later.
We visited the Fetal Medicine Unit (FMU) the following week, and the doctors spent over an hour performing a detailed scan of our baby’s heart. We were then taken to a quiet room and told our baby had a coarctation of the aorta (CoA) and a small VSD. We were told the left structures of the heart appeared to be on the small side, although functioning, so a close eye would be kept on the growth of the heart. We then had scans every six weeks to monitor this.
In the meantime, we were invited to have a growth scan at 30 weeks. This scan showed that our baby had stopped growing at 25 weeks, and was measuring very small.
Two weeks later, we had another scan at the FMU. We were told all the possible risk factors if the baby stopped growing: Down syndrome, Turner syndrome, Edward syndrome, plus others – our baby could have extreme life limitations.
Due to this, we were offered a test to look into the baby’s chromosomes. Though, this test carried risks with it, including the induction of premature labour. We decided to refuse this test as we knew we loved our baby no matter what, and being born prematurely was the last thing our little munchkin needed.
Following an additional growth scan, it was confirmed that our little bundle of joy was measuring nicely; right where she should be at this stage of pregnancy.
Around 36 weeks, we had our final scan and were given a prenatal diagnosis of hypoplastic aortic arch (HAA), severe coarctation of the aorta, plus a small VSD. We were also told the left structures of the heart were measuring small, although they were borderline to be deemed suitable for full repair.
The doctor informed us that upon our baby’s arrival, there would be numerous scans performed to evaluate the severity of her condition. From this, we would receive a formal diagnosis, and a plan to repair her heart would be made.
We were warned that when our baby arrives, if her left heart structures don’t grow to the required size, and her heart is not strong enough to support the biventricular circulation, a univentricular palliative treatment will commence.
The doctor told us it’s hard to say which path will suit our baby better, but whatever it will be, her and her team will fix it – these words kept my partner and I going through this scary, uncertain time.
Gabriella was born at 39 weeks 5 days, weighing 7lb 5oz. My labour was induced and lasted just over an hour. Gabriella came out pink and loud. We were given five minutes for cuddles before she was wheeled away to the NICU, where an infusion of prostaglandin and morphine began.
On day two (Tuesday), we met her cardiologist who confirmed that, so far, she is doing well and would soon be moved to the PICU to prepare for her open heart surgery.
Gabriella was intubated on Thursday morning, before going into deep sedation for her CT scan to get a clear image of the heart. After that scan, we were told her open heart surgery would take place the next day.
Gabriella went into theatre at 11 am, before arriving in the recovery ward around 6 pm. She was on the heart and lung bypass machine for 1hr 32min, and according to the doctors in the PICU, she had done very well.
We spoke to her surgeon later that evening who confirmed he was able to carry out the full repair of the arch and place a pulmonary band on the pulmonary artery to help the VSD close on its own over time. Gabriella’s chest was left open for 48 hours to allow the swelling to go down and to support the heart function.
After her surgery, Gabriella’s scar took a long time to heal. She needed a vacuum dressing and honey gel to draw the puss out. She later caught an infection called staphylococcus and was treated with intravenous vancomycin.
Gabriella also suffered from a severe chyle leak of between 10-30 ml per hour. Despite being on TPNs and being starved of milk, the chyle leak wasn’t easing, so the surgeon decided to fix her thoracic duct via thoracotomy. She was then put on a course of monogen (fat-free milk) for six weeks, as a precaution, in case the chyle leak were to come back.
Gabriella spent her first six weeks of life in hospital – 22 days of which were in PICU on a ventilator.
A few months after discharge, Gabriella went back to the hospital as her stools turned white, and she was diagnosed with an immobile gallstone, which was possibly caused by prolonged periods of starvation and being on TPNs.
Gabriella also had a mild laryngomalacia which worsened her reflux symptoms and made her food come back up regularly.
Looking at Gabriella now, she is the happiest girl on earth. She has been attending her cardiology clinic every three months post-discharge from the hospital, and her cardiologist has recently moved her onto six monthly appointments, hoping to switch to yearly, then two-yearly visits thereafter.
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