When Johana Luna-Dominguez was told she’d be having a second baby, she was ecstatic at the thought of growing her family.
“My first child, Citlalli, was so easy I just figured things would be the same for my second pregnancy,” reflected Johana.
But at her 20-week ultrasound, an anatomy scan revealed things were anything but usual. Johana’s OB/GYN noticed a rare and complicated defect called situs inversus whereby the baby’s liver and stomach were on the opposite side of where they were supposed to be. To make matters worse, further complications were discovered affecting the heart, lungs, spleen and intestines.
A subsequent evaluation with a pediatric cardiologist determined that the baby had very complex congenital heart disease consisting of a single ventricle (pumping chamber), a leaky valve into that ventricle, underdevelopment of the artery providing blood flow to the lungs, and abnormal connections of the veins bringing blood back from the the lungs to the heart (total anomalous pulmonary venous connections).
Single ventricle heart disease can be treated with a series of palliative cardiac operations, but the abnormal connections of the veins from the lungs to the heart meant an even grimmer outlook – a decreased chance of surviving through the first few days of life.
Despite the bleak news, Johana and her husband Martin moved forward with the pregnancy. Their Michigan Medicine team prepared them for what would happen next: the baby would need a major operation immediately after delivery.
“I think the hardest part was only being able to hold my newborn daughter (Amaris) for three minutes before she had to be taken into surgery,” remembers Johana. “But I understand why it had to be that way and I trusted my medical team.”
While the initial surgery was successful at repairing the abnormal connections of the veins from the lungs to the heart and giving her a better source of blood flow to her lungs, Amaris’ underdeveloped kidneys caused poor urine output and fluid retention. Her heart and lungs also were not functioning well enough to support her. It was determined that placing her in a life-saving device known as an ECMO would help her to heal with greater ease since the machine provides the functions of Amaris’ heart and lungs while they healed.
We collaborated with our maternal-fetal medicine, neonatology, pediatric cardiology and cardiac surgical teams and more to give this child the best chance possible.
“Perhaps the biggest benefit of Michigan Medicine for a child like Amaris is the comprehensive, multi-departmental care we provide,” says Dr. Sarah Gelehrter, pediatric cardiologist and director of the Prenatal Heart Program at C.S. Mott Children’s Hospital. “In her case, we collaborated with our maternal-fetal medicine, neonatology, pediatric cardiology and cardiac surgical teams and more to give this child the best chance possible.”
Even though Amaris made great improvements over the next four weeks, she would require a second surgery. After turning only one month old, she underwent a surgery this time to repair her leaky valve before being placed back in the ECMO again.
“Amaris has a very complex heart condition due to the combination of several defects, which even each alone would be significant,” noted Richard G. Ohye, M.D., one of Amaris’ pediatric cardiothoracic surgeons and co-director of the Congenital Heart Center at Mott. “Challenging cases like Amaris’ benefit from expertise across the wide spectrum of providers in many specialties, which is available at Michigan Medicine. Innovative medicine is also a key component of care from these complex kids. ECMO, which was crucial in allowing time for Amaris’ heart and lungs to recover, was invented at Michigan and has saved tens of thousands of lives worldwide.”
Again, surgery proved to be successful. But still underdeveloped and facing life-threatening complications from the birth defects, the decision was made to keep Amaris at the hospital until she could thrive without continuous medical care.
“It was definitely an overwhelming time, you know, keeping Amaris in the hospital for six months,” remembers a somber Johana. “She ended up even having a third surgery during that time. But as long as she showed signs of improvement, we never lost hope.”
By March of 2020, six months after giving birth, Johana and Martin were able to bring Amaris home for the first time. Though she will continue to need further surgeries, both parents are grateful for the dedicated team of doctors and specialists who have given Amaris breakthrough care.
Says Johana, “While it’s overwhelming and Amaris has a long road ahead, we know we wouldn’t have gotten this far without Michigan Medicine.”
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