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After a Fetal Diagnosis of Cor Triatriatum, a Successful Outcome

gemma_nguyenWhen Karolina Adam, M.D., found excessive fluid behind the neck of Gemma Nguyen’s baby during her 10-week nuchal translucency screening, she suspected Down syndrome or a heart defect. After ruling out Down syndrome, the Houston-area maternal-fetal medicine specialist referred her patient to Helena Gardiner, M.D., Ph.D., for a closer examination of the fetal heart.

A world-renowned fetal cardiologist who specializes in fetal echocardiography, Dr. Gardiner is co-director of the Fetal Cardiology Program at The Fetal Center at Children’s Memorial Hermann Hospital, and a professor at McGovern Medical School at UTHealth with dual appointments in the department of Obstetrics, Gynecology and Reproductive Sciences and the department of Pediatric Cardiology. She first saw Nguyen when the 34-year-old was 24 weeks and four days into her pregnancy.

“On the first fetal echocardiogram, we saw a structure in the left atrium. I thought it was an enlarged coronary sinus but could not detect any abnormal venous structures entering it, so we couldn’t reach a conclusive diagnosis,” Dr. Gardiner says. “When we did a second echocardiogram at 34 weeks, we could see the baby had cor triatriatum, a rare congenital anomaly in which a fibromuscular membrane divides an atrium – usually the left – in two. It’s important to detect this before birth because the membrane can block the return of red blood from the heart to the lungs once the baby begins to breathe. For that reason, we discussed with Dr. Adam how best to manage the delivery. In cases like this we want to be prepared for anything.”

Dr. Gardiner arranged for Nguyen to meet the pediatric cardiovascular team, including pediatric cardiothoracic surgeon William Douglas, M.D., chief of pediatric cardiovascular surgery and director of the Children’s Heart Program at Children’s Memorial Hermann Hospital. Dr. Douglas is an associate professor and director of the division of Pediatric Cardiovascular Surgery at McGovern Medical School. “At that point I was worried and anxious about everything,” Nguyen says. “I asked my family and friends to pray for my daughter and to give us a team of doctors who knew how to care for her. I received great support from my family, friends, doctors and the entire team at The Fetal Center – they were a wonderful help to me and gave me hope and strength. They made sure I understood what to expect and assured me that they would do their very best to take care of my daughter after her birth. Dr. Douglas was very optimistic about the case. If the heart doesn’t function well, he said, she will have an open heart procedure to remove the extra tissue in her atrium. It’s a rare defect but a doable procedure, they all assured me, and I had confidence in them.”

Faustina Thao-Nhien Nguyen was born on April 18, 2014, at 1:53 a.m. On the morning of the delivery, a team that included her maternal-fetal medicine specialist, pediatric cardiology, pediatric anesthesiology and pediatric cardiovascular surgery was awaiting her arrival at Children’s Memorial Hermann Hospital.

“Dr. Adam delivered Gemma at Children’s Memorial Hermann Hospital, which is not her usual practice, to allow access to immediate cardiovascular surgery should it be necessary in the first few hours after birth,” Dr. Gardiner says. “Luckily, Faustina came out crying and screaming. The membrane was still there, but it had a nice big hole in it, which was seen before birth. Sometimes after the child is born, we find the hole is smaller than expected and there is more blood passing through it, which causes an obstruction to flow into the left-sided pump. So this was a great outcome and a happy discovery for the team.”

Faustina spent the night in the Neonatal Intensive Care Unit and was sent home with her mother a few days later. She is now being followed by the UTHealth Pediatric Cardiology team. Nguyen is thankful her baby is healthy and may not need surgery for the rest of her life. “I believed a miracle could happen,” she says. “Faustina’s heart is still carefully being monitored by her pediatric cardiologist but she doesn’t have to go back as often anymore. First every four months, then six months, then only once a year.”

Her story demonstrates how early detection, prenatal diagnosis, pregnancy management and coordination of delivery at a tertiary care facility like Children’s Memorial Hermann Hospital can result in a good outcome for babies with a potentially life threatening cardiac condition. The multidisciplinary teams were able to monitor the baby throughout the pregnancy and develop a customized delivery plan with specialists ready to intervene and treat the baby as needed.