Early discoveries

November 17, 2010

Imagine the excitement of learning that you're pregnant with twins. You automatically start to consider everything in pairs: two car seats, two cribs and twice as many dirty diapers. For Laura and Todd Robertson, one thing they hadn't considered was the possibility that both of their babies could be born with identical heart defects.

But thanks to advances in fetal diagnostic capabilities, cardiac specialists on the medical staff at Children's were prepared to provide specialized care for Ryan and Reece to sustain their lives until a delicate surgical repair could be performed.

Fetal diagnosis

During an early ultrasound, Laura's doctor discovered both babies had a thickened nuchal fold (the skin behind the neck). Babies with abnormalities tend to accumulate more fluid at the back of their neck during the first trimester, causing this clear space to be larger. "We were thankful when the amniocentesis ruled out the possibility of a chromosomal disorder, but our relief was short lived," Laura said. "Following the next ultrasound, my doctor recommended we see a cardiologist to receive a fetal echocardiogram. We knew something was wrong." A fetal echocardiogram checks for abnormalities of heart structure, cardiac rhythm disturbances and disorders of cardiac function. The examination is conducted on expectant mothers using high-frequency sound waves to look at the heart and major blood vessels of the fetus.

The focused study

For Ryan and Reece, the fetal echocardiogram revealed a large hole in the center of the heart — an atrioventricular canal defect — in the wall between the heart's upper and lower chambers. The opening was allowing oxygen-rich blood from the lungs to mix with the oxygen-poor venous return from the body. The mixture was then being sent back to the lungs, filling them with extra fluid. For more than 90 percent of the cases of congenital heart disease found in identical twins, at least one twin has a normal heart. The fact that both Robertson twins were affected with the same defect made their case particularly unusual.

"After learning the diagnosis, Laura and I set out to find the best doctors and facilities to treat Ryan and Reece," Todd said. "We chose Children's, because it was important to us to have our boys cared for by a facility that specialized in pediatric cardiology, with dedicated resources and supporting infrastructure for kids with heart defects."

The Children's difference

The Robertsons scheduled a consultation with Dr. Catherine Ikemba, a cardiologist on the medical staff at Children's and assistant professor of Pediatrics at UT Southwestern. "Dr. Ikemba's thoroughness made us feel confident that Children's was the right place for us," Laura said. "She explained the diagnosis in detail and ensured that we understood the treatment plan. We have never encountered a doctor who proactively communicated with us like Dr. Ikemba has — she is one of the main reasons that we chose Children's." Dr. Ikemba said in the Robertsons' case, the purpose of the fetal echocardiograms at Children's was to prepare the family for life with twins with the same heart defect.

"After reviewing the first fetal echo I conducted on Laura, it was clear that heart surgery would be required during infancy for both children," Dr. Ikemba said. "As a pediatric cardiologist specializing in fetal echocardiography, my role extends beyond making a correct diagnosis to educating and supporting parents while preparing them for the road that lies ahead." According to Dr. Ikemba, most babies with heart defects do well in utero because during pregnancy the heart's primary function is to pump; the lungs do not put oxygen into the blood; the placenta does. Detecting the heart defects before birth allowed the medical staff at Children's to establish an effective treatment plan, increasing the twins' chance to live healthy lives. "Diagnosis of congenital heart disease or any abnormality prenatally causes mourning of the loss of a normal pregnancy," Dr. Ikemba said. "Prenatal diagnosis allows families time to progress through the stages of mourning. They are better equipped emotionally at the time of delivery to take care of a baby, or babies in this case, which require extra special attention."

A confident choice

Mrs. Robertson said that after careful consideration of the medical facilities in Dallas, she and her husband were convinced that the specialized medical staff and support services at Children's were the best choice for their sons' surgeries.

"Once we toured the facilities, met Dr. Ikemba and saw the specialization with children firsthand, we knew we were at the right place," Mrs. Robertson said. "Dr. Ikemba's personal attention and dedication to Ryan and Reece gave us the feeling that we were not just another family." Both boys were born without complications in July 2006 — Ryan first, then Reece. Over the next few months, the boys returned to Children's every two weeks for monitoring and assessment of their weight — and to track any symptoms of heart failure.

Surgical intervention

Meantime, the Robertsons met with Dr. Joseph Forbess, chief of Cardiothoracic Surgery at Children's, for a surgical consultation.

Dr. Forbess holds the Pogue Distinguished Chair in Pediatric Cardiac Surgery Research and is an associate professor of Cardiothoracic Surgery at UT Southwestern. "Dr. Forbess had counseled us prenatally, so we already had a strong confidence in his approach and expectations," Mr. Robertson said. "However, talking with him the day before surgery for Ryan made us even more confident that we had the best surgeon to fix their hearts." The Robertsons had reason to be impressed. One of the nation's leading pediatric cardiothoracic surgeons, Dr. Forbess has performed close to 120 AV canal repairs with a 98 percent success rate.

The surgeries and beyond

Inside the operating room, the medical team sets to work to accomplish its task — saving Ryan's life. The infant's heart is stopped for more than an hour, the tubes and wires of a bypass machine sustaining his life. Dr. Forbess opens the tiny heart and goes to work repairing the imperfection. During the procedure, the hole in Ryan's heart is closed. Another smaller hole and leaking valve require only a few small stitches. Nearly two weeks later, the family repeated the process with Reece.

Ryan and Reece are home and thriving. Mrs. Robertson says they continue to eat, grow and, perhaps most notably, get louder. "Their lives started out a little different than most, but thanks to Children's, they're on the road to becoming just like every other kid their age, and Todd and I couldn't be happier," she said. Big sister Reid, 2, also is happy to have her brothers home. Nowadays the twins require only routine follow-up appointments. The Robertsons said they feel fortunate to have the nearby Children's Ambulatory Care Pavilion in Legacy, located in Plano, available for care.

Dr. Ikemba said that Ryan and Reece can look forward to a bright future replete with every opportunity available to a healthy child. The thin scar on each boy's chest will serve as a reminder that any obstacle can be conquered, any adversity overcome.