Stories about: treating congenital heart defects

Our patients’ stories: Treating Haven’s pulmonary atresia

Haven

When Molly Foley was first pregnant, she admits she knew very little about congenital heart defects, or how they could affect her unborn daughter, Haven. But, around 20 weeks into her pregnancy, Molly discovered that heart defects were very real—and very scary—when an ultrasound revealed that Haven had pulmonary atresia. Also known as “blue baby syndrome,” pulmonary atresia is a condition in which the heart’s pulmonary valve is abnormal and doesn’t open.

“It never occurs to most people that their baby could have a heart defect,” Molly says, noting that many parents are all-too aware of other potential birth defects like cleft lip/palate, Down syndrome and spina bifida. “It just doesn’t come across many people’s radar screen.”

And while children born with pulmonary atresia may be rare, there is a wide range of more commonly occurring heart conditions that can affect babies. In fact, when viewed as a whole, heart defects are the number one most common type of birth defect.

Following Haven’s diagnosis, Molly says that the staff at the Boston Children’s Hospital Heart Center “worked very hard to make me psychologically prepared for what was coming next.” Doctors told Molly that Haven needed a series of three surgeries—the first coming when Haven was just four days old. In addition, Haven needed to be placed on an ECMO (Extracorporeal Membrane Oxygenation), a life-supporting technology that replaces a critically ill child’s heart and lungs. “The ECMO was invasive,” Molly remembers. “We weren’t sure what to expect.”

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The heart of innovation

On February 14, the Innovation Acceleration Program will celebrate Children’s Hospital Boston’s rich history of innovation at the hospital’s first Innovation Day. Of all the groundbreaking discoveries and procedures that have taken place within Children’s walls, few have had the impact of the surgery performed by Robert Gross, MD, one summer’s day in 1938.

“If you look at the history of cardiac surgery,” says Children’s Associate Anesthesiologist-in-Chief Mark Rockoff, MD, who also chairs the hospital’s Archives Program, “it essentially all started with Dr. Gross.”

Gross’s patient, 7-year-old Lorraine Sweeney, from Brighton, Mass., came to him with a diagnosis of patent ductus arteriosus, a congenital heart defect consisting of a persistent abnormal opening between the pulmonary artery and the aorta. In 1938, it was generally a death sentence—one that would likely end with Sweeney dying of congestive heart failure before adulthood. Accepted practice dictated that surgery was not a survivable option. Gross, the chief surgical resident at Children’s at the time, disagreed.

After two years of successful animal experiments, Gross was certain that the defect could be corrected in a human being “without undue danger.” He lobbied for the opportunity to test his theory, despite skepticism from his peers, and direct opposition from William Ladd, MD, Children’s surgeon-in-chief, and Gross’s superior.

Undaunted, Gross waited until Ladd boarded a ship bound for Europe. Then, with the blessing of Sweeney’s mother, he put his career on the line and performed a revolutionary surgery—tying off Sweeney’s patent ductus arteriosus, allowing normal flow of blood through her heart. “Dr. Gross told me that if I had died, he would never have worked again,” Sweeney recalls. “He would have ended up back on his family’s chicken farm.”

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Heart Month: Catching up with the Peerless family

You may remember Renee Peerless from the ABC documentary Boston Med, which aired last summer and featured several Children’s families and clinicians. After Renee’s unborn son, Sam, was prenatally diagnosed with a congenital heart defect known as hypoplastic left heart syndrome (HLHS), an ABC camera crew followed Renee constantly, highlighting the care required to help a baby born with a heart defect survive the delicate first few days of life. In the following post Renee relives the experience and talks about how the fetal diagnosis of Sam’s condition made an extremely trying situation easier to deal with.

Jake sees the pregnant Renee for the first time in months (photo courtesy of ABC.)

For Renee Peerless, the decision to have genetic testing done during a routine ultrasound had more to do with her husband Jake than her unborn child. Jake, a solider stationed in Iraq, was overseas for most of Renee’s pregnancy, so she initially saw the testing as a chance to have more images to share with him. “Jake felt like he was missing a lot so I wanted more pictures of Sam to send him,” she says. “I knew testing was a good precautionary measure, but my real motivation was getting a few extra ultrasound pictures.”

But as it turned out, the tests revealed that there was something seriously wrong with Sam’s heart. In a matter of minutes Renee went from giddy anticipation to the terror of learning that her unborn son’s health was in serious jeopardy. “It was like my whole world was falling apart,” she says.

The next week Renee met with a pediatric cardiologist in her home state of Connecticut and was told that Sam had a congenital heart defect called hypoplastic left heart syndrome (HLHS), where the left side of the heart is underdeveloped, limiting its ability to pump blood.

Sam had two open-heart surgeries before he was a week old.

“It was so much to take in all at once,” she says. “I didn’t even know what a congenital heart defect was and all of the sudden it was all I could think about.”

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