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Fetal Heart Surgery: HLHS

Hypoplastic left heart syndrome or HLHS is a birth defect that affects blood flow and structures on the left side of the heart. Every year in the United States, about one in 4,300 babies is born with the condition. Now, experts have developed a new technique for the highest risk patients to help restore blood flow before birth, and have made one boy the first survivor of the condition.

Stosh Frydlewicz has come a long way in three years.

Tony Frydlewicz, Stosh's father, told Ivanhoe, "Going through everything we went through, I didn't know if we'd ever be able to hold him." 

Stosh was diagnosed before he was born with hypoplastic left heart syndrome.

Jack Rychik, MD, Director of the Fetal Heart Program, and Professor of Pediatrics at The Children's Hospital of Philadelphia told Ivanhoe, "The left ventricle cannot do what it's supposed to do, and therefore such children depend upon the right ventricle to do the job of the left."

But Stosh had an additional high-risk complication. A wall separated the top chambers of the heart, blocking blood from passing into the lungs.

Cardiologists planned to place a needle through Stosh's mother Katrina's abdomen and uterus into Stosh's chest, and then deploy a stent between the blocked heart chambers. Without the fetal procedure, doctors said Stosh would not survive after birth.

Katrina explained, "If that's the only option we have, that's what we have to try."

Using ultrasound guidance, doctors plotted a trajectory for the needle. As soon as the stent was deployed, blood began to flow normally.

"I remember all I could ask was, did it work, and they said yes, it worked and I just started crying" Katrina recalled.

At 39-weeks, doctors delivered Stosh by C-section.  A larger stent was placed right after birth and he went home a happy, eight-pound boy.

Katrina told Ivanhoe, "I don't think a day goes by that I don't count my blessings."

HLHS patients traditionally need a series of surgeries after birth. Stosh had his first surgery at two days and a second surgery at two months. He is preparing for a third surgery. Surgeons have only performed the fetal heart surgery on HLHS patients with a blocked atrial wall a few times. 

 

BACKGROUND: Even though less than 1,000 babies a year have hypoplastic left heart syndrome (HLHS), the effects of the condition can lead to life-long complications. HLHS is a rare heart defect that is present at birth. In HLHS, the left side of the heart is underdeveloped, and therefore cannot effectively pump blood to the rest of the body. An underdeveloped mitral valve, aortic valve or aorta can also cause HLHS. Babies with HLHS may also have an atrial septal defect, which is a hole between the left and right atria of the heart. HLHS is diagnosed during pregnancy or right after the baby is born.  After the baby is born, the symptoms of HLHS include grayish blue skin, difficult, rapid breathing, cold hands and feet, and abnormal inactivity. HLHS can be diagnosed while still in the womb through an ultrasound. There's no known way to prevent HLHS, but family history of heart defects could play a role.

 

TREATMENT: Surgery is always needed to treat HLHS. Usually the baby will either undergo a three-step surgery or need a heart transplant. Before surgery, the baby could receive IV fluids, medication to dilate blood vessels, or be put on a ventilator. The three-step surgery first involves reconstructing the aorta and connecting it to the heart's right ventricle. Then, at 4 to 12 months of age, the work of the right ventricle is reduced by allowing the ventricle to pump blood mainly to the aorta. The final step, which is done around the age of 3, allows the rest of the blood coming back from the body to go to the lungs. Lifelong follow-ups with a cardiologist will be necessary.

 

NEW TECHNOLOGY: Stosh Frydlewicz was born with HLHS, but he had an additional complication that required fetal heart surgery. Doctors at the Children's Hospital of Philadelphia performed a surgery to place a stent between blocked heart chambers while Stosh was still in the womb. Stosh was the first HLHS patient with a blocked atrial wall to survive the specialized fetal surgery.

 

FOR MORE INFORMATION ON THIS REPORT, PLEASE CONTACT:

Ms. Joey McCool

Public Relations

267-426-6070

mccool@email.chop.edu