On September 9, 2015, doctors from Children’s Hospital Los Angeles (CHLA) performed an in Utero Atrial Septal stent Procedure on a patient whose 27-week-old fetus (now delivered and named Grayson) was diagnosed with hypoplastic left heart syndrome (HLHS) with restrictive atrial septum.
HLHS with restrictive atrial septum is known a critical cardiac condition that prevents oxygenated blood circulation from a fetus’ lungs back into the body. Babies born with this rare condition have a neonatal mortality rate of 50%. Cardiologists at CHLA advised parents Samantha and Marco Davila that their baby would require open-heart surgery after birth to correct this condition and reintroduce blood flow into the child’s system.
To improve the odds of the baby’s survival during open-heart surgery, specialists from the CHLA-USC Institute for Maternal-Fetal Health formed a special medical team to execute the successful cardiac intervention procedure in utero. The procedure was meant to open the atrium and allow blood trapped in the lungs and left upper heart chamber to flow back to the right side of the heart.
Ramen Chmait, MD, director of CHLA-US Institute for Maternal-Fetal Health’s Los Angeles Fetal Surgery branch, took the helm of the fetal intervention procedure. He worked closely with Frank Ing, MD, co-director of the Chla Heart Institute and CHLA fetal cardiologist Jay Pruetz, MD. The procedure itself involved Dr. Chmait guiding a thin needle into Mrs. Davila’s womb and into the heart of the fetus. Dr. Ing was then tasked with deploying a stent device into the fetus’ developing heart. Both physicians were guided via fetal echocardiogram provided by Dr. Pruetz.
“The work by the team demonstrated the state-of-the-art prenatal diagnosis and fetal heart therapy technology, based on a multi-disciplinary approach, available for fetuses with severe congenital heart defects,” says Ing, chief of the Division of Cardiology at CHLA. “We demonstrated that in utero procedures can ultimately alter the natural history and postnatal health outcomes to increase a child’s odds for survival. This expertise was brought together by a vision to provide these specialized interventions and top surgical care as no other institution can in Southern California.”
“Completing this highly specialized fetal intervention procedure contributed to Grayson’s ability to survive after his birth,” says Chmait, associate professor of Clinical Obstetrics and Gynecology at the Keck School of Medicine of the University of Southern California. “It successfully strengthened his lungs so the surgeons could do their job after he was born.”
The entire procedure was completed within 45 minutes and allowed Grayson’s lungs time to strengthen and develop. Samantha gave birth via planned cesarean section ten weeks after the stent procedure at Hollywood Presbyterian. Following the delivery, Grayson was immediately transported to CHLA where a pediatric cardiothoracic surgery team was standing by to perform a complete Norwood surgery. The operation was led by Vaughn Starnes, MD, co-director of the CHLA Heart Institute, 90 minutes after Grayson’s birth.
“We were able to operate quickly because we were aware of his condition and Grayson’s lungs were strong enough,” Starnes explains. “This coordination of care between the fetal therapy team and CHLA surgeons was extraordinary, starting with the transcatheter fetal cardiac procedure, the monitoring during the pregnancy leading up to the birth, and the surgery.”
Grayson was discharged from CHLA on December 14, 2015 and is set to undergo two additional heart surgeriesto route oxygen-poor blood directly to the lungs and leaving the right ventricle to act as the heart’s primary pumping chamber.
CHLA’s successful in utero atrial Septal Stent Procedure is the first of its kind for both the institute for Maternal-Fetal Health and a Southern California hospital. According to Grayson’s father, Marco Davila, “CHLA was the only institution I could find on the West Coast that offered my child the access to premium, state-of-the-art facilities and great care before and after his birth,”
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