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Pediatric Heart News - Saving babies with critical heart disease begins before day one

Pediatric Heart News Winter 2012

Saving babies with critical heart disease begins before day one

Date: March 1, 2012

Phil Spevak
Phil Spevak: “Because everything is digital, there are no barriers to consultation. Whether it’s in Baltimore or Bogata, we can consult remotely.”

The diagnosis was the troubling trifecta of congenital heart disease—tricuspid atresia, transposition of the great arteries and coarctation of the aorta. But there was a silver lining in this ominous picture—the diagnosis was made prenatally.

“If the patient had been born and this had not been recognized, the ductus could have closed, compromising perfusion to the body and potentially resulting in renal failure, liver failure and brain damage,” says pediatric cardiologist Phil Spevak, director of noninvasive imaging for congenital heart disease.

Because indications of the problem were first recognized during a routine ultrasound at the end of the first trimester, the obstetrician referred the mother to Johns Hopkins Children’s Center for Fetal Echocardiography, part of a multidisciplinary program that begins with imaging and continues through diagnosis and treatment, which happens as soon after birth as possible.

“Getting the diagnosis right is the most critical thing,” says Spevak. “The whole goal is to get the best outcome. In a sense, we have two patients, the fetus and the family.”

Depending on the heart disease of the fetus, the planning team typically includes—in addition to pediatric cardiologists—cardiac surgeons, neonatologists, perinatologists, nurses and the referring physician. In cases when surgery is not immediately needed—if the baby has an arrhythmia, for example—other interventions are begun, such as treating the mother with an anti-arrhythmia drug designed to cross over to the fetus and control the heartbeat. In this case, the newborn immediately received medication in the NICU to maintain fetal physiology and prevent the transition to postnatal circulation.

 “With a diagnosis by 18 weeks’ gestation,” says Spevak, “we know what the condition is, the people who need to be involved and what we need to do. When the baby is born, everything is prepared.” 

If immediate surgery is indicated, the mother usually delivers at The Johns Hopkins Hospital, which avoids the need to stabilize and transport the fragile newborn. In this case, the newborn underwent the first procedure days after birth and is doing well. “With the prenatal model,” Spevak says, “you have a better chance that the infant will be in good condition going into surgery.”

In the United States, still only about half of all babies with critical heart disease are diagnosed prenatally. Yet with advances in cardiac imaging that not only allow for diagnosis but for modeling surgery before birth, says Spevak, there is more hope for successful outcomes now than ever before.

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