Children with rare heart condition linked to brain abnormalities

Children with single-ventricle disease face three reconstructive surgeries to treat the complex heart defect. According to a recent study from the Children’s Hospital of Philadelphia (CHOP), such patients also experience higher rates of brain abnormalities during this multi-step process.

The study, published in Circulation, examined 168 children who underwent the reconstructive procedures from 2009 and 2014. The team found infants experience changes in cerebral blood flow that could lead to long-term neurological development.

"We have long known that children with single-ventricle disease have a strong risk of poor neurologic outcomes after surviving staged surgical reconstruction," said pediatric cardiologist Mark A. Fogel, MD, primary investigator of the CHOP team. "This was the first study to measure the incidence of brain abnormalities throughout the three stages of surgery, and to investigate a correlation between cerebral blood flow and brain lesions."

The CHOP team investigated possible links between brain abnormalities and cerebral blood flow, oxygen delivery and carbon dioxide reactivity. MRI showed the children had experienced tissue loss and changes in white matter.

Still, Fogel and colleagues recommended further examination of the relationship between the procedures and resulting brain isses.

"This study cannot identify a cause-and-effect relationship between cerebral blood flow and brain lesions," said Fogel. "A longer-range, more complicated study would be necessary to determine that." 

Children with single-ventricle disease face three reconstructive surgeries to treat the complex heart defect. According to a recent study from the Children’s Hospital of Philadelphia (CHOP), such patients also experience higher rates of brain abnormalities during this multi-step process.

The study, published in Circulation, examined 168 children who underwent the reconstructive procedures from 2009 and 2014. The team found infants experience changes in cerebral blood flow that could lead to long-term neurological development.

"We have long known that children with single-ventricle disease have a strong risk of poor neurologic outcomes after surviving staged surgical reconstruction," said pediatric cardiologist Mark A. Fogel, MD, primary investigator of the CHOP team. "This was the first study to measure the incidence of brain abnormalities throughout the three stages of surgery, and to investigate a correlation between cerebral blood flow and brain lesions."

The CHOP team investigated possible links between brain abnormalities and cerebral blood flow, oxygen delivery and carbon dioxide reactivity. MRI showed the children had experienced tissue loss and changes in white matter.

Still, Fogel and colleagues recommended further examination of the relationship between the procedures and resulting brain isses.

"This study cannot identify a cause-and-effect relationship between cerebral blood flow and brain lesions," said Fogel. "A longer-range, more complicated study would be necessary to determine that."