Study Identifies Risk Factors That Increase Risk Of Death In Children With Fontan Circulatory Failure

A multi-center study has identified captious consequence factors that summation nan likelihood of decease successful children pinch a bosom defect who are awaiting aliases person precocious undergone bosom transplantation, according to findings published successful Circulation.

Fontan circulatory nonaccomplishment (FCF) is simply a semipermanent complication successful children calved pinch single-ventricle bosom defects who person undergone a bid of surgeries that culminates pinch nan Fontan procedure. While this room helps reroute humor travel and widen life expectancy, it tin lead to chronic wellness problems, including bosom nonaccomplishment and harm to different organs. Many of these patients yet require a bosom transplant.

The caller study, conducted crossed 20 U.S. aesculapian centers, offers caller insights into really circumstantial wellness complications impact endurance successful children pinch nan condition, said co-author Anna Joong, MD, cardiologist astatine Ann & Robert H. Lurie Children's Hospital of Chicago and Associate Professor of Pediatrics astatine Northwestern University Feinberg School of Medicine.

"We person a beardown liking successful knowing patients pinch Fontan circulatory failure: really we tin amended negociate their bosom failure, erstwhile nan perfect clip is to database them for a bosom transplant, and what nan consequence factors are while waiting for a transplant," Dr. Joong said.

In nan study, investigators analyzed information from 409 patients who had undergone a Fontan process and were listed for bosom transplant betwixt 2008 and 2022. They tracked outcomes from nan clip of waitlisting done 1 twelvemonth aft transplant. They recovered that 5.9 percent died awaiting a transplant, and among those transplanted, 8.5 percent died wrong nan first year.

The investigators besides recovered that definite pre-existing conditions importantly accrued nan consequence of death, including:

• Repeated hospitalizations successful nan twelvemonth earlier being listed for transplant doubled nan consequence of death.
• Clinical cyanosis - a information successful which oxygen levels successful nan humor are dangerously debased - was associated pinch a fivefold summation successful mortality risk.
• Sleep apnea, intelligence wellness conditions requiring treatment, and anatomical complications for illustration Fontan pathway obstruction were each linked pinch a higher consequence of death.

Overall, endurance has improved for patients who person azygous ventricle physiology and who had a Fontan. A really caller uncovering of this study was that patients pinch debased oxygen levels were associated pinch mediocre outcomes. That's an important point for clinicians and families to cognize about, arsenic an further consequence facet and erstwhile to mention to precocious bosom nonaccomplishment therapies."

Dr. Anna Joong, MD, cardiologist astatine Ann & Robert H. Lurie Children's Hospital of Chicago and Associate Professor of Pediatrics astatine Northwestern University Feinberg School of Medicine

The findings should guideline clinicians successful identifying high-risk patients earlier and considering much fierce interventions, including earlier bosom nonaccomplishment squad and transplant referral, Dr. Joong said. Overall, nan study lays nan groundwork for improving transplant outcomes and tailoring attraction to nan circumstantial needs of children calved pinch single-ventricle bosom defects.

Moving forward, Joong and her collaborators will proceed to study children post-heart transplant to understand their value of life and reside immoderate functional limitations, she said.

The study was supported by a assistance from nan Additional Ventures and Enduring Hearts organizations and a gift from nan Van Hooser family.