Genome sequencing is critical for forecasting outcomes following congenital cardiac surgery

W W. Scott Watkins E Edgar J. Hernandez T Thomas A. Miller N Nathan R. Blue R Raquel Mae Zimmerman E Eric R. Griffiths E Erwin Frise D Daniel Bernstein (Department of Pediatrics, Cardiology, Stanford University) M Marko T. Boskovski M Martina Brueckner (Department of Genetics, Yale School of Medicine) W Wendy K. Chung (Department of Pediatrics, Boston Children’s Hospital, Harvard Medical School) J J. William Gaynor (Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia) B Bruce D. Gelb (Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai) E Elizabeth Goldmuntz (Division of Cardiology, Children’s Hospital of Philadelphia, Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania) P Peter J. Gruber (Department of Surgery, Yale University School of Medicine) J Jane W. Newburger (Department of Cardiology, Boston Children’s Hospital, Harvard Medical School) A Amy E. Roberts (Department of Cardiology, Boston Children’s Hospital, Harvard Medical School) S Sarah U. Morton (Division of Newborn Medicine, Department of Pediatrics, Boston Children’s Hospital) J John E. Mayer C Christine E. Seidman J Jonathan G. Seidman Y Yufeng Shen (Department of Systems Biology, Columbia University Irving Medical Center) M Michael Wagner H H. Joseph Yost (Department of Human Genetics, University of Utah and School of Medicine) M Mark Yandell (Department of Human Genetics, University of Utah and School of Medicine) M Martin Tristani-Firouzi (Division of Pediatric Cardiology, University of Utah)

Abstract

Abstract While exome and whole genome sequencing have transformed medicine by elucidating the genetic underpinnings of both rare and common complex disorders, its utility to predict clinical outcomes remains understudied. Here, we use artificial intelligence (AI) technologies to explore the predictive value of whole exome sequencing in forecasting clinical outcomes following surgery for congenital heart defects (CHD). We report results for a prospective observational cohort study of 2,253 CHD patients from the Pediatric Cardiac Genomics Consortium with a broad range of complex heart defects, pre- and post-operative clinical variables and exome sequencing. Damaging genotypes in chromatin-modifying and cilia-related genes are associated with an elevated risk of adverse post-operative outcomes, including mortality, cardiac arrest and prolonged mechanical ventilation. The impact of damaging genotypes is further amplified in the context of specific CHD phenotypes, surgical complexity and extra-cardiac anomalies. The absence of a damaging genotype in chromatin-modifying and cilia-related genes is also informative, reducing the risk for some adverse postoperative outcomes. Thus, genome sequencing enriches the ability to forecast outcomes following congenital cardiac surgery.

Article Details

Volume / Issue Vol. 16, Issue 1
Published July 10, 2025
ISSN 2041-1723
Publisher Nature Portfolio

Journal Info

Nature Communications

Nature Portfolio

ISSN: 2041-1723 Open Access Life Sciences

Authors (26)

W

W. Scott Watkins

E

Edgar J. Hernandez

T

Thomas A. Miller

N

Nathan R. Blue

R

Raquel Mae Zimmerman

E

Eric R. Griffiths

E

Erwin Frise

D

Daniel Bernstein

Department of Pediatrics, Cardiology, Stanford University

M

Marko T. Boskovski

M

Martina Brueckner

Department of Genetics, Yale School of Medicine

W

Wendy K. Chung

Department of Pediatrics, Boston Children’s Hospital, Harvard Medical School

J

J. William Gaynor

Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia

B

Bruce D. Gelb

Mindich Child Health and Development Institute, Icahn School of Medicine at Mount Sinai

E

Elizabeth Goldmuntz

Division of Cardiology, Children’s Hospital of Philadelphia, Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania

P

Peter J. Gruber

Department of Surgery, Yale University School of Medicine

J

Jane W. Newburger

Department of Cardiology, Boston Children’s Hospital, Harvard Medical School

A

Amy E. Roberts

Department of Cardiology, Boston Children’s Hospital, Harvard Medical School

S

Sarah U. Morton

Division of Newborn Medicine, Department of Pediatrics, Boston Children’s Hospital

J

John E. Mayer

C

Christine E. Seidman

J

Jonathan G. Seidman

Y

Yufeng Shen

Department of Systems Biology, Columbia University Irving Medical Center

M

Michael Wagner

H

H. Joseph Yost

Department of Human Genetics, University of Utah and School of Medicine

M

Mark Yandell

Department of Human Genetics, University of Utah and School of Medicine

M

Martin Tristani-Firouzi

Division of Pediatric Cardiology, University of Utah