Combined ventricular dysfunction and atrioventricular valve regurgitation after the Norwood procedure are associated with attrition prior to superior cavopulmonary connectionCentral MessagePerspective
Sheri L. Balsara, MD, MS,
Danielle Burstein, MD, MSCE,
Richard F. Ittenbach, PhD,
Michelle Kaplinski, MD,
Monique M. Gardner, MD,
Chitra Ravishankar, MD,
Joseph Rossano, MD,
David J. Goldberg, MD,
Marlene Mahle, RN,
Matthew J. O'Connor, MD,
Christopher E. Mascio, MD,
J. William Gaynor, MD,
Tamar J. Preminger, MD
Affiliations
Sheri L. Balsara, MD, MS
The Children's Hospital of Philadelphia, Philadelphia, Pa; Address for reprints: Sheri L. Balsara, MD, MS, Cardiac Intensive Care Unit, Department of Anesthesia and Critical Care Medicine, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104.
Danielle Burstein, MD, MSCE
The Children's Hospital of Philadelphia, Philadelphia, Pa; University of Vermont Medical Center, Burlington, Vt
Richard F. Ittenbach, PhD
Cincinnati Children's Hospital, Cincinnati, Ohio
Michelle Kaplinski, MD
Stanford Children's Health, Palo Alto, Calif
Monique M. Gardner, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
Chitra Ravishankar, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
Joseph Rossano, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
David J. Goldberg, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
Marlene Mahle, RN
The Children's Hospital of Philadelphia, Philadelphia, Pa
Matthew J. O'Connor, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
Christopher E. Mascio, MD
West Virginia University Medicine Children's Hospital, Morgantown, WVa
J. William Gaynor, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
Tamar J. Preminger, MD
The Children's Hospital of Philadelphia, Philadelphia, Pa
Background: Infants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined. Methods: A retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed. VD and AVVR were defined as moderate or severe when seen on 2 sequential echocardiograms outside the perioperative period. Attrition was defined as death, listing for heart transplant, or unsuitability for SCPC or transplant. Descriptive statistics and regression models were used for analysis. Results: A total of 397 patients were included, of whom 75% had HLHS and 57% had received a Blalock-Thomas-Taussig shunt. Isolated VD occurred in 9% of patients, AVVR occurred in 13%, and both occurred in 6%. Attrition prior to SCPC occurred in 19% of the overall cohort, in 52% of patients with combined VD and AVVR (odds ratio [OR], 5.2; 95% confidence interval [CI], 2.3-12.0; P < .01), 26% of those with VD (OR, 1.5; 95% CI, 0.7-3.3; P = .32), 25% of those with AVVR (OR, 1.5; 95% CI, 0.7-2.9; P = .27), and 15% in those with neither (OR, 0.3; 95% CI, 0.2-0.6; P < .01). Other factors associated with attrition included prematurity, total bypass time at Norwood, and extracorporeal membrane oxygenation after Norwood, whereas later year of Norwood was protective (P < .01 for all). Conclusions: The presence of combined VD and AVVR markedly increases the likelihood of attrition prior to SCPC, identifying a high-risk group.
