Faculty Collaboration Database

Earlier Pulmonary Valve Replacement in Down Syndrome Patients Following Tetralogy of Fallot Repair. Pediatr Cardiol 2017 Aug;38(6):1251-1256

Date

06/16/2017

Pubmed ID

28616649

DOI

10.1007/s00246-017-1653-2

Scopus ID

2-s2.0-85020703696 (requires institutional sign-in at Scopus site)   3 Citations

Abstract

The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis. Those with Down syndrome were compared to those without. The primary outcome of interest was time from repair to pulmonary valve replacement. Secondary outcomes included pulmonary regurgitation and indexed right ventricular volume on cardiac magnetic resonance imaging. The cohort of 284 patients included 35 (12%) with Down syndrome. Transannular patch repair was performed in 210 (74%). Down syndrome showed greater degree of pulmonary regurgitation (55 ± 14 vs. 37 ± 16%, p = 0.01) without a significantly greater rate of right ventricular dilation (p = 0.09). In multivariable analysis, Down syndrome (HR 2.3, 95% CI 1.2-4.5, p = 0.02) and transannular patch repair (HR 5.5, 95% CI 1.7-17.6, p = 0.004) were significant risk factors for valve replacement. Those with Down syndrome had significantly lower freedom from valve replacement (p = 0.03). Down syndrome is associated with an increased degree of pulmonary regurgitation and earlier pulmonary valve replacement after tetralogy of Fallot repair. These patients require earlier assessment by cardiac magnetic resonance imaging to determine timing of pulmonary valve replacement and evaluation for and treatment of preventable causes of pulmonary hypertension.

Author List

Sullivan RT, Frommelt PC, Hill GD

Author

Peter C. Frommelt MD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Cardiac Surgical Procedures
Child
Child, Preschool
Down Syndrome
Female
Heart Valve Prosthesis Implantation
Humans
Infant
Infant, Newborn
Male
Pulmonary Valve
Pulmonary Valve Insufficiency
Pulmonary Valve Stenosis
Retrospective Studies
Tetralogy of Fallot
Time Factors