Author + information
- Sophie C. Hofferberth,
- Sitaram Emani,
- Minmin Lu,
- Lynn A. Sleeper,
- Fatima Lunze,
- Gerald Marx,
- Christopher Baird,
- Pedro del Nido and
- Meena Nathan
Background: Patient-based determinants of pulmonary valve (PV) function post valve-sparing repair (VSR) of tetralogy of Fallot (ToF) are unknown. We developed a risk score for structural valve deterioration (SVD) in patients undergoing VSR of ToF.
Methods: All patients who underwent VSR of ToF at <1 year of age were assessed. Multivariable Cox regression was used to develop a risk score for SVD; defined as a composite endpoint of PV reintervention, PV peak gradient ≥ 40mmHg and ≥ moderate PV regurgitation.
Results: From 2007-2015, 207 consecutive patients (median age: 98 days, IQR: 72, 129) underwent VSR of ToF. PV morphology was tricuspid in 37 (18%), bicuspid in 151 (73%) and unicuspid in 18 (9%) patients. Median PV annulus Z-score was −2.1 (IQR: −2.4, −1.6). Freedom from SVD was 65%, 50% and 40% at 1, 3 and 5 years post VSR. Univariate analysis showed PV annulus Z score <-1.77 (p≤0.001), uni- or bicuspid PV morphology (p=0.002), dysplastic leaflets, (p=0.007) and younger age (p=0.009) were associated with SVD. Multivariable analysis identified PV annulus size (HR 0.46, CI 0.32, 0.68; p<0.001) and unicuspid PV morphology (HR 3.78, CI 1.74, 8.24; p=0.003) as independent predictors of SVD. Patients in the upper risk score categories (50-<75%tile, ≥75%tile) were more likely to develop SVD (Figure 1, p≤0.001).
Conclusions: VSR of ToF is associated with a high incidence of SVD. Risk stratification based on PV anatomy identifies patients at high-risk of early valve failure post VSR, this may be useful to guide therapy.
Moderated Poster Contributions
Congenital Heart Disease and Pulmonary Hypertension Moderated Poster Theater, Poster Hall, Hall C
Friday, March 17, 2017, 4:15 p.m.-4:25 p.m.
Session Title: Predicting Outcomes in Pediatric Heart Disase
Abstract Category: 10. Congenital Heart Disease: Pediatric
Presentation Number: 1171M-07
- 2017 American College of Cardiology Foundation