All Publications


  • Development and Validation of a Mortality Risk Score for Repaired Tetralogy of Fallot. Journal of the American Heart Association Mayourian, J., Sleeper, L. A., Lee, J. H., Lu, M., Geva, A., Mulder, B., Babu-Narayan, S. V., Wald, R. M., Sompolinsky, T., Valente, A. M., Geva, T. 2024: e034871

    Abstract

    Robust risk assessment is crucial for the growing repaired tetralogy of Fallot population at risk of major adverse clinical outcomes; however, current tools are hindered by lack of validation. This study aims to develop and validate a risk prediction model for death in the repaired tetralogy of Fallot population.Patients with repaired tetralogy of Fallot enrolled in the INDICATOR (International Multicenter Tetralogy of Fallot Registry) cohort with clinical, arrhythmia, cardiac magnetic resonance, and outcome data were included. Patients from London, Amsterdam, and Boston sites were placed in the development cohort; patients from the Toronto site were used for external validation. Multivariable Cox regression was used to evaluate factors associated with time from cardiac magnetic resonance until the primary outcome: all-cause death. Of 1552 eligible patients (n=1221 in development, n=331 in validation; median age at cardiac magnetic resonance 23.4 [interquartile range, 15.6-35.6] years; median follow up 9.5 years), 102 (6.6%) experienced the primary outcome. The multivariable Cox model performed similarly during development (concordance index, 0.83 [95% CI, 0.78-0.88]) and external validation (concordance index, 0.80 [95% CI, 0.71-0.90]) and identified older age at cardiac magnetic resonance, obesity, type of tetralogy of Fallot repair, higher right ventricular end-systolic volume index, and lower biventricular global function index as independent predictors of death. A risk-scoring algorithm dividing patients into low-risk (score ≤4) versus high-risk (score >4) groups was validated to effectively discriminate risk of death (15-year survival of 95% versus 74%, respectively; P<0.001).This externally validated mortality risk prediction algorithm can help identify vulnerable patients with repaired tetralogy of Fallot who may benefit from targeted interventions.

    View details for DOI 10.1161/JAHA.123.034871

    View details for PubMedID 38860401

  • DEVELOPMENT AND VALIDATION OF AN OUTCOME PREDICTION MODEL FOR REPAIRED TETRALOGY OF FALLOT: THE INDICATOR COHORT Mayourian, J., Sleeper, L., Lee, J., Lu, M., Geva, A., Mulder, B., Babu-Narayan, S. V., Wald, R., Sompolinsky, T., Valente, A., Geva, T. ELSEVIER SCIENCE INC. 2023: 1527
  • Improved Outcomes After Pulmonary ValveReplacement in Repaired Tetralogyof Fallot. Journal of the American College of Cardiology Bokma, J. P., Geva, T., Sleeper, L. A., Lee, J. H., Lu, M., Sompolinsky, T., Babu-Narayan, S. V., Wald, R. M., Mulder, B. J., Valente, A. M. 2023; 81 (21): 2075-2085

    Abstract

    BACKGROUND: The impact of pulmonary valve replacement (PVR) on major adverse clinical outcomes in patients with repaired tetralogy of Fallot (rTOF) is unknown.OBJECTIVES: The purpose of this study was to determine whether PVR is associated with improved survival and freedom from sustained ventricular tachycardia (VT) in rTOF.METHODS: A PVR propensity score was created to adjust for baseline differences between PVR and non-PVR patients enrolled in INDICATOR (International Multicenter TOF Registry). The primary outcome was time to the earliest occurrence of death or sustained VT. PVR and non-PVR patients were matched 1:1 on PVR propensity score (matched cohort) and in the full cohort, modeling was performed with propensity score as a covariate adjustment.RESULTS: Among 1,143 patients with rTOF (age 27 ± 14 years, 47% PVR, follow-up 8.3 ± 5.2 years), the primary outcome occurred in 82. The adjusted HR for the primary outcome for PVR vs no-PVR (matched cohort n=524) was 0.41 (95%CI: 0.21-0.81; multivariable model P = 0.010). Full cohort analysis revealed similar results. Subgroup analysis suggested beneficial effects in patients with advanced right ventricular (RV) dilatation (interaction P = 0.046; full cohort). In patients with RV end-systolic volume index >80mL/m2, PVR was associated with a lower primary outcome risk (HR: 0.32; 95%CI: 0.16-0.62; P< 0.001). There was no association between PVR and the primary outcome in patients with RV end-systolic volume index≤80mL/m2 (HR: 0.86; 95%CI: 0.38-1.92; P = 0.70).CONCLUSIONS: Compared with rTOF patients who did not receive PVR, propensity score-matched individuals receiving PVR had lower risk of a composite endpoint of death or sustained VT.

    View details for DOI 10.1016/j.jacc.2023.02.052

    View details for PubMedID 37225360

  • Priorities and Understanding of Pregnancy Among Women With Congenital Heart Disease: A Mixed-Methods Study JACC: Advances Herrick, N., Al-Rousan, ., Rodriguez, C., Lee, J., Valente, A., Stone, J., Ramos, G., Asante-Boateng, B., El-Said, H., Moceri-Casas, M., Alshawabkeh, L. 2022; 1 (4)
  • Temporal Trends of Hospitalization, Mortality, and Financial Impact Related to Preeclampsia with Severe Features in Hawai'i and the United States. Hawai'i journal of health & social welfare Lee, J. H., Zhang, G., Harvey, S., Nakagawa, K. 2019; 78 (8): 252-257

    Abstract

    The temporal trend of hospitalizations, cost, and outcomes associated with preeclampsia with severe features have been inadequately studied. The publicly available Healthcare Cost and Utilization Project (HCUP) National Inpatient Sample (NIS) database was accessed to examine the temporal trend of total number of discharges, age, death, and mean charges per admission associated with preeclampsia with severe features. Eleven-year temporal trends (2004 to 2014) of these measures were compared using linear regression and run charts using the statistical process control rule. From 2004 to 2014, the total number of discharges related to preeclampsia with severe features increased both for Hawai'i and the U.S. (United States) (Hawai'i: 104 to 231; U.S.: 35,082 to 55,235; both P<.0001). The corresponding rates of discharges per 100,000 population also both increased (Hawai'i: 8.2 to 16.3; U.S.: 12.0 to 17.3; both P<.0001). Comparing the temporal trends between Hawai'i and the U.S., Hawai'i had a significantly higher average annual increase in the rate of incidence than the national level (an annual increase rate of 9.2% in Hawai'i vs 4.2% nationally; P=.0004). The cost of hospitalization for preeclampsia with severe features also showed an increased trend for both Hawai'i and the U.S. (Hawai'i: 33.1% increase, P=.0005; U.S.: 41.1% increase, P<.0001). In the U.S., in-hospital mortality rates associated with this condition decreased from 0.09% in 2004 to 0.02% in 2014 (P=.03). In conclusion, the number of discharges related to preeclampsia with severe features increased over an 11-year period in Hawai'i and the U.S., and the rate of increase was higher in Hawai'i than the U.S. Maternal mortality rates from this condition also declined over the study period.

    View details for PubMedID 31463474

    View details for PubMedCentralID PMC6695341