Open Access

ARTICLE

Trajectory of right ventricular indices is an early predictor of outcomes in hypoplastic left heart syndrome

Andrew S. Kim¹, Colleen M. Witzenburg², Mark Conaway³, Jeffrey E. Vergales¹, Jeffrey W. Holmes^2,4, Thomas J. L’Ecuyer¹, Peter N. Dean¹

1 Division of Cardiology, Department of Pediatrics, University of Virginia, Charlottesville, Virginia
2 Department of Biomedical Engineering, University of Virginia, Charlottesville, Virginia
3 Division of Translational Research and Applied Statistics, Department of Public Health Sciences, University of Virginia, Charlottesville, Virginia
4 Department of Medicine, University of Virginia, Charlottesville, Virginia

* Corresponding Author: Andrew S. Kim, Division of Cardiology, Department of Pediatrics, University of Virginia, 1215 Lee St. Charlottesville, VA 22903. Email:

Congenital Heart Disease 2019, 14(6), 1185-1192. https://doi.org/10.1111/chd.12834

Abstract

Background: Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant‐free survival to stage 3 palliation (S3P).
Methods: HLHS patients at a single center who underwent stage 1 palliation (S1P) between 2000 and 2015 were reviewed. Echocardiographic indices of RV size and function were obtained before and following S1P and stage 2 palliation (S2P). The association between these indices and transplant‐free survival to S3P was examined.
Results: There were 61 patients enrolled in the study with 51 undergoing S2P, 20 S3P, and 18 awaiting S3P. In the stage 1 perioperative period, indexed RV end‐systolic area increased in patients who died or needed transplant following S2P, and changed little in those surviving to S3P (3.37 vs −0.04 cm² /m², P = .017). Increased indexed RV end‐systolic area was associated with worse transplant‐free survival. (OR = 0.815, P = .042). In the interstage period, indexed RV end‐diastolic area increased less in those surviving to S3P (3.6 vs 9.2, P = .03).
Conclusion: Change in indexed RV end‐systolic area through the stage 1 periopera‐ tive period was associated with transplant‐free survival to S3P. Neither the prestage nor poststage 1 indexed RV end‐systolic area was associated with transplant‐free survival to S3P. Patients with death or transplant before S3P had a greater increase in indexed RV end‐diastolic area during the interstage period. This suggests earlier se‐ rial changes in RV size which may provide prognostic information beyond RV indices in a single study.

---

Keywords

---