Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with Hypoplastic Left Heart Syndrome.
Document Type
Article
Publication Date
10-2024
Identifier
DOI: 10.1007/s00246-023-03187-w
Abstract
Despite recent advances, hypoplastic left heart syndrome (HLHS) patients subsequent to the Fontan still have significant morbidity and mortality. Some require heart transplant due to systemic ventricular dysfunction. Limited data exist on timing for transplant referral. This study aims to correlate systemic ventricular strain by echocardiography to transplant-free survival. HLHS patients who had Fontan palliation at our institution were included. Patients were divided into: 1) Required transplant or experienced mortality (composite end point); 2) Did not require transplant or survived. For those who experienced the composite endpoint, the last echocardiogram prior to the composite outcome was used, while for those who did not experience the composite endpoint the last echocardiogram obtained was used. Several qualitative and quantitative parameters were analyzed with focus on strain parameters. Ninety-five patients with HLHS Fontan palliation were identified. Sixty-six had adequate images and eight (12%) experienced transplant or mortality. These patients had greater myocardial performance index by flow Doppler (0.72 versus 0.53, p = 0.01), higher systolic/diastolic duration ratio (1.51 versus 1.13, p = 0.02), lower fractional area change (17.65 versus 33.99, p < 0.01), lower global longitudinal strain (GLS) (-8.63 versus - 17.99, p < 0.01), lower global longitudinal strain rate (GLSR) (- 0.51 versus - 0.93, p < 0.01), lower global circumferential strain (GCS) (-6.68 versus -18.25, p < 0.01), and lower (GCSR) global circumferential strain rate (-0.45 versus -1.01, p < 0.01). ROC analysis demonstrated predictive value for GLS - 7.6 (71% sensitive, 97% specific, AUC 81%), GLSR -0.58 (71% sensitive, 88% specific, AUC 82%), GCS - 10.0 (86% sensitive, 91% specific, AUC 82%), and GCSR -0.85 (100% sensitive, 71% specific, AUC 90%). GLS and GCS can help predict transplant-free survival in patients with hypoplastic left heart syndrome having undergone Fontan palliation. Higher strain values (closer to zero) may be a helpful tool in determining when transplant evaluation is warranted in these patients.
Journal Title
Pediatric cardiology
Volume
45
Issue
7
First Page
1475
Last Page
1484
MeSH Keywords
Humans; Hypoplastic Left Heart Syndrome; Fontan Procedure; Heart Transplantation; Male; Female; Retrospective Studies; Child, Preschool; Infant; Echocardiography; Child; Predictive Value of Tests; Heart Ventricles; ROC Curve
Keywords
Fontan; Hypoplastic left heart; Strain analysis; Strain rate; Transplant
Recommended Citation
Abdulkarim M, Loomba RS, Zaidi SJ, et al. Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with Hypoplastic Left Heart Syndrome. Pediatr Cardiol. 2024;45(7):1475-1484. doi:10.1007/s00246-023-03187-w