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Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with Hypoplastic Left Heart
Mubeena Abdulkarim1,2, Rohit S Loomba1, S Javed Zaidi1,3
1Advocate Children's Heart Institute, Advocate Children's Hospital, Oak Lawn, IL, USA.
Insights
Echocardiographic strain parameters, including global longitudinal strain (GLS) and global circumferential strain (GCS), can predict transplant-free survival in hypoplastic left heart syndrome (HLHS) patients post-Fontan palliation. Higher strain values indicate a potential need for transplant evaluation.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Echocardiography
Background:
- Hypoplastic left heart syndrome (HLHS) patients undergoing Fontan palliation face significant long-term morbidity and mortality.
- Systemic ventricular dysfunction is a common complication, often necessitating heart transplantation.
- Current guidelines for transplant referral timing in these patients are limited.
Purpose of the Study:
- To correlate systemic ventricular strain parameters, assessed by echocardiography, with transplant-free survival in HLHS patients post-Fontan.
- To identify echocardiographic markers that can predict the need for heart transplantation or mortality.
Main Methods:
- Retrospective analysis of HLHS patients who underwent Fontan palliation.
- Division of patients into groups based on transplant requirement/mortality versus survival.
- Analysis of echocardiographic parameters, focusing on myocardial strain (GLS, GLSR, GCS, GCSR), fractional area change, and other indices.
- Receiver Operating Characteristic (ROC) curve analysis to determine the predictive value of strain parameters.
Main Results:
- Eight out of 66 eligible HLHS patients (12%) experienced the composite endpoint of transplant or mortality.
- Patients with the composite endpoint exhibited significantly lower GLS, GLSR, GCS, and GCSR compared to survivors.
- ROC analysis indicated strong predictive value for GLS, GLSR, GCS, and GCSR in forecasting transplant-free survival.
- Specific thresholds for GLS, GLSR, GCS, and GCSR demonstrated high sensitivity and specificity.
Conclusions:
- Global longitudinal strain (GLS) and global circumferential strain (GCS) are valuable echocardiographic predictors of transplant-free survival in HLHS patients after Fontan palliation.
- Elevated strain values (closer to zero) may serve as an indicator for timely transplant evaluation in this high-risk population.
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.
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