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Longitudinal Assessment of Right Ventricular Function in Hypoplastic Left Heart Syndrome

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Abstract

Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan–Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5 years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p < 0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from −2.9 ± 1.3 to −9.7 ± 1.3 (p < 0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC >35% and TAPSE Z-score > -5, patients with RV FAC  ≤ 35% and TAPSE Z-score  ≤ -5 had a significantly lower transplant-free survival (p < 0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.

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Abbreviations

HLHS:

Hypoplastic left heart syndrome

RV:

Right ventricle

FAC:

Fractional area change

TAPSE:

Tricuspid annular systolic excursion

VTI:

Velocity time integral

GLS:

Global longitudinal strain

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Authors and Affiliations

  1. Division of Cardiology, Department of Pediatrics, Stanford Medical School, 750 Welch Road, Stanford, CA, USA

    Sowmya Balasubramanian, Shea N. Smith, Theresa A. Tacy, Frank L. Hanley, Sharon Chen, Gail E. Wright, Lynn F. Peng & Rajesh Punn

  2. Department of Mathematics, Cleveland State University, Cleveland, OH, USA

    Parthasarathy Srinivasan

  3. Division of Pediatric Cardiology, 1540 East Hospital Drive, Ann Arbor, MI, 48109, USA

    Sowmya Balasubramanian

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  1. Sowmya Balasubramanian
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  2. Shea N. Smith
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Balasubramanian, S., Smith, S.N., Srinivasan, P. et al. Longitudinal Assessment of Right Ventricular Function in Hypoplastic Left Heart Syndrome. Pediatr Cardiol 42, 1394–1404 (2021). https://doi.org/10.1007/s00246-021-02624-y

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