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Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation

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Abstract

Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.

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Acknowledgements

The authors would like to acknowledge Zhanna Roytman for data collection and Matthew’s Hearts of Hope Research Grant for financial support that was used towards statistical and Tomtec Software support.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, USA

    Ruchika Karnik, Santosh C. Uppu, Meghan Tozzi, Irene D. Lytrivi, Miwa Geiger, Ira A. Parness, Rajesh Shenoy, Hari Rajagopal & Shubhika Srivastava

  2. Department of Environmental Medicine and Public Health, Icahn School of Medicine at Mount Sinai, New York, USA

    John Doucette

  3. TomTec Imaging Systems, GmbH, Munich, Germany

    Berthold Klas

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  1. Ruchika Karnik
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  2. Santosh C. Uppu
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  3. Meghan Tozzi
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  4. John Doucette
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  5. Irene D. Lytrivi
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Correspondence to Ruchika Karnik.

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Mr. Klas is a full time employee of TomTec Imaging Systems. No disclosures for other authors.

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Karnik, R., Uppu, S.C., Tozzi, M. et al. Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation. Pediatr Cardiol 39, 1172–1180 (2018). https://doi.org/10.1007/s00246-018-1877-9

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  • DOI: https://doi.org/10.1007/s00246-018-1877-9

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