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Tricuspid Atresia with Non-compaction: An Early Experience with Implications for Surgical Palliation

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Abstract

Left ventricle non-compaction (LVNC) has worse outcomes when associated with congenital heart defects (CHD). The co-occurrence and outcomes of LVNC with tricuspid atresia (TA) are not well described. Our study aims to determine the prevalence of LVNC with functionally single ventricle due to TA, and to describe the early outcomes of surgical palliation. A retrospective database search for patients (n = 167,566) and echocardiograms (n = 44,053) was performed in order to collect clinical, echocardiographic, and hemodynamic data of pediatric patients with TA and LVNC at St. Louis Children’s Hospital, Missouri, USA, from January 1, 2008, to August 31, 2013. The prevalence of TA and LVNC was 0.015 and 0.08%, respectively. Eight patients with TA had LVNC (32%, group 1). Seventeen patients had only TA (68%, group 2). Five patients from group 1 and 8 patients from group 2 were surgically palliated with the Fontan procedure. They exhibited similar remodeling indices, and hemodynamics (median LV end-diastolic pressure ≤10 mmHg; median mean pulmonary artery pressure ≤15 mmHg) that allowed for completion of the Fontan procedure. All Fontan patients were in New York Heart Association class I after a 3-year (median) follow-up period. Our data show that TA with LVNC patients, who have acceptable cardiac remodeling indices, LV systolic function, and hemodynamics (LVEDP ≤ 10 mmHg, mean pulmonary artery pressure ≤ 15 mmHg) can have successful completion of the Fontan procedure and positive early outcomes.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Cardiology, Washington University in St. Louis School of Medicine, 660 S. Euclid Ave., Campus Box 8116-NWT, St. Louis, 63110, MO, USA

    Hoang H. Nguyen, Rabia Khan & Gautam K. Singh

  2. Department of Pediatrics, Division of Cardiology, Rush University Medical College, 1753 W. Congress Pkwy., Pavilion 654, Chicago, IL, 60612, USA

    Hoang H. Nguyen

  3. Department of Pediatrics, Division of Cardiology, Stanford University School of Medicine, 750 Welch Rd., Suite 305, Palo Alto, 94304, CA, USA

    Norman H. Silverman

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  1. Hoang H. Nguyen
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  2. Rabia Khan
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  4. Gautam K. Singh
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Correspondence to Hoang H. Nguyen.

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Nguyen, H.H., Khan, R., Silverman, N.H. et al. Tricuspid Atresia with Non-compaction: An Early Experience with Implications for Surgical Palliation. Pediatr Cardiol 38, 495–505 (2017). https://doi.org/10.1007/s00246-016-1541-1

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  • DOI: https://doi.org/10.1007/s00246-016-1541-1

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