Visualization and Quantification of the Unrepaired Complete Atrioventricular Canal Valve Using Open-Source Software

Hannah H. Nam, Christian Herz, Andras Lasso, Alana Cianciulli, Maura Flynn, Jing Huang, Zi Wang, Beatriz Paniagua, Jared Vicory, Saleha Kabir, John Simpson, David Harrild, Gerald Marx, Meryl S. Cohen, Andrew C. Glatz, Matthew A. Jolley*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Repair of complete atrioventricular canal (CAVC) is often complicated by residual left atrioventricular valve regurgitation. The structure of the mitral and tricuspid valves in biventricular hearts has previously been shown to be associated with valve dysfunction. However, the three-dimensional (3D) structure of the entire unrepaired CAVC valve has not been quantified. Understanding the 3D structure of the CAVC may inform optimized repair. Methods: Novel open-source work flows were created in SlicerHeart for the modeling and quantification of CAVC valves on the basis of 3D echocardiographic images. These methods were applied to model the annulus, leaflets, and papillary muscle (PM) structure of 35 patients (29 with trisomy 21) with CAVC using transthoracic 3D echocardiography. The mean leaflet and annular shapes were calculated and visualized using shape analysis. Metrics of the complete native CAVC valve structure were compared with those of normal mitral valves using the Mann-Whitney U test. Associations between CAVC structure and atrioventricular valve regurgitation were analyzed. Results: CAVC leaflet metrics varied throughout systole. Compared with normal mitral valves, the left CAVC PMs were more acutely angled in relation to the annular plane (P < .001). In addition, the anterolateral PM was laterally and inferiorly rotated in CAVC, while the posteromedial PM was more superiorly and laterally rotated, relative to normal mitral valves (P < .001). Lower native CAVC atrioventricular valve annular height and annular height–to–valve width ratio before repair were both associated with moderate or greater left atrioventricular valve regurgitation after repair (P < .01). Conclusions: It is feasible to model and quantify 3D CAVC structure using 3D echocardiographic images. The results demonstrate significant variation in CAVC structure across the cohort and differences in annular, leaflet, and PM structure compared with the mitral valve. These tools may be used in future studies to catalyze future research intended to identify structural associations of valve dysfunction and to optimize repair in this vulnerable and complex population.

Original languageEnglish
Pages (from-to)985-996.e11
JournalJournal of the American Society of Echocardiography
Volume35
Issue number9
DOIs
Publication statusPublished - Sep 2022
Externally publishedYes

Bibliographical note

Funding Information:
This work was supported a Pediatric Heart Network Scholars Award ( National Institutes of Health grant U24 HL135691 ), National Institutes of Health grant 1R01HL153166 , National Institute of Biomedical Imaging and Bioengineering grant R01EB021391 , Big Hearts to Little Hearts , a Children’s Hospital of Philadelphia Cardiac Center Innovation Award, the Cora Topolewski Fund at the Children’s Hospital of Philadelphia Pediatric Valve Center , and the CANARIE Research Software Foundation .

Funding Information:
This work was supported a Pediatric Heart Network Scholars Award (National Institutes of Health grant U24 HL135691), National Institutes of Health grant 1R01HL153166, National Institute of Biomedical Imaging and Bioengineering grant R01EB021391, Big Hearts to Little Hearts, a Children's Hospital of Philadelphia Cardiac Center Innovation Award, the Cora Topolewski Fund at the Children's Hospital of Philadelphia Pediatric Valve Center, and the CANARIE Research Software Foundation.

Publisher Copyright:
© 2022 American Society of Echocardiography

Keywords

  • Congenital heart disease
  • Echocardiography
  • Valvular heart disease

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