Journal of the American Society of Echocardiography
Clinical InvestigationsEchocardiography in ChildrenVisualization and Quantification of the Unrepaired Complete Atrioventricular Canal Valve Using Open-Source Software
Section snippets
Subjects
Institutional databases at the Children’s Hospital of Philadelphia, Boston Children’s Hospital, and Evelina London Children’s Hospital were used to identify patients with CAVC in whom transthoracic apical four-chamber 3DE had been collected for clinical studies. To augment these studies, dedicated acquisitions in the operating room before repair were obtained at the Children’s Hospital of Philadelphia. These studies were obtained with parental consent per an institutional review board–approved
Results
The CAVC leaflets were modeled in end-diastole, mid-systole, and end-systole in all 35 patients. PM analysis was performed in 34 of the 35 patients with CAVC in mid-systole. The median age of the patients with CAVC at echocardiography was 0.3 years (IQR, 0.2-0.3 years). Twenty patients had Rastelli type A anatomy, and 15 patients had Rastelli type C anatomy. Twenty-nine of the 35 patients had trisomy 21. Thirty-two patients had qualitatively normal left ventricular systolic function, two had
Discussion
We present a novel 3DE-based modeling work flow that we have used to visualize and quantify the native CAVC valve before repair. Our major findings are as follows: (1) modeling of the native CAVC valve annulus, leaflets, and PM from 3DE is feasible using our newly released open-source framework; (2) both the PM angle relative to the annulus and the papillary rotational positions in CAVC differ from normal mitral valves; (3) the CAVC annulus is flatter than a normal mitral valve; and (4) a more
Conclusion
Three-dimensional echocardiography–derived modeling and quantifying the 3D structure of the unrepaired CAVC leaflets, annulus, and PMs is feasible using novel software. We demonstrate significant differences in annular, leaflet, and PM structure compared with the normal mitral valve. Further research is needed to identify 3D structural associations of valve dysfunction and inform the development of 3D image–based customized repairs in this vulnerable population.
Acknowledgment
We acknowledge the sonographers at participating institutions for the outstanding images used for this research.
References (34)
- et al.
Predisposing factors of valve regurgitation in complete atrioventricular septal defect
J Am Coll Cardiol
(1998) - et al.
Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect
Ann Thorac Surg
(2005) - et al.
3-Dimensional echocardiographic analysis of the tricuspid annulus provides new insights into tricuspid valve geometry and dynamics
JACC Cardiovasc Imaging
(2019) - et al.
Three-dimensional mitral valve morphology in children and young adults with Marfan syndrome
J Am Soc Echocardiogr
(2018) - et al.
The value of preoperative 3-dimensional over 2-dimensional valve analysis in predicting recurrent ischemic mitral regurgitation after mitral annuloplasty
J Thorac Cardiovasc Surg
(2016) - et al.
Partial zone of apposition closure in atrioventricular septal defect: are papillary muscles the clue
Ann Thorac Surg
(2013) - et al.
Left valvar morphology is associated with late regurgitation in atrioventricular canal defect
Ann Thorac Surg
(2020) - et al.
Dynamic annular modeling of the unrepaired complete atrioventricular canal annulus
Ann Thorac Surg
(2022) - et al.
Three-dimensional mitral valve morphology and age-related trends in children and young adults with structurally normal hearts using transthoracic echocardiography
J Am Soc Echocardiogr
(2017) - et al.
Dynamic three-dimensional geometry of the tricuspid valve annulus in hypoplastic left heart syndrome with a Fontan circulation
J Am Soc Echocardiogr
(2019)
Subxyphoid 2-dimensional echocardiographic identification of left ventricular papillary muscle anomalies in complete common atrioventricular canal
Am J Cardiol
Left atrioventricular valve after surgical repair in atrioventricular septal defect with separate valve orifices (“ostium primum atrial septal defect”): an echo-Doppler study
Am J Cardiol
Quantitative real-time three-dimensional echocardiography provides new insight into the mechanisms of mitral valve regurgitation post-repair of atrioventricular septal defect
J Am Soc Echocardiogr
The angle of the components of the common atrioventricular valve predicts the outcome of surgical correction in patients with atrioventricular septal defect and common atrioventricular junction
J Am Soc Echocardiogr
3D Slicer as an image computing platform for the Quantitative Imaging Network
Magn Reson Imaging
Computational analysis of virtual echocardiographic assessment of functional mitral regurgitation for validation of proximal isovelocity surface area methods
J Am Soc Echocardiogr
Surgical outcome of reoperation due to left atrioventricular valve regurgitation after previous correction of complete atrioventricular septal defect
J Card Surg
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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.
Drs. Paniagua and Vicory are employees of Kitware.
Girish S. Shirali, MD, FASE, served as guest editor for this report.