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Central Surgical Association

49th Annual Meeting

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Computed Tomographic Angiography (CTA) Provides Reproducible and Reliable Identification of Right Ventricular Dependent Coronary Circulation in Infants with Pulmonary Atresia and Intact Ventricular Septum
LaDonna J. Malone2, Lorna P. Browne2, Gareth J. Morgan3, *Max B. Mitchell1, *David N. Campbell1, *James Jaggers1, *Matthew L. Stone1
1Surgery, Division of Pediatric Cardiac Surgery, University of Colorado School of Medicine, Denver, Colorado, United States, 2Radiology, Division of Pediatric Radiology, University of Colorado School of Medicine, Aurora, Colorado, United States, 3Pediatrics, Division of Pediatric Interventional Cardiology, University of Colorado School of Medicine, Aurora, Colorado, United States

Objective: Incorrect identification of RVDCC (defined as significant stenosis in two of the three major coronary arteries) in infants with pulmonary atresia and intact ventricular septum (PA/IVS) prior to decompression of the right ventricle may result in fatal myocardial ischemia. Transcatheter aortography has served as the traditional method for coronary evaluation yet is limited by both diagnostic accuracy and the potential for induced physiologic impairment. CTA has emerged as a promising strategy for neonatal coronary evaluation yet applications in infants with PA/IVS remain undefined. Thus, the purpose of this study was to review a single institution experience with first-line CTA in the identification of coronary artery stenoses and RVDCC to define candidacy for neonatal orthotopic heart transplantation (OHT) versus biventricular repair or single-ventricle palliation.
Methods: Infants diagnosed with PA/IVS at our institution from 2015-2021 that underwent first-line CTA coronary artery evaluation were retrospectively reviewed. CTA findings were compared to post-CTA invasive catheterization findings, if performed, and subsequent treatment strategy to determine diagnostic accuracy of CTA in defining RVDCC. Infants with RVDCC were considered candidates for OHT and did not undergo RV decompression.
Results: 12 infants were born with a diagnosis of PA/IVS during the study period and all underwent successful first-line cardiac CTA imaging (Age: 1-48 days). 3/12 (25%) infants required repeated imaging sequencing for undefined coronary anatomy. Coronary sinusoids were visualized in 8/12 (66.7%) infants and 4/12 (33.3%) infants demonstrated significant coronary artery dilation. Of those with coronary sinusoids, 2/8 (25%) infants demonstrated findings consistent with RVDCC. 1/12 (8.3%) infant had non-visualized coronary arteries on CTA and underwent confirmatory catheterization which demonstrated markedly abnormal, nearly atretic coronary arteries. 5/12 (41.7%) infants underwent single ventricle palliation while biventricular repairs were achieved in 5/12 (41.7%). 2/12 (16.7%) infants with RVDCC are currently awaiting OHT.
Conclusions: The findings of the present study support the diagnostic accuracy and utility of cardiac CTA as the first-line imaging modality for infants with PA/IVS to define RVDCC and transplantation candidacy. CTA should be considered over invasive catheterization for infants with PA/IVS given diagnostic accuracy, reproducibility, and lower ionizing radiation dose; yet there remains a role for confirmatory aortography in select infants. Standardized, prospective study is needed to further define the utility of cardiac CTA in the palliation and treatment of infants with PA/IVS and RVDCC.


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