Evaluate the use of coronary CTA as an initial assessment for determining Right Ventricle Dependent Coronary Circulation (RVDCC) in neonates with Pulmonary Atresia with Intact Ventricular Septum (PA IVS). Retrospective review of cases with coronary CTA and compare with available catheter angiography, pathology, surgical reports, and outcomes from Mar 2015 to May 2022. In our cohort of 16 patients, 3 were positive for RVDCC, confirmed by pathologic evaluation, and there was concordance for presence or absence of RVDCC with catheter angiography in 5 patients (4 negatives for RVDCC, 1 positive). Clinical follow up for the 8 patients that underwent RV decompression had no clinical evidence of myocardial ischemia. Our findings suggest that coronary CTA is reliable as first-line imaging for determination of RVDCC in neonates with PA IVS. These findings, if supported by further prospective study, may reserve invasive coronary angiography for cases with diagnostic uncertainty or at the time of necessary transcatheter interventions.
Abbreviations:PA IVS (Pulmonary Atresia with Intact Ventricular Septum), RVDCC (Right Ventricular Dependent Coronary Circulation), CTA (Computed Tomographic Angiography), LAD (Left Anterior Descending), ECG (Electrocardiographic Gating), MPR (Multiplanar Reconstructions), ECMO (Extracorporeal Membrane Oxygenation), mBTS (modified Blalock Taussig Shunt), BSA (Body Surface Area), PDA (Patent Ductus Arteriosus), RF (Radiofrequency), BDG (Bidirectional Glenn shunt), CMR (Cardiac Magnetic Resonance), 2V (Two Ventricle (biventricular repair)), 1V (One Ventricle (single ventricle palliation))
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Published online: October 13, 2022
Publication stageIn Press Journal Pre-Proof
WTSA Meeting Presentation
Funding: There was no funding for this study.
Conflict of interest: The authors have no financial disclosures.
IRB approval was obtained on November 10, 2021, IRB # 21-4728.
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