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Clinical Variables Associated with Pre-Fontan Aortopulmonary Collateral Burden

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Abstract

Aortopulmonary collaterals (APCs) develop universally, but to varying degrees, in patients with single ventricle congenital heart disease (CHD). Despite their ubiquitous presence, APCs remain poorly understood. We sought to evaluate the association between APC burden and common non-invasive clinical variables. We conducted a single center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent pre-Fontan cardiac magnetic resonance (CMR) imaging from 3/2018 to 3/2021. CMR was used to quantify APC flow, which was normalized to aortic (APC/QAo) and pulmonary vein (APC/QPV) blood flow. Univariate, multivariable, and classification and regression tree (CART) analyses were done to investigate the potential relationship between CMR-quantified APC burden and clinical variables. A total of 29 patients were included, all of whom had increased APC flow (APC/QAo: 26.9, [22.0, 39.1]%; APC/QPV: 39.4 [33.3, 46.9]%), but to varying degrees (APC/QAo: range 11.9–44.4%; APC/QPV: range 17.7–60.0%). Pulmonary artery size (Nakata index, at pre-Fontan CMR) was the only variable associated with APC flow on multivariable analysis (APC/QAo: p = 0.020, R2 = 0.19; APC/QPV: p = 0.0006, R2 = 0.36) and was the most important variable associated with APC burden identified by CART analysis (size inversely related to APC flow). APC flow is universally increased but highly variable in patients with single ventricle CHD and Glenn circulation. Small branch pulmonary artery size is a key factor associated with increased APC burden; however, the pathogenesis of APCs is likely multifactorial. Further research is needed to better understand APC pathogenesis, including predisposing and mitigating factors.

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Funding

ADS was supported by the National Institutes of Health from the National Heart, Lung, and Blood Institute (K08HL157510) and by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health (UL1TR001436). Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIH.

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Authors and Affiliations

  1. Division of Cardiology, Department of Pediatrics, Herma Heart Institute, Medical College of Wisconsin, Children’s Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI, 53226, USA

    David E. Segar, Daniel I. McLennan, Steven J. Kindel, Stephanie S. Handler, Salil Ginde, Benjamin H. Goot & Andrew D. Spearman

  2. Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI, 53226, USA

    David E. Segar, Amy Y. Pan, Daniel I. McLennan, Steven J. Kindel, Stephanie S. Handler, Salil Ginde, Ronald K. Woods, Benjamin H. Goot & Andrew D. Spearman

  3. Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Children’s Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI, 53226, USA

    Amy Y. Pan

  4. Division of Cardiology, Department of Medicine, Medical College of Wisconsin, 8701 West Watertown Plank, Milwaukee, WI, 53226, USA

    Salil Ginde

  5. Division of Congenital Cardiac Surgery, Department of Surgery, Medical College of Wisconsin, 8701 West Watertown Plank, Milwaukee, WI, 53226, USA

    Ronald K. Woods

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  1. David E. Segar
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  2. Amy Y. Pan
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  8. Benjamin H. Goot
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  9. Andrew D. Spearman
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Contributions

Data collection: DES, BHG, ADS. Data analysis and interpretation: DES, AYP, BHG, ADS. Statistical analysis: DES, AYP, ADS. Wrote and edited the manuscript: DES, AYP, SSH, RKW, BHG, ADS. All authors participated in study design, reviewed the manuscript, and approved the final version.

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Correspondence to Andrew D. Spearman.

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The authors declare no competing interests.

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This study was approved by the Local Institutional Review Board.

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Segar, D.E., Pan, A.Y., McLennan, D.I. et al. Clinical Variables Associated with Pre-Fontan Aortopulmonary Collateral Burden. Pediatr Cardiol 44, 228–236 (2023). https://doi.org/10.1007/s00246-022-03014-8

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  • DOI: https://doi.org/10.1007/s00246-022-03014-8

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