Clinical Variables Associated with Pre-Fontan Aortopulmonary Collateral Burden

Pediatr Cardiol. 2023 Jan;44(1):228-236. doi: 10.1007/s00246-022-03014-8. Epub 2022 Sep 25.


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.

Keywords: Aortopulmonary collaterals; Fontan; Glenn; Single ventricle.

MeSH terms

  • Collateral Circulation
  • Fontan Procedure* / methods
  • Heart Defects, Congenital* / diagnostic imaging
  • Heart Defects, Congenital* / surgery
  • Heart Ventricles / surgery
  • Humans
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Artery / surgery
  • Pulmonary Circulation
  • Retrospective Studies
  • Treatment Outcome
  • Univentricular Heart*