Abstract

IntroductionA 2-year-old female with hypoplastic left heart syndrome (HLHS)-variant, a complex congenital heart defect (CHD) characterized by the underdevelopment of the left ventricle, presented with complications following single ventricle palliation. Diagnostic work-up revealed elevated Fontan pathway pressures, as well as significant dilation of the inferior Fontan pathway with inefficient swirling flow and hepatic venous reflux. Due to the frail condition of the patient, the clinical team considered an endovascular revision of the Fontan pathway. In this work, we performed a computational fluid dynamics (CFD) analysis informed by data on anatomy, flow, and pressure to investigate the hemodynamic effect of the endovascular Fontan revision.MethodsA patient-specific anatomical model of the Fontan pathway was constructed from magnetic resonance imaging (MRI) data using the cardiovascular modeling software CardiovasculaR Integrated Modeling and SimulatiON (CRIMSON). We first created and calibrated a pre-intervention 3D-0D multi-scale model of the patient’s circulation using fluid-structure interaction (FSI) analyses and custom lumped parameter models (LPMs), including the Fontan pathway, the single ventricle, arterial and venous systemic, and pulmonary circulations. Model parameters were iteratively tuned until simulation results matched clinical data on flow and pressure. Following calibration of the pre-intervention model, a custom bifurcated endograft was introduced into the anatomical model to virtually assess post-intervention hemodynamics.ResultsThe pre-intervention model successfully reproduced the clinical hemodynamic data on regional flow splits, pressures, and hepatic venous reflux. The proposed endovascular repair model revealed increases of mean and pulse pressure at the inferior vena cava (IVC) of 6 and 29%, respectively. Inflows at the superior vena cava (SVC) and IVC were each reduced by 5%, whereas outflows at the left pulmonary artery (LPA) and right pulmonary artery (RPA) increased by 4%. Hepatic venous reflux increased by 6%.ConclusionOur computational analysis indicated that the proposed endovascular revision would lead to unfavorable hemodynamic conditions. For these reasons, the clinical team decided to forgo the proposed endovascular repair and to reassess the management of this patient. This study confirms the relevance of CFD modeling as a beneficial tool in surgical planning for single ventricle CHD patients.

Highlights

  • A 2-year-old female with hypoplastic left heart syndrome (HLHS)-variant, a complex congenital heart defect (CHD) characterized by the underdevelopment of the left ventricle, presented with complications following single ventricle palliation

  • The current treatment paradigm for HLHS and/or HLHSvariants consists of multiple staged reconstructive surgeries, aimed at creating a Fontan circulation in which venous blood is redirected into the lungs and oxygenated blood is pumped into the systemic circulation, supported by a single ventricle [i.e., the morphological right ventricle (RV)] (Feinstein et al, 2012)

  • We evaluated the feasibility of an endovascular repair considered by the pediatric cardiology team at the University of Michigan C.S

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Summary

Introduction

A 2-year-old female with hypoplastic left heart syndrome (HLHS)-variant, a complex congenital heart defect (CHD) characterized by the underdevelopment of the left ventricle, presented with complications following single ventricle palliation. We performed a computational fluid dynamics (CFD) analysis informed by data on anatomy, flow, and pressure to investigate the hemodynamic effect of the endovascular Fontan revision. Hypoplastic left heart syndrome (HLHS), is a complex single ventricle congenital heart defect (CHD), characterized by the underdevelopment of the left heart, including left atrium, mitral valve, left ventricle, and aorta (Noonan and Nadas, 1958). A stage 2 superior cavopulmonary connection, which, depending on patient anatomy and/or institutional preference can either be a bi-directional Glenn or Hemi-Fontan procedure (Figure 1B), is performed at 4–6 months of age. At our institution the preferred surgical approach for stage 3 Fontan completion in over 90% of cases consists of an intra-atrial lateral tunnel Fontan procedure (Douglas et al, 1999; Hirsch et al, 2008) (Figure 1C)

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