Patient-specific modeling of right coronary circulation vulnerability post-liver transplant in Alagille's syndrome

PLoS One. 2018 Nov 8;13(11):e0205829. doi: 10.1371/journal.pone.0205829. eCollection 2018.

Abstract

Objectives: Cardiac output (CO) response to dobutamine can identify Alagille's syndrome (ALGS) patients at higher risk of cardiovascular complications during liver transplantation. We propose a novel patient-specific computational methodology to estimate the coronary autoregulatory responses during different hemodynamic conditions, including those experienced in a post-reperfusion syndrome (PRS), to aid cardiac risk-assessment.

Material and methods: Data (pressure, flow, strain and ventricular volumes) from a 6-year-old ALGS patient undergoing catheter/dobutamine stress MRI (DSMRI) were used to parameterize a closed-loop coupled-multidomain (3D-0D) approach consisting of image-derived vascular models of pulmonary and systemic circulations and a series of 0D-lumped parameter networks (LPN) of the heart chambers and the distal arterial and venous circulations. A coronary microcirculation control model (CMCM) was designed to adjust the coronary resistance to match coronary blood flow (and thus oxygen delivery) with MVO2 requirements during Rest, Stress and a virtual PRS condition.

Results: In all three simulated conditions, diastolic dominated right coronary artery (RCA) flow was observed, due to high right ventricle (RV) afterload. Despite a measured 45% increase in CO, impaired coronary flow reserve (CFR) (~1.4) at Stress was estimated by the CMCM. During modeled PRS, a marked vasodilatory response was insufficient to match RV myocardial oxygen requirements. Such exhaustion of the RCA autoregulatory response was not anticipated by the DSMRI study.

Conclusion: Impaired CFR undetected by DSMRI resulted in predicted myocardial ischemia in a computational model of PRS. This computational framework may identify ALGS patients at higher risk of complications during liver transplantation due to impaired coronary microvascular responses.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alagille Syndrome / diagnosis
  • Alagille Syndrome / diagnostic imaging
  • Alagille Syndrome / physiopathology*
  • Cardiac Output
  • Catheterization
  • Child
  • Coronary Circulation / physiology
  • Coronary Vessels / diagnostic imaging
  • Coronary Vessels / physiopathology
  • Echocardiography, Stress
  • Heart / diagnostic imaging
  • Heart / physiopathology*
  • Humans
  • Liver Transplantation / adverse effects
  • Male
  • Models, Cardiovascular
  • Myocardial Ischemia / diagnosis
  • Myocardial Ischemia / diagnostic imaging
  • Myocardial Ischemia / physiopathology*
  • Myocardial Reperfusion / adverse effects
  • Patient-Specific Modeling*
  • Regional Blood Flow / physiology