Marc Delaney1, Vincent Cleveland2, Paige Mass2, Francesco Capuano3, Yue-Hin Loke4, and Laura Olivieri4
1Pediatrics, Children's National Hospital, Washington, DC, United States, 2Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Hospital, Washington, DC, United States, 3Industrial Engineering, Universita di Napoli Federico II, Naples, Italy, 4Pediatric Cardiology, Children's National Hospital, Washington, DC, United States
Synopsis
Repair of D- transposition of great arteries (DTGA) involves pulmonary
artery (PA) manipulation that alters shape and flow patterns. Many patients
experience increased right ventricular afterload and the etiology remains
unclear. We examined the contribution of PA flow separation to afterload in
these patients using 4D flow CMR imaging of a mock circulatory system
incorporating 3D-printed PA replicas. We found that 2 distinct markers of flow
inefficiency correlated with afterload. These data emphasize the utility of 4D
flow CMR in quantifying abnormal blood flow and identifying important early clinical predictors
of complications in DTGA.
Introduction:
Surgical treatment of
D- transposition of great arteries (DTGA) involves the Arterial Switch
Operation (ASO) and the LeCompte maneuver, where the pulmonary artery (PA) is
translocated anterior to the neoaortic root, exaggerating branch pulmonary
artery bending and stretching. In many
patients, right ventricular dysfunction and increased right RV afterload is
observed following ASO1,2. While pulmonary stenosis is a common
postoperative complication, flow separation, flow inefficiencies, and energy
loss created by this altered PA geometry may contribute to elevated RV afterload
in a manner independent of stenosis. Our aim was to understand the correlation
between RV afterload and flow characteristics produced by the ASO using 4D flow
imaging of a mock circulatory system incorporating 3D printed replicas of ASO
patients. Methods:
CMR imaging was
analyzed from 16 patients with DTGA (average age 9.2 (+/-8.8) years, 69% male)
with (31%) and without (69%) ventricular septal defect, who underwent ASO with
LeCompte maneuver. Patients with pulmonary artery
(PA) stents, pulmonary hypertension, or other anatomical confounders were
excluded. Segmentation was performed on contrast-enhanced angiograms to create PA
models that were 3D printed and mounted in an MRI-compatible mock circulatory
system (MCS) with simulated PA resistance, compliance, and patient-specific pulsatile
flow generated from an MRI-compatible flow pump. Patient-specific flow and
resistance was calibrated in the circuit using iterative design and patients
were excluded if PA flow curve kinetics could not be accurately reproduced
(i.e. delayed systolic flow to branch PAs, n=3). Pressure transducers were utilized
during flow simulations to gather peak systolic pressure change from circuit
inlet to branch PA as an analog of RV afterload (ΔP). 4D Flow CMR (1.5T Siemens
Aera) sequences of each simulation was obtained and flow patterns in systole were
quantified using ITFlow© software (Cardio Flow Design, Japan). Results:
In post-ASO DTGA patients,
analysis 4D flow data in MCS simulation reveals ΔP is significantly correlated
to maximum systolic energy loss (mW/m3) (p = 0.021, r = 0.57) and maximum
systolic PA wall shear stress (Pa) (p <0.001, r = 0.85). Discussion:
4D flow CMR allows
the observation and quantification of flow inefficiencies in the post-ASO PA. Quantitative
markers of flow inefficiency of peak energy loss and wall shear stress correlate
strongly with simulated RV afterload in MCS simulations of post-ASO anatomy.Conclusion:
4D flow is a powerful
tool to provide deeper understanding of flow field inefficiencies and
subsequent energy loss in repaired DTGA. Inefficient PA flow patterns as
quantified by 4D flow CMR are likely mechanistically related to RV afterload in
this population and, with further research, may prove to be a clinically useful
predictive factor for the management of complications in this growing patient
population.Acknowledgements
This project was supported by
Award Number R38AI140298 from the NIH National Center of Allergy and Infectious Diseases. Its contents are
solely the responsibility of the authors and do not necessarily represent the official views of the National
Center of Allergy and Infectious Diseases or the National Institutes of Health.References
1. Loke
Y-H, Capuano F, Mandell J et al. Abnormal Pulmonary Artery Bending Correlates
With Increased Right Ventricular Afterload Following the Arterial Switch
Operation. World Journal for Pediatric and Congenital Heart Surgery
2019;10:572-581.
2. Klitsie LM, Roest AA, Kuipers IM,
Hazekamp MG, Blom NA, Ten Harkel AD. Left and right ventricular performance
after arterial switch operation. The Journal of thoracic and cardiovascular
surgery 2014;147:1561-1567.