Right ventricle in hypoplastic left heart syndrome exhibits altered hemodynamics in the human fetus

Zebhi, Banafsheh; Wiputra, Hadi; Howley, Lisa; Cuneo, Bettina F.; Park, Dawn; Hoffman, Hilary A.; Gilbert, Lisa; Yap, Choon Hwai; Bark, David

doi:10.1016/j.jbiomech.2020.110035

Cited by 8 publications

(6 citation statements)

References 30 publications

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“…Another computational model based on patient-specific RV anatomies reconstructed from magnetic resonance imaging revealed the strong dependence of interventricular pressure gradients, filling dynamics and capacity on RV shape and temporal deformation patterns ( 76 ). To date, the only quantification of the native pre-surgical HLHS RV hemodynamics was performed numerically in a fetal heart in the context of cardiac development ( 77 ). This investigation, which assessed fetal blood flow using 4D spatiotemporal image correlation ultrasound and numerical modeling, revealed that despite a larger right-ventricular cavity size and a greater cardiac output, HLHS fetal hearts generated essentially the same global interventricular hemodynamics as a normal heart.…”

Section: Hlhs Outcomesmentioning

confidence: 99%

Current state of the art in hypoplastic left heart syndrome

Birla

Brimmer²,

Short³

et al. 2022

Front. Cardiovasc. Med.

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Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.

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Section: Hlhs Outcomesmentioning

confidence: 99%

Current state of the art in hypoplastic left heart syndrome

Birla

Brimmer²,

Short³

et al. 2022

Front. Cardiovasc. Med.

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show abstract

“…The blood flow in HLHS hearts is disturbed as a consequence of the developmental disorders. As a result, the heart’s shear stress distribution and hemodynamics are seriously altered compared to the healthy fetal hearts ( Zebhi et al, 2020 ).…”

Section: Introductionmentioning

confidence: 99%

“…On the other hand, only few research studies have been published in the literature that employ CFD modeling to elucidate the flow patterns in human embryonic hearts ( DeGroff et al, 2003 ; Pennati et al, 2008 ; Saw et al, 2017 ; Wiputra et al, 2018 ). In several computational modeling studies, the volumetric contractions of the left ( Lai et al, 2016 ) and right ventricles ( Wiputra et al, 2016 ; Zebhi et al, 2020 ) are analyzed to determine the flow characteristics in human fetal hearts. Furthermore, hemodynamic evaluations and computational models for the human fetal hearts can provide a thorough knowledge of CHDs, particularly for demystifying the mechanobiological pathways of HLHS development.…”

Section: Introductionmentioning

confidence: 99%

Hemodynamic and Structural Comparison of Human Fetal Heart Development Between Normally Growing and Hypoplastic Left Heart Syndrome-Diagnosed Hearts

et al. 2022

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Congenital heart defects (CHDs) affect a wide range of societies with an incidence rate of 1.0–1.2%. These defects initiate at the early developmental stage and result in critical health disorders. Although genetic factors play a role in the formation of CHDs, the occurrence of cases in families with no history of CHDs suggests that mechanobiological forces may also play a role in the initiation and progression of CHDs. Hypoplastic left heart syndrome (HLHS) is a critical CHD, which is responsible for 25–40% of all prenatal cardiac deaths. The comparison of healthy and HLHS hearts helps in understanding the main hemodynamic differences related to HLHS. Echocardiography is the most common imaging modality utilized for fetal cardiac assessment. In this study, we utilized echocardiographic images to compare healthy and HLHS human fetal hearts for determining the differences in terms of heart chamber dimensions, valvular flow rates, and hemodynamics. The cross-sectional areas of chamber dimensions are determined from 2D b-mode ultrasound images. Valvular flow rates are measured via Doppler echocardiography, and hemodynamic quantifications are performed with the use of computational fluid dynamics (CFD) simulations. The obtained results indicate that cross-sectional areas of the left and right sides of the heart are similar for healthy fetuses during gestational development. The left side of HLHS heart is underdeveloped, and as a result, the hemodynamic parameters such as flow velocity, pressure, and wall shear stress (WSS) are significantly altered compared to those of healthy hearts.

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“…It is essential to assess prenatal RV function in HLHS fetuses. Although, prenatally, the fetuses compensate by increasing output from the RV that should be stroked from the LV [ 4 , 5 , 6 ], the existing studies found abnormal fetal RV function using the SDI index and tissue Doppler technology [ 7 , 8 ]. Furthermore, normal right ventricular contraction is determined by the integrity of the free wall and interventricular septum [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Right Ventricular Function and Myocardial Microstructure in Fetal Hypoplastic Left Heart Syndrome

Yuan

Zhang

et al. 2022

JCM

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Right ventricular (RV) function is one of the critical factors affecting the prognosis of fetuses with hypoplastic left heart syndrome (HLHS). Our study objectives included assessment of cardiac function and comprehensive measurement of cardiac microstructure. We retrospectively studied 42 fetuses diagnosed as HLHS by echocardiography. Myocardial deformation of the right ventricular wall was calculated automatically in offline software. Postmortem cardiac imaging for three control fetal hearts and four HLHS specimens was performed by a 9.4T DTI scanner. Myocardial deformation parameters of the RV (including strain, strain rate, and velocity) were significantly lower in HLHS fetuses (all p < 0.01). FA values increased (0.18 ± 0.01 vs. 0.21 ± 0.02; p < 0.01) in HLHS fetuses, but MD reduced (1.3 ± 0.15 vs. 0.88 ± 0.13; p < 0.001). The HLHS fetuses’ RV lateral base wall (−7.31 ± 51.91 vs. −6.85 ± 31.34; p = 0.25), middle wall (1.71 ± 50.92 vs. −9.38 ± 28.18; p < 0.001), and apical wall (−6.19 ± 46.61 vs. −11.16 ± 29.86, p < 0.001) had HA gradient ascent but HA gradient descent in the anteroseptal wall (p < 0.001) and inferoseptal wall (p < 0.001). RV basal lateral wall HA degrees were correlated with RVGLS (R2 = 0.97, p = 0.02). MD values were positively correlated with RVGLS (R2 = 0.93, p = 0.04). Our study found morphological and functional changes of the RV in HLHS fetuses, and cardiac function was related to the orientation patterns of myocardial fibers. It may provide insight into understanding the underlying mechanisms of impaired RV performance in HLHS.

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Right ventricle in hypoplastic left heart syndrome exhibits altered hemodynamics in the human fetus

Cited by 8 publications

References 30 publications

Current state of the art in hypoplastic left heart syndrome

Current state of the art in hypoplastic left heart syndrome

Hemodynamic and Structural Comparison of Human Fetal Heart Development Between Normally Growing and Hypoplastic Left Heart Syndrome-Diagnosed Hearts

Evaluation of Right Ventricular Function and Myocardial Microstructure in Fetal Hypoplastic Left Heart Syndrome

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