scholarly journals Deficient Myocardial Organization and Pathological Fibrosis in Fetal Aortic Stenosis—Association of Prenatal Ultrasound with Postmortem Histology

2021 ◽  
Vol 8 (10) ◽  
pp. 121
Author(s):  
Fleur Zwanenburg ◽  
Marco C. DeRuiter ◽  
Lambertus J. Wisse ◽  
Conny J. van Munsteren ◽  
Margot M. Bartelings ◽  
...  

In fetal aortic stenosis (AS), it remains challenging to predict left ventricular development over the course of pregnancy. Myocardial organization, differentiation and fibrosis could be potential biomarkers relevant for biventricular outcome. We present four cases of fetal AS with varying degrees of severity and associate myocardial deformation on fetal ultrasound with postmortem histopathological characteristics. During routine fetal echocardiography, speckle tracking recordings of the cardiac four-chamber view were performed to assess myocardial strain as parameter for myocardial deformation. After pregnancy termination, postmortem cardiac specimens were examined using immunohistochemical labeling (IHC) of key markers for myocardial organization, differentiation and fibrosis and compared to normal fetal hearts. Two cases with critical AS presented extremely decreased left ventricular (LV) strain on fetal ultrasound. IHC showed overt endocardial fibro-elastosis, which correlated with pathological fibrosis patterns in the myocardium and extremely disturbed cardiomyocyte organization. The LV in severe AS showed mildly reduced myocardial strain and less severe disorganization of the cardiomyocytes. In conclusion, the degree of reduction in myocardial deformation corresponded with high extent to the amount of pathological fibrosis patterns and cardiomyocyte disorganization. Myocardial deformation on fetal ultrasound seems to hold promise as a potential biomarker for left ventricular structural damage in AS.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yumin Li ◽  
Jia Liu ◽  
Yukun Cao ◽  
Xiaoyu Han ◽  
Guozhu Shao ◽  
...  

AbstractMyocardial fibrosis assessed by late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) is associated with cardiovascular outcomes in hypertrophic cardiomyopathy (HCM) patients, but little is known about the utility of non-invasive markers for detecting LGE. This study aims to explore the association between cardiac-specific biomarkers, CMR myocardial strain, left ventricular (LV) hypertrophy and LGE in HCM patients with preserved ejection fraction (EF) and investigate the predictive values of these indexes for LGE. We recruited 33 healthy volunteers and 86 HCM patients with preserved EF to undergo contrast-enhanced CMR examinations. In total, 48 of 86 HCM patients had the presence of LGE. The LGE-positive patients had significant higher serum high-sensitivity cardiac troponin I (hs-cTnI) and N-terminal pro b-type natriuretic peptide (Nt-proBNP) levels and lower global longitudinal (GLS) and circumferential (GCS) strains than the LGE-negative group. The LGE% was independently associated with the Nt-proBNP levels, GCS, LV end-diastolic maximum wall thickness (MWT) and beta-blocker treatment. In the receiver operating characteristic curve analysis, the combined parameters of Nt-proBNP ≥ 108.00 pg/mL and MWT ≥ 17.30 mm had good diagnostic performance for LGE, with a specificity of 81.25% and sensitivity of 70.00%. These data indicate that serum Nt-proBNP is a potential biomarker associated with LGE% and, combined with MWT, were useful for identifying myocardial fibrosis in HCM patients with preserved EF. Additionally, LV GCS may be a more sensitive indicator for reflecting the presence of myocardial fibrosis than GLS.


2020 ◽  
Author(s):  
Yumin Li ◽  
Jia Liu ◽  
Yukun Cao ◽  
Xiaoyu Han ◽  
Guozhu Shao ◽  
...  

Abstract Myocardial fibrosis assessed by late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) is associated with cardiovascular outcomes in hypertrophic cardiomyopathy (HCM) patients, but little is known about the utility of non-invasive markers for detecting LGE. This study aims to explore the association between cardiacspecific biomarkers, CMR myocardial strain, left ventricular (LV) hypertrophy and LGE in HCM patients with preserved ejection fraction (EF) and investigate the predictive values of these indexes for LGE. We recruited 33 healthy volunteers and 86 HCM patients with preserved EF to undergo contrast-enhanced CMR examinations. In total, 48 of 86 HCM patients had the presence of LGE. Serum high-sensitivity cardiac troponin I (hs-cTnI) and N-terminal pro b-type natriuretic peptide (Nt-proBNP) levels were elevated in LGE-positive patients compared with LGE-negative patients. The LGE-positive patients had lower global longitudinal (GLS) and circumferential (GCS) strains than the LGE-negative group and the healthy controls. The LGE% was independently associated with the Nt-proBNP levels, GCS, MWT and beta-blocker treatment. In the receiver operating characteristic curve analysis, the combined parameters of Nt-proBNP≥108 pg/mL and MWT≥17.3 mm had good diagnostic performance for LGE, with a specificity of 81.3% and sensitivity of 70.0%. These data indicate that serum Nt-proBNP is a potential biomarker associated with LGE% and, combined with MWT, were useful for identifying myocardial fibrosis in HCM patients with preserved EF. Additionally, LV GCS may be a more sensitive indicator for reflecting the presence of myocardial fibrosis than GLS.


2006 ◽  
Vol 16 (S1) ◽  
pp. 43-46 ◽  
Author(s):  
Elsa Suh ◽  
James Quintessenza ◽  
James Huhta ◽  
Ruben Quintero

Various physiologic mechanisms have been proposed to account for the development of hypoplasia of the left heart. The mechanism thus far most widely accepted suggests that the entity starts as severe or critical aortic stenosis during fetal gestation. Obstruction at the level of the abnormal aortic valve is then held to increase left ventricular afterload, resulting in decreased systolic and diastolic function. Shunting across the patent oval foramen is then reversed, so that blood flows from left to right. This reversal of flow during fetal gestation decreases the volume of blood crossing the mitral valve, thus decreasing the further potential for growth of the left ventricle.1 Additional support for this postulated physiologic mechanism was provided with the advent of fetal echocardiography during the 1980s.2–4 It was the group of Allan, working at Guy's Hospital in London, which first documented the fetal development of hypoplasia of the left heart by serial echocardiographic observation.4 In their retrospective study of 7000 pregnancies, 462 fetuses were diagnosed to have a structural cardiac defect at the time of the initial echocardiogram. Among those, 28 patients had dilated and dysfunctional left ventricles and aortic valves. The majority of these patients were also found to have concomitant endocardial fibroelastosis. Out of 15 patients in the series who were followed with serial echocardiograms, five progressed to develop hypoplasia of the left heart. With echocardiographic technology undergoing refinement over the same period, it was during this era that the first fetal cardiac intervention was performed using echocardiographic guidance.2,5,6 With still further technologic advances, fetal diagnosis of hypoplasia of the left heart can now be made as early as 13 weeks gestational age.7 One entity which is frequently associated with the hypoplastic left ventricle and aortic stenosis is endocardial fibroelastosis. There is an overlap of pathology between these three entities.8–10 In this report, we describe our own experience in intervention in a fetus suspected of developing hypoplasia of the left heart.


2021 ◽  
Vol 5 (2) ◽  
pp. 244-253
Author(s):  
Prof. Dr. dr. Hj. Yusrawati SpOG(K)-KFM ◽  
Nanda Tri Wahdini ◽  
Hauda El Rasyid ◽  
Muhammad Riendra

Objective : To report the diagnosis and management of congenital total AV block on VSD and PDA in pregnancy.Method : A case reportCase : A 37-year-old multiparous woman G4P3A0H2 24- 25 weeks of preterm pregnancy with fetal bradycardia, VSD, PDA with ultrasonography and CTG results was FHR 70 bpm. At 37- 38 weeks of pregnancy, termination of pregnancy was performed by cesarean delivery with preparation for complication of fetal AV block. A male baby was born with weight 2600 gram and APGAR score of 8/9. Immediate echocardiography result was situs solitus, VSD PM LR shunt, PDA LR shunt, good left ventricular function, left aortic arch and EF 74%. ECG result was sinus bradycardia, total AV block with junctional escape rhythm. Sternotomy and PPM implantation was performed by cardiothoracic surgeon three hours after the baby was born. Post PPM implantation, ECG results was HR 165 bpm and chest X- rays interpretation was cardiomegaly with plethora. Mother and baby came home in good condition on the 6th day of treatment. On the next baby’s control at 4.5 months obtained a weight of 5.4 kg with the echocardiography results was solitus, VSD PM LàR shunt, VSD muscular multiple 3 pieces LàR shunt, PDA LàR shunt, good right and left ventricular function, and left arch. The child got captopril 2x1.5 mg and planned for a 6-month repeat echocardiography.Conclusion : Congenital of total AV block on VSD and PDA is confirmed by prenatal diagnosis and preparation for comprehensive multidisciplinary management.Keywords: congenital total AV block, fetal bradycardia, fetal echocardiography, PPM, ultrasound


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Alfarih ◽  
C Leu ◽  
J Moon ◽  
A Hughes ◽  
P Nihoyannopoulos ◽  
...  

Abstract Introduction Aortic stenosis (AS) is the most prevalent form of acquired valvular heart disease, it affects ∼2% of people aged over 75. Series of compensatory mechanisms occur, in order for LV to adapt to high pressure overload. Aortic valve replacement has been the mainstay AS treatment either surgically or percutaneously. The evaluation of myocardial strains after Transcatheter Aortic Valve Implantation (TAVI) and Surgical Aortic Valve Replacement (SAVR) is still underexplored and there is no single study to date scouting the difference between TAVI and SAVR. Aim To assess the impact of unloading LV after TAVI and SAVR on LV remodelling. Methods In this prospective study, we have recruited 111 patients (75±11 years, 63% were females) with varying degrees of aortic stenosis. Of the 111 patients, 43 patients and 11 patients underwent TAVI and SAVR respectively between November 2017 and May 2018. Demographics, clinical and echocardiographic measurements along with speckle tracking parameters were recorded for all participants and again 4±2 weeks after intervention. Results Pre-TAVI LV-GLS mean was −10.8±3.5% and after implantation of aortic prosthesis immediate improvement of the myocardial deformation to −13.98±2.9% was observed after one month of the intervention, mean difference of −3.16% following procedure. There was an evidence of significant improvement in LV-GRS after TAVI (44.86±12.9% to 49.77±10.8%, P value= 0.047). Per contra, when comparing pre and post TAVI LV-GCS, no statistical evidence was noted. However, a difference of −2.4% in GCS following the intervention might be clinically important, but no previous evidence can support this. This is attributed to the poor reproducibility and yet not available standardisation. Table 1 Variables TAVI (n=43) SAVR (n=11) P value† Pre Post P* value Pre Post P* value GLS (%) −10.82±3.5 −13.98±2.9 <0.001 −12.75±4.3 −16.1±2 0.021 0.152 GCS (%) −30.1±8.1 −32.49±9.2 0.134 −27±9.8 −33.9±4.69 0.063 0.062 GRS (%) 44.86±12.9 49.77±10.8 0.047 36.6±13.3 44.97±4.9 0.074 0.058 Data are expressed as mean ± SD. Comparisons were performed using paired Student's t tests. *Pre and post intervention. †Post TAVI vs. post SAVR. Comparison done using unpaired t test of the differences. Conclusion Significant improvement was evident in myocardial deformation parameters – in particular GLS – after weeks of the intervention demonstrating a strong evidence of reversed remodelling following SAVR and TAVI.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
G Faganello ◽  
L Pagura ◽  
E Croatto ◽  
D Collia ◽  
S Furlotti ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction. Aortic stenosis (AS) is one of the most common valvular heart diseases; however, the association between left ventricular (LV) myocardial deformation and hemodynamic forces (HDFs) is still mostly  unexplored. Purpose. This study aimed to assess the differences in LV myocardial deformation and HDFs in a large cohort of patients with aortic stenosis retrospectively. Methods. Two-hundred fifty-four subjects (median age 77 years, 50% women) with preserved LV ejection fraction (LVEF), and mild (n = 87), moderate (n = 92) or severe (n = 75) AS, were included in the study. The 2D LV global longitudinal strain (GLS), circumferential strain (GCS), and HDFs were measured with new software that allowed us to calculate all these values and parameters from the three apical views. Results. When comparing severe AS to mild AS, LV mass appeared increased while the LV hypertrophy phenotype was concentric (p &lt;0.0001). Along with the progression of the AS, LVEF was decreased. All GLS, GCS, and HDFs parameters were uniformly reduced in severe AS compared to mild AS (p &lt;0.0001), in the same way, LV longitudinal force, LV longitudinal systolic force, and LV impulse have proven to be accurate on ROC curves (AUC 70%, 73% and 73% respectively). Conclusion. The integrated approach of deformation and cardiac mechanics allows the description of pathophysiological changes during the progression of mild to severe aortic stenosis. Abstract Figure. Strain parameters and aortic stenosis


2019 ◽  
Vol 18 (1) ◽  
Author(s):  
Preneet Cheema Brar ◽  
Anne Chun ◽  
Xiazhou Fan ◽  
Vivek Jani ◽  
Mary Craft ◽  
...  

Abstract Background It is unknown that dysglycemia in obese adolescents has effects on myocardial deformation that are more pronounced when compared to obesity alone. We hypothesized that obesity associated abnormal glucose tolerance (dysglycemia) would have adverse effects on two-dimensional speckle tracking echocardiography derived longitudinal, radial and circumferential strain (LS, RS, CS) compared to age and gender lean controls. We also examined if changes in deformation would be reflected in abnormal ventricular vascular coupling indices (VVI). Methods In a prospective cross-sectional design 39 obese adolescents (15.9 ± 1.7 years; 101.5 ± 39 kg; female − 58%) were compared to age and gender matched lean controls (15.7 ± 1.8 yrs, 60 ± 12.8 kg). Based on results from an oral glucose tolerance test (OGTT), obese adolescents were categorized as obese normoglycemic (ONG, n = 25) or obese dysglycemic (ODG, n = 14). Left ventricular (LV) global and average LS, CS, RS and strain rate were measured. LV ejection fraction and mass index were measured and VVI approximated as ratio of arterial elasticity (Ea) and end-systolic elastance (Ees). Results Adolescents with ODG had significantly (P = 0.005) impaired global LS (− 20.98% ± 2.8%) compared to controls (− 23.01% ± 2.3%). A similar (P = 0.0027) reduction was observed in average LS for adolescents with ODG (18.87% ± 2.5%) compared to controls (20.49% ± 2%). Global CS was also decreased (P = 0.03) in ODG (− 23.95%) compared to ONG (− 25.80). A similar trend was observed in average CS after multivariate regression for BMI and blood pressure. CS correlated with HbA1c in both groups (P = 0.05). VVI had a negative correlation with both LS (r = − 0.4, P = 0.025) and CS rate (r = − 0.36, P = 0.04). Conclusions Myocardial strain and strain rate were significantly altered in obese adolescents. Unfavorable subclinical reductions in global and average CS were more pronounced in adolescents with dysglycemia compared to obese adolescents with normoglycemia and controls. These data indicate progressive worsening of subendocardial function across the spectrum of glucose tolerance. Strain rate was predictive of VVI in obese adolescents, suggesting strain rate may be a sensitive marker for cardiac remodeling in abnormal glucose homeostasis states.


2020 ◽  
Vol 320 ◽  
pp. 133-138
Author(s):  
S. Chandra-Bose Reddy ◽  
Jin Zhang ◽  
Vivek Jani ◽  
Steven B. Wolfe ◽  
David Danford ◽  
...  

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