P6093D echocardiography versus cardiovascular magnetic resonance in the evaluation of the right ventricle in patients with congenital heart disease after pulmonary valve replacement

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
F R Pluchinotta ◽  
M Panebianco ◽  
L Piazza ◽  
P Tarzia ◽  
L Fusini ◽  
...  

Abstract Background Cardiac MR (CMR) is the gold standard for right ventricular (RV) quantification. Three-dimensional echo (3DE) is a relatively new technique which may offer a rapid alternative for the examination of the right heart. The purpose of this study was to investigate the clinical significance and interchangeability of these modalities to evaluate patients with congenital heart disease (CHD) who underwent percutaneous pulmonary valve implantation (PPVI) for RV outflow tract dysfunction. Methods 36 patients who underwent PPVI were evaluated with 3DE and CMR to quantify the RV. RV volumes and ejection fraction (EF) were measured for both imaging techniques with commercially available softwares (Tomtec-Germany for 3DE and Medimatic-Netherlands for CMR data). Paired t-test, Bland-Altman analysis, and Pearson's correlation analysis were used as most appropriate to compare both measured techniques with CMR regarded as the reference standard. Results 86% of the patients (31 patients) had adequate image quality on 3DE and was included in the study. Patients underwent both 3D echo and CMR within a mean of 9 days of each other and at a mean time of 3 years after PPVI. Compared to CMR, 3D echo significantly underestimated volumes in all patients and overestimate RV ejection fraction (EF). Mean RV End-diastolic Volumes (EDV) and End-Systolic Volumes (ESV) were significantly greater when measured by CMR compared to 3D echo (EDV: 99 ml/m2 vs. 85 ml/m2; p<0.01, ESV: 52 ml/m2 vs. 41 ml/m2; p<0.01). Mean RV EF was lower when measured by CMR compared to 3D echo (48% vs 52%; p<0.05). Linear regression analysis showed high correlation coefficients between 3DE and CMR (r=0,68 for EDV, r=0,62 for ESV, and r=0,57 for EF; p<0.001). Bland-Altman analysis demonstrated that for both RV EDV and RV ESV there was a significant and systematic under-estimation of volume by 3D echo compared to CMR. Both 3DE and CMR measurements were found to be highly reproducible in terms of intra-observer variability. Conclusions Statistically significant and clinically meaningful differences in volumetric measurements were observed between 3DE and CMR in the evaluation of RV volumes and function in patients with CHD after PPVI. Despite linear regression and Bland-Altman analysis showed that the two techniques are related and present some degree of agreement, 3D Echocardiography systematically underestimates volumes and overestimates EF and this would have to be considered in the clinical practice.

2014 ◽  
Vol 25 (4) ◽  
pp. 794-796 ◽  
Author(s):  
Zahra Khajali ◽  
Ali Mohammadzadeh ◽  
Marzieh Khayatzadeh

AbstractWe present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Nadya Al-Wakeel-Marquard ◽  
Tiago Ferreira da Silva ◽  
Sarah Jeuthe ◽  
Sanaz Rastin ◽  
Frédéric Muench ◽  
...  

AbstractThe right ventricle´s (RV) characteristics—thin walls and trabeculation—make it challenging to evaluate extracellular volume (ECV). We aimed to assess the feasibility of RV ECV measurements in congenital heart disease (CHD), and to introduce a novel ECV analysis tool. Patients (n = 39) and healthy controls (n = 17) underwent cardiovascular magnetic resonance T1 mapping in midventricular short axis (SAX) and transverse orientation (TRANS). Regions of interest (ROIs) were evaluated with regard to image quality and maximum RV wall thickness per ROI in pixels. ECV from plane ROIs was compared with values obtained with a custom-made tool that derives the mean T1 values from a “line of interest” (LOI) centered in the RV wall. In CHD, average image quality was good (no artifacts in the RV, good contrast between blood/myocardium), and RV wall thickness was 1–2 pixels. RV ECV was not quantifiable in 4/39 patients due to insufficient contrast or wall thickness < 1 pixel. RV myocardium tended to be more clearly delineated in SAX than TRANS. ECV from ROIs and corresponding LOIs correlated strongly in both directions (SAX/TRANS: r = 0.97/0.87, p < 0.001, respectively). In conclusion, RV ECV can be assessed if image quality allows sufficient distinction between myocardium and blood, and RV wall thickness per ROI is ≥ 1 pixel. T1 maps in SAX are recommended for RV ECV analysis. LOI application simplifies RV ECV measurements.


2006 ◽  
Vol 29 ◽  
pp. S279-S285 ◽  
Author(s):  
Alessandro Frigiola ◽  
Alessandro Giamberti ◽  
Massimo Chessa ◽  
Marisa Di Donato ◽  
Raul Abella ◽  
...  

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ismee A Williams ◽  
Howard Andrews ◽  
Michael M Myers ◽  
William Fifer

Objectives: Children with congenital heart disease (CHD) are at risk for abnormal neurodevelopment (ND). We evaluated associations between fetal Doppler and biometry measures, neonatal electroencephalogram (EEG) and 18-month ND. Methods: Fetuses with hypoplastic left heart syndrome (HLHS), transposition of the great arteries (TGA), and tetralogy of Fallot (TOF) had middle cerebral (MCA) and umbilical artery (UA) Doppler velocities, as well as biometry such as head (HC) and abdominal circumference (AC), prospectively recorded at 20-25 (F1), 26-32 (F2), and 33-39 (F3) wks gestational age (GA). Pulsatility indices (PI) with GA-derived z-scores and cerebral-to-placental resistance (CPR) ratios were calculated. Neonatal high-density EEG was preformed preoperatively and the Bayley Scales of Infant Development-III were assessed at 18-months. Factor analysis was used to reduce the number of EEG predictors used in regression analysis. Results: Among 56 CHD fetuses (N=19 HLHS, N=16 TGA, N=21 TOF) who underwent preoperative EEG, ND scores are available for 33 to date. Cardiac subtype was highly associated with EEG and was considered in all models. Cognition scores were predicted by CPR< 1 ever (B=-15.7, P=0.002) and HC/AC at F2 (B=-130, P=0.013, R 2 =0.42). Language scores were predicted by UA PI z-score at F1 (B=-9.6, P=0.005, R 2 =0.27). Motor scores were predicted by UA PI z-score at F1 (B=-3.9, P=0.085), HLHS (B=-15, P<0.001), EFW%ile (B=0.374, P=0.007), and delta band right parietal and right temporal log power in active sleep (B=3.9, P=0.045, R 2 =0.61). Conclusion: Lower umbilical artery pulsatility at 20-25 wks GA was associated with higher 18-month Language and Motor scores. A diagnosis of HLHS predicted poorer Motor scores. Increased EEG power in the parietal and temporal region of the right brain predicted higher Motor scores. A larger abdomen relative to head at 26-32 wks was associated with improved cognition while diminished cerebrovascular compared with placental resistance predicted poorer cognition, similar to what has been observed in the growth restricted fetus. Further investigation is needed to confirm these hypothesis-generating findings.


PEDIATRICS ◽  
1983 ◽  
Vol 71 (1) ◽  
pp. 144-145
Author(s):  
KARL-GEORG EVERS ◽  
PETER GRONECK

To the Editor.— Congenital asymmetric crying facies is generally considered to be due to unilateral agenesis or hypoplasia of the anguli oris depressor muscle (HAODM). Electromyographic (EMG) examinations of the affected sides have revealed absent spontaneous activity or diminished motor unit activity.1-3 Association of HAODM syndrome with congenital heart disease, the "cardiofacial syndrome," has been described.4 Major defects of other organ systems and minor congenital defects may be associated with asymmetric crying facies as well.5 Monreal6 reported five patients with asymmetric congenital crying facies syndroms who besides this anomaly displayed juxtaoral defects, egm atresia of one side of the jaw and soft palate, cleft lip, hypoplasis of mandible and ear.


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