Postmortem Assessment of Isolated Congenital Heart Defects Remains Essential Following Termination of Pregnancy.

OBJECTIVES: To investigate the correlation between prenatal ultrasound (US) and autopsy findings in pregnancies terminated due to isolated congenital heart defects (CHDs), including CHDs associated with heterotaxy syndrome. MATERIALS AND METHODS: The material consists of 67 fetuses with prenatally detected isolated CHDs or CHDs associated with heterotaxy syndrome at a tertiary center in Norway between 1985 and 2014. The main CHDs were categorized into subdiagnoses of CHDs in accordance with ICD-10. The US and autopsy findings were categorized according to degree of concordance. RESULTS: Gestational age at termination was 12 + 0-22 + 6 weeks. Hypoplastic left heart syndrome was the most common main diagnosis among the 67 fetuses (32.8%). There was full agreement between US and autopsy findings in 97.4% (222/228) of all subdiagnoses. The discrepant findings in three fetuses had no influence on the decision to terminate the pregnancy. CONCLUSIONS: The correlation was high between prenatal US and postmortem findings in fetuses with isolated CHDs. Meticulous assessment of cardiac anatomy is particularly necessary when the decision to terminate relies on isolated CHDs. The trend of earlier termination challenges verification of diagnoses at autopsy. Consequently, the fetus should be examined at a tertiary center with fetal medicine specialists, pediatric cardiologists and perinatal pathologists.

Quantitative Assessment of 3D Printed Model Accuracy in Delineating Congenital Heart Disease.

BACKGROUND: Three-dimensional (3D) printing is promising in medical applications, especially presurgical planning and the simulation of congenital heart disease (CHD). Thus, it is clinically important to generate highly accurate 3D-printed models in replicating cardiac anatomy and defects. The present study aimed to investigate the accuracy of the 3D-printed CHD model by comparing them with computed tomography (CT) images and standard tessellation language (STL) files. METHODS: Three models were printed, comprising different CHD pathologies, including the tetralogy of Fallot (ToF), ventricular septal defect (VSD) and double-outlet right-ventricle (DORV). The ten anatomical locations were measured in each comparison. Pearson's correlation coefficient, Bland-Altman analysis and intra-class correlation coefficient (ICC) determined the model accuracy. RESULTS: All measurements with three printed models showed a strong correlation (r = 0.99) and excellent reliability (ICC = 0.97) when compared to original CT images, CT images of the 3D-printed models, STL files and 3D-printed CHD models. CONCLUSION: This study demonstrated the high accuracy of 3D-printed heart models with excellent correlation and reliability when compared to multiple source data. Further investigation into 3D printing in CHD should focus on the clinical value and the benefits to patients.

Congenital heart disease in low-and-middle-income countries: Focus on sub-Saharan Africa.

The etiology of congenital heart disease (CHD) is multifactorial. The birth prevalence of CHD is shaped by a wide variety of maternal, fetal, and neonatal risk factors, along with the rates of prenatal diagnosis and terminations of pregnancy, all of which have geographic variability Epidemiology data availability from low-and-middle-income countries (LMIC) on CHD prevalence, morbidity, and mortality are far more limited than from high income countries. Data on specific genetic, environmental, and prenatal risk associated with CHD are almost nonexistent. In this article, we will focus on defining what data are available, genetic risk factors, birth and overall prevalence, morbidity, and the impact of limited access to interventions, both surgery and cardiac catheterizations. We will highlight CHD in sub-Saharan Africa to detail epidemiology studies in the poorest regions of the world. Existing literature as well as estimates from the Global Burden of Disease Study (http://ghdx.healthdata.org) form the basis for this review. The intersection of poverty, high fertility rates, and limited access to care results in a unique profile of CHD in LMIC. CHD is not a preventable disease (by most standards), so early detection and access are our key interventions to improve the dire outcomes for children in low-resources settings of the world.

Quantitative assessment of technical performance during hands-on surgical training of the arterial switch operation using 3-dimensional printed heart models.

OBJECTIVE: Data supporting the use of hands-on simulation in congenital heart surgery are promising but primarily qualitative. This study aimed to demonstrate if there was an objective improvement in time and technical performance of the arterial switch procedure on 3-dimensional printed heart models by surgeons using a validated assessment method. METHODS: A total of 30 surgeons of varying experience performed the arterial switch procedure twice on 3-dimensional printed models with transposition of the great arteries during the Hands-on Surgical Training courses. Surgeons' performances were recorded and retrospectively assessed for both time and performance using the Hands-on Surgical Training-Congenital Heart Surgery tool, a validated procedure-specific assessment tool for the arterial switch. RESULTS: A total of 60 videos were scored. Eighty percent of surgeons (24/30) had improved from their first attempt. The mean total score of the first attempt performance compared with the second was 103 and 120, respectively, with a mean difference in score of 17 (95% confidence interval, 10-24). All surgeons were statistically significantly quicker in their second attempt. The mean time for the first attempt compared with the second was 1 hour, 28 minutes, 4 seconds and 1 hour, 5 minutes, and 45 seconds, respectively, with a mean difference of 0 hours, 22 minutes, 19 seconds (95% confidence interval, 0 hours, 15 minutes, 22 seconds to 0 hours, 25 minutes, 34 seconds). CONCLUSIONS: This is the first study to demonstrate an objective improvement in time and technical performance of the arterial switch procedure on 3-dimensional printed heart models. This supports the evidence that simulation in the form of deliberate practice with constructive, objective feedback is fundamental in the training of future congenital heart surgeons. These simulations and assessments should be incorporated to create structured, standardized training curricula within congenital heart surgery.

Assessment of CT angiographic findings in comparison with echocardiography findings of chest among patients with aortic arch anomalies.

The objective of this study was to examine the findings of computed tomographic (CT) angiography in patients with aortic arch anomalies in comparison with transthoracic echocardiography findings who referred to a private imaging center in Tehran during 2009-2012. The cases included 203 patients with clinical symptoms or echocardiogram of congenital heart disease to assess the presence of aortic arch anomalies among patients referred to imaging center. This study is a retrospective study based on the CT angiographic findings in comparison with transthoracic echocardiography findings of chest among patients with aortic arch anomalies. In this study, 203 patients with congenital anomalies were enrolled in the study, among those, 107 patients were men and 96 were female. The most common anomaly of the aortic arch was found to be coarctation (19.7%), followed by right sided arch with mirror image branching (19.2%). Furthermore, the most common cardiac anomalies associated with aortic arch anomalies were VSD, PA and PDA. The sensitivity and specificity of transthoracic echocardiography in the diagnosis of aortic arch anomalies was 59% and 100% in comparison with CT angiography. In addition, the agreement between the two methods (kappa) in the diagnosis of aortic arch anomalies was 0.72. But, transthoracic echocardiography is the first diagnostic method for patients with congenital heart disease. In some patients, the ability of this method was limited to the detection of coronary artery anomalies and thoracic vessels. Therefore, CT is used for morphological evaluation of congenital heart disease (CHD) due to its main advantages including fast acquisition time, large anatomical coverage, high speed, and great spatial resolution. Moreover, CT is essential for proper evaluation of CHD regarding its high spatial and temporal resolution.

Prenatal echocardiographic assessment of right aortic arch.

OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern in relation to the trachea, and to establish whether the echocardiographic 'V-shaped' or 'U-shaped' appearance of the junction between the DA and aortic arch (AA) in the fetal upper mediastinal view is sufficiently accurate for assessment of fetal AA anatomy. METHODS: This was a retrospective study of pregnancies with a prenatal diagnosis of fetal RAA that had postnatal confirmation of AA anatomy, referred to our tertiary center during 2011-2017. Prenatal and postnatal medical records, including echocardiographic and computed tomography (CT)/magnetic resonance imaging (MRI) scan reports, were reviewed, and cardiac and extracardiac abnormalities and the results of genetic testing were recorded. RESULTS: Of 55 consecutive pregnancies with a prenatal diagnosis of fetal RAA, six were lost to follow-up, one was terminated and three were excluded due to lack of postnatal confirmation of AA anatomy. Of the remaining 45 pregnancies, AA anatomy was assessed postnatally by CT in 39, by MRI in one and by direct examination at cardiac surgery in five. A U-shaped appearance was found in 37/45 (82.2%) patients, all of which had a complete vascular ring (CVR). Of these 37 patients, on postnatal confirmation, 21 (56.8%) had RAA with Kommerell's diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11 (29.7%) had a double AA (DAA), four (10.8%) had RAA with Kommerell's diverticulum, LPDA and mirror-image (MI) branching (RAA/LPDA/MI), and one (2.7%) had RAA with Kommerell's diverticulum, LPDA and aberrant left innominate artery (ALIA) (RAA/LPDA/ALIA). A V-shaped appearance was found in 3/45 (6.7%) patients, all of which had RAA with right DA not forming a CVR and MI branching. In the 5/45 (11.1%) fetuses with neither U- nor V-shaped appearance, RAA with left anterior DA arising from the left innominate artery and MI branching, not forming a CVR, was found. Twelve (26.7%) fetuses had a congenital heart defect (CHD). RAA forming a CVR (U-shaped appearance) was associated with a septal defect in 6/37 (16.2%) fetuses, while RAA not forming a CVR (V-shaped appearance or no U- or V-shaped appearance) was associated with major CHD in 6/8 (75.0%) fetuses. CONCLUSIONS: In fetuses with RAA, V-shaped appearance of the junction between the DA and AA indicates only that the transverse AA and DA run together on the same side of the thorax (trachea) while a U-shaped appearance is always a sign of a CVR. Among fetuses with a CVR, RAA/LPDA/MI is more frequent than described previously. Finally, RAA forming a CVR is not usually associated with complex CHD, as opposed to RAA not forming a CVR. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Noninvasive Liver Assessment in Adult Patients With Fontan Circulation Using Acoustic Radiation Force Impulse Elastography and Hepatic Magnetic Resonance Imaging.

BACKGROUND: Patients who have undergone the Fontan procedure are at risk of developing hepatic dysfunction. However, broad recommendations regarding liver monitoring are limited. The purpose of this study was to characterize the frequency of liver disease in adult Fontan patients using multimodality imaging (hepatic magnetic resonance imaging [MRI], acoustic radiation force impulse [ARFI] elastography, or hepatic ultrasound). METHODS: In a prospective cross-sectional analysis of adult patients palliated with a Fontan procedure, hepatic MRI, ARFI, and hepatic ultrasound were used to assess for liver disease. The protocol compared (1) varying prevalence of liver disease based on each imaging technique, (2) agreement between different techniques, and (3) association between noninvasive imaging diagnosis of liver disease and clinical variables, including specific liver disease biomarkers. RESULTS: Thirty-seven patients were enrolled. The ARFI results showed high wave propagation velocity in 35 patients (94.6%). All patients had some abnormality in the hepatic MRI. Specifically, 8 patients (21.6%) showed signs of chronic liver disease, 10 patients (27%) had significant liver fibrosis, and 27 patients (73%) had congestion. No correlation was found between liver stiffness measured as propagation velocity and hepatic MRI findings. Only 7 patients had an abnormal hepatic ultrasound study. CONCLUSIONS: There is an inherent liver injury in adult Fontan patients. Signs of liver disease were observed in most patients by both hepatic MRI and ARFI elastography but not by ultrasound imaging. Increased liver stiffness did not identify specific disease patterns from MRI, supporting the need for multimodality imaging to characterize liver disease in Fontan patients.

Single centre experience of the application of self navigated 3D whole heart cardiovascular magnetic resonance for the assessment of cardiac anatomy in congenital heart disease.

BACKGROUND: For free-breathing cardiovascular magnetic resonance (CMR), the self-navigation technique recently emerged, which is expected to deliver high-quality data with a high success rate. The purpose of this study was to test the hypothesis that self-navigated 3D-CMR enables the reliable assessment of cardiovascular anatomy in patients with congenital heart disease (CHD) and to define factors that affect image quality. METHODS: CHD patients ≥2 years-old and referred for CMR for initial assessment or for a follow-up study were included to undergo a free-breathing self-navigated 3D CMR at 1.5T. Performance criteria were: correct description of cardiac segmental anatomy, overall image quality, coronary artery visibility, and reproducibility of great vessels diameter measurements. Factors associated with insufficient image quality were identified using multivariate logistic regression. RESULTS: Self-navigated CMR was performed in 105 patients (55% male, 23 ± 12y). Correct segmental description was achieved in 93% and 96% for observer 1 and 2, respectively. Diagnostic quality was obtained in 90% of examinations, and it increased to 94% if contrast-enhanced. Left anterior descending, circumflex, and right coronary arteries were visualized in 93%, 87% and 98%, respectively. Younger age, higher heart rate, lower ejection fraction, and lack of contrast medium were independently associated with reduced image quality. However, a similar rate of diagnostic image quality was obtained in children and adults. CONCLUSION: In patients with CHD, self-navigated free-breathing CMR provides high-resolution 3D visualization of the heart and great vessels with excellent robustness.

Hospitalizations and associated costs in a population-based study of children with Down syndrome born in Florida.

BACKGROUND: Our objective was to examine differences in hospital resource usage for children with Down syndrome by age and the presence of other birth defects, particularly severe and nonsevere congenital heart defects (CHDs). METHODS: This was a retrospective, population-based, statewide study of children with Down syndrome born 1998 to 2007, identified by the Florida Birth Defects Registry (FBDR) and linked to hospital discharge records for 1 to 10 years after birth. To evaluate hospital resource usage, descriptive statistics on number of hospitalized days and hospital costs were calculated. Results were stratified by isolated Down syndrome (no other coded major birth defect); presence of severe and nonsevere CHDs; and presence of major FBDR-eligible birth defects without CHDs. RESULTS: For 2552 children with Down syndrome, there were 6856 inpatient admissions, of which 68.9% occurred during the first year of life (infancy). Of the 2552 children, 31.7% (n = 808) had isolated Down syndrome, 24.0% (n = 612) had severe CHDs, 36.3% (n = 927) had nonsevere CHDs, and 8.0% (n = 205) had a major FBDR-eligible birth defect in the absence of CHD. Infants in all three nonisolated DS groups had significantly higher hospital costs compared with those with isolated Down syndrome. From infancy through age 4, children with severe CHDs had the highest inpatient costs compared with children in the other sub-groups. CONCLUSION: Results support findings that for children with Down syndrome the presence of other anomalies influences hospital use and costs, and children with severe CHDs have greater hospital resource usage than children with other CHDs or major birth defects without CHDs.

Preoperative assessment of the aortic arch in children younger than 1 year with congenital heart disease: utility of low-dose high-pitch dual-source computed tomography. A single-centre, retrospective analysis of 62 cases.

OBJECTIVES: To evaluate the feasibility, image quality and impact of 3D imaging in low-dose high-pitch dual-source computed tomography (DSCT) to assess arbitrary anatomical malformations of the aortic arch in children <1 year of age with congenital heart disease (CHD). METHODS: Between January 2010 and May 2013, DSCT was performed to assess the aortic arch anatomy in a total of 62 consecutive patients with CHD (aged 0-348 days). DSCT was used whenever conventional echocardiography was not sufficient to display the complex anatomy entirely. Image data acquisition was realized within a single cardiac cycle using prospective ECG triggering. 3D reconstruction for surgical planning was performed. Image quality was assessed retrospectively, using a 4-point scale from '1 = no artefacts' to '4 = uninterpretable'. The accuracy and impact of the 3D reconstructions was compared with intraoperative findings using a 5-point scale (from '1 = essential' to '5 = misleading'). Administered radiation exposure was evaluated. RESULTS: Imaging was successful in all patients, image quality was rated 1.34 on the 4-point scale and the impact of the 3D reconstructions for surgical planning was 2.05 on the 5-point scale. Mean dose-length product was 6.8 ± 2.6 mGy cm, and the effective dose was 0.45 ± 0.13 mSv (0.21-0.74). CONCLUSIONS: DSCT is a fast and appropriate imaging modality in the preoperative assessment of the aortic arch for surgical planning in CHD.

Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease.

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease, and is now predominantly among patients with uncorrected left-to-right shunts. A growing population is characterized by persistent or recurrent PAH after surgical or interventional correction of left-to-right shunts; the latter having a worse prognosis than other forms of PAH associated with congenital heart disease. New treatments for PAH have been shown to be effective in improving PAH exercise capacity and hemodynamics, raising the hope for making previously inoperable congenital heart defects operable and shifting the framework for the assessment of operability. This review focuses on current methods for assessing operability in PAH associated with congenital heart disease, and the possibility of "treat-and-repair" vs. "repair-and-treat" strategies for patients with inoperable or borderline PAH.

Subaortic membrane and aorto-septal angle: an echocardiographic assessment and surgical outcome.

BACKGROUND: Development of a subaortic membrane is not fully understood. Recurrence after surgical removal continues to be high. We sought to assess the differences in aorto-septal angles (AoSA) to possibly explain alterations within the left ventricular outflow tract, hence in subaortic membrane formation. METHODS: A total of 113 patients who underwent subaortic membrane resection were matched by age and sex with 113 controls. The subaortic membrane resection group included isolated subaortic membranes (n = 34, group I), associated with ventricular septal defect (n = 29, group II), or patent ductus arteriosus (n = 50, group III). RESULTS: Mean (± standard deviation) AoSA (in degrees) were not different between subaortic membrane groups I, II, and III but were steeper than their control groups (126.2 ± 9.2 vs 138.6 ± 7.0, 129.2 ± 9.9 vs 137.7 ± 10.0, and 126.2 ± 8.1 vs 135 ± 8.5, respectively; all Ps < .05). Additionally, group II had lower preoperative gradients (28.8 ± 20.7 mm Hg) compared to groups I and III (67.0 ± 32.9 and 66.2 ± 33.1 mm Hg, respectively, P < .001). Follow-up ranged from 3 to 132 months. In 22 (32%) patients, a subaortic membrane recurred. Early postoperative residual gradients and development of aortic regurgutation were associated with the need for reoperation (P < .05). CONCLUSIONS: These findings suggest a contributing role of the AoSA in the development of subaortic membrane. Further rheological experiments are warranted. Whether the steeper the angle the higher the risk of recurrence may be revealed by longer follow-up periods.

Assessment of right heart dilatation with magnetic resonance imaging and multidetector computed tomography angiography: spectrum of disease findings.

Right heart chamber enlargement can be caused by a diverse and heterogeneous group of conditions with highly varied clinical symptoms and signs. An appreciation of the pathophysiology, causes, and imaging features of right heart enlargement is paramount in recognizing and potentially ameliorating the development of right heart dysfunction or adverse cardiac events. Chest x-ray and transthoracic echocardiography have traditionally been, and still are, the mainstay in initial evaluation of right heart dilatation; however, recent advances in both multidetector computed tomography and cardiovascular magnetic resonance imaging now permit a comprehensive assessment of the causes and consequences of right heart dilatation.

Nitrogen biobank for cardiovascular research.

Biobanks play a crucial role in "-Omics" research providing well-annotated samples to study major diseases, their pathways and mechanisms. Accordingly, there are major efforts worldwide to professionalize biobanks in order to provide high quality preservation and storage of biological samples with potentially greater scientific impact. Biobanks are an important resource to elucidate relevant disease mechanisms as well as to improve the diagnosis, prognosis, and treatment of both pediatric and adult cardiovascular disease. High-quality biological sample collections housed in specialized bio-repositories are needed to discover new genetic factors and molecular mechanisms of congenital heart disease and inherited cardiomyopathies in order to prevent the potential risk of having a fatal cardiac condition as well as to facilitate rational drug design around molecular diseases (personalized medicine). Biological samples are also required to improve the understanding the environmental mechanisms of heart disease (environmental cardiology). The goal of this paper is to focus on preanalytical issues (informed consent, sample type, time of collection, temperature and processing procedure) related to collection of biological samples for research purposes. In addition, the paper provides an overview of the efforts made recently by our Institute in designing and implementing a high-security liquid nitrogen storage system (-196°C). We described the implementations of reliable preservation technologies and appropriate quality control (the right temperature, the right environment, fully traceable with all possible back-up systems) in order to ensure maximum security for personnel as well as the quality and suitability of the stored samples.

Individual risk factors and complexity associated with congenital heart disease in a pediatric medicaid cohort.

OBJECTIVES: To determine the sex and race differences associated with specific congenital heart diseases (CHDs) and the patterns of concomitant conditions associated with eight severe, complex lesions. METHODS: A 15-year Medicaid dataset (1996-2010) from one state was analyzed for 14,496 patients aged 17 years and younger and diagnosed as having a CHD on one or more service visits to a pediatrician or pediatric cardiologist. RESULTS: Controlling for all other diagnosed CHDs, boys were more likely to be diagnosed as having transposition of the great arteries, hypoplastic left heart syndrome, aortic stenosis, and coarctation of the aorta, whereas African Americans were more likely to be diagnosed as having tricuspid regurgitation, atrial septal defect sinus venosus, coronary artery anomaly, and pulmonary stenosis. Ventricular septal defects, atrial septal defects secundum, patent ductus arteriosus, and pulmonary stenosis were the most prevalent isolated CHDs, whereas tetralogy of Fallot, atrioventricular canal/endocardial cushion defect, common/single ventricle, double outlet right ventricle, and transposition of the great arteries were the most prevalent severe, complex lesions. The complexity of some severe cardiac anomalies appears to be increasing over time. CONCLUSIONS: Changes over time in pediatric CHD caseload mix may affect care management and result in prognosis or outcome differences. These changes present important opportunities for pediatricians and pediatric cardiologists to collaborate, especially in the care of the most severe anomalies.

Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment.

Adult patients with congenital heart disease (ACHD) represent a growing population due to progress in management. Surgical procedures generally fall short of restoring entirely normal anatomical and functional relations. Further procedures can be needed and lifelong follow-up is required. The right ventricle (RV) plays an important role in congenital heart disease and cardiac magnetic resonance (CMR) imaging has become the imaging method of choice for its assessment. CMR can provide relatively accurate measurements of RV volume and function, and arterial flow, with additional anatomical information provided by three-dimensional contrast angiography and late gadolinium imaging of fibrosis. Here we focus our review on three categories of ACHD in which evaluation of the RV is important: repaired tetralogy of Fallot, the systemic RV and Ebstein anomaly. We demonstrate how CMR contributes to decision-making regarding the types and timings of interventions. A dedicated CMR service should be regarded as a necessary facility of a centre specializing in the care of ACHD patients.

Cost-effectiveness analysis of TAVR.

Transcather aortic valve replacement (TAVR) has rapidly gained worldwide acceptance for treating very high-risk patients with symptomatic severe aortic stenosis. Two valve systems are currently in common use worldwide and under trial in the United States. The Edwards SAPIEN valve has completed its PARTNER trial and has been approved for use in nonoperative patients. The Medtronic CoreValve is currently completing its US pivotal trial. Both plan studies of intermediate-risk patients. The use of TAVR in Europe has grown rapidly and is now about 23% of the total aortic valve replacements done in which a tissue valve is chosen (generally patients over 60 to 65 years of age). This technology is used in a patient population that was either not receiving any surgical therapy due to extreme risk or was considered very high risk for conventional surgery. The procedure requires a highly trained TAVR team, advanced imaging, and the devices themselves, which are expensive. Medical device trials are generally designed to establish if the device works as planned. For TAVR in today's world of rising health care costs, the additional question of cost effectiveness is important to address. Fortunately, the PARTNER trial addressed this and the CoreValve trial has built this into the trial design as well. This article examines what is currently known about the cost-effectiveness of TAVR.

Morphological assessment of the aortic valve using coronary computed tomography angiography, cardiovascular magnetic resonance, and transthoracic echocardiography: comparison with intraoperative findings.

To compare the diagnostic accuracies of coronary computed tomography angiography (CCTA), cardiovascular magnetic resonance (CMR), and transthoracic echocardiography (TTE) in aortic valve (AV) morphological assessments with operative findings. We retrospectively enrolled 262 patients who underwent CCTA, CMR, and TTE before AV surgery. Two independent blinded observers assessed AV morphology as being tricuspid, bicuspid, or quadricuspid using three imaging modalities. Interobserver and intermodality agreements were obtained with kappa statistics. The diagnostic accuracies of CCTA, CMR, and TTE for identifying AV morphology (tricuspid vs. non-tricuspid) were compared with intraoperative findings as the reference standard. At surgery, tricuspid AV, bicuspid AV, and quadricuspid AV were present in 179, 80, and 3 patients, respectively. The CCTA and CMR image qualities were all diagnostic. Thirteen cases of TTE were not evaluable due to severe AV calcification. An excellent correlation between CMR and CCTA was seen for the identification of AV morphology (κ = 0.97). Good correlations existed between CCTA and TTE (κ = 0.72) and between CMR and TTE (κ = 0.74). CCTA, CMR, and TTE had an excellent or good interobserver agreement (κ = 0.90, 0.95, and 0.72, respectively). Sensitivity, specificity, and positive and negative predictive values for AV morphology assessment (tricuspid vs. non-tricuspid) were: 97, 95, 98, and 94 % with CCTA (n = 262); 98, 96, 98, and 95 % with CMR (n = 262); and 98, 88, 95, and 96 % with TTE (n = 249). CCTA and CMR are highly accurate for identifying AV morphology.

Computational assessment of bicuspid aortic valve wall-shear stress: implications for calcific aortic valve disease.

The bicuspid aortic valve (BAV) is associated with a high prevalence of calcific aortic valve disease (CAVD). Although abnormal hemodynamics has been proposed as a potential pathogenic contributor, the native BAV hemodynamic stresses remain largely unknown. Fluid-structure interaction models were designed to quantify the regional BAV leaflet wall-shear stress over the course of CAVD. Systolic flow and leaflet dynamics were computed in two-dimensional tricuspid aortic valve (TAV) and type-1 BAV geometries with different degree of asymmetry (10 and 16% eccentricity) using an arbitrary Lagrangian­Eulerian approach. Valvular performance and regional leaflet wallshear stress were quantified in terms of valve effective orifice area (EOA), oscillatory shear index (OSI) and temporal shear magnitude (TSM). The dependence of those characteristics on the degree of leaflet calcification was also investigated. The models predicted an average reduction of 49% in BAV peak-systolic EOA relative to the TAV. Regardless of the anatomy, the leaflet wall-shear stress was side-specific and characterized by high magnitude and pulsatility on the ventricularis and low magnitude and oscillations on the fibrosa. While the TAV and non-coronary BAV leaflets shared similar shear stress characteristics, the base of the fused BAV leaflet fibrosa exhibited strong abnormalities, which were modulated by the degree of calcification (6-fold, 10-fold and 16-fold TSM increase in the normal, mildly and severely calcified BAV, respectively, relative to the normal TAV). This study reveals the existence of major differences in wall-shear stress pulsatility and magnitude on TAV and BAV leaflets. Given the ability of abnormal fluid shear stress to trigger valvular inflammation, the results support the existence of a mechano-etiology of CAVD in the BAV.