Right-left ventricular shape variations in tetralogy of Fallot: associations with pulmonary regurgitation.

BACKGROUND: Relationships between right ventricular (RV) and left ventricular (LV) shape and function may be useful in determining optimal timing for pulmonary valve replacement in patients with repaired tetralogy of Fallot (rTOF). However, these are multivariate and difficult to quantify. We aimed to quantify variations in biventricular shape associated with pulmonary regurgitant volume (PRV) in rTOF using a biventricular atlas. METHODS: In this cross-sectional retrospective study, a biventricular shape model was customized to cardiovascular magnetic resonance (CMR) images from 88 rTOF patients (median age 16, inter-quartile range 11.8-24.3 years). Morphometric scores quantifying biventricular shape at end-diastole and end-systole were computed using principal component analysis. Multivariate linear regression was used to quantify biventricular shape associations with PRV, corrected for age, sex, height, and weight. Regional associations were confirmed by univariate correlations with distances and angles computed from the models, as well as global systolic strains computed from changes in arc length from end-diastole to end-systole. RESULTS: PRV was significantly associated with 5 biventricular morphometric scores, independent of covariates, and accounted for 12.3% of total shape variation (p < 0.05). Increasing PRV was associated with RV dilation and basal bulging, in conjunction with decreased LV septal-lateral dimension (LV flattening) and systolic septal motion towards the RV (all p < 0.05). Increased global RV radial, longitudinal, circumferential and LV radial systolic strains were significantly associated with increased PRV (all p < 0.05). CONCLUSION: A biventricular atlas of rTOF patients quantified multivariate relationships between left-right ventricular morphometry and wall motion with pulmonary regurgitation. Regional RV dilation, LV reduction, LV septal-lateral flattening and increased RV strain were all associated with increased pulmonary regurgitant volume. Morphometric scores provide simple metrics linking mechanisms for structural and functional alteration with important clinical indices.

Two cases of warfarin-induced tracheobronchial calcification after Fontan surgery.

This study identified tracheobronchial cartilage calcification in children with congenital heart disease. Calcification of the tracheobronchial airways has been found previously in adults receiving warfarin and in children receiving warfarin after mitral valve replacement. A 9-year-old girl who had received a Fontan repair 6 years previously underwent a cardiac computed tomography (CT) scan to evaluate pulmonary artery size. The result was an incidental finding of extensive tracheobronchial cartilage calcification. A retrospective review of all pediatric Fontan patients who had undergone cardiac CT was conducted to search for calcification of the tracheobronchial cartilage. The study investigated ten pediatric Fontan patients who had undergone cardiac CT scanning. Two patients with extensive calcification of the tracheobronchial airways were identified. The index case had hypoplastic left heart syndrome, and the patient had undergone a staged repair with the Fontan at the age of 3 years. A 16-year-old boy with tricuspid atresia had undergone staged repair and Fontan at the age of 3.5 years. These two patients had received continuous warfarin therapy for 6 and 13 years, respectively. Other common causes of airway calcification were excluded from the study. This report describes warfarin-induced tracheobronchial calcification in patients after the Fontan procedure. This finding has possible implications for airway growth and vascular calcification.

Biventricular shape modes discriminate pulmonary valve replacement in tetralogy of Fallot better than imaging indices.

Current indications for pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) rely on cardiovascular magnetic resonance (CMR) image-based indices but are inconsistently applied, lead to mixed outcomes, and remain debated. This study aimed to test the hypothesis that specific markers of biventricular shape may discriminate differences between rTOF patients who did and did not require subsequent PVR better than standard imaging indices. In this cross-sectional retrospective study, biventricular shape models were customized to CMR images from 84 rTOF patients. A statistical atlas of end-diastolic shape was constructed using principal component analysis. Multivariate regression was used to quantify shape mode and imaging index associations with subsequent intervention status (PVR, n = 48 vs. No-PVR, n = 36), while accounting for confounders. Clustering analysis was used to test the ability of the most significant shape modes and imaging indices to discriminate PVR status as evaluated by a Matthews correlation coefficient (MCC). Geometric strain analysis was also conducted to assess shape mode associations with systolic function. PVR status correlated significantly with shape modes associated with right ventricular (RV) apical dilation and left ventricular (LV) dilation (p < 0.01), RV basal bulging and LV conicity (p < 0.05), and pulmonary valve dilation (p < 0.01). PVR status also correlated significantly with RV ejection fraction (p < 0.05) and correlated marginally with LV end-systolic volume index (p < 0.07). Shape modes discriminated subsequent PVR better than standard imaging indices (MCC = 0.49 and MCC = 0.28, respectively) and were significantly associated with RV and LV radial systolic strain. Biventricular shape modes discriminated differences between patients who did and did not require subsequent PVR better than standard imaging indices in current use. These regional features of cardiac morphology may provide insight into adaptive vs. maladaptive types of structural remodeling and point toward an improved quantitative, patient-specific assessment tool for clinical use.

Right ventricular morphology on catheter angiography: variations and its implications for the diagnosis of arrhythmogenic right ventricular cardiomyopathy.

BACKGROUND: Catheter angiography is one modality used to diagnose right ventricular (RV) structural abnormalities in suspected arrythmogenic right ventricular dysplasia or cardiomyopathy (ARVC) patients. The appearance of the normal RV on angiography is poorly defined. This study aimed to assess RV morphology in a control group to define the range of normal appearances. METHODS: RV angiography was performed in 46 subjects (mean age 59 years; 70% male) undergoing coronary angiography for suspected coronary artery disease. Qualitative assessment for RV dilatation, regional wall motion abnormalities (RWMAs), pattern of trabeculae, and presence of micro-aneurysms was performed. Right ventricular outflow tract (RVOT) diameter was measured. RESULTS: Regional or global RV dilatation was considered to be present in 17 patients, RWMA in 13, an abnormal trabecular pattern in 10, and microaneurysms noted in two. The RVOT diameter ranged from 1.78 to 3.51 cm in right anterior oblique view and 2.33 to 4.38 cm in left anterior oblique view. CONCLUSION: The apparent prevalence of abnormal RV morphology in individuals who have no known RV pathology implies that detection of such is not necessarily of diagnostic significance in suspected ARVC. Significant inter-observer variation limits the usefulness of qualitative assessment; quantitative assessment is preferred therefore.

COVID Vaccine-Associated Myocarditis in Adolescent Siblings: Does It Run in the Family?

The development of myocarditis after receiving messenger RNA vaccination against COVID-19 is well documented, particularly in adolescent and young adult males. We report a case of vaccine-associated myocarditis in adolescent brothers following their second dose of the BNT162b2 mRNA vaccine (Pfizer-BioNTech, Mainz, Germany). This report illustrates the need to better understand the mechanisms leading to myocarditis after mRNA vaccination.

Right Ventricular Flow Vorticity Relationships With Biventricular Shape in Adult Tetralogy of Fallot.

Remodeling in adults with repaired tetralogy of Fallot (rToF) may occur due to chronic pulmonary regurgitation, but may also be related to altered flow patterns, including vortices. We aimed to correlate and quantify relationships between vorticity and ventricular shape derived from atlas-based analysis of biventricular shape. Adult rToF (n = 12) patients underwent 4D flow and cine MRI imaging. Vorticity in the RV was computed after noise reduction using a neural network. A biventricular shape atlas built from 95 rToF patients was used to derive principal component modes, which were associated with vorticity and pulmonary regurgitant volume (PRV) using univariate and multivariate linear regression. Univariate analysis showed that indexed PRV correlated with 3 modes (r = -0.55,-0.50, and 0.6, all p < 0.05) associated with RV dilatation and an increase in basal bulging, apical bulging and tricuspid annulus tilting with more severe regurgitation, as well as a smaller LV and paradoxical movement of the septum. RV outflow and inflow vorticity were also correlated with these modes. However, total vorticity over the whole RV was correlated with two different modes (r = -0.62,-0.69, both p < 0.05). Higher vorticity was associated with both RV and LV shape changes including longer ventricular length, a larger bulge beside the tricuspid valve, and distinct tricuspid tilting. RV flow vorticity was associated with changes in biventricular geometry, distinct from associations with PRV. Flow vorticity may provide additional mechanistic information in rToF remodeling. Both LV and RV shapes are important in rToF RV flow patterns.

Transseptal fine needle aspiration of a large left atrial tumour.

The diagnosis of cardiac tumours is often based on images without tissue diagnosis or tissue obtained at surgery. Percutaneous myocardial biopsy via a transvenous approach has been described in literatures but this technique is not feasible with left atrial tumours. We report a patient presenting with heart failure and left atrial tumour. The diagnosis of spindle cell neoplasm was established pre-operatively via successful transseptal fine needle aspiration of cells from a left atrial tumour. We believe this technique worth consideration to aid pre-surgery diagnosis.

Giant congenital coronary artery aneurysm in pulmonary atresia with intact septum.

Congenital coronary artery fistula is frequently associated with pulmonary atresia and intact ventricular septum (PA/IVS). Aneurysmal dilatation is rare, and can be complicated by rupture, distal thrombi and myocardial ischaemia.

A randomized trial of the aldosterone-receptor antagonist eplerenone in asymptomatic moderate-severe aortic stenosis.

BACKGROUND: The aim of the study is to determine whether the selective aldosterone-receptor antagonist eplerenone delays onset of left ventricular (LV) systolic dysfunction or reduces LV hypertrophy in asymptomatic patients with moderate to severe aortic stenosis. Effects of eplerenone on LV diastolic function and progression of valve stenosis were also evaluated. METHODS: Sixty-five asymptomatic patients with a peak aortic valve velocity >3.0 m/s and normal LV function were randomized double blind to eplerenone, 100 mg daily (n = 33), or placebo (n = 32) for a median of 19 (interquartile range 15 to 25) months. Cardiac magnetic resonance imaging and echocardiography were performed and N-terminal pro-brain natriuretic peptide was measured at baseline and follow-up. RESULTS: Symptomatic deterioration occurred in 13 subjects randomized to eplerenone and 11 to placebo (P = .34). Change in LV mass index (mean change +/- SD -0.3 +/- 14.6 vs +5.1 +/- 15 g/m(2) per year, P = .3), LV ejection fraction (+0.0% +/- 5.7% vs +0.8% +/- 5.7% per year, P = .9), and LV end-systolic volume index (-1.2 +/- 9 vs +0.04 +/- 12 mL/m(2) per year, P = .8) were small and similar for patients randomized to eplerenone and placebo, respectively. Decrease of aortic valve area (-0.11 +/- 0.22 vs -0.18 +/- 0.24 cm(2)/y, P = .2), worsening of LV diastolic dysfunction by echo-Doppler (E/E' +0.49 +/- 0.7 vs +1.32 +/- 2.0/year, P = .4), increase in the plasma level of N-terminal pro-brain natriuretic peptide (+63% vs +12% per year, P = .1), and decline in physical function score (9 +/- 34 vs 12 +/- 37/year, P = .7) were similar for subjects randomized to eplerenone and placebo, respectively. CONCLUSIONS: In asymptomatic patients with moderate-severe aortic stenosis, eplerenone did not slow onset of LV systolic or diastolic dysfunction, decrease LV mass, or reduce progression of valve stenosis.

Mapping of mitral regurgitant defects by cardiovascular magnetic resonance in moderate or severe mitral regurgitation secondary to mitral valve prolapse.

PURPOSE: In mitral valve prolapse, determining whether the valve is suitable for surgical repair depends on the location and mechanism of regurgitation. We assessed whether cardiovascular magnetic resonance (CMR) could accurately identify prolapsing or flail mitral valve leaflets and regurgitant jet direction in patients with known moderate or severe mitral regurgitation. METHODS: CMR of the mitral valve was compared with trans-thoracic echocardiography (TTE) in 27 patients with chronic moderate to severe mitral regurgitation due to mitral valve prolapse. Contiguous long-axis high temporal resolution CMR cines perpendicular to the valve commissures were obtained across the mitral valve from the medial to lateral annulus. This technique allowed systematic valve inspection and mapping of leaflet prolapse using a 6 segment model. CMR mapping was compared with trans-oesophageal echocardiography (TOE) or surgical inspection in 10 patients. RESULTS: CMR and TTE agreed on the presence/absence of leaflet abnormality in 53 of 54 (98%) leaflets. Prolapse or flail was seen in 36 of 54 mitral valve leaflets examined on TTE. CMR and TTE agreed on the discrimination of prolapse from flail in 33 of 36 (92%) leaflets and on the predominant regurgitant jet direction in 26 of the 27 (96%) patients. In the 10 patients with TOE or surgical operative findings available, CMR correctly classified presence/absence of segmental abnormality in 49 of 60 (82%) leaflet segments. CONCLUSION: Systematic mitral valve assessment using a simple protocol is feasible and could easily be incorporated into CMR studies in patients with mitral regurgitation due to mitral valve prolapse.

Estimation of myocardial strain from non-rigid registration and highly accelerated cine CMR.

Sparsely sampled cardiac cine accelerated acquisitions show promise for faster evaluation of left-ventricular function. Myocardial strain estimation using image feature tracking methods is also becoming widespread. However, it is not known whether highly accelerated acquisitions also provide reliable feature tracking strain estimates. Twenty patients and twenty healthy volunteers were imaged with conventional 14-beat/slice cine acquisition (STD), 4× accelerated 4-beat/slice acquisition with iterative reconstruction (R4), and a 9.2× accelerated 2-beat/slice real-time acquisition with sparse sampling and iterative reconstruction (R9.2). Radial and circumferential strains were calculated using non-rigid registration in the mid-ventricle short-axis slice and inter-observer errors were evaluated. Consistency was assessed using intra-class correlation coefficients (ICC) and bias with Bland-Altman analysis. Peak circumferential strain magnitude was highly consistent between STD and R4 and R9.2 (ICC = 0.876 and 0.884, respectively). Average bias was -1.7 ± 2.0 %, p < 0.001, for R4 and -2.7 ± 1.9 %, p < 0.001 for R9.2. Peak radial strain was also highly consistent (ICC = 0.829 and 0.785, respectively), with average bias -11.2 ± 18.4 %, p < 0.001, for R4 and -15.0 ± 21.2 %, p < 0.001 for R9.2. STD circumferential strain could be predicted by linear regression from R9.2 with an R2 of 0.82 and a root mean squared error of 1.8 %. Similarly, radial strain could be predicted with an R2 of 0.67 and a root mean squared error of 21.3 %. Inter-observer errors were not significantly different between methods, except for peak circumferential strain R9.2 (1.1 ± 1.9 %) versus STD (0.3 ± 1.0 %), p = 0.011. Although small systematic differences were observed in strain, these were highly consistent with standard acquisitions, suggesting that accelerated myocardial strain is feasible and reliable in patients who require short acquisition durations.

The effect of reducing spatial resolution by in-plane partial volume averaging on peak velocity measurements in phase contrast magnetic resonance angiography.

BACKGROUND: The aim of this study was to quantify the degree of the effect of in-plane partial volume averaging on recorded peak velocity in phase contrast magnetic resonance angiography (PCMRA). METHODS: Using cardiac optimized 1.5 Tesla MRI scanners (Siemens Symphony and Avanto), 145 flow measurements (14 anatomical locations; ventricular outlets, aortic valve (AorV), aorta (5 sites), pulmonary arteries (3 sites), pulmonary veins, superior and inferior vena cava)- in 37 subjects (consisting of healthy volunteers, congenital and acquired heart disease patients) were analyzed by Siemens Argus default voxel averaging technique (where peak velocity = mean of highest velocity voxel and four neighbouring voxels) and by single voxel technique (1.3×1.3×5 or 1.7×1.7×5.5 mm3) (where peak velocity = highest velocity voxel only). The effect of scan protocol (breath hold versus free breathing) and scanner type (Siemens Symphony versus Siemens Avanto) were also assessed. Statistical significance was defined as P<0.05. RESULTS: There was a significant mean increase in peak velocity of 7.1% when single voxel technique was used compared to voxel averaging (P<0.0001). Significant increases in peak velocity were observed by single voxel technique compared to voxel averaging regardless of subject type, anatomical flow location, scanner type and breathing command. Disabling voxel averaging did not affect the volume of flow recorded. CONCLUSIONS: Reducing spatial resolution by the use of voxel averaging produces a significant underestimation of peak velocity. While this is of itself not surprising this is the first report to quantify the size of the effect. When PCMRA is used to assess peak velocity recording pixel averaging should be disabled.

Improving assessment of congenital heart disease through rapid patient specific modeling.

Congenital heart disease is the most common birth defect, with an incidence of 75 in every 1000 births. As a result of improved interventions, 90% of people with congenital heart disease now survive to adulthood. They must undergo regular imaging to assess their biventricular (left and right ventricular) function. Analysis of the images is problematic due to the large variety of shapes and complex geometry. In this paper we extend a biventricular modeling method to improve the analysis of MR images from congenital heart disease patients. We used a subdivision surface method to create three customizable exemplars, representing common manifestations of anatomy, and incorporated these as priors into an interactive biventricular customization procedure. The CHD-specific priors were tested on 60 cases representing a variety of congenital heart diseases for which the gold standard manual contours were available. The introduction of multiple priors showed a significant decrease in analysis time while maintaining good correlation between the two methods (R2 >.82).

Regeneration of the heart in diabetes by selective copper chelation.

Heart disease is the major cause of death in diabetes, a disorder characterized by chronic hyperglycemia and cardiovascular complications. Although altered systemic regulation of transition metals in diabetes has been the subject of previous investigation, it is not known whether changed transition metal metabolism results in heart disease in common forms of diabetes and whether metal chelation can reverse the condition. We found that administration of the Cu-selective transition metal chelator trientine to rats with streptozotocin-induced diabetes caused increased urinary Cu excretion compared with matched controls. A Cu(II)-trientine complex was demonstrated in the urine of treated rats. In diabetic animals with established heart failure, we show here for the first time that 7 weeks of oral trientine therapy significantly alleviated heart failure without lowering blood glucose, substantially improved cardiomyocyte structure, and reversed elevations in left ventricular collagen and beta(1) integrin. Oral trientine treatment also caused elevated Cu excretion in humans with type 2 diabetes, in whom 6 months of treatment caused elevated left ventricular mass to decline significantly toward normal. These data implicate accumulation of elevated loosely bound Cu in the mechanism of cardiac damage in diabetes and support the use of selective Cu chelation in the treatment of this condition.

Creating shape templates for patient specific biventricular modeling in congenital heart disease.

Survival rates for infants with congenital heart disease (CHD) are improving, resulting in a growing population of adults with CHD. However, the analysis of left and right ventricular function is very time-consuming owing to the variety of congenital morphologies. Efficient customization of patient geometry and function depends on high quality shape templates specifically designed for the application. In this paper, we combine a method for creating finite element shape templates with an interactive template customization to patient MRI examinations. This enables different templates to be chosen depending on patient morphology. To demonstrate this pipeline, a new biventricular template with 162 elements was created and tested in place of an existing 82-element template. The method was able to provide fast interactive biventricular analysis with 0.31 sec per edit response time. The new template was customized to 13 CHD patients with similar biventricular topology, showing improved performance over the previous template and good agreement with clinical indices.

Three-dimensional assessment of left ventricular systolic strain in patients with type 2 diabetes mellitus, diastolic dysfunction, and normal ejection fraction.

Left ventricular (LV) diastolic dysfunction often occurs in patients with type 2 diabetes mellitus (DM) independent of atherosclerotic coronary artery disease, myocardial ischemia, and regional wall motion anomalies. Limited information exists on LV myocardial tissue strain in this patient group. We measured 3-dimensional (3-D) parameters of LV systolic and diastolic functions in 28 patients who had type 2 DM (age 33 to 70 years), standard echocardiographic evidence of LV diastolic dysfunction, and normal LV ejection fraction, and 31 normal control subjects (age 19 to 74 years) who had no evidence of cardiac disease, with multislice cine anatomic and tagged magnetic resonance imaging. Three-dimensional analysis of the resulting images showed that peak systolic mitral valve plane displacement was 12% smaller (p = 0.040) and peak diastolic mitral valve plane velocity was 21% lower (p = 0.008) in patients who had DM than in normal controls. Peak systolic circumferential and longitudinal strains and principal 3-D shortening strain were 14%, 22%, and 10% smaller, respectively, in the DM group (p <0.001 for each). Peak diastolic rate of relaxation of circumferential and longitudinal strains and principal 3-D shortening strain were 35%, 32%, and 33% lower, respectively, in the DM group (p <0.001 for each). Thus, LV systolic circumferential, longitudinal and 3-D principal strains, and diastolic strain rates are impaired in patients who have type 2 DM, LV diastolic dysfunction, and normal LV ejection fraction.

Rapid D-Affine biventricular cardiac function with polar prediction.

Although many solutions have been proposed for left ven-tricular functional analysis of the heart, right and left (bi-) ventricular function has been problematic due to the complex geometry and large motions. Biventricular function is particularly important in congenital heart disease, the most common type of birth defects. We describe a rapid interactive analysis tool for biventricular function which incorporates 1) a 3D+ time finite element model of biventricular geometry, 2) a fast prediction step which estimates an initial geometry in a polar coordinate system, and 3) a Cartesian update which penalizes deviations from affine transformations (D-Affine) from a prior. Solution times were very rapid, enabling interaction in real time using guide point modeling. The method was applied to 13 patients with congenital heart disease and compared with the clinical gold standard of manual tracing. Results between the methods showed good correlation (R2 > 0.9) and good precision (volume < 17 ml; mass < 11g) for both chambers.

Consider charity with caution.

Cardiac CT offers a non-invasive diagnostic alternative to coronary angiography in the diagnosis of spontaneous coronary dissection.