Blood and Imaging Biomarkers in the Long-term Follow-up of Bicuspid Aortic Valve Patients.

Background: Bicuspid aortic valve (BAV) is a common congenital heart defect. Patients with BAV are at risk for long-term complications such as valve stenosis and regurgitation. This study aimed to investigate sex differences in blood and imaging biomarkers and to describe the long-term prognostic value of blood and echocardiographic biomarkers. Methods: Patients were included from 2 prospective observational cohort studies; they underwent venous blood sampling and transthoracic echocardiography including speckle tracking. Analyzed blood biomarkers were red-cell distribution width (RDW), creatinine, C-reactive protein (CRP), troponin T, N-terminal pro B-type natriuretic peptide (NT-proBNP), and transforming growth factor-beta (TGF-ß). Sex differences were analyzed at baseline. Associations between biomarkers and arrhythmia-free and intervention-free survival were determined by Cox regression, adjusted for age and sex. Results: A total of 182 patients with BAV were included: median age 34; interquartile range [IQR]: 23-46 years; 55.5% male. CRP, NT-proBNP, and RDW were higher in women, whereas creatinine, troponin T and TGF-ß were higher among men. After a median follow-up time of 6.9 (IQR: 6.5-9.9) years, arrhythmia-free and intervention-free survival was, 81.0% and 73.1%, respectively. NT-proBNP was associated with both arrhythmia-free and intervention-free survival (hazard ratio [HR], 1.94, P = 0.005 and HR, 2.06, P = 0.002, respectively). On echocardiography higher left atrial (LA) size, left ventricular end-diastolic diameter (LVEDD), left ventricular (LV) mass index and E/e' ratio were associated with lower arrhythmia-free survival, whereas higher LA size, LV mass index, aortic valve peak velocity, and aortic regurgitation were associated with lower intervention-free survival. Conclusions: Differences were observed in blood biomarkers between men and women with BAV. Besides LV systolic parameters, diastolic LV function and NT-proBNP should have a more prominent role as prognostic markers in clinical care.

Contexte: La bicuspide valvulaire aortique (BVA) est une anomalie cardiaque congénitale fréquente. Les patients atteints d'une BVA présentent des risques de complications à long terme, comme la sténose valvulaire ou la régurgitation valvulaire. Cette étude visait 1) à évaluer les différences entre les sexes en ce qui concerne les biomarqueurs sanguins et les biomarqueurs à l'imagerie; et 2) à décrire la valeur pronostique à long terme des biomarqueurs sanguins et échocardiographiques. Méthodologie: Des patients de 2 études de cohortes observationnelles prospectives ont été inclus dans l'analyse. Des échantillons de sang veineux ont été prélevés, et des échocardiographies transthoraciques, y compris le suivi des marqueurs acoustiques, ont été effectuées. Les biomarqueurs sanguins analysés étaient les suivants : indice de distribution des globules rouges (IDR), créatinine, protéine C-réactive (CRP), troponine T, propeptide natriurétique de type B N-terminal (NT-proBNP) et facteur de croissance transformant ß (TGF-ß). Les différences entre les sexes ont été analysées au départ. Les liens entre les biomarqueurs et la survie sans arythmie et sans intervention ont été déterminés par la régression de Cox, avec correction en fonction de l'âge et du sexe. Résultats: Cent quatre-vingt-deux patients présentant une BVA étaient inclus (âge médian de 34 [écart interquartile : 23-46] ans, 55,5 % hommes). La CRP, la NT-proBNP et l'IDR étaient plus élevées chez les femmes, alors que la créatinine, la troponine T et le TGF-ß étaient plus élevés chez les hommes. Après une période de suivi médiane de 6,9 (écart interquartile : 6,5-9,9) ans, les taux de survie sans arythmie et sans intervention étaient respectivement de 81,0 % et de 73,1 %. La NT-proBNP a été associée à la survie sans arythmie (rapport des risques instantanés [RRI] : 1,94, p = 0,005) et à la survie sans intervention (RRI : 2,06, p = 0,002). À l'échocardiographie, des valeurs élevées pour la taille de l'oreillette gauche, le diamètre télédiastolique du ventricule gauche (VG), l'indice de masse du VG et le rapport E/e' étaient associées à un faible taux de survie sans arythmie, alors que des valeurs élevées pour la taille de l'oreillette gauche, l'indice de masse du VG, la vitesse maximale aortique et la régurgitation aortique étaient associées à un faible taux de survie sans intervention. Conclusions: Les biomarqueurs sanguins variaient en fonction du sexe des personnes présentant une BVA. Outre les paramètres systoliques du VG, la fonction VG diastolique et la NT-proBNP devraient être davantage utilisées comme marqueurs pronostiques en soins cliniques.

Nonsustained Ventricular Tachycardia Is Independently Associated With Sustained Ventricular Arrhythmias in Nonischemic Dilated Cardiomyopathy.

BACKGROUND: Spontaneous nonsustained ventricular tachycardia (NSVT) on Holter, VT inducibility during electrophysiology study, and late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) have been associated with sustained ventricular arrhythmias (SVAs) in nonischemic dilated cardiomyopathy (DCM). This study aimed to analyze whether these parameters carry independent prognostic value for spontaneous SVA in DCM. METHODS: Between 2011 and 2018, patients with the DCM clinical spectrum and documented SVA, suspected SVA, or considered to be at intermediate or high risk for SVA were enrolled in the prospective Leiden Nonischemic Cardiomyopathy Study. Patients underwent a comprehensive evaluation including 24-hour Holter, LGE-CMR, and electrophysiology study. Holters were assessed for the presence of NSVT (≥3 beats; rate, ≥120 bpm; lasting <30 s) and NSVT characteristics (coupling interval, duration, cycle length, morphology, regularity). Patients were followed at 6 to 12 monthly intervals. RESULTS: Of all 115 patients (age, 59±12 years; 77% men; left ventricular ejection fraction, 33±13%; history of SVA, 36%; LGE in 63%; median LGE mass, 13 g; interquartile range, 8-23 g), 62 (54%) had NSVT on Holter, and sustained monomorphic VT was inducible in 34 of 114 patients (30%). NSVT was not associated with LGE on CMR or VT inducibility during electrophysiology study nor were its features (all P>0.05). During 4.0±1.8 years of follow-up, SVA occurred in 39 patients (34%). NSVT (HR, 4.47 [95% CI, 1.87-10.72]; P=0.001) and VT inducibility (HR, 3.08 [95% CI, 1.08-8.81]; P=0.036) were independently associated with SVA during follow-up. A bivariable model including only noninvasively acquired parameters also allowed identification of a high-risk subgroup (ie, those with both NSVT and LGE on CMR). The findings remained similar when only patients without prior SVA were included. CONCLUSIONS: In patients with DCM, NSVT on Holter and VT inducibility during electrophysiology study predict SVA during follow-up independent of LGE on CMR. NSVTs may serve as an initiator, and sustained VT inducibility indicates the presence of the substrate for SVA in DCM. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT01940081.

Extent of Coronary Artery Disease in Patients With Stenotic Bicuspid Versus Tricuspid Aortic Valves.

Background Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, which is often complicated by aortic valve stenosis (AoS). In tricuspid aortic valve (TAV), AoS strongly associates with coronary artery disease (CAD) with common pathophysiological factors. Yet, it remains unclear whether AoS in patients with BAV is also associated with CAD. This study investigated the association between the aortic valve morphological features and the extent of CAD. Methods and Results A single-center study was performed, including all patients who underwent an aortic valve replacement attributable to AoS between 2006 and 2019. Coronary sclerosis was graded on preoperative coronary angiographies using the coronary artery greater even than scoring method, which divides the coronaries in 28 segments and scores nonobstructive (20%-49% sclerosis) and obstructive coronary sclerosis (>49% sclerosis) in each segment. Multivariate analyses were performed, controlling for age, sex, and CAD risk factors. A total of 1296 patients (931 TAV and 365 BAV) were included, resulting in 548 matched patients. Patients with TAV exhibited more CAD risk factors (odds ratio [OR], 2.66; 95% CI, 1.79-3.96; P<0.001). Patients with BAV had lower coronary artery greater even than 20 (1.61±2.35 versus 3.60±2.79) and coronary artery greater even than 50 (1.24±2.43 versus 3.37±3.49) scores (P<0.001), even after correcting for CAD risk factors (P<0.001). Patients with TAV more often needed concomitant coronary revascularization (OR, 3.50; 95% CI, 2.42-5.06; P<0.001). Conclusions Patients with BAV who are undergoing surgery for AoS carry a lower cardiovascular risk profile, correlating with less coronary sclerosis and a lower incidence of concomitant coronary revascularization compared with patients with TAV.

Effectiveness of a multidisciplinary clinical pathway for women with systemic lupus erythematosus and/or antiphospholipid syndrome.

OBJECTIVES: SLE and/or antiphospholipid syndrome (SLE/APS) are complex and rare systemic autoimmune diseases that predominantly affect women of childbearing age. Women with SLE/APS are at high risk of developing complications during pregnancy. Therefore, clinical practice guidelines recommend that patients with SLE/APS should receive multidisciplinary counselling before getting pregnant. We investigated the clinical effectiveness of implementing a multidisciplinary clinical pathway including prepregnancy counselling of patients with SLE/APS. METHODS: A clinical pathway with specific evaluation and prepregnancy counselling for patients with SLE/APS was developed and implemented in a tertiary, academic hospital setting. Patients were prospectively managed within the clinical pathway from 2014 onwards and compared with a retrospective cohort of patients that was not managed in a clinical pathway. Primary outcome was a combined outcome of disease flares for SLE and thromboembolic events for APS. Secondary outcomes were maternal and fetal pregnancy complications. RESULTS: Seventy-eight patients with 112 pregnancies were included in this study. The primary combined outcome was significantly lower in the pathway cohort (adjusted OR (aOR) 0.20 (95% CI 0.06 to 0.75)) which was predominantly determined by a fivefold risk reduction of SLE flares (aOR 0.22 (95% CI 0.04 to 1.09)). Maternal and fetal pregnancy complications were not different between the cohorts (respectively, aOR 0.91 (95% CI 0.38 to 2.17) and aOR 1.26 (95% CI 0.55 to 2.88)). CONCLUSIONS: The outcomes of this study suggest that patients with SLE/APS with a pregnancy wish benefit from a multidisciplinary clinical pathway including prepregnancy counselling.

Abnormal Aortic Wall Properties in Women with Turner Syndrome.

BACKGROUND: Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS. METHODS: In this cross-sectional study, 52 women with TS aged 35 ± 13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ascending aortic distensibility (AAD) and aortic arch pulse wave velocity (AA-PWV) by magnetic resonance imaging (MRI). As control group, 13 women with BAV without TS and 48 healthy patients were included. RESULTS: Women with TS showed a higher AA-PWV (ß = 1.08, confidence interval [CI]: 0.54-1.62) after correcting for age and comorbidities compared with controls. We found no significant difference in AAD and CF-PWV. In women with TS, the presence of BAV, coarctation of the aorta, or monosomy (45, X) was not associated with aortic stiffness. In addition, aortic tissue samples were investigated with routine and immunohistochemical stains in five additional women with TS who were operated. The tissue showed more compact smooth muscle cell layers with abnormal deposition and structure of elastin and diminished or absent expression of contractile proteins desmin, actin, and caldesmon, as well as the progesterone receptor. CONCLUSION: Both aortic arch stiffness measurements on MRI and histomorphological changes point toward an inherent abnormal thoracic aortic wall in women with TS.

Cardiac complications in patients hospitalised with COVID-19.

AIMS: To determine the frequency and pattern of cardiac complications in patients hospitalised with coronavirus disease (COVID-19). METHODS AND RESULTS: CAPACITY-COVID is an international patient registry established to determine the role of cardiovascular disease in the COVID-19 pandemic. In this registry, data generated during routine clinical practice are collected in a standardised manner for patients with a (highly suspected) severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection requiring hospitalisation. For the current analysis, consecutive patients with laboratory confirmed COVID-19 registered between 28 March and 3 July 2020 were included. Patients were followed for the occurrence of cardiac complications and pulmonary embolism from admission to discharge. In total, 3011 patients were included, of which 1890 (62.8%) were men. The median age was 67 years (interquartile range 56-76); 937 (31.0%) patients had a history of cardiac disease, with pre-existent coronary artery disease being most common (n=463, 15.4%). During hospitalisation, 595 (19.8%) patients died, including 16 patients (2.7%) with cardiac causes. Cardiac complications were diagnosed in 349 (11.6%) patients, with atrial fibrillation (n=142, 4.7%) being most common. The incidence of other cardiac complications was 1.8% for heart failure (n=55), 0.5% for acute coronary syndrome (n=15), 0.5% for ventricular arrhythmia (n=14), 0.1% for bacterial endocarditis (n=4) and myocarditis (n=3), respectively, and 0.03% for pericarditis (n=1). Pulmonary embolism was diagnosed in 198 (6.6%) patients. CONCLUSION: This large study among 3011 hospitalised patients with COVID-19 shows that the incidence of cardiac complications during hospital admission is low, despite a frequent history of cardiovascular disease. Long-term cardiac outcomes and the role of pre-existing cardiovascular disease in COVID-19 outcome warrants further investigation.

Left ventricular global longitudinal strain in bicupsid aortic valve patients: head-to-head comparison between computed tomography, 4D flow cardiovascular magnetic resonance and speckle-tracking echocardiography.

Left ventricular global longitudinal strain (LVGLS) analysis is a sensitive measurement of myocardial deformation most often done using speckle-tracking transthoracic echocardiography (TTE). We propose a novel approach to measure LVGLS using feature-tracking software on the magnitude dataset of 4D flow cardiovascular magnetic resonance (CMR) and compare it to dynamic computed tomography (CT) and speckle tracking TTE derived measurements. In this prospective cohort study 59 consecutive adult patients with a bicuspid aortic valve (BAV) were included. The study protocol consisted of TTE, CT, and CMR on the same day. Image analysis was done using dedicated feature-tracking (4D flow CMR and CT) and speckle-tracking (TTE) software, on apical 2-, 3-, and 4-chamber long-axis multiplanar reconstructions (4D flow CMR and CT) or standard apical 2-, 3-, and 4-chamber acquisitions (TTE). CMR and CT GLS analysis was feasible in all patients. Good correlations were observed for GLS measured by CMR (- 21 ± 3%) and CT (- 20 ± 3%) versus TTE (- 20 ± 3%, Pearson's r: 0.67 and 0.65, p < 0.001). CMR also correlated well with CT (Pearson's r 0.62, p < 0.001). The inter-observer analysis showed moderate to good reproducibility of GLS measurement by CMR, CT and TTE (Pearsons's r: 0.51, 0.77, 0.70 respectively; p < 0.05). Additionally, ejection fraction (EF), end-diastolic and end-systolic volume measurements (EDV and ESV) correlated well between all modalities (Pearson's r > 0.61, p < 0.001). Feature-tracking GLS analysis is feasible using the magnitude images acquired with 4D flow CMR. GLS measurement by CMR correlates well with CT and speckle-tracking 2D TTE. GLS analysis on 4D flow CMR allows for an integrative approach, integrating flow and functional data in a single sequence. Not applicable, observational study.

Blood biomarkers in patients with bicuspid aortic valve disease.

BACKGROUND: Patients with a bicuspid aortic valve (BAV) are at risk of developing valve deterioration and aortic dilatation. We aimed to investigate whether blood biomarkers are associated with disease stage in patients with BAV. METHODS: Serum levels of high sensitivity C-reactive protein (hsCRP), high sensitivity troponin T (hsTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and total transforming growth factor-beta 1 (TGF-ß1) were measured in adult BAV patients with valve dysfunction or aortic pathology. Age-matched general population controls were included for TGFß-1 measurements. Correlation analyses and multivariable linear regression were used to determine the association between (2log-transformed) biomarker levels and aortic valve regurgitation, aortic valve stenosis, aortic dilatation, or left ventricular function. RESULTS: hsCRP and hsTnT were measured in the total group of 183 patients (median age 34 years, 25th-75th percentile 23-46), NT-proBNP in 162 patients, and TGF-ß1 beta in 108 patients. Elevated levels of NT-proBNP were found in 20% of the BAV patients, elevated hsTnT in 6%, and elevated hsCRP in 7%. Higher hsTnT levels were independently associated with aortic regurgitation [odds ratio per doubling (OR2log) 1.34, 95% CI 1.01;1.76] and higher NT-proBNP levels with aortic valve maximal velocity (ß2log 0.17, 95%CI 0.07;0.28) and aortic regurgitation (OR2log 1.41, 95%CI 1.11;1.79). Both BAV patients with (9.9 ± 2.7 ng/mL) and without aortic dilatation (10.4 ± 2.9 ng/mL) showed lower TGF-ß1 levels compared to general population controls (n = 85, 11.8 ± 3.2 ng/mL). CONCLUSIONS: Higher NT-proBNP and hsTNT levels were associated with aortic valve disease in BAV patients. TGF-ß1 levels were lower in BAV patients than in the general population, and not related to aortic dilatation. Longitudinal data are needed to further investigate the prognostic value of biomarkers in these patients.

Endothelial Colony Forming Cells as an Autologous Model to Study Endothelial Dysfunction in Patients with a Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with an increased prevalence of aortic dilation, aortic rupture and aortic valve calcification. Endothelial cells (ECs) play a major role in vessel wall integrity. Little is known regarding EC function in BAV patients due to lack of patient derived primary ECs. Endothelial colony forming cells (ECFCs) have been reported to be a valid surrogate model for several cardiovascular pathologies, thereby facilitating an in vitro system to assess patient-specific endothelial dysfunction. Therefore, the aim of this study was to investigate cellular functions in ECFCs isolated from BAV patients. Outgrowth and proliferation of ECFCs from patients with BAV (n = 34) and controls with a tricuspid aortic valve (TAV, n = 10) were determined and related to patient characteristics. Interestingly, we were only able to generate ECFCs from TAV and BAV patients without aortic dilation, and failed to isolate ECFC colonies from patients with a dilated aorta. Analyzing EC function showed that while proliferation, cell size and endothelial-to-mesenchymal transition were similar in TAV and BAV ECFCs, migration and the wound healing capacity of BAV ECFCs is significantly higher compared to TAV ECFCs. Furthermore, calcification is blunted in BAV compared to TAV ECFCs. Our results reveal ECs dysfunction in BAV patients and future research is required to unravel the underlying mechanisms and to further validate ECFCs as a patient-specific in vitro model for BAV.

Entropy as a Novel Measure of Myocardial Tissue Heterogeneity for Prediction of Ventricular Arrhythmias and Mortality in Post-Infarct Patients.

OBJECTIVES: This study proposed entropy as a new late gadolinium enhanced cardiac magnetic resonance-derived parameter to evaluate tissue inhomogeneity, independent of signal intensity thresholds. This study hypothesized that entropy within the scar is associated with ventricular arrhythmias (VAs), whereas entropy of the entire left ventricular (LV) myocardium is associated with mortality. BACKGROUND: In patients after myocardial infarction, the heterogeneity of fibrosis determines the substrate for VA. Fibrosis in remote areas has been associated with heart failure and mortality. Late gadolinium-enhanced cardiac magnetic resonance has been used to delineate fibrosis, but available methods depend on signal intensity thresholds and results have been inconsistent. METHODS: Consecutive post-myocardial infarction patients undergoing late gadolinium enhanced cardiac magnetic resonance prior to implantable cardioverter-defibrillator implantation were included. From cardiac magnetic resonance imaging, total scar size, scar gray zone, scar transmurality, and tissue entropy were derived. Patients were followed for appropriate implantable cardioverter-defibrillator therapy and mortality. RESULTS: A total of 154 patients (age 64 ± 10 years, 84% male, LV ejection fraction 29 ± 10%, 47% acute revascularization) were included. During a median follow-up of 56 (interquartile range: 40 to 73) months, appropriate implantable cardioverter-defibrillator therapy occurred in 46 patients (30%), and 41 patients (27%) died. From multivariable analysis, higher entropy of the scar (hazard ratio [HR]: 1.9; 95% confidence interval [CI]: 1.0 to 3.5; p = 0.042) was independently associated with VA, after adjusting for multivessel disease, acute revascularization, LV ejection fraction, scar gray zone, and transmurality. Entropy of the entire LV was independently associated with mortality (HR: 3.2; 95% CI: 1.1 to 9.9; p = 0.038). CONCLUSIONS: High entropy within the scar was associated with VA and may indicate an arrhythmogenic scar. High entropy of the entire LV was associated with mortality and may reflect a fibrosis pattern associated with adverse remodeling.

Intermodality variation of aortic dimensions: How, where and when to measure the ascending aorta.

BACKGROUND: No established reference-standard technique is available for ascending aortic diameter measurements. The aim of this study was to determine agreement between modalities and techniques. METHODS: In patients with aortic pathology transthoracic echocardiography, computed tomography angiography (CTA) and magnetic resonance angiography (MRA) were performed. Aortic diameters were measured at the sinus of Valsalva (SoV), sinotubular junction (STJ) and tubular ascending aorta (TAA) during mid-systole and end-diastole. In echocardiography both the inner edge-to-inner edge (I-I edge) and leading edge-to­leading edge (L-L edge) methods were applied, and the length of the aortic annulus to the most cranial visible part of the ascending aorta was measured. In CTA and MRA the I-I method was used. RESULTS: Fifty patients with bicuspid aortic valve (36 ±â€¯13 years, 26% female) and 50 Turner patients (35 ±â€¯13 years) were included. Comparison of all aortic measurements showed a mean difference of 5.4 ±â€¯2.7 mm for the SoV, 5.1 ±â€¯2.0 mm for the STJ and 4.8 ±â€¯2.1 mm for the TAA. The maximum difference was 18 mm. The best agreement was found between echocardiography L-L edge and CTA during mid-systole. CTA and MRA showed good agreement. A mean difference of 1.5 ±â€¯1.3 mm and 1.8 ±â€¯1.5 mm was demonstrated at the level of the STJ and TAA comparing mid-systolic with end-diastolic diameters. The visible length of the aorta increased on average 5.3 ±â€¯5.1 mmW during mid-systole. CONCLUSIONS: MRA and CTA showed best agreement with L-L edge method by echocardiography. In individual patients large differences in ascending aortic diameter were demonstrated, warranting measurement standardization. The use of CTA or MRA is advised at least once.

Coronary anatomy in Turner syndrome versus patients with isolated bicuspid aortic valves.

OBJECTIVE: Variations in coronary anatomy, like absent left main stem and left dominant coronary system, have been described in patients with Turner syndrome (TS) and in patients with bicuspid aortic valves (BAV). It is unknown whether coronary variations in TS are related to BAV and to specific BAV subtypes. AIM: To compare coronary anatomy in patients with TS with/without BAV versus isolated BAV and to study BAV morphology subtypes in these groups. METHODS: Coronary anatomy and BAV morphology were studied in 86 patients with TS (20 TS-BAV, 66 TS-tricuspid aortic valve) and 86 patients with isolated BAV (37±13 years vs 42±15 years, respectively) by CT. RESULTS: There was no significant difference in coronary dominance between patients with TS with and without BAV (25% vs 21%, p=0.933). BAVs with fusion of right and left coronary leaflets (RL BAV) without raphe showed a high prevalence of left coronary dominance in both TS-BAV and isolated BAV (both 38%). Absent left main stem was more often seen in TS-BAV as compared with isolated BAV (10% vs 0%). All patients with TS-BAV with absent left main stem had RL BAV without raphe. CONCLUSION: The equal distribution of left dominance in RL BAV without raphe in TS-BAV and isolated BAV suggests that presence of left dominance is a feature of BAVs without raphe, independent of TS. Both TS and RL BAV without raphe seem independently associated with absent left main stems. Awareness of the higher incidence of particularly absent left main stems is important to avoid complications during hypothermic perfusion.

High spatial resolution free-breathing 3D late gadolinium enhancement cardiac magnetic resonance imaging in ischaemic and non-ischaemic cardiomyopathy: quantitative assessment of scar mass and image quality.

PURPOSE: To compare breath-hold (BH) with navigated free-breathing (FB) 3D late gadolinium enhancement cardiac MRI (LGE-CMR) MATERIALS AND METHODS: Fifty-one patients were retrospectively included (34 ischaemic cardiomyopathy, 14 non-ischaemic cardiomyopathy, three discarded). BH and FB 3D phase sensitive inversion recovery sequences were performed at 3T. FB datasets were reformatted into normal resolution (FB-NR, 1.46x1.46x10mm) and high resolution (FB-HR, isotropic 0.91-mm voxels). Scar mass, scar edge sharpness (SES), SNR and CNR were compared using paired-samples t-test, Pearson correlation and Bland-Altman analysis. RESULTS: Scar mass was similar in BH and FB-NR (mean ± SD: 15.5±18.0 g vs. 15.5±16.9 g, p=0.997), with good correlation (r=0.953), and no bias (mean difference ± SD: 0.00±5.47 g). FB-NR significantly overestimated scar mass compared with FB-HR (15.5±16.9 g vs 14.4±15.6 g; p=0.007). FB-NR and FB-HR correlated well (r=0.988), but Bland-Altman demonstrated systematic bias (1.15±2.84 g). SES was similar in BH and FB-NR (p=0.947), but significantly higher in FB-HR than FB-NR (p<0.01). SNR and CNR were lower in BH than FB-NR (p<0.01), and lower in FB-HR than FB-NR (p<0.01). CONCLUSION: Navigated free-breathing 3D LGE-CMR allows reliable scar mass quantification comparable to breath-hold. During free-breathing, spatial resolution can be increased resulting in improved sharpness and reduced scar mass. KEY POINTS: • Navigated free-breathing 3D late gadolinium enhancement is reliable for myocardial scar quantification. • High-resolution 3D late gadolinium enhancement increases scar sharpness • Ischaemic and non-ischaemic cardiomyopathy patients can be imaged using free-breathing LGE CMR.

Prosthesis Dislocation After Transapical Valve-In-Valve Mitral Valve Implantation.

Transapical valve-in-valve mitral valve implantation (TA-MVI) has been described as an alternative treatment option in patients with degenerated bioprostheses in the mitral position. We report the first case of prosthesis dislocation and migration into the left atrium after TA-MVI. A new prosthesis was implanted using the same approach. The dislocated prosthesis was successfully removed through the left atrial appendage through an extended anterolateral thoracotomy without the use of cardiopulmonary bypass.

Biplane versus short-axis measures of the left atrium and ventricle in patients with systolic dysfunction assessed by magnetic resonance.

PURPOSE: This study aims to investigate if the time saving biplane method can be an alternative to short-axis measurements for left atrium (LA) and left ventricle (LV) in cardiac magnetic resonance of patients with extensive LV remodeling. MATERIALS AND METHODS: In 45 patients with coronary artery disease and systolic LV dysfunction [ejection fraction (EF)<50%], LA and LV end-diastolic volume, end-systolic volume, stroke volume, and EF were measured by the biplane area length and by the short-axis volume method. RESULTS: No statistical differences between the biplane area length and short-axis volume were found for the LA end-systolic or the LV end-diastolic volumes. However, the biplane area length method overestimated LA stroke volume by 7.1±12.3 ml, LA EF by 6.7±8.8% (both P<.001), LV stroke volume by 6.2±12.9 ml (P=.002), and LV EF by 2.6±4.6% (P<.001). The differences in LV measurements were more evident in patients with severely distorted LV. For LA volume parameters, overall consistency between biplane area length and short-axis approach was good [all intraclass correlation coefficient (ICC)≥0.74] and for LV volume parameters, overall consistency between both approaches was excellent (all ICC≥0.93). CONCLUSION: In patients with LV systolic dysfunction, the short-axis volume is superior to the biplane method and should be the first choice, when accurate determination of LA and LV EF is the main clinical indication for cardiac magnetic resonance.

The pros and cons of preserving a functioning arteriovenous fistula after kidney transplantation.

The autologous arteriovenous fistula (AVF) for hemodialysis burdens the cardiovascular system with increased cardiac output and pulmonary artery pressure, increasing cardiovascular risk. This article reviews literature on the benefits and drawbacks of a functioning AVF after kidney transplantation and discusses the cardiovascular effects of AVF closure. Several cohort studies demonstrate a significant cardiac burden of an AVF and improvement of cardiac dimensions after AVF ligation. However, no randomized trials have been conducted on routine AVF closure after successful kidney transplantation. Therefore, clinical trials are warranted to evaluate whether the cardiovascular benefits of routine AVF closure outweigh the potential harm for patients after successful kidney transplantation.

Coronary anatomy as related to bicuspid aortic valve morphology.

OBJECTIVE: Variable coronary anatomy has been described in patients with bicuspid aortic valves (BAVs). This was never specified to BAV morphology, and prognostic relevance of coronary vessel dominance in this patient group is unclear. The purpose of this study was to evaluate valve morphology in relation to coronary artery anatomy and outcome in patients with isolated BAV and with associated aortic coarctation (CoA). METHODS: Coronary anatomy was evaluated in 186 patients with BAV (141 men (79%), 51±14 years) by CT and invasive coronary angiography. Correlation of coronary anatomy was made with BAV morphology and coronary events. RESULTS: Strictly bicuspid valves (without raphe) with left-right cusp fusion (type 1B) had more left dominant coronary systems compared with BAVs with left-right cusp fusion with a raphe (type 1A) (48% vs. 26%, p=0.047) and showed more separate ostia (28% vs. 9%, p=0.016). Type 1B BAVs had more coronary artery disease than patients with type 1A BAV (36% vs. 19%, p=0.047). More left dominance was seen in BAV patients with CoA than in patients without (65% vs. 24%, p<0.05). CONCLUSIONS: The incidence of a left dominant coronary artery system and separate ostia was significantly related to BAVs with left-right fusion without a raphe (type 1B). These patients more often had significant coronary artery disease. In patients with BAV and CoA, left dominancy is more common.

Myocardial scar predicts monomorphic ventricular tachycardia but not polymorphic ventricular tachycardia or ventricular fibrillation in nonischemic dilated cardiomyopathy.

BACKGROUND: The relation between myocardial scar and different types of ventricular arrhythmias in patients with nonischemic dilated cardiomyopathy (NIDCM) is unknown. OBJECTIVES: The purpose of this study was to analyze the effect of myocardial scar, assessed by late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR), on the occurrence and type of ventricular arrhythmia in patients with NIDCM. METHODS: Consecutive patients with NIDCM who underwent LGE-CMR and implantable cardioverter-defibrillator (ICD) implantation at either of 2 centers were included. LGE was defined by signal intensity ≥35% of maximal signal intensity, subdivided into core and border zones (≥50% and 35%-50% of maximal signal intensity, respectively), and categorized according to location (basal or nonbasal) and transmurality. ICD recordings and electrocardiograms were reviewed to determine the occurrence and type of ventricular arrhythmia during follow-up. RESULTS: Of 87 patients (age 56 ± 13 y, 62% male, left ventricular ejection fraction 29% ± 12%), 55 (63%) had LGE (median 6.3 g, interquartile range 0.0-13.8 g). During a median follow-up of 45 months, monomorphic ventricular tachycardia (VT) occurred in 18 patients (21%) and polymorphic VT/ventricular fibrillation (VF) in 10 (11%). LGE predicted monomorphic VT (log-rank, P < .001), but not polymorphic VT/VF (log-rank, P = .40). The optimal cutoff value for the extent of LGE to predict monomorphic VT was 7.2 g (area under curve 0.84). Features associated with monomorphic VT were core extent, basal location, and area with 51%-75% LGE transmurality. CONCLUSIONS: Myocardial scar assessed by LGE-CMR predicts monomorphic VT, but not polymorphic VT/VF, in NIDCM. The risk for monomorphic VT is particularly high when LGE shows a basal transmural distribution and a mass ≥7.2 g. Importantly, patients without LGE on CMR remain at risk for potentially fatal polymorphic VT/VF.

CMR-based identification of critical isthmus sites of ischemic and nonischemic ventricular tachycardia.

OBJECTIVES: This study evaluates whether contrast-enhanced (CE) cardiac magnetic resonance (CMR) can be used to identify critical isthmus sites for ventricular tachycardia (VT) in ischemic and nonischemic heart disease. BACKGROUND: Fibrosis interspersed with viable myocytes may cause re-entrant VT. CE-CMR has the ability to accurately delineate fibrosis. METHODS: Patients who underwent VT ablation with CE-CMR integration were included. After the procedure, critical isthmus sites (defined as sites with a ≥11 of 12 pacemap, concealed entrainment, or VT termination during ablation) were projected on CMR-derived 3-dimensional (3D) scar reconstructions. The scar transmurality and signal intensity at all critical isthmus, central isthmus, and exit sites were compared to the average of the entire scar. The distance to >75% transmural scar and to the core-border zone (BZ) transition was calculated. The area within 5 mm of both >75% transmural scar and the core-BZ transition was calculated. RESULTS: In 44 patients (23 ischemic and 21 nonischemic, left ventricular ejection fraction 44 ± 12%), a total of 110 VTs were induced (cycle length 290 ± 67 ms). Critical isthmus sites were identified for 78 VTs (71%) based on ≥11 of 12 pacemaps (67 VTs), concealed entrainment (10 VTs), and/or termination (30 VTs). The critical isthmus sites, and in particular central isthmus sites, had high scar transmurality and signal intensity compared with the average of the entire scar. Of the pacemap, concealed entrainment, and termination sites, 74%, 100%, and 84% were within 5 mm of >75% transmural scar, and 67%, 100%, and 94% were within 5 mm of the core-BZ transition, respectively. The areas within 5 mm of both >75% transmural scar and the core-BZ transition (median 13% of LV) contained all concealed entrainment sites and 77% of termination sites. CONCLUSIONS: Both in ischemic and nonischemic VT, critical isthmus sites are typically located in close proximity to the CMR-derived core-BZ transition and to >75% transmural scar. These findings suggest that CMR-derived scar characteristics may guide to critical isthmus sites during VT ablation.

Pulse wave velocity and flow in the carotid artery versus the aortic arch: effects of aging.

PURPOSE: To explore differences in arterial stiffness of the aorta and carotid artery, assessed by pulse wave velocity (PWV), to evaluate the blood flow volume distribution towards the carotid circulation and to assess the effect of aging on the coupling between aortic and carotid PWV using velocity-encoded magnetic resonance imaging (MRI). MATERIALS AND METHODS: Sixteen adult younger volunteers (age <30 years) and 16 older volunteers (age >45 years) underwent 3T MRI examination to assess aortic and carotid flow volumes and PWV using the transit time method. RESULTS: Aortic versus carotid PWV-ratio was 1.2 for younger volunteers and 0.95 for older volunteers, demonstrating leveling of wall stiffness. Furthermore, flow volume per minute in the internal carotid artery was lower for older versus younger volunteers (mean volume 177 ± 42 mL/min/m(2) vs. 147 ± 32 mL/min/m(2), P = 0.028), whereas aorta and common carotid artery flow volumes were not different. Consequently, the fraction of blood flow volume towards the brain was smaller for older versus younger volunteers (61 ± 9% versus 71 ± 8%, P = 0.002). CONCLUSION: PWV-leveling between aorta and carotid artery at older age is associated with a reduction in blood flow volume towards the brain. Velocity-encoded MRI can be used to evaluate PWV and flow volume distribution in the aortic arch and the carotid circulation.