Derivation and Validation of a Novel Right-Sided Heart Failure Model After Implantation of Continuous Flow Left Ventricular Assist Devices: The EUROMACS (European Registry for Patients with Mechanical Circulatory Support) Right-Sided Heart Failure Risk Score.

BACKGROUND: The aim of the study was to derive and validate a novel risk score for early right-sided heart failure (RHF) after left ventricular assist device implantation. METHODS: The EUROMACS (European Registry for Patients with Mechanical Circulatory Support) was used to identify adult patients undergoing continuous-flow left ventricular assist device implantation with mainstream devices. Eligible patients (n=2988) were randomly divided into derivation (n=2000) and validation (n=988) cohorts. The primary outcome was early (<30 days) severe postoperative RHF, defined as receiving short- or long-term right-sided circulatory support, continuous inotropic support for ≥14 days, or nitric oxide ventilation for ≥48 hours. The secondary outcome was all-cause mortality and length of stay in the intensive care unit. Covariates found to be associated with RHF (exploratory univariate P<0.10) were entered into a multivariable logistic regression model. A risk score was then generated using the relative magnitude of the exponential regression model coefficients of independent predictors at the last step after checking for collinearity, likelihood ratio test, c index, and clinical weight at each step. RESULTS: A 9.5-point risk score incorporating 5 variables (Interagency Registry for Mechanically Assisted Circulatory Support class, use of multiple inotropes, severe right ventricular dysfunction on echocardiography, ratio of right atrial/pulmonary capillary wedge pressure, hemoglobin) was created. The mean scores in the derivation and validation cohorts were 2.7±1.9 and 2.6±2.0, respectively (P=0.32). RHF in the derivation cohort occurred in 433 patients (21.7%) after left ventricular assist device implantation and was associated with a lower 1-year (53% versus 71%; P<0.001) and 2-year (45% versus 58%; P<0.001) survival compared with patients without RHF. RHF risk ranged from 11% (low risk score 0-2) to 43.1% (high risk score >4; P<0.0001). Median intensive care unit stay was 7 days (interquartile range, 4-15 days) versus 24 days (interquartile range, 14-38 days) in patients without versus with RHF, respectively (P<0.001). The c index of the composite score was 0.70 in the derivation and 0.67 in the validation cohort. The EUROMACS-RHF risk score outperformed (P<0.0001) previously published scores and known individual echocardiographic and hemodynamic markers of RHF. CONCLUSIONS: This novel EUROMACS-RHF risk score outperformed currently known risk scores and clinical predictors of early postoperative RHF. This novel score may be useful for tailored risk-based clinical assessment and management of patients with advanced HF evaluated for ventricular assist device therapy.

Determinants of success and hemodynamic impact of balloon postdilatation of self-expanding transcatheter aortic valves.

OBJECTIVES: To explore the rate, the determinants of success, and the hemodynamic impact of balloon postdilatation (BPD) of self-expanding transcatheter heart valves (SE-THVs) BACKGROUND: BPD is commonly used to optimize valve expansion and reduce paravalvular leakage (PVL) after transcatheter aortic valve implantation (TAVI) without clearly knowing its hemodynamic benefits. METHODS: Patients (n = 307) who received a SE-THV were stratified according to whether a BPD was performed or not. Patients who received BPD were stratified according to the severity of PVL remaining after BPD into two groups: Successful BPD (≤mild PVL + BPD) and Failed BPD (moderate-severe PVL + BPD). RESULTS: BPD was performed in 121 patients (39.4%) and was successful in 106 patients (87.6% of attempts). A ratio of the postdilatation balloon diameter to the annulus diameter ≤0.95 was an independent predictor of BPD failure (OR: 10.72 [2.02-56.76], P = .005). Peak transvalvular pressure gradient (PG) was lower in the Successful BPD group (14[12-22] mm Hg) than in the Failed BPD group (18[16-23] mm Hg, P = .029), and did not rise in either group during follow-up (median [IQR], 364[161-739] days). CONCLUSION: BPD was performed in 39% of patients who received a SE-THV, and was successful in the majority of attempts. BPD failure was more likely in patients with a small postdilatation balloon-to-annulus diameter ratio. Effective BPD improved THV hemodynamic performance, and this was maintained in the intermediate-term post-TAVI.

Role of Computed Tomography in Planning the Appropriate X-Ray Gantry for Quantitative Aortography of Post-transcatheter Aortic Valve Implantation Regurgitation.

BACKGROUND: The clinical robustness of contrast-videodensitometric (VD) assessment of aortic regurgitation (AR) after transcatheter aortic valve implantation (TAVI) has been demonstrated. Correct acquisition of aortic root angiography for VD assessment, however, is hampered by the opacified descending aorta and by individual anatomic peculiarities. The aim of this study was to use preprocedural multi-slice computed tomography (MSCT) to optimize the angiographic projection in order to improve the feasibility of VD assessment.Methods and Results:In 92 consecutive patients, post-TAVI AR (i.e., left ventricular outflow tract [LVOT] AR) was assessed on aortic root angiograms using VD software. The patients were divided into 2 groups: The first group of 54 patients was investigated prior to the introduction of the standardized acquisition protocol; the second group of 38 consecutive patients after implementation of the standardized acquisition protocol, involving MSCT planning of the optimal angiographic projection. Optimal projection planning has dramatically improved the feasibility of VD assessment from 57.4% prior to the standardized acquisition protocol, to 100% after the protocol was implemented. In 69 analyzable aortograms (69/92; 75%), LVOT-AR ranged from 3% to 28% with a median of 12%. Inter-observer agreement was high (mean difference±SD, 1±2%), and the 2 observers' measurements were highly correlated (r=0.94, P<0.0001). CONCLUSIONS: Introduction of computed tomography-guided angiographic image acquisition has significantly improved the analyzability of the angiographic VD assessment of post-TAVI AR.

Inter-Technique Consistency and Prognostic Value of Intra-Procedural Angiographic and Echocardiographic Assessment of Aortic Regurgitation After Transcatheter Aortic Valve Implantation.

BACKGROUND: We investigated the relationship between intraprocedural angiographic and echocardiographic AR severity after TAVI, and the clinical robustness of angiographic assessment. Methods and Results: In 74 consecutive patients, the echocardiographic circumferential extent (CE) of the paravalvular regurgitant jet was retrospectively measured and graded based on the VARC-2 cut-points; and angiographic post-TAVI AR was retrospectively quantified using contrast videodensitometry (VD) software that calculates the ratio of the contrast time-density integral in the LV outflow tract to that in the ascending aorta (LVOT-AR). Seventy-four echocardiograms immediately after TAVI were analyzable, while 51 aortograms were analyzable for VD. These 51 echocardiograms and VD were evaluated. Median LVOT-AR across the echocardiographic AR grades was as follows: none-trace, 0.07 (IQR, 0.05-0.11); mild, 0.12 (IQR, 0.09-0.15); and moderate, 0.17 (IQR, 0.15-0.22; P<0.05 for none-trace vs. mild, and mild vs. moderate). LVOT-AR strongly correlated with %CE (r=0.72, P<0.0001). At 1 year, the rate of the composite end-point of all-cause death or HF re-hospitalization was significantly higher in >mild AR patients compared with no-mild AR on intra-procedural echocardiography (41.5% vs. 12.4%, P=0.03) as well as in patients with LVOT-AR >0.17 compared with LVOT-AR ≤0.17 (59.5% vs. 16.6%, P=0.03). CONCLUSIONS: VD (LVOT-AR) has good intra-procedural inter-technique consistency and clinical robustness. Greater than mild post-TAVI AR, but not mild post-TAVI AR, is associated with late mortality.

Angiographic assessment of aortic regurgitation by video-densitometry in the setting of TAVI: Echocardiographic and clinical correlates.

OBJECTIVES: We sought to investigate a new angiographic method for aortic regurgitation (AR) severity assessment in the setting of transcatheter aortic valve implantation (TAVI). BACKGROUND: AR after TAVI is common but challenging to quantitate, especially in the cath-lab. METHODS: In 228 patients, AR was quantitated before and after TAVI by echocardiography and by video-densitometric analysis of aortograms. Contrast time-density curves for the aortic root (the reference region) and the left ventricular outflow tract, LVOT were generated. LVOT-AR was calculated as the area under the curve of the LVOT as a fraction of the area under the curve of the reference region. RESULTS: LVOT-AR was 0.10 ± 0.08, 0.13 ± 0.10 and 0.28 ± 0.14 in none-trace, mild and moderate-severe post-TAVI AR as defined by echocardiography (P < 0.001) and a cutpoint of >0.17 corresponded to moderate-severe AR on echocardiography (area under the curve = 0.84). At follow-up (median, 496 days), patients with LVOT-AR ≤ 0.17 showed a significant reduction of LV mass index (LVMi; 121 [95-148] vs. 140 [112-169] g/m2 , P = 0.009) and the prevalence of LV hypertrophy (LVH; 64 vs. 88%, P = 0.001) compared to baseline. In patients with LVOT-AR > 0.17, LVMi (149 [121-178] vs. 166 [144-188] g/m2 , P = 0.14) and the prevalence of LVH (74 vs. 87%, P = 0.23) did not show a significant change. Compared to patients with LVOT-AR ≤ 0.17, those with LVOT-AR > 0.17 had an increased 30-day (16.4% vs. 7.1%, P = 0.035) and one year mortality (32.9 vs. 14.2%, log rank P value = 0.001; HR: 2.690 [1.461-4.953], P = 0.001). CONCLUSIONS: LVOT-AR > 0.17 corresponds to greater than mild AR as defined by echocardiography and predicts impaired LV reverse remodeling and increased early and midterm mortality after TAVI. © 2017 Wiley Periodicals, Inc.

Accuracy of an automated transthoracic echocardiographic tool for 3D assessment of left heart chamber volumes.

AIMS: The feasibility and accuracy of an automated adaptive algorithm (Heart Model) for the quantification of left heart chamber volumes and left ventricular ejection fraction has been reported earlier. An improved version of the algorithm is available, and we aimed to test its accuracy compared with manual 3D echocardiography. METHODS AND RESULTS: Apical 3D transthoracic datasets were obtained in 67 patients. Acquisitions covering ventricles and atria were performed for analysis using the automated software (Heart Model, Philips Healthcare). In addition, acquisitions focused on the left ventricle were acquired for left ventricle manual 3D measurements (QLAB 10 3DQA, Philips Healthcare). Automated results using endocardial contouring settings at 50% showed a strong correlation with manual 3D measurements (r=.84-.97). Left ventricular end-diastolic volumes were underestimated (bias -5.9 mL, LOA ±38.5 mL), with no significant differences in other parameters. Intra-observer variability using the automated algorithm was zero for all parameters given the lack of human interaction. Manual corrections of the automatic algorithm introduced small but significant differences in volumes but not in ejection fraction when compared with automatic results. Manually corrected results of the automatic algorithm were not significantly different from those obtained with manual 3D echocardiography, except for a small underestimation of left atrial volumes (bias -2.1 mL, LOA ±15.2 mL). CONCLUSIONS: The current improved version of the automated adaptive algorithm is accurate for the assessment of left heart chamber volumes, albeit a small underestimation of left ventricular end-diastolic volume is seen, when compared with manual 3D echocardiography.

Adjudicating paravalvular leaks of transcatheter aortic valves: a critical appraisal.

Paravalvular leakage (PVL) is an important complication of transcatheter aortic valve implantation (TAVI). It contributed to the erosion of the clinical benefits of TAVI and confidence of its adoption as a default therapy in low surgical-risk patients. Newer TAVI technologies are provided with effective paravalvular sealing as well as retrieval/reposition mechanisms that are believed to considerably lower the risk of PVL. Meanwhile, developments in timely detection and accurate quantitation of PVL remain lagging behind those technological advances. The Valve Academic Research Consortium-standardized criteria of PVL assessment are based on echocardiography and are, according to experts' opinion, not adequately validated. Peri-procedural diagnosis, based on angiographic, haemodynamic, and/or echocardiographic methods, is so far without standardization of acquisition or interpretation. The aim of this report is to review the strengths and limitations of the current technologies used for PVL adjudication. Understanding this strengths/limitations ratio is important to define an appropriate scheme for detection and quantitation of PVLs both in clinical trials and in routine clinical practice.

Limitations and difficulties of echocardiographic short-axis assessment of paravalvular leakage after corevalve transcatheter aortic valve implantation.

To make assessment of paravalvular aortic leakage (PVL) after transcatheter aortic valve implantation (TAVI) more uniform the second Valve Academic Research Consortium (VARC) recently updated the echocardiographic criteria for mild, moderate and severe PVL. In the VARC recommendation the assessment of the circumferential extent of PVL in the short-axis view is considered critical. In this paper we will discuss our observational data on the limitations and difficulties of this particular view, that may potentially result in overestimation or underestimation of PVL severity.

A Novel 13-Segment Standardized Model for Assessment of Right Ventricular Function Using Two-Dimensional iRotate Echocardiography.

AIMS: The aim of this study was to evaluate the feasibility of transthoracic two-dimensional (2D) iRotate, a new echo modality, to assess the whole right ventricle (RV) from a single transducer position based on anatomic landmarks. METHODS AND RESULTS: The anatomic landmarks were first defined based on three-dimensional echocardiographic datasets using multiplane reconstruction analyses. Thereafter, we included 120 healthy subjects (51% male, age range 21-67 years). Using 2D iRotate, four views of the RV could be acquired based on these landmarks. The anterior, lateral, inferior wall (divided into three segments: basal-mid-apical), and right ventricular outflow tract (RVOT) anterior wall of the RV were determined. The feasibility of visualization of RV segments and tricuspid annular plane systolic excursion (TAPSE) and tissue Doppler imaging (TDI) measurements were assessed. To evaluate this model for diseased RVs, a small pilot study of 20 patients was performed. In 98% of healthy subjects and 100% of patients, iRotate mode was feasible to assess the RV from one single transducer position. In total, 86% and 95%, respectively, of the RV segments could be visualized. The visualization of the RVOT anterior wall was worse 23% and 75%, respectively. TAPSE and TDI measurements on all four views were feasible 93% and 92%, respectively, of the healthy subjects and in 100% of the patients. CONCLUSION: With 2D iRotate, a comprehensive evaluation of the entire normal and diseased RV is feasible from a fixed transducer position based on anatomic landmarks. This is less time-consuming than the multiview approach and enhances accuracy of RV evaluation. Imaging of the RVOT segment remains challenging.

Assessment of subendocardial contractile function in aortic stenosis: a study using speckle tracking echocardiography.

BACKGROUND: Angina and an electrocardiographic strain pattern are potential manifestations of subendocardial ischemia in aortic stenosis (AS). Left ventricular (LV) twist is known to increase proportionally to the severity of AS, which may be a result of loss of the inhibiting effect of the subendocardial fibers due to subendocardial dysfunction. It has also been shown that the ratio of LV twist to circumferential shortening of the endocardium (twist-to-shortening ratio [TSR]) is a reliable parameter of subendocardial dysfunction. The aim of this study was to investigate whether these markers are increased in AS patients with angina and/or electrocardiographic strain. METHODS: The study comprised 60 AS patients with an aortic valve area <2.0 cm(2) and LV ejection fraction >50%, and 30 healthy-for age and gender matched-control subjects. LV rotation parameters were determined by speckle tracking echocardiography. RESULTS: Comparison of patients without angina and strain (n = 22), with either angina or strain (n = 28), and with both angina and strain (n = 8), showed highest peak systolic LV apical rotation, peak systolic LV twist, and TSR, in patients with more signs of subendocardial ischemia. In a multivariate linear regression model, only severity of AS and the presence of angina and/or strain could be identified as independent predictors of peak systolic LV twist and TSR. CONCLUSIONS: Peak systolic LV twist and TSR are increased in AS patients and related to the severity of AS and symptoms (angina) or electrocardiographic signs (strain) compatible with subendocardial ischemia.

Diastolic abnormalities in normal phenotype hypertrophic cardiomyopathy gene carriers: a study using speckle tracking echocardiography.

BACKGROUND: Tissue Doppler imaging (TDI) of the mitral annulus has been proposed as an alternative for the identification of hypertrophic cardiomyopathy (HCM) genetically affected subjects without left ventricular hypertrophy (G+/LVH-). Unfortunately, conflicting results have been described in the literature, potentially caused by the angle-dependency of TDI. This study sought to assess abnormalities in mitral annular velocities in G+/LVH- subjects as detected by speckle tracking echocardiography (STE). METHODS: The study population consisted of 23 consecutive genotyped family members without major or minor criteria for the diagnosis of HCM (mean age 37 ± 13 years, 9 men) and 23 healthy volunteers (age 38 ± 12 years, 12 men) who prospectively underwent STE. RESULTS: There were no significant differences in global peak systolic annular velocity (7.4 ± 1.2 vs. 7.1 ± 1.0 cm/sec) and early diastolic annular velocity (10.2 ± 2.5 vs. 11.3 ± 2.2 cm/sec) between G+/LVH- and control subjects. Global peak late diastolic annular velocity was higher in G+/LVH- subjects (8.1 ± 1.7 vs. 5.7 ± 1.1 cm/sec, P < 0.001). Regionally, this difference was seen in all 6 studied LV walls. CONCLUSIONS: This STE study confirms our previous TDI observations on increased peak late diastolic annular velocities in G+/LVH- subjects. Because of the complete overlap in early diastolic annular velocities this parameter cannot be used in the genotypes we studied to differentiate genotype (+) from genotype (-) individuals.

The prognosis of implantable defibrillator patients treated with cardiac resynchronization therapy: comorbidity burden as predictor of mortality.

AIMS: Comorbidity, such as myocardial infarction, diabetes, and renal failure, plays a pivotal role in the prognosis of a patient with arrhythmias. However, data on the prognostic impact of comorbiditiy in heart failure patients with cardiac resynchronization therapy and defibrillation (CRT-D) are scarce. The purpose of this study was to determine the impact of comorbidity on survival in CRT-D patients. METHODS AND RESULTS: The study population consisted of 463 heart failure patients who received a CRT-D between 1999 and 2008 in Rotterdam and Basel. The Charlson comorbidity index (CCI) is often used as an adjusting variable in prognostic models. The Cox proportional hazards analysis was performed to determine the independent effect of comorbidity on survival. During a median follow-up of 30.5 months, 85 patients died. Mortality rates at 1 and 7 years were 6.3 and 32.3%. Cumulative incidence of implantable cardioverter defibrillator (ICD) therapy at 7 years was 50%, and death without ICD therapy was observed in 9% of patients. At least three comorbid conditions were observed in 81% of patients. Patients who died had a higher CCI score compared with those who survived (3.9 ± 1.5 vs. 2.9 ± 1.5; P < 0.001). An age-adjusted CCI score ≥ 5 was a predictor of mortality (hazard ratio 3.69, 95% CI 2.06-6.60; P < 0.001) independent from indication for ICD therapy, and from ICD interventions during the clinical course. CONCLUSION: Comorbidity is often present in heart failure patients, and a high comorbidity burden was a significant predictor of mortality in CRT-D recipients. Comorbidity cannot predict appropriate ICD therapy. Death without prior ICD therapy occurs in a minor proportion of patients.

Contrast echocardiography improves interobserver agreement for wall motion score index and correlation with ejection fraction.

BACKGROUND: The wall motion score index (WMSI) is a surrogate for left ventricular ejection fraction (LV-EF), which becomes unreliable in poor echo windows. The value of contrast LV opacification (LVO) for WMSI assessment is not well known. OBJECTIVES: We sought to compare interobserver agreement for WMSI and the correlation between the LVO-WMSI and LV-EF using two-dimensional second harmonic (SH) and LVO echocardiography. METHODS: The study comprised 100 consecutive patients (57 ± 13 years, 85% males). Two independent physicians assessed LV segmental quality and wall motion for both the SH and LVO studies according to a 17-segment model. Systolic wall motion was defined as: normokinesia, hypokinesia (systolic inward endocardial motion <7 mm), akinesia, and dyskinesia. LV-EF was assessed from the LVO images according to the biplane modified Simpson's method. RESULTS: Of the 1,700 analyzed segments, 453 (26.6%) were poorly visualized with SH imaging, and 173 (10.2%) with LVO (P < 0.0001). The two observers agreed on segmental wall motion score in 1,299 segments (agreement 76%, Kappa 0.60) with SH imaging and in 1,491 segments (agreement 88%, Kappa 0.78) with LVO. Interobserver correlation (r(2) ) was 0.86 for the SH-WMSI and 0.93 for the LVO-WMSI. The limits-of-agreement for interobserver LVO-WMSI (mean difference -1.0%± 6.8%, agreement -14.6%, 12.6%) was lower than that for SH-WMSI (mean difference -2.3%± 10.1%, agreement -22.5, 17.9). The LVO-WMSI correlated well with LV-EF (r(2) = 0.71). LV-EF could be estimated according to the formula 1.01 - 0.32 × WMSI. CONCLUSION: Echo-contrast improves interobserver agreement for wall motion scoring and the WMSI. The LVO-imaged WMSI correlates well with LV-EF.

Alterations in left ventricular untwisting with ageing.

BACKGROUND: In order to gain further insight into age-associated changes of left ventricular (LV) diastolic function, the purpose of the current study was to investigate alterations in LV untwisting with ageing. METHODS AND RESULTS: The study comprised 75 healthy volunteers, classified into 3 groups: age 16-35 (n=25), 36-55 (n=25) and 56-75 (n=25) years. LV untwisting (as a percentage of peak systolic twist) at 5%, 10%, 15% and 50% of diastole, peak diastolic untwisting velocity, time-to-peak diastolic untwisting velocity and untwisting rate (mean untwisting velocity during the time interval from peak systolic twist to mitral valve opening) were assessed using speckle-tracking echocardiography. Untwisting at 5%, 10%, 15% and 50% of diastole decreased with ageing. Although the peak diastolic untwisting velocity and untwisting rate were not significantly different between the age groups, when normalized for LV peak systolic twist, these parameters decreased with advancing age (both P<0.01). Time-to-peak diastolic untwisting velocity increased with ageing (P<0.01). CONCLUSIONS: Impairment of the relative peak diastolic untwisting velocity and untwisting rate, resulting in delayed LV untwisting, may help to explain diastolic dysfunction in the elderly. (Circ J 2010; 74: 101 - 108).

Left ventricular untwisting in restrictive and pseudorestrictive left ventricular filling: novel insights into diastology.

BACKGROUND: Conceptually, an ideal therapeutic agent should target the underlying mechanisms that cause left ventricular (LV) diastolic dysfunction. The objective of our study was to gain further insight into the mechanics of diastology by comparison of LV untwisting measured by speckle tracking echocardiography (STE) in young healthy adults with normal and "pseudorestrictive" LV filling, and dilated cardiomyopathy (DCM) patients with "true restrictive" LV filling. METHODS: The study comprised 20 healthy volunteers with a Doppler LV-inflow pattern compatible with restrictive LV filling but a diastolic early phase filling velocity/early diastolic velocity of the mitral annulus (E/Em) ratio <8 ("pseudorestrictive"), 20 for age and gender-matched healthy volunteers with normal LV filling and an E/Em ratio <8, and 10 DCM patients with "true restrictive" LV filling and an E/Em ratio >15. LV untwisting parameters were determined by STE. RESULTS: Compared to healthy subjects, DCM patients had decreased peak diastolic untwisting velocity (-62 +/- 33 degrees/s vs -113 +/- 25 degrees/s, P < 0.01) and untwisting rate (-15 +/- 9 degrees/s vs -51 +/- 24 degrees/s, P < 0.01). Compared to healthy subjects with normal LV filling, healthy subjects with "pseudorestrictive" LV filling had increased peak diastolic untwisting velocity (-123 +/- 25 degrees/s vs -104 +/- 30 degrees/s, P < 0.05) and untwisting rate (-59 +/- 23 degrees/s vs -44 +/- 22 degrees/s, P < 0.05). CONCLUSION: Faster LV untwisting plays a pivotal role in the rapid early diastolic filling occasionally seen in young healthy individuals. In contrast, in DCM patients untwisting is severely delayed and this impairment to utilize suction may reduce LV filling.

Thrombus in a normal left ventricle: a cardiac manifestation of pheochromocytoma.

In this case report, a patient with a thrombus in a normal-sized and functional left ventricular is described. The thrombus was most likely formed during pheochromocytoma crisis with severe transient wall motion abnormalities.

Recovery of long-axis left ventricular function after aortic valve replacement in patients with severe aortic stenosis.

BACKGROUND: Patients with aortic stenosis (AS) should undergo aortic valve replacement (AVR) before irreversible LV dysfunction has developed. Assessment of long-axis left ventricular (LV) function may assist in proper timing of AVR. OBJECTIVES: To assess serial changes in long-axis LV function before and after AVR in patients with severe AS and preserved LV ejection fraction. METHODS: The study comprised 27 consecutive patients (mean age 64.9 ± 11.7 years, 15 males) with symptomatic severe AS, scheduled for AVR. Seventeen subjects without known cardiac disease, matched for age, gender, LV ejection fraction and cardiovascular risk factors, served as a control group. Long-axis LV function assessment was done with tissue Doppler imaging at 3 weeks, 6 months, and 12 months after AVR. RESULTS: Mean aortic valve area in the AS group was 0.70 ± 0.24 cm². Pre-AVR peak systolic mitral annular velocities were significantly lower compared to controls (6.7 ± 1.5 vs. 8.9 ± 2.0 cm/s, P < 0.05). Post-AVR peak systolic mitral annular velocities improved to 9.1 ± 2.9 at 3 weeks, 8.6 ± 2.7 at 6 months, and 8.1 ± 1.7 cm/s at 12 months (P < 0.05). Improvements were seen over the whole range of pre-AVR peak systolic mitral annular velocities. Patients with improved Sm after AVR (defined as ≥ 10% compared to baseline values) did not differ in baseline characteristics as compared to those who did not improve. CONCLUSIONS: In patients with severe AS and preserved LV ejection fraction, abnormal systolic mitral annular velocities improve after AVR, independent of the pre-AVR value.

Disease penetrance and risk stratification for sudden cardiac death in asymptomatic hypertrophic cardiomyopathy mutation carriers.

AIMS: To investigate the outcome of cardiac evaluation and the risk stratification for sudden cardiac death (SCD) in asymptomatic hypertrophic cardiomyopathy (HCM) mutation carriers. METHODS AND RESULTS: Seventy-six HCM mutation carriers from 32 families identified by predictive DNA testing underwent cardiac evaluation including history, examination, electrocardiography, Doppler echocardiography, exercise testing, and 24 h Holter monitoring. The published diagnostic criteria for HCM in adult members of affected families were used to diagnose HCM. Thirty-three (43%) men and 43 (57%) women with a mean age of 42 years (range 16-79) were examined; in 31 (41%) HCM was diagnosed. Disease penetrance was age related and men were more often affected than women (P = 0.04). Myosin Binding Protein C (MYBPC3) mutation carriers were affected at higher age than Myosin Heavy Chain (MYH7) mutation carriers (P = 0.01). Risk factors for SCD were present in affected and unaffected carriers. CONCLUSION: Hypertrophic cardiomyopathy was diagnosed in 41% of carriers. Disease penetrance was age dependent, warranting repeated cardiologic evaluation. The MYBPC3 mutation carriers were affected at higher age than MYH7 mutation carriers. Risk factors for SCD were present in carriers with and without HCM. Follow-up studies are necessary to evaluate the effectiveness of risk stratification for SCD in this population.

[Evaluation of the mitral valve by transthoracic real-time three-dimensional echocardiography]. / A mitralis billentyu vizsgálata transthoracalis real-time háromdimenziós echokardiográfiával.

Recently, echocardiography is the most widely used routine non-invasive diagnostic method, with which morphology and function of the mitral valve can be characterized. The aim of this review is to demonstrate the role of one of the newest echocardiographic developments, the transthoracic real-time three-dimensional echocardiography in the evaluation of mitral valve.

Diagnosis of biventricular non-compaction cardiomyopathy by real-time three-dimensional echocardiography.

Non-compaction of the ventricular myocardium is a recently recognized rare disorder of the endomyocardial morphogenesis. The disease can be characterized by systolic and diastolic heart failure, ventricular arrhythmias and systemic embolization. The present case suggests the clinical role of real-time three-dimensional echocardiography in the spatial evaluation of both ventricles in suspected biventricular non-compaction cardiomyopathy.