Three-dimensional echocardiography for the assessment of left ventricular geometry and papillary muscle morphology in hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HC) is characterized by left ventricular (LV) hypertrophy and associated with papillary muscle (PM) abnormalities. The aim of this study was to evaluate the utility of three-dimensional echocardiography (3DE) for the geometric assessment of LV hypertrophy and PM morphology. METHODS: The study included 24 patients with an established diagnosis of HC and 31 healthy controls. 3DE was performed using an iE33 or EPIQ 7C ultrasound system with an X5-1 transducer. QLAB software was used for the 3D analysis of LV wall thickness (LVWT) and PM morphology and hypertrophy; the number and cross-sectional area (CSA) of anterolateral and posteromedial PMs; and the presence of bifid or accessory PMs. RESULTS: Patients with HC had a larger LVWT compared to controls in all segments (p < 0.001), and LVWT was largest in the midventricular septal segment (2.12 ± 0.68 cm). The maximum LVWT followed a spiral pattern from the LV base to the apex. The CSA of both anterolateral and posteromedial PMs was larger in patients with HC than in controls (1.92 vs. 1.15 cm2; p = 0.001 and 1.46 vs. 1.08 cm2; p = 0.033, respectively). The CSA of the posteromedial PM was larger in patients with LVOT obstruction than in those without (2.64 vs 1.16 cm2, p = 0.021). CONCLUSIONS: 3DE allows the assessment of LV geometry and PM abnormalities in patients with HC. 3DE demonstrated that the maximum hypertrophy was variable and generally located in a spiral from the LV base to the apex.

Transthoracic 3D echocardiographic left heart chamber quantification in patients with bicuspid aortic valve disease.

Integration of volumetric heart chamber quantification by 3D echocardiography into clinical practice has been hampered by several factors which a new fully automated algorithm (Left Heart Model, (LHM)) may help overcome. This study therefore aims to evaluate the feasibility and accuracy of the LHM software in quantifying left atrial and left ventricular volumes and left ventricular ejection fraction in a cohort of patients with a bicuspid aortic valve. Patients with a bicuspid aortic valve were prospectively included. All patients underwent 2D and 3D transthoracic echocardiography and computed tomography. Left atrial and ventricular volumes were obtained using the automated program, which did not require manual contour detection. For comparison manual and semi-automated measurements were performed using conventional 2D and 3D datasets. 53 patients were included, in four of those patients no 3D dataset could be acquired. Additionally, 12 patients were excluded based on poor imaging quality. Left ventricular end-diastolic and end-systolic volumes and ejection fraction calculated by the LHM correlated well with manual 2D and 3D measurements (Pearson's r between 0.43 and 0.97, p < 0.05). Left atrial volume (LAV) also correlated significantly although LHM did estimate larger LAV compared to both 2DE and 3DE (Pearson's r between 0.61 and 0.81, p < 0.01). The fully automated software works well in a real-world setting and helps to overcome some of the major hurdles in integrating 3D analysis into daily practice, as it is user-independent and highly reproducible in a group of patients with a clearly defined and well-studied valvular abnormality.

Relation between calcium burden, echocardiographic stent frame eccentricity and paravalvular leakage after corevalve transcatheter aortic valve implantation.

AIMS: Paravalvular aortic leakage (PVL) after transcatheter aortic valve implantation (TAVI) is a complication with potentially severe consequences. The relation between native aortic root calcium burden, stent frame eccentricity and PVL was not studied before. METHODS AND RESULTS: Two-hundred-and-twenty-three consecutive patients with severe aortic stenosis who underwent TAVI with a Medtronic CoreValve System© and who had available pre-discharge transthoracic echocardiography were studied. Echocardiographic stent inflow frame eccentricity was defined as major-minor diameter in a short-axis view >2 mm. PVL was scored according to the updated Valve Academic Research Consortium (VARC-2) recommendations. In a subgroup of 162 (73%) patients, the calcium Agatston score was available. Stent frame eccentricity was seen in 77 (35%) of patients. The correlation between the Agatston score and stent frame eccentricity was significant (ρ = 0.241, P = 0.003). Paravalvular leakage was absent in 91 cases (41%), mild in 67 (30%), moderate in 51 (23%), and severe in 14 (6%) cases. The correlation between stent frame eccentricity and PVL severity was significant (ρ = 0.525, P < 0.0001). There was a relation between particular eccentric stent frame shapes and the site of PVL. CONCLUSION: Calcification of the aortic annulus is associated with a subsequent eccentric shape of the CoreValve prosthesis. This eccentric shape results in more PVL, with the localization of PVL related to the shape of stent frame eccentricity.

Delayed and decreased LV untwist and unstrain rate in mutation carriers for hypertrophic cardiomyopathy.

BACKGROUND: The echocardiographic focus to detect abnormalities in genetically hypertrophic cardiomyopathy (HCM) affected subjects without left ventricular (LV) hypertrophy (G+/LVH-) has been on diastolic abnormalities in transmitral flow and longitudinal myocardial function with tissue Doppler imaging. The aim of this study was to assess diastolic LV unstrain and untwist. METHODS AND RESULTS: Forty-one consecutive genotyped family members of HCM patients (mean age 37 ± 11 years, 16 men) and 41 age- and gender-matched healthy volunteers underwent speckle-tracking echocardiography to measure untwist and unstrain. No significant differences between G+/LVH- and control subjects were seen in maximal systolic twist and global longitudinal strain. In diastole, the early peak untwist rate was significantly lower in G+/LVH- subjects compared with control subjects (62 ± 19°s - 1 vs. 76 ± 30°s - 1, P <0.05), whereas the late peak untwist rate tended to be higher. Untwist from maximal twist until the first 20% of diastole was delayed in G+/LVH- subjects (39.3 ± 12.9% vs. 51.3 ± 15.6%, P <0.005). Late diastolic unstrain rate was significantly higher in G+/LVH- subjects in the inferoseptal wall (111 ± 33 s - 1 vs. 94 ± 32 s - 1, P = 0.024), the inferolateral wall (105 ± 42 vs. 75 ± 35 s - 1, P = 0.007) and the anteroseptal wall (97 ± 26 vs. 80 ± 23 s - 1, P = 0.010). Unstrain from maximal twist until the first 20% of diastole was delayed in G+/LVH- subjects in the inferoseptal (18.9 ± 14.0% vs. 30.1 ± 17.7%, P = 0.005), inferolateral (27.1 ± 16.3% vs. 39.2 ± 18.0%, P = 0.015) and anteroseptal (19.1 ± 14.7% vs. 35.8 ± 18.5%, P = 0.0003) segments. CONCLUSIONS: In mutation carriers, for HCM LV, untwist and unstrain are delayed and untwist rate and unstrain rate are decreased.

Quantitative assessment of the entire right ventricle from one acoustic window: an attractive approach.

AIMS: The aim of this prospective study was to evaluate the feasibility and establish normal values of functional right ventricle (RV) parameters as assessed in our four, long-axis view RV model using iRotate echocardiography. Furthermore, we evaluated the potential use of this model in patients with abnormally loaded RVs. METHOD AND RESULTS: One hundred and fifty-five healthy subjects aged 20-72 years (≥28 subjects per decile) were prospectively recruited. We used non-dedicated RV speckle-tracking software to test the feasibility and to establish normal range values of peak systolic global longitudinal RV strain (RV-GLS) from the RV free-walls (septum was excluded). Also normal range values for: dimensions, tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (TDI-S') were established. The feasibility of RV-GLS was 88, 84, and 62%, respectively, in the lateral, inferior, and anterior free wall. Mean RV-GLS normal values were -24.5 ± 4.9% for lateral wall and -25.4 ± 5.0% for anterior wall. Mean RV-GLS in the inferior wall was -23.2 ± 4.4% in the aortic (Ao) view and -20.7 ± 5.0% in the coronal (CV) view. The feasibility of mean RV-GLS was 100% in the anterior, lateral, and inferior walls in abnormally dilated RVs. CONCLUSION: The feasibility of all RV parameters assessed in the four-view iRotate model is good to excellent. Normal values for RV dimension and function of the anterior, inferior, and lateral RV walls have been established. Further studies and dedicated RV speckle-tracking software are warranted to discover the full potential of this new technique.

Quantitative Doppler for Estimation of Paravalvular Leakage after Transcatheter Aortic Valve Implantation.

BACKGROUND: The echocardiographic grading of paravalvular aortic leakage (PVL) after transcatheter aortic valve implantation (TAVI) severity is challenging. The study aim was to assess the value of quantitative Doppler echocardiography to monitor PVL severity. METHODS: A total of 100 subjects was enrolled in the study, including 65 consecutive patients who had undergone TAVI with a CoreValve prosthesis and without valvular aortic regurgitation, and 35 normal controls. The PVL volume was calculated using the quantitative Doppler method as the difference of left and right ventricular stroke volume (SV). PVL severity was assessed both visually and quantitatively as the circumferential extent on a short-axis view (SAX). RESULTS: The inter-observer variabilities for SVs in TAVI patients were disappointing: 14 ± 11% for the left ventricular SV and 18 ± 14% for right ventricular SV. The correlation (r2) between the averaged regurgitant PVL volume and circumferential SAX extent of PVL was 0.02 (p = NS). The relationship between PVL volumes and categories, defined quantitatively by the circumferential SAX extent of PVL and qualitatively by visual assessment of severity of PVL were poor. The results improved when only patients with optimal quality images were included but were still statistically non-significant. CONCLUSIONS: The relationship between calculated PVL volume in TAVI patients and other estimates of PVL severity was poor, most likely due to intrinsic errors made in the quantitative Doppler method. Therefore, one should be prudent to include the quantitative Doppler method in TAVI patients in clinical trials and clinical decision-making, in particular in patients with reduced image quality.

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.

Normal myocardial strain values using 2D speckle tracking echocardiography in healthy adults aged 20 to 72 years.

AIMS: Evaluation of left ventricular (LV) myocardial deformation by speckle tracking echocardiography (STE) is useful for clinical and research purposes. However, strain measurements depend on the used software. Normative data for QLAB 10 (Philips) are scarce. Additionally, little is known about the influence of anthropometric factors. We aimed to establish normal adult STE-derived strain and strain rate values and to evaluate associations with anthropometrics. METHODS: One hundred fifty-five healthy subjects aged 20 to 72 years (≥28 subjects per decile) were prospectively gathered and examined with electrocardiography and two-dimensional echocardiography. With STE, we assessed peak systolic LV global longitudinal strain (GLS), segmental longitudinal strain, and strain rate from the three standard apical views. RESULTS: We included 147 healthy subjects (age 44.6±13.7 years, 50% female, GLS -20.8±2.0%). Men had significantly lower GLS than women (-20.1±1.8% vs -21.5±2.0%, P<.001). GLS was significantly lower in subjects with age above 55 years (P=.029), higher blood pressure (P<.001), higher body surface area (BSA, P<.001), larger LV end-systolic and end-diastolic volumes (both P<.001), lower LV ejection fraction (P<.001), and some indices of diastolic function. After multivariable regression analysis, the correlation with systolic blood pressure, E-wave, and LV end-systolic volume remained significant. The systolic strain rates of most segments correlated with BSA. CONCLUSIONS: Our study resulted in normative LV GLS values assessed with QLAB 10. Male sex, higher BSA, and higher blood pressure negatively influence GLS. Therefore, these factors should be taken into account for strain interpretation in clinical practice.

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.

Prediction of paravalvular leakage after transcatheter aortic valve implantation.

Significant paravalvular leakage (PVL) after transcatheter aortic valve implantation (TAVI) is related to patient mortality. Predicting the development of PVL has focused on computed tomography (CT) derived variables but literature targeting CoreValve devices is limited, controversial, and did not make use of standardized echocardiographic methods. The study included 164 consecutive patients with severe aortic stenosis that underwent TAVI with a Medtronic CoreValve system©, with available pre-TAVI CT and pre-discharge transthoracic echocardiography. The predictive value for significant PVL of the CT-derived Agatston score, aortic annulus size and eccentricity, and "cover index" was assessed, according to both echocardiographic Valve Academic Research Consortium (VARC) criteria and angiographic Sellers criteria. Univariate predictors for more than mild PVL were the maximal diameter of the aortic annulus size (for both angiographic and echocardiographic assessment of PVL), cover index (for echocardiographic assessment of PVL only), and Agatston score (for both angiographic and echocardiographic assessment of PVL). The aortic annulus eccentricity index was not predicting PVL. At multivariate analysis, Agatston score was the only independent predictor for both angiographic and echocardiographic assessment of PVL. Agatston score is the only independent predictor of PVL regardless of the used imaging technique for the definition of PVL.

Single-beat real-time three-dimensional echocardiographic automated contour detection for quantification of left ventricular volumes and systolic function.

To assess the feasibility and accuracy in measuring left ventricular (LV) end-diastolic volume (EDV), end-systolic volume (ESV) and ejection fraction (EF) with Siemens single-beat real-time 3D transthoracic echocardiography. The LV volumes and EF were measured in 3D datasets acquired by six imaging modes (time-1-harmonic (T1H), time-1-fundamental, time-2-harmonic, time-2-fundamental, space-1-harmonic (S1H), and space-1-fundamental) in 41 patients using the automated contouring algorithm and compared with manually corrected 3DE QLAB measurements. The main determinates of the temporal and spatial resolutions of 3D datasets acquired were the fundamental and harmonic modes. Consequently, the S1H mode had the lowest volume rate and highest spatial resolution. Compared with the 3DE QLAB analysis, the S1H mode resulted in the best LV volumes and EF estimates in all patients (0 ± 10 % for EF, -7 ± 44 ml for EDV, -7 ± 39 ml for ESV) and in the 10 patients with correct LV contour tracking according to a visual assessment from the multiplanar reconstruction views in all six modes (0 ± 9 % for EF, -3 ± 23 ml for EDV, -2 ± 14 ml for ESV). The T1H mode was the best alternative. Overall 28 patients (68 %) could be analysed automatically and satisfyingly with the S1H and T1H modes: 0 ± 8 % (EF), 0 ± 27 ml (EDV) and -1 ± 16 ml (ESV). The accuracy of the Siemens automated RT-3D algorithm in measuring LV volumes and EF is significantly influenced by the different imaging modes. The S1H mode may be the preferred 3D acquisition mode, supplemented by the T1H mode in enlarged LVs that do not fit in the S1H acquisition sector.

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.

Regional left ventricular rotation and back-rotation in patients with reverse septal curvature hypertrophic cardiomyopathy.

AIMS: This study sought to investigate regional left ventricular (LV) rotation in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: The study comprised 44 patients with HCM with a typical reverse septal curvature (age 40 ± 14 years, 33 men) and 44 healthy volunteers (age 39 ± 14 years, 32 men) in whom LV rotation could be assessed at the basal and apical LV level with speckle-tracking echocardiography, using the QLAB Advanced Quantification Software version 6.0 (Philips, Best, The Netherlands). In HCM patients, lower values of initial counter-clockwise rotation at the basal LV level (1.5 ± 1.2 vs. 0.6 ± 0.9°, P < 0.001) were seen, in particular in the septal segment (1.7 ± 1.6 vs. 0.4 ± 0.7°, P < 0.001). After this period, the direction of rotation changed to clockwise with a peak basal rotation of -4.8 ± 2.0° in controls vs. -6.1 ± 2.5° in HCM patients (P < 0.05). Peak basal rotation in HCM patients was in particular higher in the anterior (-6.6 ± 3.0 vs. -4.4 ± 2.4°, P < 0.01) and septal (-5.4 ± 2.6 vs. -3.9 ± 1.9°, P < 0.05) segments. The normalized (corrected for peak basal rotation) global back-rotation rate was lower in HCM patients (4.1 ± 3.1 vs. 6.3 ± 4.9 s(-1), P < 0.05), in particular driven by a lower rate in the septal segment (3.8 ± 2.6 vs. 6.4 ± 4.8 s(-1), P < 0.01). At the apical level, changes in rotation and back-rotation were more homogeneous. CONCLUSION: Changes in rotation and back-rotation at the LV basal level in HCM patients are mainly caused by regional changes in the basal septal and anterior segments, the segments mostly involved in the hypertrophic process.

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.

Geometric errors of the pulsed-wave Doppler flow method in quantifying degenerative mitral valve regurgitation: a three-dimensional echocardiography study.

BACKGROUND: The aim of this study was to estimate geometric errors made by the two-dimensional (2D) transthoracic echocardiographic (TTE) pulsed-wave Doppler flow (PWDF) method in calculating regurgitant volume (RVol) and effective regurgitant orifice area (EROA) in degenerative mitral regurgitation (MR) by comparison with the three-dimensional (3D) transesophageal echocardiographic (TEE) PWDF method. METHODS: RVol and EROA were calculated in 22 patients with degenerative MR using the conventional 2D TTE PWDF method on the basis of monoplanar dimensions and a circular geometric assumption of the cross-sectional areas (CSAs) of the mitral annulus (MA) and the left ventricular outflow tract (LVOT) and the 3D TEE PWDF method, in which the CSAs of the MA and LVOT were measured directly in "en face" views. Diameters of the MA and LVOT were also measured in similar views as with TTE imaging in 3D TEE data sets. RESULTS: Both the MA and LVOT were oval. Mean MA diameters were 41 ± 4 mm (3D TEE major axis), 31 ± 4 mm (3D TEE minor axis), 39 ± 5 mm (2D TTE imaging), and 38 ± 5 mm (2D TEE imaging). Mean LVOT diameters were 29 ± 4 mm (3D TEE major axis), 21 ± 2 mm (3D TEE minor axis), 22 ± 2 mm (2D TTE imaging), and 23 ± 2 mm (2D TEE imaging). Compared with 3D TEE measurements, mitral annular CSA was overestimated by 13 ± 12% on 2D TTE imaging and by 7 ± 14% on 2D TEE imaging, while LVOT CSA was underestimated by 23 ± 10% and 17 ± 10%, respectively. Mean values of RVol were 95 ± 43 mL (3D TEE PWDF), 137 ± 56 mL (2D TTE PWDF), 120 ± 45 mL (2D TEE PWDF), and 111 ± 49 mL (flow convergence). Mean EROAs were 69 ± 34 mm2 (3D TEE PWDF), 98 ± 45 mm2 (2D TTE PWDF), 88 ± 42 mm2 (2D TEE PWDF), and 79 ± 36 mm2 (flow convergence). Observer variability for 3D TEE imaging was better than for 2D imaging. CONCLUSIONS: The 2D TTE PWDF method overestimates mitral RVol and EROA significantly because monoplanar 2D measurements represent mitral annular major-axis diameter and LVOT minor-axis diameter, and assumed circular CSAs of the MA and LVOT are oval.

Heart Failure Due to Severe Hypertrophic Cardiomyopathy Reversed by Low Calorie, High Protein Dietary Adjustments in a Glycogen Storage Disease Type IIIa Patient.

In glycogen storage disease type III (GSD III), deficiency of the debranching enzyme causes storage of an intermediate glycogen molecule (limit dextrin) in the affected tissues. In subtype IIIa hepatic tissue, skeletal- and cardiac muscle tissue is affected, while in subtype IIIb only hepatic tissue is affected. Cardiac storage of limit dextrin causes a form of cardiomyopathy, which resembles primary hypertrophic cardiomyopathy on cardiac ultrasound. We present a 32-year-old GSD IIIa patient with severe left ventricular hypertrophy (LVH) first diagnosed at the age of 8 years. LVH remained stable and symptomless until the patient presented at age 25 years with increasing dyspnea, fatigue, obesity, and NYHA (New York Heart Association) functional classification two out of four. Dyspnea, fatigue, and obesity progressed, and at age 28 years she was severely symptomatic with NYHA classification 3+ out of 4. On echocardiogram and electrocardiogram, the LVH had progressed as well. Initially, she was rejected for cardiac transplantation because of severe obesity. Therefore, a 900 cal, high protein diet providing 37% of total energy was prescribed during 4 months on which 10 kg weight loss was achieved. However, her symptoms as well as the electrocardiographic and echocardiographic LVH indices had improved dramatically - ultimately deferring cardiac transplantation. Thereafter, the caloric intake was increased to 1,370 cal per day, and the high protein intake was continued providing 43% of total energy. After 3 years of follow-up, the patient remains satisfied with reasonable exercise tolerance and minor symptoms in daily life.

Diagnostic uncertainties and future perspectives in noncompaction cardiomyopathy.

BACKGROUND: Noncompaction cardiomyopathy (NCCM) is a new pathoanatomic entity, disputably believed to result from abnormal arrest in embryonic endomyocardial morphogenesis. During almost three decades of research of NCCM, more knowledge has developed alongside diagnostic uncertainties and precise definition. In this article, we present these uncertainties and provide perspectives on how to overcome these challenges. AREAS COVERED: The uncertainties, about NCCM regarding nomenclature, classification, pathophysiology, and limitations of the current diagnostic criteria will be reviewed. The application of newer imaging modalities will be contrasted in relation to conventional assessments. Finally, future aspirations will be outlined providing a more thoughtful appraisal toward NCCM diagnosis. EXPERT OPINION: Our current understanding of NCCM is limited by heterogeneity of disease spectrum and phenotype-genotype overlap with other cardiac anomalies. Selection bias, small sampling, and retrospective nature limit most of published studies on NCCM. There are three main research fields related to NCCM: pathoanatomic studies, imaging studies, and genetic screening. Besides conventional echocardiography, imaging should include both structural (cardiac MRI, contrast and 3D echocardiography) and functional diagnosis using deformation imaging. These research aspects should be integrated in a collaborative international registry of nonselective populations in order to achieve better understanding and optimal diagnosis of NCCM. Moreover, it holds the promise of the detection of earlier stages of disease. A clear pathoanatomic cut-off definition of NCCM should be the initial step toward uniform imaging diagnosis.

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.

Diagnostic value of rigid body rotation in noncompaction cardiomyopathy.

BACKGROUND: The diagnosis of noncompaction cardiomyopathy (NCCM) remains subject to controversy. Because NCCM is probably caused by an intrauterine arrest of the myocardial fiber compaction during embryogenesis, it may be anticipated that the myocardial fiber helices, normally causing left ventricular (LV) twist, will also not develop properly. The resultant LV rigid body rotation (RBR) may strengthen the diagnosis of NCCM. The purpose of the current study was to explore the diagnostic value of RBR in a large group of patients with prominent trabeculations. METHODS: The study comprised 15 patients with dilated cardiomyopathy, 52 healthy subjects, and 52 patients with prominent trabeculations, of whom a clinical expert in NCCM defined 34 as having NCCM. LV rotation patterns were determined by speckle-tracking echocardiography and defined as follows: pattern 1A, completely normal rotation (initial counterclockwise basal and clockwise apical rotation, followed by end-systolic clockwise basal and counterclockwise apical rotation); pattern 1B, partly normal rotation (normal end-systolic rotation but absence of initial rotation in the other direction); and pattern 2, RBR (rotation at the basal and apical level predominantly in the same direction). RESULTS: The majority of normal subjects had LV rotation pattern 1A (98%), whereas the 18 subjects with hypertrabeculation not fulfilling diagnostic criteria for NCCM predominantly had pattern 1B (71%), and the 34 patients with NCCM predominantly had pattern 2 (88%). None of the patients with dilated cardiomyopathy showed RBR. Sensitivity and specificity of RBR for differentiating NCCM from "hypertrabeculation" were 88% and 78%, respectively. CONCLUSIONS: RBR is an objective, quantitative, and reproducible functional criterion with good predictive value for the diagnosis of NCCM as determined by expert opinion.