Right ventricular involvement in hypertrophic cardiomyopathy

The aim of this study was to assess, using cardiovascular magnetic resonance (CMR), whether morphologic right ventricular (RV) abnormalities are present in patients with hypertrophic cardiomyopathy (HC). Left ventricular hypertrophy has been considered the predominant phenotypic expression of HC. Whether structural abnormalities of the right ventricle are also present in HC is unknown. CMR provides complete coverage of both ventricles with high spatial resolution. CMR was applied to study RV morphology in HC. CMR was performed on 46 subjects with HC (mean age 39 +/- 16 years; 70% men) free of pulmonary hypertension and 22 healthy subjects (mean age 44 +/- 16 years; 50% men). Mass, wall thickness, chamber volume, the ejection fraction, and fibrosis were assessed for both ventricles. Maximum RV wall thickness was increased in patients with HC compared with referent controls (7 +/- 2 vs 5 +/- 1 mm, p <0.001), including 15 (33%) with maximum wall thicknesses > or =8 mm (> or =2 SDs higher than the mean for controls) and 4 (9%) with extreme hypertrophy (> or =10 mm). RV hypertrophy was predominantly a diffuse process involving the entire or a significant proportion of the RV wall in most patients (n = 8 [53%]). The RV wall mass index was also increased in patients with HC (28 +/- 9 vs 22 +/- 4 g, p <0.001). A significant correlation was found between maximum RV and left ventricular wall thickness (R(2) = 0.4, p <0.001) and between RV and left ventricular mass (R(2) = 0.4, p <0.001). Only 1 (2%) patient with HC had evidence of RV wall fibrosis. In conclusion, morphologic RV abnormalities are present in a substantial proportion of patients with HC.     

Rapid and accurate measurement of LV mass by biplane real-time 3D echocardiography in patients with concentric LV hypertrophy: comparison to CMR.

AIMS: To evaluate the accuracy of real-time three-dimensional echocardiography (RT3DE) using a biplane and multiplane method in determining left ventricular (LV) mass compared to cardiac magnetic resonance imaging (CMR). METHODS AND RESULTS: LV mass was measured in 18 adult patients with congenital aortic stenosis using CMR and echocardiography (M-mode, two-dimensional echocardiography (2DE), and RT3DE). RT3DE data were analysed using a biplane and multiplane method. No geometric assumptions were necessary using the multiplane RT3DE method. With regard to biplane or multiplane RT3DE, no tendency of over- or underestimation of LV mass was observed. Pearson's correlation coefficients for RT3DE versus CMR were 0.84 and 0.90 for the biplane and multiplane method, respectively. In addition, the accuracy of both RT3DE methods were comparable (Fisher's R-to-Z transformation: Z = 0.69, P = NS). Finally, off-line analysis using biplane RT3DE was significantly faster than multiplane RT3DE (3.8 +/- 1.2 vs. 7.8 +/- 1.7 minutes, P < 0.001). CONCLUSIONS: Biplane RT3DE provided an accurate estimate of LV mass in patients with concentric left ventricular hypertrophy, which was not improved by multiplane RT3DE. The accuracy and speed of analysis renders biplane RT3DE an attractive tool in daily clinical practice for assessing the degree of LV hypertrophy.     

Gadolinium delayed enhancement cardiovascular magnetic resonance correlates with clinical measures of myocardial infarction

OBJECTIVES: The current study tested the hypothesis that gadolinium delayed enhancement assessment of infarct size correlates with clinical indices of myocardial infarction (MI) in humans. Acute infarct mass by cardiac magnetic resonance (CMR) was compared with peak troponin I, acute and chronic left ventricular (LV) systolic function, and chronic infarct mass in patients imaged after recent acute MI. BACKGROUND: Cardiac magnetic resonance accurately determines myocardial viability in patients with chronic ischemic heart disease but is not well validated for recent MI. METHODS: Patients with first acute MI (n = 33) or chronic MI (n = 10) underwent cine CMR followed by gadolinium delayed enhancement imaging. A follow-up CMR scan was performed on 20 of the 33 acute MI patients and all of the chronic MI patients. RESULTS: In patients with acute percutaneous coronary intervention, acute MI mass correlated with peak troponin I (r = 0.83, p < 0.001, n = 23). In the 20 acute infarct patients with follow-up CMR scans, the acute infarct size correlated well with the follow-up LV ejection fraction (r = 0.86, p < 0.001). The transmural extent of delayed enhancement imaged acutely correlated inversely with wall thickening measured acutely (p < 0.001) and at follow-up (p < 0.001). Although chronic infarct size was reproducible (11 +/- 4% vs. 12 +/- 7%, p = NS), acute infarct size decreased from 16 +/- 12% to 11 +/- 9% (p < 0.003). CONCLUSION: In humans imaged shortly after acute MI, gadolinium delayed enhancement acute CMR infarct size correlates with acute and chronic indices of infarct size but will appear to diminish in size on follow-up.     

Clinical and morphologic features of hypertrophic cardiomyopathy in elderly patients 85 years or older.

We studied the distinctive morphology of the left ventricle (LV) and attempted to relate advanced age and hypertension to this characteristic feature in elderly patients with hypertrophic cardiomyopathy (HC). Fourteen elderly patients > or = 85 years old (mean age 90 +/- 5 years) with HC were compared with 45 young patients < or = 40 years (mean age 34 +/- 4 years) with this disease. More mild hypertension in the elderly (10/14, 71%) than in the young (0%), and more syncope in the young (10/45, 22%) than in the elderly (0%) were observed. Echocardiography showed that the elderly patients had relatively mild LV wall thickening, generally confined to the septum (elderly vs young: 18 +/- 4 vs 25 +/- 8 mm, p < 0.001), with more basal septal bulging (elderly vs young: 12/14, 86% vs 0%, p < 0.001) and anterior septal hypertrophy of LV (elderly vs young: 11/14, 79% vs 0%, p < 0.001). Elderly patients with mild hypertension showed a predominantly basal septal bulging (10/10, 100%) and anterior septal hypertrophy of LV (9/10, 90%). HC in elderly patients > or = 85 years old has a striking LV morphology. Mild hypertension and advanced age may contribute to the distinctive geometry.     

Differentiation of hypertrophic cardiomyopathy from other forms of left ventricular hypertrophy by means of three-dimensional echocardiography

In clinical practice, differential diagnosis among different forms of left ventricular (LV) hypertrophy is not always easy, and hypertrophic cardiomyopathy (HC) can be misdiagnosed. In this study, it was hypothesized that a 3-dimensional echocardiographically derived index of LV regional mass distribution could be useful in differentiating HC from other forms of LV hypertrophy. Sixty-eight subjects underwent 2- and 3-dimensional echocardiography; of these, 20 were healthy volunteers, 18 were top-level athletes, 15 had essential hypertension, and 15 had HC. In off-line analysis, a 12-segment model was generated for segmental mass measurement. A mass dispersion index (MDI) was calculated as the average of the SDs of segmental mass values at the basal, middle, and apical layers. The ratio of ventricular septal thickness to posterior wall thickness was also calculated using 2-dimensional echocardiography. Patients with HC had significantly higher MDI values (1.75 +/- 0.43) than healthy volunteers (0.39 +/- 0.13) (p <0.0001), athletes (0.49 +/- 0.12) (p <0.0001), and patients with hypertension (0.38 +/- 0.10) (p <0.0001). The ratio of ventricular septal thickness to posterior wall thickness was significantly higher in patients with HC (1.31 +/- 0.23) than normal subjects (1.04 +/- 0.05) (p <0.0001), highly trained athletes (1.03 +/- 0.06) (p = 0.001), and patients with hypertension (1.06 +/- 0.06) (p = 0.002). However, receiver-operating characteristic analysis showed a higher sensitivity for MDI (93.3% for the cut-off value of 1.13) than the ratio of ventricular septal thickness to posterior wall thickness (66.7% for the cut-off value of 1.20), with excellent specificity for both (100%) in identifying patients with HC. In conclusion, the 3-dimensional echocardiographically derived MDI could be considered a useful and reliable additional tool in differentiating HC from other forms of LV hypertrophy.     

Degree of left ventricular hypertrophy in patients with hypertrophic cardiomyopathy and chronic atrial fibrillation.

It has been generally assumed that most patients with hypertrophic cardiomyopathy (HC) who develop atrial fibrillation (AF) have marked left ventricular (LV) hypertrophy and subaortic obstruction. The morphologic and functional features of this subset of patients with HC have not been systematically investigated. The LV morphology and functional profile of 46 patients with HC and chronic AF were compared with those of 81 control patients with HC and normal sinus rhythm. Contrary to expectations, LV hypertrophy (assessed with 2-dimensional echocardiography) was substantially less marked in the patients with AF than in the control patients, and prevalence of subaortic obstruction was similar in the 2 groups. Maximal LV wall thickness and wall thickness index were lower in patients with AF (18 +/- 2 and 56 +/- 7 mm, respectively) than in control patients (22 +/- 6 and 67 +/- 16 mm, respectively; p less than 0.001). Furthermore, mild LV hypertrophy (maximal LV wall thickness less than or equal to 17 mm confined to 1 ventricular segment) was almost twice as frequent in patients with AF (63%) than in control patients (36%; p less than 0.005). Subaortic obstruction was present in 9 patients with AF (20%) and in 28 control patients (35%; p greater than 0.05). In a subgroup of 22 patients with AF who were followed for 4 to 10 years, 5 patients had marked LV wall thinning (greater than or equal to 5 mm, range 5 to 14). In conclusion, these results demonstrate that most patients with HC and chronic AF have the nonobstructive form of HC, and relatively mild LV hypertrophy.     

Usefulness of left ventricular mass in predicting recovery of left ventricular systolic function in patients with symptomatic idiopathic dilated cardiomyopathy

Prognosis of idiopathic dilated cardiomyopathy (IDC) is variable. We determined the prognostic value of left ventricular (LV) mass and systolic and diastolic function in patients with IDC of <12 months duration. Clinical and echocardiographic assessment was performed at baseline and at 8+/-6 months follow-up in 25 patients (47+/-13 years) with IDC and an LV ejection fraction (LVEF1) of <40% (22+/-7%). Based on a follow-up LVEF (LVEF2) of < or >40%, patients were divided into unimproved (n = 13, LVEF2 = 21+/-9%) and improved groups (n = 12, LVEF2 = 51+/-11%). There was no difference in the LVEF1 (22+/-8% vs. 22+/-6%), LV end-systolic (5.7+/-0.8 vs. 5.8+/-0.9 cm) or end-diastolic (6.5+/-0.6 vs. 6.6+/-0.9 cm) dimension, wall stress (102+/-26 vs 99+/-28 g/cm2), end-systolic (1.7+/-0.3 vs. 1.8+/-0.2) or end-diastolic (1.7+/-0.3 vs. 1.6+/-0.1) sphericity, dp/dt (582+/-163 vs. 678+/-222 mm Hg/s), or right ventricular fractional shortening (20+/-9% vs. 27+/-7%, p = 0.06) in unimproved and improved groups. LV mass was lower (1.00+/-0.21 vs. 1.38+/-0.27 g/ml, p = 0007) and mitral inflow E-wave deceleration time shorter (97+/-42 vs. 164+/-58 ms, p = 0007) in the unimproved versus the improved group. On Pearson correlation analysis, LV mass (r = 0.62, p = 0.001), deceleration time (r = 0.68, p = 0.0002), wall motion score index (r = -0.47, p = 02), and dp/dt (r = 0.52, p = 03) were the significant predictors of LVEF2. There was correlation between LV mass (grams per milliliter) and deceleration time (r = 0.61, p = 0.001). During follow-up, death occurred in 1, and readmission for worsening heart failure in 4 patients in the unimproved group versus no hospitalization in the improved group. Thus, in patients with recent onset IDC, LV mass and diastolic function determine late outcome.     

Comparison of interstudy reproducibility of cardiovascular magnetic resonance with two-dimensional echocardiography in normal subjects and in patients with heart failure or left ventricular hypertrophy

Fast breath-hold cardiovascular magnetic resonance (CMR) shows excellent results for interstudy reproducibility of left ventricular (LV) volumes, ejection fraction, and mass, which are thought to be superior to results of 2-dimensional echocardiography. However, there is no direct comparison of the interstudy reproducibility of both methods in the same subjects. A total of 60 subjects (normal volunteers [n = 20], or patients with heart failure [n = 20] or LV hypertrophy [n = 20]) underwent 2 CMRs and 2 echocardiographic studies for assessment of LV volumes, function, and mass. The interstudy reproducibility coefficient of variability was superior for CMR in all groups for all parameters. Statistical significance was reached for end-systolic volume (4.4% to 9.2% vs 13.7% to 20.3%, p <0.001), ejection fraction (2.4% to 7.3% vs 8.6% to 19.4%, p <0.001), and mass (2.8% to 4.8% vs 11.6% to 15.7% p <0.001), with a trend for end-diastolic volume (2.9% to 4.9% vs 5.5% to 10.5%, p = 0.17). The superior interstudy reproducibility resulted in considerably lower calculated sample sizes (reductions of 55% to 93%) required by CMR compared with echocardiography to show clinically relevant changes in LV dimensions and function. Thus, CMR has excellent interstudy reproducibility in normal, dilated, and hypertrophic hearts, and is superior to 2-dimensional echocardiography.     

Relation between number of component views and accuracy of left ventricular mass determined by three-dimensional echocardiography

Three-dimensional echocardiography (3DE) allows the accurate determination of left ventricular (LV) mass, but the optimal number of component or extracted 2-dimensional (2D) image planes that should be used to calculate LV mass is not known. This study was performed to determine the relation between the number of 2D image planes used for 3DE and the accuracy of LV mass, using cardiovascular magnetic resonance (CMR) imaging as the reference standard. Three-dimensional echocardiography data sets were analyzed using 4, 6, 8, 10 and 20 component 2D planes as well as biplane 2D echocardiography and CMR in 25 subjects with a variety of LV pathologies. Repeated-measures analysis of variance and the Bland-Altman method were used to compare measures of LV mass. To further assess the potential clinical impact of reducing the number of component image planes used for 3DE, the number of discrepancies between CMR and each of the 3DE estimates of LV mass at prespecified levels (i.e., > or =5%, > or =10%, and > or =20% difference from CMR LV mass) was tabulated. The mean LV mass by magnetic resonance imaging was 177 +/- 56 g (range 91 to 316). Biplane 2-dimensional echocardiography significantly underestimated CMR LV mass (p <0.05), but LV mass by 3DE was not statistically different from that by CMR regardless of the number of planes used. However, error variability and Bland-Altman 95% confidence intervals decreased with the use of additional image planes. In conclusion, transthoracic 3DE measures LV mass more accurately than biplane 2-dimensional echocardiography when > or =6 component 2D image planes are used. The use of >6 planes further increases the accuracy of 3DE, but at the cost of greater analysis time and potentially increased scanning times.     

Papillary Muscle Contribution to Ventricular Ejection in Normal and Hypertrophic Ventricles: A Transesophageal Echocardiographic Study

BACKGROUND: The interrelationship between left ventricular (LV) volume, stroke volume, and papillary muscle (PM) volume have not been studied. These volumes are relevant in understanding LV ejection mechanics in normal chambers and ascertaining whether differences exist between normal and hypertrophied LV chambers. METHODS AND RESULTS: PM basal areas were measured in short-axis transesophageal echocardiographic views and lengths were measured in long-axis views. PM volume was estimated by the formula for volume of a cone: 1/3 x PM base area x PM length. The formula for LV volume was as follows: LV volume = 2/3 x LV area x LV length. Of the initial 82 subjects with normal LV function studied by TEE, data on 71 are presented in this report. Thirty-two patients had normal LV size and wall thickness, and 39 had LV hypertrophy (LVH). PM volume/LV volume % in end-diastole (ED) and end-systole (ES) in normal muscles was 3.1 +/- 1.0 and 9.6 +/- 4.9, respectively. In LVH, the respective values were 5.1 +/- 2.0 (P < 0.05) and 13.5 +/- 4.9 (P < 0.05). For those with severe LVH, the values were 7.1 +/- 2.5 (P < 0.001) and 15.9 +/- 4.1 (P < 0.001), respectively, for ED and ES. Similar trends were seen in the PM volume/stroke volume relationships in normal and hypertrophic ventricles. CONCLUSIONS: PMs are larger and form a larger fraction of LV volume in LVH than in normal muscles. In patients with severe LVH, the contribution of PMs to ventricular ejection is more pronounced. PMs may, therefore, play a larger role in LV ejection in LVH than in normal ventricles (i.e., hypertrophied PM enhance the pump efficiency of LV ejection).     

Comparison of two- and three-dimensional echocardiography with sequential magnetic resonance imaging for evaluating left ventricular volume and ejection fraction over time in patients with healed myocardial infarction

Echocardiographic follow-up of left ventricular (LV) volumes is difficult because of the test-retest variation of 2-dimensional echocardiography (2DE). We investigated whether the accuracy and reproducibility of real-time 3-dimensional echocardiography (RT3DE) would make this modality more feasible for serial follow-up of LV measurements. We performed 2DE and RT3DE and cardiac magnetic resonance imaging (MRI) in 50 patients with previous infarction and varying degrees of LV function (44 men; 61 +/- 11 years of age) at baseline and after 1-year follow-up. Images were obtained during breath-hold and measurements of LV volumes and ejection fraction were made offline. Over follow-up, end-diastolic volume decreased from 192 +/- 53 to 187 +/- 60 ml (p <0.01), end-systolic volume decreased from 104 +/- 51 to 95 +/- 53 ml (p <0.01), and ejection fraction increased from 48 +/- 12% to 51 +/- 12% (p <0.01). MRI showed that LV mass shrank from 183 +/- 39 to 182 +/- 37 g (p <0.01). The correlation between change in RT3DE and change in MRI was greater than the correlations of 2DE with MRI for measurement of end-diastolic volume (r = 0.47 vs 0.02, p <0.01), end-systolic volume (r = 0.44 vs 0.17, p <0.01), and ejection fraction (r = 0.58 vs -0.03, p <0.01). The change in end-diastolic volume between baseline and follow-up with RT3DE (-4 +/- 20, p <0.01) was similar to that with MRI but was unrecognized by 2DE (4 +/- 19, p = 0.09). There was good test-retest and inter- and intraobserver correlation within RT3DE for volumes, ejection fraction, and mass. In conclusion, if sequential measurement of LV volumes is used to guide management decisions, 3DE appears preferable to 2DE.     

New generation 3-dimensional echocardiography for left ventricular volumetric and functional measurements: comparison with cardiac magnetic resonance.

AIMS: Non-invasive assessment of left ventricular (LV) structure and function is important in the evaluation of cardiac patients. This study was designed to test the accuracy and reproducibility of new generation 3-dimensional echocardiography (3DE) in measuring volumetric and functional LV indices as compared with current "gold standard" of non-invasive cardiac imaging, cardiac magnetic resonance (CMR). METHODS AND RESULTS: Sixty-four subjects with good acoustic windows, including 40 cardiac patients with LV ejection fraction (EF)<45%, 14 patients with EF>45% and 10 normal volunteers underwent 3DE using a commercially available Philips Sonos 7500 scanner equipped with a matrix phase-array x4 xMATRIX transducer, and CMR on a 1.5 T Signa CV/i scanner (GE Medical Systems). Volumetric assessment was performed with analytical 4D-LV-Analysis software (TomTec) for 3DE and MRI-Mass software (Medis) for CMR. We found no significant differences in LV end-diastolic volume (EDV), end-systolic volume (ESV) and EF with excellent correlations between the indices measured using 3DE and CMR (r=0.97, r=0.98, and r=0.94, respectively). Bland-Altman analysis showed bias of 7 ml for EDV, 3 ml for ESV and -1% for EF with 3DE with corresponding limits of agreement (2SD) of 28 ml, 22 ml and 10%, respectively. Intraobserver and interobserver variabilities were for EDV: 3% and 4% (3DE) vs 2% and 2% (CMR), for ESV: 3% and 6% (3DE) vs 2% and 3% (CMR), and for EF: 4% and 4% (3DE) vs 2% and 4% (CMR), respectively. CONCLUSION: New generation 3DE provides accurate and reproducible quantification of LV volumetric and functional data in subjects with good acoustic windows as compared with CMR.     

Usefulness of N-terminal pro-B-type natriuretic peptide levels to predict exercise capacity in hypertrophic cardiomyopathy

Most patients with hypertrophic cardiomyopathy (HC) have reduced maximal oxygen consumption (VO2max) during exercise. The degree of impairment is poorly predicted by the magnitude of hypertrophy, left ventricular (LV) outflow tract obstruction, and other conventional markers of disease severity. The aim of this study was to determine the usefulness of N-terminal-pro-B-type natriuretic peptide (NT-pro-BNP) as a marker of exercise performance in HC. Plasma NT-pro-BNP was measured in 171 consecutive patients (mean age 46 +/- 18 years) who underwent echocardiography and cardiopulmonary exercise testing. The mean log NT-pro-BNP was 2.79 +/- 0.5; log NT-pro-BNP levels were higher in women patients (p = 0.001) and patients with chest pain (p = 0.010), in New York Heart Association class > or = II (p = 0.009), with atrial fibrillation (p < 0.001), with systolic impairment (p = 0.025), and with LV outflow tract obstructions (p < 0.0001). NT-pro-BNP levels were also correlated with maximal wall thickness (r = 0.335, p < 0.0001), left atrial size (r = 0.206, p = 0.007), and the mitral Doppler E/A ratio (r = 0.197, p = 0.012). The mean percent VO2max achieved was 73.8 +/- 22.6%; percent VO2max was smaller in patients with systolic impairment (p = 0.044) and LV outflow tract obstructions (p = 0.025). There were inverse correlations between percent VO2max and NT-pro-BNP (r = -0.352, p = 0.001), LV end-systolic cavity size (r = -0.182, p = 0.031), and left atrial size (r = -0.251, p = 0.003). On multivariate analysis, only NT-pro-BNP was correlated with percent VO2max. A NT-pro-BNP level of 316 ng/L had 78% sensitivity and 44% specificity (area under the curve 0.616) for predicting percent VO2max < 80%. In conclusion, NT-pro-BNP levels correlate with peak oxygen consumption in HC and are more predictive of functional impairment than other conventional markers of disease severity.     

Right ventricular infarction: two-dimensional echocardiographic evaluation

Seventeen patients with predominant right ventricular infarction (RVMI) were studied with two-dimensional echocardiography (2DE). On initial 2DE all had abnormal wall motion (AWM), defined as akinesis plus dyskinesis, in the inferior right ventricle (RV), inferior interventricular septum, and inferior left ventricle (LV). The extent of RV vs LV AWM in short-axis sections at mitral, chordal, and papillary levels was 58% vs 29%, 56% vs 38%, and 59% vs 38%, respectively. The calculated topographic extent of AWM was greater in the RV than in the LV (58% vs 36%, p less than 0.05), and the RV/LV ratio (1.65) exceeded (p less than 0.001) unity. Peak creatine phosphokinase levels correlated significantly (p less than 0.001) with the topographic extent of LV AWM (r = 0.79) or RV + LV AWM (r = 0.75). Although all patients had RV dilatation, eight also had LV dilatation. Serial studies detected the cause of mechanical complications (n = 13), mural echo densities suggesting thrombi (LV in six and RV in seven), and persistent AWM in survivors. Thus, 2DE provided diagnostic data, and assessment of RV and LV AWM confirmed predominant RV involvement.     

Left ventricular twist mechanics in hypertrophic cardiomyopathy assessed by three-dimensional speckle tracking echocardiography

Left ventricular (LV) twist represents a phenomenon that links systolic contraction with diastolic relaxation and plays a major role in cardiac physiology; thus, the study of twist mechanics is of particular interest in hypertrophic cardiomyopathy (HC). Three-dimensional speckle tracking echocardiography (3D-STE) has the potential to overcome the limitations of 2-dimensional imaging and provide a greater understanding of LV twist in HC. We aimed to examine LV twist mechanics in HC using 3D-STE. Echocardiograms from subjects with a diagnosis of HC were examined for 3D-STE analysis. Age- and gender-matched healthy subjects were tested as a control group. Forty patients with HC (age 37 ± 16 years; 42.5% women) and 40 control subjects (age 35 ± 10 years; 42.5% women) were examined. Compared with the controls, the patients with HC showed increased peak LV twist (16.5 ± 4.7° vs 12.0 ± 3.9°, p <0.001) mainly because of increased apical rotation of those with LV outflow tract obstruction (obstruction, 12.7 ± 4.4° vs nonobstruction, 9.7 ± 2.8°, p = 0.02). In addition, the patients with HC displayed onset of torsion recoil occurring closer to the aortic valve closure (94 ± 6% vs 85 ± 6%, p <0.001; time normalized by the length of systole), limited completion of untwist during early diastole (31 ± 12% vs 62 ± 15%, p <0.001), and delayed peak untwist velocity (22 ± 7% vs 13 ± 9%, p <0.001; time normalized by the length of diastole). In conclusion, the evaluation of twist mechanics using 3D-STE provides novel insight regarding alterations in LV mechanics in patients with HC. Elucidating the characteristics of the wringing motion of the heart might help to broaden the understanding of the hyperdynamic contraction and impaired relaxation observed in these patients.     

Effect of successful alcohol septal ablation on microvascular function in patients with obstructive hypertrophic cardiomyopathy

We hypothesized that relief of obstruction in patients with hypertrophic cardiomyopathy (HC) by percutaneous transluminal septal myocardial ablation (PTSMA) improves microvascular dysfunction by relief of extravascular compression. Microvascular dysfunction in obstructive HC is related to extravascular compression by increased left ventricular (LV) mass and LV end-diastolic pressure. The study included 14 patients with obstructive HC (mean age 55+/-12 years, 11 men) who underwent successful PTSMA and 14 healthy volunteers (mean age 31+/-4 years, 11 men). LV hemodynamics (by Doppler echocardiography) and intramyocardial flow dynamics (by adenosine myocardial contrast echocardiography) were evaluated in healthy volunteers and before and 6 months after PTSMA in patients with HC. LV end-diastolic pressure was estimated from the ratio of transmitral early LV filling velocity to early diastolic mitral annular velocity. PTSMA reduced the invasively measured LV outflow tract gradient (119+/-35 vs 17+/-16 mm Hg, p<0.0001) and LV end-diastolic pressure (23+/-3 vs 16+/-2 mm Hg, p<0.001). Six months after PTSMA, myocardial flow reserve improved (2.73+/-0.56 vs 3.21+/-0.49, p<0.001), but did not normalize compared with healthy controls (vs 3.95+/-0.77, p<0.001). Also, septal hyperemic endo-to-epi myocardial blood flow ratio improved (0.70+/-0.11 vs 0.92+/-0.07, p<0.001). Changes in LV end-diastolic pressure, LV mass index, and LV outflow tract peak systolic gradient correlated well with changes in hyperemic perfusion (all p<0.05). In conclusion, microvascular dysfunction improves after PTSMA due to relief of extravascular compression forces.     

Influence of left ventricular cavity size on clinical presentation in hypertrophic cardiomyopathy

The aim of this study was to assess whether left ventricular (LV) cavity size relates to functional impairment and syncope in patients with hypertrophic cardiomyopathy (HC). LV diastolic dysfunction influences functional limitation in HC. A reduced LV end-diastolic dimension may underlie impaired diastolic properties and be implicated in hemodynamic syncope. Eighty-two consecutive patients with HC (off drugs, in sinus rhythm) underwent echocardiography to measure LV end-diastolic dimension in the short-axis view (indexed to the body surface area) and radionuclide angiography (n = 50) to calculate peak filling rate (normalized to stroke counts/s). Patients in New York Heart Association functional classes II to IV had smaller LV end-diastolic dimension (23.2 +/- 2.6 vs 25.5 +/- 2.5 mm/M2, p = 0.0001) and lower peak filling rate (4.3 +/- 1.4 vs 5.1 +/- 1.3 stroke counts/s, p = 0.036) than those in New York Heart Association class I. LV end-diastolic diameter was correlated to peak filling rate (r = 0.37; p = 0.008). The most potent predictors of functional limitation were LV end-diastolic dimension (relative risk [RR] 0.63, confidence interval [CI] 0.45 to 0.88; p = 0.008), age (RR 1.09, CI 1.03 to 1.17; p = 0.003), and LV thickness score (RR 1.08, CI 1.02 to 1.13; p = 0.003). LV cavity size was smaller in patients with functional limitation irrespective of obstruction and hypertrophy. Patients with differed from those without a history of syncope for a smaller LV end-diastolic dimension (23.2 +/- 2.5 vs 25.0 +/- 2.7 mm/M2, p = 0.008), which was the only independent predictor of syncope (RR 0.77, CI 0.63 to 0.95; p = 0.013). Thus, a small LV cavity size is associated with functional limitation and history of syncope in HC.     

Importance of overweight in studies of left ventricular hypertrophy and diastolic function in mild systemic hypertension

The relations of Metropolitan Life Insurance Co. Relative Weight values and blood pressure (BP) to minimal forearm vascular resistance, ventricular septal and posterior wall thickness, left ventricular (LV) mass index and cardiac diastolic function were assessed in 31 men, 37 +/- 2 (mean +/- standard error of the mean) years of age. Eighteen patients with untreated mild hypertension were compared with 13 normotensive control subjects of similar age and weight. The hypertensives had higher clinic (137 +/- 3/96 +/- 2 vs 121 +/- 4/81 +/- 3 mm Hg, p less than 0.001/less than 0.001) and home (p less than 0.001) BP. Despite higher BP, the hypertensives did not have significantly greater values than normotensives, respectively, for minimal forearm vascular resistance (2.20 +/- 0.12 vs 2.04 +/- 0.11 U), ventricular septal (9.9 +/- 0.5 vs 10.2 +/- 0.3 mm) and posterior wall thickness (10.2 +/- 0.4 vs 10.0 +/- 0.3 mm) or LV mass index (106 +/- 6 vs 107 +/- 6 g/m2). Furthermore, diastolic peak filling rate, an index of LV diastolic function, was virtually identical in the 2 groups (2.71 +/- 0.14 vs 2.69 +/- 0.07 liters/s, difference not significant). Correlates of peak filling rate included relative weight (r = -0.62, p less than 0.001), posterior wall thickness (r = -0.51, p less than 0.01) and age (r = -0.45, p less than 0.05). Relative weight also correlated significantly with posterior wall (r = 0.59, p less than 0.005), ventricular septal (r = 0.47, p less than 0.005) and LV mass index (r = 0.38, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

Relation of left ventricular thickness to age and gender in hypertrophic cardiomyopathy

Left ventricular (LV) wall thickening is the most consistent clinical marker of hypertrophic cardiomyopathy (HC), and characteristically increases substantially during adolescence. In this study, we used 2-dimensional echocardiography to develop a cross-sectional profile of LV wall thicknesses in adult patients with HC. We studied a regional community-based cohort of 239 consecutively enrolled patients (aged 18 to 91 years). On average, maximum LV wall thickness decreased relative to increasing age (p = 0.007) within 4 age groups: 22.8 +/- 5.1 mm (18 to 39 years) to 22.1 +/- 5.1 mm (40 to 59 years) to 21.1 +/- 3.7 mm (60 to 74 years) to 20.8 +/- 3.6 mm (>or=75 years). The LV thickness index (summation of wall thicknesses in all 4 segments) also decreased with age (p = 0.017): 63.0 +/- 12.2 mm to 59.8 +/- 11.9 mm to 58.3 +/- 10.4 mm to 57.9 +/- 9.8 mm. Decreasing magnitude of LV hypertrophy was independently associated with increasing age, but not with other relevant disease variables, such as symptoms and outflow obstruction. However, when separated by gender, this inverse relation between age and LV wall thickness was statistically significant only for women (p = 0.007). In conclusion, in an unselected HC cohort, cross-sectional analysis showed a modest but statistically significant inverse relation between age and LV hypertrophy that was largely gender-specific for women. This association constitutes another facet of the natural history of this complex and heterogenous disease and may reflect disproportionate occurrence of premature death in young patients with HC with marked hypertrophy or possibly gradual LV remodeling.     

Wasting of the left ventricle in patients with cardiac cachexia: a cardiovascular magnetic resonance study

BACKGROUND: The "cachectic heart" has been described as a pathologic decrease in the size and mass of the heart, but no in vivo studies have shown changes in cardiac dimensions or left ventricular (LV) mass over time in chronic heart failure (CHF) associated with body wasting (cardiac cachexia). Cardiovascular magnetic resonance (CMR) has high reproducibility and is more sensitive than other techniques. METHODS: CMR studies of LV volumes and mass were performed at baseline and a mean of 15 months later in nine CHF patients with cardiac cachexia and 28 matched CHF controls without cachexia (mass index 23 +/- 1 vs. 29 +/- 5 kg/m2, P=0.0005). RESULTS: At baseline, LV end-diastolic volume (197 +/- 78 vs. 203 +/- 65 ml), end-systolic volume (131 +/- 75 vs. 126 +/- 63 ml), LV mass (213 +/- 44 vs. 222 +/- 62 g), and LV ejection fraction (38 +/- 19% vs. 40 +/- 16%) did not differ between cachectic patients and controls (all P>0.10). During follow-up, there was a significant decrease in LV mass in patients with cachexia (-16 g, P<0.05) and a trend to increase in LV mass in patients without cachexia (+7 g, P=0.12, comparison between groups: P=0.010). CONCLUSIONS: The direction of changes over time in LV mass differs in CHF patients with cachexia as compared with non-cachectic controls. A significant decrease in LV mass occurs in patients with cardiac cachexia. This study documents in vivo the occurrence of wasting of the left ventricle in patients with CHF who demonstrate general body wasting.