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.
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).
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.
RBR is an objective, quantitative, and reproducible functional criterion with good predictive value for the diagnosis of NCCM as determined by expert opinion.
Previous observational studies demonstrated that patients with hypertrophic cardiomyopathy at risk for sudden cardiac death (SCD) may benefit from implantable cardioverter defibrillator (ICD) ...therapy. A complete overview of outcome and complications after ICD therapy is currently not available. This study pools data from published studies on outcome and complications after ICD therapy in patients with hypertrophic cardiomyopathy.
A PubMed database search returned 27 studies on 16 cohorts reporting outcome and complications after ICD therapy in patients with hypertrophic cardiomyopathy. In case of >1 publications on a particular cohort, the publication with the largest number of patients was included in the meta-analysis. ICD interventions, complications, and mortality rates were extracted, pooled, and analyzed. There were 2190 patients (mean age, 42 years; 38% women), most of whom (83%) received an ICD for primary prevention of SCD. Risk factors for SCD were left ventricular wall thickness ≥30 mm (20%), family history of SCD (43%), nonsustained ventricular tachycardia (46%), syncope (41%), and abnormal blood pressure response (25%). During the 3.7-year follow-up, the annualized cardiac mortality rate was 0.6%, the noncardiac mortality rate was 0.4%, and the appropriate ICD intervention rate was 3.3%. The annualized inappropriate ICD intervention rate was 4.8% and the annualized ICD-related complication rate was 3.4%.
This meta-analysis demonstrates a low cardiac and noncardiac mortality rate after ICD therapy in patients with hypertrophic cardiomyopathy. Appropriate ICD intervention occurred at a rate of 3.3%/year, thereby, most probably, preventing SCD. Inappropriate ICD intervention and complications are not uncommon.
Diastolic Abnormalities as First Feature of Hypertrophic Cardiomyopathy in Dutch Myosin-Binding Protein C Founder Mutations Michelle Michels, Osama I. I. Soliman, Marcel J. Kofflard, Yvonne M. ...Hoedemaekers, Dennis Dooijes, Danielle Majoor-Krakauer, Folkert J. ten Cate Family screening in hypertrophic cardiomyopathy by echocardiography is complicated by the absence of left ventricular hypertrophy in a significant number of mutation carriers. Previous studies have shown tissue Doppler imaging (TDI) to identify carriers of some mutations. This study shows that carriers of mutations in cardiac myosin-binding protein C were not identified by reduced TDI velocities. It seems likely that TDI is able to recognize myocardial changes earlier than when the presence of hypertrophy becomes apparent with standard echocardiography. However, there is an even earlier stage when the phenotype has not developed sufficiently to be apparent with imaging.
Proliferation of the adventitial vasa vasorum (VV) is inherently linked with early atherosclerotic plaque development and vulnerability. Recently, direct visualization of arterial VV and intraplaque ...neovascularization has emerged as a new surrogate marker for the early detection of atherosclerotic disease. This clinical review focuses on contrast-enhanced ultrasound (CEUS) as a noninvasive application for identifying and quantifying carotid and coronary artery VV and intraplaque neovascularization. These novel approaches could potentially impact the clinician's ability to identify individuals with premature cardiovascular disease who are at high risk. Once clinically validated, the uses of CEUS may provide a method to noninvasively monitor therapeutic interventions. In the future, the therapeutic use of CEUS may include ultrasound-directed, site-specific therapies using microbubbles as vehicles for drug and gene delivery systems. The combined applications for diagnosis and therapy provide unique opportunities for clinicians to image and direct therapy for individuals with vulnerable lesions.
Real-time 3-dimensional echocardiography (RT3DE) allows simultaneous timing of regional volumetric changes as a net result of longitudinal, radial, circumferential left ventricular (LV) contraction, ...hence LV systolic dyssynchrony. We sought to examine real-time 3-dimensional echocardiographically derived dyssynchrony for prediction of long-term response to cardiac resynchronization therapy (CRT) in a prospective study. Ninety consecutive patients with heart failure (mean age 60 ± 12 years, 73% men, New York Heart Association class III in 97%) underwent clinical and echocardiographic assessments at baseline and at 12 months after CRT including real-time 3-dimensional echocardiographically derived LV systolic dyssynchrony index. The systolic dyssynchrony index (SDI) was defined as the SD of time to minimum systolic volume of the 16 LV segments, expressed in percent RR duration. CRT response was defined as a >15% decrease in LV end-systolic volume on real-time 3-dimensional echocardiogram. After 12 months of CRT, 68 patients (76%) were responders. Feasibility of the SDI was 94%. An SDI >10% predicted CRT response with good sensitivity (96%), specificity (88%), positive likelihood ratio (8), and negative likelihood ratio (0.05). Patients with an SDI >10% had mean change (−21%, −31%, 39% vs −13%, −10%, 10%) in LV end-diastolic volume, LV end-systolic volume, and LV ejection fraction, respectively, compared with baseline versus patients with an SDI <10% (p <0.01). Mean acquisition and analysis duration of single-patient RT3DE was 8 minutes (range 6 to 13). Interobserver variabilities of LV end-systolic volume and SDI were 5% and 11%, respectively. In conclusion, RT3DE provides accurate identification of reverse volumetric LV remodeling after CRT. From these accurate volumetric data, RT3DE provides more intuitive assessment of dyssynchrony and response to CRT as a simple, reproducible, and fast technique. CRT can be individually tailored using RT3DE and seems very effective in patients with heat failure with real-time 3-dimensional echocardiographic evidence of dyssynchrony.
The panel focused largely on the management of this complex disease and derived prudent, practical, and contemporary treatment strategies for the many subgroups of patients comprising the broad HCM ...disease spectrum. Because of the relatively low prevalence of HCM in general cardiologic practice (50), its diverse presentation, and mechanisms of death and disability and skewed patterns of patient referral (7,11,13,36-38,42,51-59), the level of evidence governing management decisions for drugs or devices has often been derived from non-randomized and retrospective investigations. Large-scale controlled and randomized study designs, such as those that have provided important answers regarding the management of coronary artery disease (CAD) and congestive heart failure (60-62), have generally not been available in HCM as a result of these factors. ...treatment strategies have necessarily evolved based on available data that have frequently been observational in design, sometimes obtained in relatively small patient groups, or derived from the accumulated clinical experience of individual investigators, and reasonable inferences drawn from other cardiac diseases.
Background:The Frank–Starling law describes the relation between left ventricular volume and function. However, only a few studies have described the relation between left atrial volume (LAV) and ...function.Objective:To describe an LA Frank–Starling law by studying changes in LAV measured by real-time, three-dimensional echocardiography (RT3DE).Methods:LAV was calculated by RT3DE in 70 patients at end-systole (LAVmax), end-diastole (LAVmin) and pre-atrial contraction (LAVpre-A). According to LAVmax, patients were classified into three groups: LAVmax <50 ml (group I), LAVmax 50–70 ml (group II) and LAVmax >70 ml (group III). Calculated indices of LA pump function were active atrial stroke volume (SV), defined as LAVpre-A – LAVmin, and active atrial emptying fraction (EF), defined as active atrial SV/LAVpre-A ×100%Results:Active atrial SV was significantly higher in group II than in group I (mean (SD) 19.0 (9.2) vs 8.2 (4.9) ml, p<0.0001), in group III it was non-significantly lower than in group II (16.7 (12.5) vs 19.0 (9.2) ml). Active atrial SV correlated well with LAVpre-A (r = 0.56, p<0.001), but decreased with larger LAVpre-A. Active atrial EF tended to be higher in group II than in group I (43.1 (18.2) vs 33.2 (17.5), p<0.10), in group III it was significantly lower than in group II (26.2 (18.5) vs 43.1 (18.2), p<0.01).Conclusion:A Frank–Starling mechanism in the left atrium could be described by RT3DE, shown by an increase in LA contractility in response to an increase in LA preload up to a point, beyond which LA contractility decreased.
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.
Department of Cardiology, The Thoraxcenter, Erasmus University Medical Center, Rotterdam, The Netherlands
Submitted 22 April 2009
; accepted in final form 20 October 2009
The dynamic interaction ...between subendocardial and subepicardial fibre helices in the left ventricle (LV) leads to a twisting deformation, which has an important role in LV function. This study sought to assess the influence of cardiac shape on LV twist in the normal and dilated human heart. The study comprised 45 dilated cardiomyopathy (DCM) patients and 60 for age- and gender-matched healthy volunteers. Speckle tracking echocardiography was used to determine basal and apical LV peak systolic rotation (Rot max ) and instantaneous LV peak systolic twist (Twist max ). LV sphericity index was calculated by dividing the LV maximal long-axis internal dimension by the maximal short-axis internal dimension at end-diastole. A parabolic relation between the sphericity index and apical Rot max or Twist max was identified in the total study population ( R 2 = 0.56 and R 2 = 0.54, respectively; both P < 0.001) and healthy volunteers ( R 2 = 0.39 and R 2 = 0.25, respectively; both P < 0.001), whereas these relations were linear in DCM patients ( R 2 = 0.40 and R 2 = 0.43, respectively; both P < 0.001). In a multivariate analysis, LV sphericity index was the strongest independent predictor of apical Rot max and Twist max . In conclusion, LV apical rotation and twist are significantly influenced by LV configuration. Taking the important function of LV twist into account, this finding highlights the vital influence of cardiac shape on LV systolic function.
cardiac mechanics; cardiac function; left ventricular twist; dilated cardiomyopathy; echocardiography
Address for reprint requests and other correspondence: M. L. Geleijnse, Erasmus Univ. Medical Center, Thoraxcenter, Rm. BA 302, 's-Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands (e-mail: m.geleijnse{at}erasmusmc.nl ).
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