Olivier Huttin

Prevalence and clinical phenotype of hereditary transthyretin amyloid cardiomyopathy in patients with increased left ventricular wall thickness

AIMS Increased left ventricular wall thickness (LVWT) is a common finding in cardiology. It is not known how often hereditary transthyretin-related familial amyloid cardiomyopathy (mTTR-FAC) is responsible for LVWT. Several therapeutic modalities for mTTR-FAC are currently in clinical trials; thus, it is important to establish the prevalence of TTR mutations (mTTR) and the clinical characteristics of the patients with mTTR-FAC. METHODS AND RESULTS In a prospective multicentre, cross-sectional study, the TTR gene was sequenced in 298 consecutive patients diagnosed with increased LVWT in primary cardiology clinics in France. Among the included patients, median (25-75th percentiles) age was 62 [50;74]; 74% were men; 23% were of African origin; and 36% were in NYHA Class III-IV. Median LVWT was 18 (16-21) mm. Seventeen (5.7%; 95% confidence interval [CI]: [3.4;9.0]) patients had mTTR of whom 15 (5.0%; 95% CI [2.9;8.2]) had mTTR-FAC. The most frequent mutations were V142I (n = 8), V50M (n = 2), and I127V (n = 2). All mTTR-FAC patients were older than 63 years with a median age of 74 [69;79]. Of the 15 patients with mTTR-FAC, 8 were of African descent while 7 were of European descent. In the African descendants, mTTR-FAC median age was 74 [72;79] vs. 55 [46;65] years in non-mTTR-FAC (P < 0.001). In an adjusted multivariate model, African origin, neuropathy, carpal tunnel syndrome, electrocardiogram (ECG) low voltage, and late gadolinium enhancement (LGE) at cardiac-magnetic resonance imaging were all independently associated with mTTR-FAC. CONCLUSION Five per cent of patients diagnosed with hypertrophic cardiomyopathy have mTTR-FAC. Mutated transthyretin genetic screening is warranted in elderly subjects with increased LVWT, particularly, those of African descent with neuropathy, carpal tunnel syndrome, ECG low voltage, or LGE.

Risk of atrial fibrillation according to the initial presentation of a preexcitation syndrome

BACKGROUND Atrioventricular reentrant tachycardia (AVRT) is frequent in Wolff-Parkinson-White syndrome (WPW). Atrial fibrillation (AF) is rare. The purpose of the study was to determine the factors of spontaneous AF in WPW according to the initial presentation. METHODS AND RESULTS Electrophysiological study (EPS) was performed among 709 patients with a preexcitation syndrome. First event was AF in 44 patients. Remaining patients were studied for AVRT (314), syncope (94), adverse presentation without AF (9) or systematically (248 asymptomatic patients). Patients with AF were older than other patients (44 ± 16 years vs 34.5 ± 17) (0.0003); maximal rate conducted over accessory pathway (AP) was higher in patients with AF than in other patients except in adverse presentation (0.0002); AVRT was induced more frequently in patients with AF than in asymptomatic patients (57% vs 14.5%) but less than in patients with AVRT (89%). AF was induced more frequently in patients with AF than in other patients except in adverse presentation (<0.0001). During follow-up AF occurred more frequently in patients with AF (5; 11%) than in patients with AVRT (7; 2%), with syncope (1%) and asymptomatic patients (4; 1.6%). Older age predicted recurrence (54 ± 16 vs 40 ± 17). CONCLUSIONS AF was the first event in only 6% of patients with WPW and was a rare event in other patients. They are older but 10% are less than 18 years and have a more rapid conduction over AP than other patients.

Reproducibility in Echocardiographic Assessment of Diastolic Function in a Population Based Study (The STANISLAS Cohort Study)

Introduction There is limited evidence regarding intra-observer and inter-observer variations in echocardiographic measurements of diastolic function. This study aimed to assess this reproducibly within a population-based cohort study. Methods Sixty subjects in sinus rhythm were randomly selected among 4th visit participants of the STANISLAS Cohort (Lorraine region, France). This 4th examination systematically included M-mode, 2-dimensional, DTI and pulsed-wave Doppler echocardiograms. Reproducibility of variables was studied by intra-class correlation coefficients (ICC) and Bland Altman plots. Results Our population was on average middle-aged (50 ± 14y), overweight (BMI = 26 ± 6kg/m2) and non-smoking (87%) with a quarter of the participants having self-declared hypertension or treated with anti-hypertensive medication(s). Intra-observer ICC were > 0.90 for all analyzed parameters except for left ventricular ejection fraction (LVEF) which was 0.89 (0.81–0.93). The mean relative intra-observer differences were small and limits of agreement of relative differences were narrow for all considered parameters (<5% and <15% respectively). Inter-observer ICC were > 0.90 for all analyzed parameters except for LVEF (ICC = 0.87) and both mitral and pulmonary A wave duration (0.83 and 0.73 respectively). The mean relative inter-observer differences were <5% for all parameters except for pulmonary A wave duration (mean difference = 6.5%). Limits of agreement of relative differences were narrow (<15%), except for mitral A wave duration and velocity (both <20%) as well as left ventricular mass and pulmonary A wave duration (both <30%). Intra-observer agreements with regard to the presence and severity of diastolic dysfunction were excellent (Kappa = 0.93 (0.83–1.00) and 0.88 (0.75–0.99), respectively). Conclusion In this validation study within the STANISLAS cohort, diastolic function echocardiographic parameters were found to be highly reproducible. Diastolic dysfunction consequently appears as a highly effective clinical and research tool.

All you need to know about the tricuspid valve: Tricuspid valve imaging and tricuspid regurgitation analysis

The acknowledgment of tricuspid regurgitation (TR) as a stand-alone and progressive entity, worsening the prognosis of patients whatever its aetiology, has led to renewed interest in the tricuspid-right ventricular complex. The tricuspid valve (TV) is a complex, dynamic and changing structure. As the TV is not easy to analyse, three-dimensional imaging, cardiac magnetic resonance imaging and computed tomography scans may add to two-dimensional transthoracic and transoesophageal echocardiographic data in the analysis of TR. Not only the severity of TR, but also its mechanisms, the mode of leaflet coaptation, the degree of tricuspid annulus enlargement and tenting, and the haemodynamic consequences for right atrial and right ventricular morphology and function have to be taken into account. TR is functional and is a satellite of left-sided heart disease and/or elevated pulmonary artery pressure most of the time; a particular form is characterized by TR worsening after left-sided valve surgery, which has been shown to impair patient prognosis. A better description of TV anatomy and function by multimodality imaging should help with the appropriate selection of patients who will benefit from either surgical TV repair/replacement or a percutaneous procedure for TR, especially among patients who are to undergo or have undergone primary left-sided valvular surgery.

Prediction of Left Ventricular Remodeling after a Myocardial Infarction: Role of Myocardial Deformation: A Systematic Review and Meta-Analysis

Aims Left ventricular (LV) adverse or reverse remodeling after ST-segment elevation myocardial infarction (MI) is the best outcome to assess the benefit of revascularization. Speckle tracking echocardiography (STE) may accurately identify early deformation impairment, while also being predictive of LV remodeling during follow-up. This systematic analysis aimed to provide a comprehensive review of current findings on STE as a predictor of LV remodeling after MI. Methods PubMed databases were searched through December 2014 to identify studies in adults targeting the association between LV remodeling and STE. Meta-regression was performed for longitudinal analysis. Results A total of 23 prospective studies (3066 patients) were found eligible. Eleven studies reported an association between STE and adverse remodeling and twelve studies with reverse remodeling. Using peak systolic longitudinal strain, the most accurate cut-off to predict adverse remodeling and reverse remodeling ranged from -12.8% to -10.2% and from -13.7% to -9.5%, respectively. In smaller studies, assessment of circumferential strain and torsion showed additive value in predicting remodeling. Meta-regression analysis revealed that longitudinal STE was associated with adverse remodeling (pooled univariable OR = 1.27, 1.17–1.38, p<0.001; pooled multivariable OR = 1.38, 1.13–1.70, p = 0.002) while pooled ORs of longitudinal STE only tended to predict reverse remodeling (pooled OR = 0.75, 0.54–1.06, p = 0.09). Conclusions This systematic review suggests that STE is associated with changes in LV volume or function regardless of underlying mechanisms and deformation direction. Meta-regression demonstrates a strong association between peak longitudinal systolic strain and adverse remodeling. Added STE predictive value over other clinical, biological and imaging variables remains to be proven.

Impact of Changes in Consensus Diagnostic Recommendations on the Echocardiographic Prevalence of Diastolic Dysfunction

The diagnosis of diastolic dysfunction (DD) is based on consensus recommendations [(1,2)][1], although classification is difficult because of a multiplicity of echocardiographic indexes and substantial overlap between values in healthy subjects and in patients. Recently, a joint task force of the

Dramatic Response to Tocilizumab Before Emergency Surgery in Severe Active Takayasu Disease

A 19-year-old man was admitted in our tertiary care center in August 2014 for a 10-kg weight loss in a few months (48 kg, 1.80 m, and body mass index, 14.8 kg/m2), claudication in the lower limbs after a 500-m distance and a postprandial abdominal pain associated with mild renal insufficiency and proteinuria with no hypertension (100/80 mm Hg). The patient had no medical history and was an active smoker (tobacco and cannabis, estimated consumption: 2 pack-years). Laboratory parameters were elevated leukocyte count (13×109/L), C-reactive protein level (55 mg/L), creatinine (14 mg/L), and B-natriuretic peptide (4246 pg/mL). No thrombophilia, autoimmune disorders, or viral infections were identified. The patient underwent a B-mode Doppler ultrasound that showed multiple proximal arterial occlusions and stenoses of large vessels confirmed by a computed tomographic angiography: occlusions of bilateral subclavian arteries, superior and inferior mesenteric arteries, and right renal artery with kidney atrophy were found (Figure 1A). Furthermore, significant stenoses of celiac artery, left renal artery (Figure 1B), right internal iliac artery, and bilateral superficial femoral arteries were identified. A discrete circumferential thickening of the abdominal aorta was suggestive of aortitis. Cervical magnetic resonance angiography showed a complete occlusion of both subclavian and vertebral arteries (Figure 2). Transthoracic echocardiography revealed a severe left ventricular (LV) systolic dysfunction (LV ejection fraction …

Assessment of Left Ventricular Ejection Fraction Calculation on Long-axis Views From Cardiac Magnetic Resonance Imaging in Patients With Acute Myocardial Infarction.

AbstractTo assess left ventricular ejection fraction (LVEF) accurately, cardiac magnetic resonance (CMR) can be indicated and lays on the evaluation of multiple slices of the left ventricle in short axis (CMRSAX). The objective of this study was to assess another method consisting of the evaluation of 2 long-axis slices (CMRLAX) for LVEF determination in acute myocardial infarction.One hundred patients underwent CMR 2 to 4 days after acute myocardial infarction. LVEF was computed by the area-length method on horizontal and vertical CMRLAX images. Those results were compared to reference values obtained on contiguous CMRSAX images in one hand, and to values obtained from transthoracic echocardiography (TTE) in the other hand. For CMRSAX and TTE, LVEF was computed with Simpson method. Reproducibility of LVEF measurements was additionally determined. The accuracy of volume measurements was assessed against reference aortic stroke volumes obtained by phase-contrast MR imaging.LVEF from CMRLAX had a mean value of 47 ± 8% and were on average 5% higher than reference LVEF from CMRSAX (42 ± 8%), closer to routine values from TTELAX (49 ± 8%), much better correlated with the reference LVEF from CMRSAX (R = 0.88) than that from TTE (R = 0.58), obtained with a higher reproducibility than with the 2 other techniques (% of interobserver variability: CMRLAX 5%, CMRSAX 11%, and TTE 13%), and obtained with 4-fold lower recording and calculation times than for CMRSAX. Apart from this, CMRLAX stroke volume was well correlated with phase-contrast values (R = 0.81).In patients with predominantly regional contractility abnormalities, the determination of LVEF by CMRLAX is twice more reproducible than the reference CMRSAX method, even though the LVEF is consistently overestimated compared with CMRSAX. However, the CMRLAX LVEF determination provides values closer to TTE measurements, the most available and commonly used method in clinical practice, clinical trials, and guidelines in ischemic cardiomyopathy. Moreover, LVEF determination by CMRLAX allows a 63% gain of acquisition/reading time compared with CMRSAX. Thus, despite the fact that LVEF obtained from CMRSAX remains the gold standard, CMRLAX should be considered to shorten the overall imaging acquisition and reading time as a putative replacement.

Right ventricular dysfunction in patients with idiopathic dilated cardiomyopathy: Prognostic value and predictive factors

BACKGROUND Right ventricular (RV) dysfunction is an important predictor of impaired prognosis in idiopathic dilated cardiomyopathy. AIMS To determine the prognostic role of RV dysfunction, independent of left ventricular (LV) dysfunction. METHODS A total of 136 consecutive patients (73% men; mean age 59.0±13.2 years) with idiopathic dilated cardiomyopathy (LV ejection fraction ≤ 45%) were enrolled retrospectively. Thirty-four patients (25%, group 1) presented with RV dysfunction, defined as tricuspid annular plane systolic excursion (TAPSE) ≤ 15 mm; 102 patients (group 2) had preserved RV function. RESULTS Mean LV ejection fraction was 27.5±8.7%. Mean TAPSE was 18.6±5.4 mm (15-21.8 mm). Multivariable predictors of RV dysfunction were LV outflow tract time-velocity integral (odds ratio 0.8, 95% confidence interval [CI] 0.7-0.9; P=0.003) and E-wave deceleration time ≤ 145 ms (odds ratio 4.1, 95% CI 1.3-12.8; P=0.017). Major adverse cardiac event-free survival rates at 1 and 2 years were 64% and 55%, respectively, in group 1 and 87% and 79%, respectively, in group 2 (P=0.002). Both by multivariable analysis and after stratification using a propensity score, RV dysfunction emerged as an independent predictor for major adverse cardiac events (hazard ratio 3.2, 95% CI 1.3-7.6; P=0.009), along with right atrium area and age. CONCLUSION In idiopathic dilated cardiomyopathy, RV dysfunction with TAPSE ≤ 15 mm offers additional prognostic information, independent of the extent of LV dysfunction.

Reproducibility of echocardiographic assessment of 2D-derived longitudinal strain parameters in a population-based study (the STANISLAS Cohort study)

Global peak systolic longitudinal strain (PLS) derived from speckle tracking echocardiography (STE) is a widely used left ventricular deformation parameter. Modern ultrasound systems with improved temporal resolution and new software now allow automated multilayer analysis; however, there is limited evidence regarding its reproducibility. We performed intra- and inter-observer analyses within a population-based cohort study using conventional quantitative strain analysis (GE Healthcare). Fifty patients (49 ± 14 years) were randomly selected among the fourth visit of the STANISLAS Cohort. Multilayer PLS (transmural, subendocardial, and subepicardial), and strain rate (peak systolic, early and late diastolic) were evaluated. Peak systolic shortening (PSS) and early positive systolic strain (EPS) were calculated, as well as post-systolic index (PSI) and pre-stretch index (PST), two additional strain-derived parameters. Intra-observer intraclass correlation coefficients (ICC) were >0.75 for all analyzed parameters. The mean relative intra-observer differences were <5% for all considered parameters, and their 1.96 SDs were <15% for multilayer PLS, strain rate and PSS, but not for EPS, PSI and PST. Inter-observer ICCs were >0.70 (the majority being >0.80). The mean relative inter-observer differences were <7.5% for all considered parameters, with 1.96 SDs of relative differences being <21% for multilayer PLS, strain rate and PSS, but not for EPS, PSI and PST. In this population-based study, in subjects without or with a limited number of cardiovascular risk factors and no previous cardiovascular events, deformation parameters were found to be highly reproducible, except for EPS, PSI and PST, which showed moderately higher variability. Quantitative strain analysis appears to be an effective clinical and research tool, providing insights regarding longitudinal deformation using a simple three-step post-processing procedure.