scholarly journals 813 The added value of transthoracic three dimensional echocardiography in apical hypertrophic cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Valeria Rella ◽  
Mara Gavazzoni ◽  
Michele Tomaselli ◽  
Giorgio Oliverio ◽  
Valentina Volpato ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Wall Thickness ◽  
Cardiac Death ◽  
Contrast Echocardiography ◽  
Three Dimensional ◽  
Added Value ◽  
Maximal Wall Thickness ◽  
Apical Hypertrophy

Abstract We present the case of a 73-year-old patient with a recent diagnosis of hypertrophic cardiomyopathy (HCM). He was asymptomatic and has no family history of sudden cardiac death (SCD), syncope or ventricular arrhythmias. An echocardiogram performed at the moment of diagnosis (2020), showed left ventricular (LV) asymmetric apical hypertrophy with maximal wall thickness of 21 mm. Cardiac magnetic resonance (CMR) confirmed LV apical hypertrophy with mid-ventricular obliteration, and late gadolinium enhancement in the apical segments, without wall motion abnormalities present at rest. According to 2014 ESC guidelines, his calculated risk score for sudden cardiac death was low (1.23% at 5 years). On 2021, a comprehensive transthoracic echocardiographic examination including advanced techniques (three-dimensional echo-3DE-, and two-dimensional speckle-tracking-2DSTE) was done as part of his routine follow-up in our cardiomyopathy outpatient clinic. The echo study showed an asymmetric pattern of LV hypertrophy with a maximal wall thickness of 21 mm at the level of the anterolateral apical segment, normal LV volumes (end-diastolic volume 55 mL/m2) and ejection fraction (69%) by 3DE. LV longitudinal strain analysis by 2DSTE showed impaired LV myocardial deformation mainly at the apical LV segments (GLS = −13.6%). There was evidence of dynamic intracavitary obstruction (maximal gradient 32 mmHg at rest and raised to 52 mmHg during Valsalva manoeuvre). 3DE views of the LV (both multi-slice display and 3D rendered image) allowed to avoid foreshortening of the LV apical views, and to appreciate the actual wall motion at the real LV apex. They revealed a LV apical aneurysm which was not detected in the conventional LV-focused apical 2D views (Figure 1A and B). Apical hypertrophic cardiomyopathy (ApHCM) is a variant of HCM that is characteristic of focal thickening of the LV apical myocardium and was reported to have a more benign course than other non-apical forms. However, the presence of LV aneurysm in ApHCM patients is associated with an increased risk for ventricular arrhythmias, sudden cardiac death and thromboembolism. Accordingly, the detection of apical LV aneurysms has significant impact on patient management. Guidelines recommend the use of contrast echocardiography or CMR when the apical region of the LV is suboptimally visualized by conventional 2D echocardiography. However, contrast echocardiography may still be affected by apical foreshortening resulting in suboptimal accuracy, as it is a 2D technique. On the other end, CMR may be contraindicated or not widely available for routine yearly follow-up for all HCM patients requiring regular imaging follow-up. Our clinical case emphasizes the added value of 3DE to increase the sensitivity of transthoracic echocardiography in detecting apical LV aneurysms in patients with apical HCM with important clinical implications for the management of the patient. 813 Figure 1(A) 2D 4chamber-view showing maximal wall thickness in the apical segments (21 mm) with apical obliteration. At a first evaluation, apical aneurism is not easily detected. (B) 4D rendering of the apex showing the apical aneurism.

scholarly journals Sudden cardiac death risk in hypertrophic cardiomyopathy: comparison between echocardiography and magnetic resonance imaging

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Mateusz Śpiewak ◽  
Mariusz Kłopotowski ◽  
Ewa Kowalik ◽  
Agata Kubik ◽  
Natalia Ojrzyńska-Witek ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Wall Thickness ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Maximal Wall Thickness ◽  
Final Population ◽  
Unselected Sample ◽  
Sudden Cardiac Death Risk ◽  
Risk Categories

AbstractIn hypertrophic cardiomyopathy (HCM) patients, left ventricular (LV) maximal wall thickness (MWT) is one of the most important factors determining sudden cardiac death (SCD) risk. In a large unselected sample of HCM patients, we aimed to simulate what changes would occur in the calculated SCD risk according to the European HCM Risk-SCD calculator when MWT measured using echocardiography was changed to MWT measured using MRI. All consecutive patients with HCM who underwent cardiac MRI were included. MWT measured with echocardiography and MRI were compared, and 5-year SCD risk according to the HCM Risk-SCD calculator was computed using four different models. The final population included 673 patients [389 (57.8%) males, median age 50 years, interquartile range (36–60)]. The median MWT was lower measured by echocardiography than by MRI [20 (17–24) mm vs 21 (18–24) mm; p < 0.0001]. There was agreement between echocardiography and MRI in the measurement of maximal LV wall thickness in 96 patients (14.3%). The largest differences between echo and MRI were − 13 mm and + 9 mm. The differences in MWT by echocardiography and MRI translated to a maximal difference of 8.33% in the absolute 5-year risk of SCD, i.e., the echocardiography-based risk was 8.33% lower than the MRI-based estimates. Interestingly, 13.7% of patients would have been reclassified into different SCD risk categories if MRI had been used to measure MWT instead of echocardiography. In conclusion, although there was high general intermodality agreement between echocardiography and MRI in the MWT measurements, the differences in MWT translated to significant differences in the 5-year risk of SCD.

scholarly journals 1228 New predictors of sudden cardiac death in hypertrophic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D M Adamczak ◽  
A Rogala ◽  
M Antoniak ◽  
Z Oko-Sarnowska
Keyword(s):  
Risk Factors ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Volume Index ◽  
Lv Function ◽  
Icd Implantation ◽  
Significant Difference

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. HCM is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias. However, in most patients, HCM has a benign course. That is why it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the risk of SCD. The parameters included in the model at evaluation are: age, maximum left ventricular (LV) wall thickness, left atrial (LA) dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia (nsVT) and unexplained syncope. Nevertheless, there is potential to improve and optimize the effectiveness of this tool in clinical practice. Therefore, the following new risk factors are proposed: LV global longitudinal strain (GLS), LV average strain (ASI) and LA volume index (LAVI). GLS and ASI are sensitive and noninvasive methods of assessing LV function. LAVI more accurately characterizes the size of the left atrium in comparison to the LA dimension. METHODS 252 HCM patients (aged 20-88 years, of which 49,6% were men) treated in our Department from 2005 to 2018, were examined. The follow-up period was 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All patients underwent an echocardiographic examination. The medical and family histories were collected and ICD examinations were performed. RESULTS 76 patients underwent an ICD implantation during the follow-up period. 20 patients have reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. There were statistically significant differences of GLS and ASI values between SCD and non-SCD groups; p = 0.026389 and p = 0.006208, respectively. The average GLS in the SCD group was -12.4% ± 3.4%, and -15.1% ± 3.5% in the non-SCD group. The average ASI values were -9.9% ± 3.8% and -12.4% ± 3.5%, respectively. There was a statistically significant difference between LAVI values in SCD and non-SCD groups; p = 0.005343. The median LAVI value in the SCD group was 45.7 ml/m2 and 37.6 ml/m2 in the non-SCD group. The ROC curves showed the following cut-off points for GLS, ASI and LAVI: -13.8%, -13.7% and 41 ml/m2, respectively. Cox’s proportional hazards model for the parameters used in the Calculator was at the borderline of significance; p = 0.04385. The model with new variables (GLS and LAVI instead of LA dimension) was significant; p = 0.00094. The important factors were LAVI; p = 0.000075 and nsVT; p = 0.012267. CONCLUSIONS The proposed new SCD risk factors were statistically significant in the study population and should be taken into account when considering ICD implantation.

Prognostic role of coronary flow velocity reserve in hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Tesic ◽  
Q Ciampi ◽  
A Djordjevic-Dikic ◽  
B Beleslin ◽  
L Cortigiani ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Flow Velocity ◽  
Wall Thickness ◽  
Coronary Flow ◽  
Multivariable Analysis ◽  
Coronary Microvascular Dysfunction ◽  
Coronary Flow Velocity Reserve ◽  
Coronary Flow Velocity ◽  
Maximal Wall Thickness

Abstract Background A reduction in coronary flow velocity reserve (CFVR) related to coronary microvascular dysfunction is a major mechanism for ischemia in hypertrophic cardiomyopathy (HCM). Hypothesis To assess the functional correlates and prognostic value of CFVR during stress echocardiography (SE) in HCM. Methods We enrolled 201 HCM patients (age 51±14 years, 105 male, 52%; maximal wall thickness: 18±3 mm) studied with CFVR during exercise (n=33, 16.4%), dipyridamole (n=89, 44.3%) or adenosine (n=79, 39.3%) SE in 6 certified centers. CFVR was assessed using pulsed wave Doppler sampling in left anterior descending coronary artery. All patients completed the clinical follow-up. Results During SE mean value of CFVR was 2.11±0.46. No patients showed regional wall motion abnormalities during stress. LV outflow tract obstruction (LVOTO) was present in 34 (16.9%) patients at rest and in 47 (23.4%) at peak stress. CFVR was inversely related to age (r=−0.229, p=0.001) and maximal wall thickness (r=−0.197, p=0.031). During a median follow-up of 26 months (IQ range: 12–48 months), 75 events in 63 patients occurred: 10 deaths, 33 new hospital admission for acute heart failure, 8 sustained ventricular tachycardias and 24 atrial fibrillations. Patients in the lowest tertile (≤1.88) showed the worse prognosis with higher incidence of follow-up events compared to median tertile (1.89–2.29) and highest tertile (≥2.30) (see figure). At multivariable analysis, NYHA functional class (HR: 2.234, 95% CI: 1.398–3.517, p=0.001), presence of LVOTO at rest (HR: 2.958, 95% CI: 1.074–3.570, p=0.028) and lowest tertile of CFVR (HR: 2.144, 95% CI: 1.126–4.081, p=0.011) were the independent predictors of follow-up events. Conclusions In HCM patients, reduction in CFVR is associated to a clearly worse outcome. The spectrum of prognostic stratification is expanded if the response is titrated according to a continuous scale. Figure 1 Funding Acknowledgement Type of funding source: None

scholarly journals Validation of the 2020 AHA/ACC Risk Stratification for Sudden Cardiac Death in Chinese Patients With Hypertrophic Cardiomyopathy

2021 ◽  
Vol 8 ◽  
Author(s):  
Yan Dong ◽  
Wen Yang ◽  
Chongchong Chen ◽  
Jiamei Ji ◽  
Wei Zheng ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Implantable Cardioverter Defibrillator ◽  
Cardiac Death ◽  
Area Under The Curve ◽  
Chinese Patients ◽  
Successful Resuscitation ◽  
Cardioverter Defibrillator

Background: Sudden cardiac death (SCD) is a common cause of death in hypertrophic cardiomyopathy (HCM), but identification of patients at a high risk of SCD is challenging. The study aimed to validate the three SCD risk stratifications recommended by the 2011 ACCF/AHA guideline, the 2014 ESC guideline, and the 2020 AHA/ACC guideline in Chinese HCM patients.Methods: The study population consisted of a consecutive cohort of 511 patients with HCM without a history of SCD event. The endpoint was a composite of SCD or an equivalent event (appropriate implantable cardioverter defibrillator therapy or successful resuscitation after cardiac arrest).Results: During a follow-up of 4.7 ± 1.7 years, 15 patients (2.9%) reached the SCD endpoint and 12 (2.3%) were protected by implantable cardioverter defibrillator for primary prevention. A total of 13 (2.8%) patients experiencing SCD events were misclassified as low-risk patients by the 2011 ACCF/AHA guideline, 12 (2.3%) by the 2014 ESC model, and 7 (1.6%) by the 2020 AHA/ACC guideline. The SCD risk stratification in the 2020 AHA/ACC guideline showed greater area under the curve (0.71; 95% CI 0.56–0.87, p &lt; 0.001) than the one in the 2011 ACCF/AHA guideline (0.52; 95% CI 0.37–0.67, p = 0.76) and 2014 ESC guideline (0.68; 95% CI 0.54–0.81, p = 0.02).Conclusion: The SCD risk stratification recommended by the 2020 AHA/ACC guideline showed a better discrimination than previous stratifications in Chinese patients with HCM. A larger multicenter, independent, and prospective study with long-term follow-up would be warranted to validate our result.

scholarly journals Radial global strain by cardiac magnetic resonance imaging predicts arrhythmic outcomes in hypertrophic cardiomyopathy

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
P Martinez Vives ◽  
A Cecconi ◽  
A Vera ◽  
T Nogales-Romo ◽  
B Lopez-Melgar ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Score ◽  
Cardiac Death ◽  
Strain Analysis ◽  
Short Axis ◽  
Analysis Software ◽  
Tissue Tracking

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Hypertrophic cardiomyopathy (HCM) is a relatively prevalent condition associated with arrhythmic events and sudden cardiac death. Several tools are currently available to identify which HCM patients are at risk of developing these events. Purpose We aimed to evaluate the association of Tissue Tracking strain analysis by cardiac magnetic resonance (CMR) and the development of arrhythmic events in patients with HCM. Methods We prospectively analyzed 136 consecutive patients with HCM diagnosis (established according to current clinical practice guidelines) from January 2006 to October 2017. Every routine 24 hours ECG-monitoring test was registered and looked for sustained or non-sustained ventricular tachycardia (any VT). CMR studies were performed following our predefined CMR protocol for HCM with 1.5T magnets. Cine images were obtained with standard, retrospectively gated, steady-state free-precession (SSFP) sequences in 2, 3 and 4 chambers views and in 10–15 contiguous short-axis slices covering the ventricles from the base to the apex, with breath holding. The strain evaluation was performed by a commercially available Tissue Tracking analysis software, manually defining the endocardial border in short axis, 4, 3 and 2 chambers views and, after verifying adequate identification of the different structures, running the strain analysis (Figure 1, displaying myocardium identification by the strain analysis software). Results Mean follow-up was 49 ± 45 months. Mean age was 61 ± 15 years old (p for the comparation between the group with arrhythmic and the group without arrhythmic events 0.212) and 31% of patients were women (p 0.420). Mean ejection fraction was 69 ± 9.21% (p 0.223) and mean HCM-SCD (hypertrophic cardiomyopathy sudden cardiac death) risk score was 2.20 ± 1.34 (p &lt;0.001). Median percentage of total myocardium showing late gadolinium enhancement (LGE) was 0.61 (interquartile range 2.9; p 0.170). Mean global radial strain (GRS) was 26.23 ± 8.78% (p &lt;0.001). 21 VT episodes were recorded during follow-up. GRS showed an area under de ROC curve of 0.75 predicting VT during follow-up, selecting the value of 27% as the best sensitivity/specificity cutoff point. Statistically significant differences were not found when analyzing global circumferential strain (GCS) and global longitudinal strain (GLS) as VT predictors after adjusting for possible confusion factors (GRS, GCS and GLS distributions depicted in Figure 2). A binary GRS ≥27%/&lt;27% variable was included in a logistic regression model adjusted by age, percent of total myocardium mass showing LGE and HCM-SCD risk score. Significantly more arrhythmic events were found to occur in patients with a GRS &lt;27% (OR 7.33; 95% confidence interval 1.07 – 50.41; p 0.043) after adjusting by age, percent of total myocardium mass showing LGE, and HCM-SCD risk score Conclusions A GRS value of &lt;27% on CMR appears to be a good predictor of worse arrhythmic prognosis in patients with HCM.

scholarly journals Prevention of sudden cardiac death by the implantable cardioverter-defibrillator in adults with inherited heart disease. long term follow-up

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
M Herruzo Rojas ◽  
A Gomez Lopez ◽  
MA Martin Toro ◽  
PJ Gonzalez Perez ◽  
FJ Morales Ponce
Keyword(s):  
Heart Disease ◽  
Genetic Testing ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Supraventricular Arrhythmias ◽  
Icd Implantation

Abstract Funding Acknowledgements Type of funding sources: None. BACKGROUND Inherited heart diseases include all inheritable channelopathies or cardiomyopathies. Sudden cardiac death (SCD) might be the first and last clinical presentation. ICD implantation  is established as a therapeutic tool according to risk criteria. OBJETIVES The aim of this study is to analyze the population characteristics in ICD/ CRT-ICD recipients in patients with inherited heart disease and establish possible risk predictor factors of arrhytmogenic events during follow-up. MÉTHODS: This is a prospective single-center registry. We included all patients from January1, 2012, to December 31, 2020 who subsequently underwent ICD/ ICD-CRT implantation. RESULTS A total of 172 patients were included with a mean age of 60.47 ± 13,1 years and a mean follow-up duration of 49.71 ± 41.8 months. The most frequent underlying cardiac condition was dilated cardiomyopathy (58.1%), followed by hypertrophic cardiomyopathy (15.7%), arrythmogenic cardiomyopathy (2.3%), long QT syndrome (1.7%), Brugada syndrome (1.2%), catecholaminergic (2.9%) , idiopathic (7%) and others (11%). 89 ICDs (51.7%) and 83 ICD-CRTs (48.2%) were implanted for primary prevention of SCD. During follow-up, 13.4% of the patients experienced arrhythmogenic events. Only 4.1% of the patients experienced inappropriate shock, secondary to supraventricular arrhythmias all of them. Genetic testing was done in only 14.1% of our patients. In the 27 cases of hypertrophic cardiomyopathy, 25 (92.6%) underwent ICD implantation for primary prevention. 14 patients (51.9%) had family history of SCD, 8 (29.6%) had unexplained syncope and 16 (59.3%) had ventricular tachycardia in the Holter monitoring. The mean left atrial size was 44.15 ± 7.2mm, mean maximum LV-wall thickness 22.85 ± 5,25mm, and a mean LVOT gradient of 30.56 ± 31.3mmHg, with a mean actual HCM SCD-score of SCD over 5 years of 5.84 ± 3.53%. According to traditional parameters, 100% of the HCM sample meet criteria for ICD implantation in primary prevention compared to 66.7% according to the new score. During follow-up 2 patients (7.4% ) experienced arrhythmogenic events, and 3 patients (11.1%) had inappropriate shocks secondary to supraventricular arrhythmias. Genetic testing was done in 22.2% of the patients and was positive for a known pathogenic mutation in half of the cases. None of the parameters evaluated in the current score, a high risk of SCD according to it, having old high risk criteria or implanted of ICD in secondary prevention were related in our study with more arrhytmogenic events. CONCLUSIONS ICD implatation for primary prevention is the main indication in our population. Either classic factors or the new HCM SCD-score were no related in our study with the presence of arrhytmogenic events during follow-up.

scholarly journals A systematic approach to echocardiography in hypertrophic cardiomyopathy: a guideline protocol from the British Society of Echocardiography

2015 ◽  
Vol 2 (1) ◽  
pp. G1-G7 ◽  
Author(s):  
Nicola Smith ◽  
Richard Steeds ◽  
Navroz Masani ◽  
Julie Sandoval ◽  
Gill Wharton ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Systematic Approach ◽  
British Society ◽  
The Core ◽  
Echocardiographic Study ◽  
Cardiac Condition ◽  
Minimum Dataset

Hypertrophic cardiomyopathy (HCM) is a relatively common inherited cardiac condition with a prevalence of approximately one in 500. It results in otherwise unexplained hypertrophy of the myocardium and predisposes the patient to a variety of disease-related complications including sudden cardiac death. Echocardiography is of vital importance in the diagnosis, assessment and follow-up of patients with known or suspected HCM. The British Society of Echocardiography (BSE) has previously published a minimum dataset for transthoracic echocardiography, providing the core parameters necessary when performing a standard echocardiographic study. However, for patients with known or suspected HCM, additional views and measurements are necessary. These additional views allow more subtle abnormalities to be detected or may provide important information in order to identify patients with an adverse prognosis. The aim of this Guideline is to outline the additional images and measurements that should be obtained when performing a study on a patient with known or suspected HCM.

RESEARCH THE VALUE OF THE COMBINATION OF T WAVE ALTERNANS AND NT-PROBNT IN PREDICTING SUDDEN CARDIAC DEATH

2012 ◽  
pp. 74-83
Author(s):  
Anh Tien Hoang ◽  
Nhat Quang Nguyen
Keyword(s):  
Myocardial Infarction ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Healthy Person ◽  
Treadmill Test ◽  
T Wave ◽  
T Wave Alternans

Background: Decades of research now link TWA with inducible and spontaneous clinical ventricular arrhythmias. This bench-to-bedside foundation makes TWA, NT-ProBNP a very plausible index of susceptibility to ventricular arrythmia, and motivates the need to define optimal combination of TWA and NT-ProBNP in predicting ventricular arrythmia in myocardial infarction patients. We research this study with 2 targets: 1. To evaluate the role of TWA in predicting sudden cardiac death in myocardial infarction patients. 2. To evaluate the role of NT-ProBNP in predicting sudden cardiac death in myocardial infarction patients 3. Evaluate the role of the combined NT-ProBNP and TWA in predicting sudden cardiac death in myocardial infarction patients. Methods: Prospective study with follow up the mortality in 2 years: 71 chronic myocardial infarction patients admitted to hospital from 5/2009 to 5/20011 and 50 healthy person was done treadmill test to caculate TWA; ECG, echocardiography, NT-ProBNP. Results: Cut-off point of NT-ProBNP in predicting sudden cardiac death is 3168 pg/ml; AUC = 0,86 (95% CI: 0,72 - 0,91); Cut-off point of TWA in predicting sudden cardiac death is 107 µV; AUC = 0,81 (95% CI: 0,69 - 0,87); NT-ProBNP can predict sudden cardiac death with OR= 7,26 (p<0,01); TWA can predict sudden cardiac death with OR= 8,45 (p<0,01). The combined NT-ProBNP and TWA in predicting ventricular arrythmia in heart failure patients: OR= 17,91 (p<0,001). Conclusions: The combined NT-ProBNP and TWA have the best predict value of sudden cardiac death in myocardial infarction patients, compare to NT-ProBNP or TWA alone

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