Prominent para-cardiac fat mimicking left atrial appendage thrombus on echocardiography: Utility of cardiac computed tomography.

Transesophageal echocardiography is the most frequently used imaging modality for exclusion of left atrial appendage thrombus prior to cardioversion. Echocardiographers should be aware of rare conditions that may mimic left atrial appendage thrombus. Here, we describe a rare case of prominent para-cardiac fat mimicking left atrial appendage thrombus on transesophageal echocardiographic imaging. Multimodality imaging with cardiac computed tomography was instrumental in providing further anatomical delineation and characterization of the echodensity as prominent para-cardiac fat in this case.

Diastolic Stress Echocardiography in Patients With Hypertrophy Cardiomyopathy: Association With Exercise Capacity.

Diastolic dysfunction plays a major role in precipitating congestive heart failure in patients with hypertrophic cardiomyopathy (HCM). In many such patients, symptoms are unmasked only during exercise because left ventricular (LV) filling pressure is normal at rest but increase with exercise. We sought to establish whether abnormal postexercise diastolic parameters are associated with reduced exercise capacity in patients with HCM. We examined 590 patients with asymptomatic/minimally symptomatic HCM (age 54 ± 14 years, 57% men, body mass index 30 ± 6 kg/m2, 84% on ß blockers) with HCM by 2-dimensional and Doppler echocardiography at rest and after maximal treadmill exercise echocardiography (TSE). A complete echocardiogram (including LV ejection fraction, LV thickness, LV outflow tract [LVOT] gradient, degree of mitral regurgitation) was recorded. Diastolic parameters (septal and lateral [e'] velocities of the mitral annulus, peak early [E] and late [A] mitral inflow velocity, E/A ratio, E/e', right ventricular systolic pressure [RVSP], and left atrial volume index) were recorded at rest and after TSE. Exercise functional capacity was recorded and divided into <85% or >85% of age-gender predicted metabolic equivalents (AGP-METs). After maximal exercise, 32% patients had at least moderate mitral regurgitation, mean LVOT gradient was 61 ± 59 mm Hg, E/A ratio was 1.2 ± 1.0, average E/e' ratio 12.9 ± 1.0, and peak RVSP was 36 ± 15 mm Hg. Only 42% of patients achieved >85% of AGP-METs; the mean METs was 7 ± 3. On multivariable logistic regression analysis, higher body mass index (odds ratio [OR] 1.05), ß-blocker use (OR 2.58), higher left atrial volume index (OR 1.02), higher peak stress LVOT gradient (LVOTG) (OR 1.06), peak stress E/e' (OR 1.04), and higher RVSP (OR 1.03) were independently associated with <85% AGP-METs achieved (all p <0.05). In conclusion, in patients with asymptomatic/minimally symptomatic HCM who underwent TSE, there is a significant and independent association between abnormal diastolic response to exercise and reduced exercise capacity in HCM. Incorporating diastolic parameters during stress echocardiography could provide incremental diagnostic utility in deciphering the exact etiology of dyspnea in such patients.

Measurements of the Interventricular Septum and Mitral Leaflet Length in Hypertrophic Cardiomyopathy Patients Who Underwent Surgical Myectomy: A Prospective Comparative Multimodality Imaging Study.

Transesophageal echocardiography (TEE) plays an important role for real-time procedural guidance during surgical smyectomy (SM) for hypertrophic obstructive cardiomyopathy (HOCM). We aimed to compare (1) interventricular septum (IVS) thickness using 2- (2D) and 3-dimensional (3D) intraoperative TEE and preoperative cardiac magnetic resonance (CMR) and (2) mitral valve (MV) leaflet length using 2D, 3D TEE, automatic quantification of mitral valve (AMVQ) and preoperative CMR. We prospectively studied 50 patients with HOCM (age 59 ± 12 years, 44% men) who underwent SM during 2018 to 2019. The maximal basal, mid, and distal anteroseptum (AS) and inferoseptum (IS) were measured by multiplanar 3D reconstruction on TEE and by short-axis imaging on preoperative CMR and classified as mild (≤18 mm), moderate (18 to 25 mm), or severe (≥25 mm) groups based on AS and IS thickness on CMR. MV leaflet lengths were evaluated by preoperative CMR and intraprocedural 2D TEE, zoom 3D TEE, and AMVQ (EchoPAC, General Electric, Wisconsin). There was a moderate correlation between AS and IS thickness on 3D TEE and CMR (R2 = 0.46, p <0.01 and R2 = 0.41, p <0.01, respectively), with 3D TEE showing an average overestimation of 3.8 and 4.7 mm versus CMR. The 3D TEE overestimated 14 patients (56%) with mild thickness as moderate and 5 patients (22%) with moderate thickness as severe. Assuming 3D TEE as the gold standard, the closest correlation for anterior mitral leaflet length was with CMR (average overestimation by CMR of 0.5 mm [root mean square deviation (RMSE%) 17]), intermediate correlation with 2D TEE (average deviation of 0.6 mm [RMSE% 21]) and no correlation with AMVQ (average deviation of 0.7 mm [RMSE% 24]). In conclusion, 3D TEE overestimates IVS thickness versus CMR in patients with HOCM who underwent SM, with greater discrepancy in those with thinner IVS. There are significant differences in MV lengths measured using different imaging techniques.

Patient reported outcomes in obstructive hypertrophic cardiomyopathy undergoing myectomy: Results from SPIRIT-HCM study.

BACKGROUND: Patient reported outcomes (PRO) can assess quality of life (QOL) in obstructive hypertrophic cardiomyopathy (oHCM). In symptomatic oHCM patients, we sought to study the correlation between various PROs, their association with physician reported New York Heart Association (NYHA) class and changes after surgical myectomy. METHODS: We prospectively studied 173 symptomatic oHCM patients undergoing myectomy (age 51 years, 62% men) between 3/17-6/20. PROs, including a) Kansas City Cardiomyopathy Questionnaire (KCCQ) summary score b) Patient-Reported Outcomes Measurement Information System [PROMIS] c) Duke Activity Status Index [DASI] & d) European QOL score [EQ-5D], along with NYHA class, 6-min walk test (6MWT) distance and peak left ventricular outflow tract gradient (PLVOTG) were recorded at baseline and 12 month follow-up. RESULTS: The median baseline PRO scores (KCCQ summary, PROMIS physical, PROMIS mental, DASI, EQ-5D) were 50, 67, 63, 25, 50, 37, 44, 25 and 0.61, respectively; 6MWT distance was 366 m. There were significant correlations between various PROs (r-values between 0.66 and 0.92, p < 0.001), but only modest correlations with 6MWT and provokable LVOTG (r-values between 0.2 and 0.5, p < 0.01). At baseline, 35-49% patients in NYHA class II had PROs worse than median, while 30-39% patients in NYHA Class III/IV had PROs better than median. At follow-up, a 20 point improvement in KCCQ summary score was observed in 80%, 4 point improvement in DASI score in 83%, 4 point improvement in PROMIS physical score 86% and a 0.04 point improvement in EQ-5D in 85%); along with improvements in NYHA class (67% in Class I) and peak LVOTG (median 13 mmHg) and 6MWT (median distance 438 m). CONCLUSIONS: In a prospective study of symptomatic oHCM patients, surgical myectomy significantly improved PROs, LVOT obstruction, and functional capacity, with a high correlation between various PROs. However, there was high rate of discordance between PROs and NYHA class. STUDY REGISTRATION: ClinicalTrials.gov: NCT03092843.

Quantitative Echocardiographic Assessment and Optimal Criteria for Early Intervention in Asymptomatic Tricuspid Regurgitation.

BACKGROUND: Significant tricuspid regurgitation (TR) is associated with poor outcome and high operative mortality resulting from late presentation. Yet, the optimal timing for intervention is unknown. OBJECTIVES: The purpose of this study was to evaluate the prognostic value of echocardiographic parameters to inform early intervention in asymptomatic TR. METHODS: Using the Cleveland Clinic echocardiography database 2004 to 2018, the authors identified a consecutive cohort of asymptomatic patients with moderate to severe (3+) or severe (4+) TR. Quantitative TR and right heart parameters were retrospectively determined, and their prognostic utility for all-cause mortality was assessed. RESULTS: In 325 asymptomatic patients (mean age: 67.9 years; 79.4% female) with at least 3+ TR, there were 132 deaths (40.6%), with a median survival time of 9.9 years (95% CI: 7.9-12.7 years). By contrast, the median survival time in an age- and sex-matched cohort of symptomatic TR patients was 4.4 years (95% CI: 2.8-5.9 years). Among all the echocardiographic parameters evaluated, right ventricle free wall strain (RVFWS) and tricuspid regurgitant volume (RVol) were the strongest predictors of mortality in asymptomatic TR. The optimal discriminatory thresholds for these parameters were RVFWS <-19% and RVol >45 mL. The 5-year survival rates by number of risk factors (RF) were 93% (95% CI: 86%-96%), 65% (95% CI: 55%-74%), and 38% (95% CI: 26%-49%) for no RF, 1 RF, and both RFs, respectively. Compared with symptomatic TR, mortality was lower for asymptomatic TR with no RF (HR: 0.10; 95% CI: 0.04-0.29) or 1 RF (HR: 0.29; 95% CI: 0.14-0.58), but similar for asymptomatic TR with both RFs (HR: 1.11; 95% CI: 0.56-2.19). CONCLUSIONS: RVFWS and RVol are key prognostic markers that can be serially monitored to inform optimal timing of intervention for severe asymptomatic TR.

Rilonacept for the treatment of recurrent pericarditis.

INTRODUCTION: Recurrent pericarditis (RP) is a debilitating disease that has an underlying autoinflammatory pathophysiology mediated by cytokine interleukin (IL)-1. Rilonacept, a recombinant dimeric fusion protein that blocks IL-1α and IL-1ß signaling has emerged as a valuable therapeutic option of RP. Rilonacept has been evaluated in Phase 2 and 3 clinical trials and was recently approved for RP treatment. AREAS COVERED: This article reviews available clinical trials assessing the efficacy and safety of rilonacept for the treatment of RP. EXPERT OPINION: Findings from the Rhapsody study) trial suggest that rilonacept represents a promising new therapy for those patients with colchicine resistant or glucocorticoid-dependent disease. Treatment leads to rapid clinical response, with a median resolution of symptoms in 5 days, normalization of C-reactive protein (CRP) in a median of 7 days, and successful weaning from glucocorticoids. This novel therapy also reduces recurrence rates compared with placebo. Rilonacept has also demonstrated a good safety profile, with the most common adverse events including injection-site reactions and upper respiratory tract infections. This anti-IL 1 agent has emerged as an efficacious treatment for RP, with potential use for glucocorticoid-free regimens and as monotherapy. Future trials are needed to explore these treatment options and to clarify the appropriate therapy duration.

Prognostic value of age-sex adjusted NT-proBNP ratio in obstructive hypertrophic cardiomyopathy.

BACKGROUND: We sought to determine the incremental prognostic value of age-sex adjusted N-terminal prohormone brain natriuretic peptide (NT-pro BNP) ratio in obstructive hypertrophic cardiomyopathy (oHCM) patients. METHODS: The study included 2119 consecutive oHCM patients (age 55 ± 13 years, 53% men, maximal LVOT ≥30 mmHg) evaluated between 6/2002-12/2018 with BNP or NT-pro BNP measured at baseline. NT-pro BNP ratio was calculated as: NT-proBNP/ upper limit of normal NT-proBNP derived from age-sex matched controls. Septal reduction therapy (SRT) during follow-up was recorded. Primary endpoint was death, need for cardiac transplantation or appropriate internal cardioverter defibrillator (ICD) discharge. RESULTS: Median NT-proBNP ratio was 5.4 (IQR 2.1-12.3). Using spline analysis, log-transformed NT-pro BNP ratio of 2 (corresponding to NT-pro BNP ratio of 6) was the optimal value where primary endpoint hazards crossed 1; there were 966 patients with high and 1153 patients with low NT-pro BNP ratio. 1665 (79%) patients underwent SRT at 47 days (IQR 7-128 days). At 5.4 years of follow-up (IQR 2.8-9.2 years), the primary outcome occurred in 315 (15%) patients (deaths = 270). High NT-pro BNP ratio was associated with higher risk of primary outcome in unadjusted (30.1 vs. 17.2 events/1000 person-year, hazard ratio or (HR) 1.73, 1.37-2.17, P < 0.001) and adjusted analysis (aHR 1.69, 95% 1.19-2.38, P = 0.003) vs. low NT-pro BNP ratio. Even in asymptomatic patients, NT-pro BNP ratio remained associated with primary outcome (aHR 1.28, 95% CI 1.06-1.54, P = 0.01). CONCLUSIONS: Age-sex adjusted NT-pro BNP ratio is independently associated with long-term outcomes in oHCM patients, including in a subgroup of asymptomatic patients.

Tricuspid valve endocarditis: Cardiovascular imaging evaluation and management.

Right-sided infective endocarditis is an increasingly recognized disease entity, with tricuspid valve being most frequently involved. Risk factors for tricuspid valve endocarditis (TVIE) include intravenous drug use, cardiac implantable electronic devices and indwelling catheters. Staphylococcus aureus is the predominant causative organism in TVIE. The diagnosis of infective endocarditis (IE) is based on clinical manifestations, blood cultures, and the presence of valvular vegetations detected by echocardiography. Complementary imaging is helpful when there is ongoing clinical suspicion for IE following initially negative echocardiography. Multislice computed tomography allows for assessment of extra-cardiac complications in TVIE, including pulmonary septic emboli. 18F-fluorodeoxyglucose positron emission tomography/computed tomography and radiolabelled white blood cell, single-photon emission computed tomography provide important clinical information concerning the presence of IE in right-sided prosthetic valves or cardiac implantable electronic devices. The aim of this review is to provide an update on TVIE, discussing the role of multimodality imaging in TVIE and the management of these patients.

Characteristics and Outcomes of Elderly Patients With Hypertrophic Cardiomyopathy.

Background We report characteristics and outcomes of elderly patients with hypertrophic cardiomyopathy (HCM) with basal septal hypertrophy and dynamic left ventricular outflow tract obstruction. Methods and Results We studied 1110 consecutive elderly patients with HCM (excluding moderate or greater aortic stenosis or subaortic membrane, age 80±5 years [range, 75-92 years], 66% women), evaluated at our center between June 2002 and December 2018. Clinical and echocardiographic data, including maximal left ventricular outflow tract gradient, were recorded. The primary outcome was death and appropriate internal defibrillator discharge. Hypertension was observed in 72%, with a Society of Thoracic Surgeons (STS) score (8.6±6); while 80% had no HCM-related sudden cardiac death risk factors. Left ventricular mass index, basal septal thickness, and maximal left ventricular outflow tract gradient were 127±43 g/m2, 1.7±0.4 cm, and 49±31 mm Hg, respectively. A total of 597 (54%) had a left ventricular outflow tract gradient >30 mm Hg, of which 195 (33%) underwent septal reduction therapy (SRT; 79% myectomy and 21% alcohol ablation). At 5.1±4 years, 556 (50%) had composite events (273 [53%] in nonobstructive, 220 [55%] in obstructive without SRT, and 63 [32%] in obstructive subgroup with SRT). One- and 5-year survival, respectively were 93% and 63% in nonobstructive, 90% and 63% in obstructive subgroup without SRT, and 94% and 84% in the obstructive subgroup with SRT. Following SRT, there were 5 (2.5%) in-hospital deaths (versus an expected Society of Thoracic Surgeons mortality of 9.2%). Conclusions Elderly patients with HCM have a high prevalence of traditional cardiovascular rather than HCM risk factors. Longer-term outcomes of the obstructive SRT subgroup were similar to a normal age-sex matched US population.

Standardized measurement of coronary inflammation using cardiovascular computed tomography: integration in clinical care as a prognostic medical device.

AIMS: Coronary computed tomography angiography (CCTA) is a first-line modality in the investigation of suspected coronary artery disease (CAD). Mapping of perivascular fat attenuation index (FAI) on routine CCTA enables the non-invasive detection of coronary artery inflammation by quantifying spatial changes in perivascular fat composition. We now report the performance of a new medical device, CaRi-Heart®, which integrates standardized FAI mapping together with clinical risk factors and plaque metrics to provide individualized cardiovascular risk prediction. METHODS AND RESULTS: The study included 3912 consecutive patients undergoing CCTA as part of clinical care in the USA (n = 2040) and Europe (n = 1872). These cohorts were used to generate age-specific nomograms and percentile curves as reference maps for the standardized interpretation of FAI. The first output of CaRi-Heart® is the FAI-Score of each coronary artery, which provides a measure of coronary inflammation adjusted for technical, biological, and anatomical characteristics. FAI-Score is then incorporated into a risk prediction algorithm together with clinical risk factors and CCTA-derived coronary plaque metrics to generate the CaRi-Heart® Risk that predicts the likelihood of a fatal cardiac event at 8 years. CaRi-Heart® Risk was trained in the US population and its performance was validated externally in the European population. It improved risk discrimination over a clinical risk factor-based model [Δ(C-statistic) of 0.085, P = 0.01 in the US Cohort and 0.149, P < 0.001 in the European cohort] and had a consistent net clinical benefit on decision curve analysis above a baseline traditional risk factor-based model across the spectrum of cardiac risk. CONCLUSION: Mapping of perivascular FAI on CCTA enables the non-invasive detection of coronary artery inflammation by quantifying spatial changes in perivascular fat composition. We now report the performance of a new medical device, CaRi-Heart®, which allows standardized measurement of coronary inflammation by calculating the FAI-Score of each coronary artery. The CaRi-Heart® device provides a reliable prediction of the patient's absolute risk for a fatal cardiac event by incorporating traditional cardiovascular risk factors along with comprehensive CCTA coronary plaque and perivascular adipose tissue phenotyping. This integration advances the prognostic utility of CCTA for individual patients and paves the way for its use as a dual diagnostic and prognostic tool among patients referred for CCTA.

Advanced imaging for risk stratification of sudden death in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac condition, which typically manifests as left ventricular hypertrophy. A small subset of patients with HCM have an increased risk of sudden cardiac death (SCD) from ventricular arrhythmias. Risk of SCD can be effectively reduced following implantation of implantable cardiac defibrillators (ICD), although this treatment carries a risk of complications such as inappropriate shocks. With this in mind, we turn to advances in cardiac imaging to guide risk stratification for SCD and to select the appropriate individual who may benefit from ICD implantation. In this review, we have taken the opportunity to briefly summarise the role of imaging in the diagnosis of HCM before focusing on how specific imaging features influence risk of SCD in patients with HCM.

Sonothrombolysis Improves Myocardial Dynamics and Microvascular Obstruction Preventing Left Ventricular Remodeling in Patients With ST Elevation Myocardial Infarction.

BACKGROUND: It has recently been demonstrated that high-energy diagnostic transthoracic ultrasound and intravenous microbubbles dissolve thrombi (sonothrombolysis) and increase angiographic recanalization rates in patients with ST-segment-elevation myocardial infarction. We aimed to study the effect of sonothrombolysis on the myocardial dynamics and infarct size obtained by real-time myocardial perfusion echocardiography and their value in preventing left ventricular remodeling. METHODS: One hundred patients with ST-segment-elevation myocardial infarction were randomized to therapy (50 patients treated with sonothrombolysis and percutaneous coronary intervention) or control (50 patients treated with percutaneous coronary intervention only). Left ventricular volumes, ejection fraction, risk area (before treatment), myocardial perfusion defect over time (infarct size), and global longitudinal strain were determined by quantitative real-time myocardial perfusion echocardiography and speckle tracking echocardiography imaging. RESULTS: Risk area was similar in the control and therapy groups (19.2±10.1% versus 20.7±8.9%; P=0.56) before treatment. The therapy group presented a behavior significantly different than control group over time (P<0.001). The perfusion defect was smaller in the therapy at 48 to 72 hours even in the subgroup of patients with no recanalization at first angiography (12.9±6.5% therapy versus 18.8±9.9% control; P=0.015). The left ventricular global longitudinal strain was higher in the therapy than control immediately after percutaneous coronary intervention (14.1±4.1% versus 12.0±3.3%; P=0.012), and this difference was maintained until 6 months (17.1±3.5% versus 13.6±3.6%; P<0.001). The only predictor of left ventricular remodeling was treatment with sonothrombolysis: the control group was more likely to exhibit left ventricular remodeling with an odds ratio of 2.79 ([95% CI, 0.13-6.86]; P=0.026). CONCLUSIONS: Sonothrombolysis reduces microvascular obstruction and improves myocardial dynamics in patients with ST-segment-elevation myocardial infarction and is an independent predictor of left ventricular remodeling over time.

Clinical Applications of Echo Strain Imaging: a Current Appraisal.

PURPOSE OF REVIEW: This article reviews recent advances in echocardiographic strain imaging, particularly in its ability to prognosticate in cardiovascular outcomes and impact clinical decision making. RECENT FINDINGS: Strain has been proposed as a sensitive tool in detecting early ventricular dysfunction. Left ventricular global longitudinal strain (LV-GLS) detects subtle changes in myocardial function, often not quantifiable by ejection fraction alone. Thus, LV-GLS provides the opportunity for early decision-making, and the implementation of more effective treatments, improving outcomes in a variety of diseases such as valvular heart diseases, cardio-oncology, ischemic heart disease, cardiomyopathies, heart transplantation, and pericardial diseases and cardiomyopathies. Strain is a promising tool for the early detection of myocardial dysfunction in patients with preserved left ventricular ejection fraction and can prognosticate long-term outcomes.

Sonothrombolysis in ST-Segment Elevation Myocardial Infarction Treated With Primary Percutaneous Coronary Intervention.

BACKGROUND: Preclinical studies have demonstrated that high mechanical index (MI) impulses from a diagnostic ultrasound transducer during an intravenous microbubble infusion (sonothrombolysis) can restore epicardial and microvascular flow in acute ST-segment elevation myocardial infarction (STEMI). OBJECTIVES: This study tested the clinical effectiveness of sonothrombolysis in patients with STEMI. METHODS: Patients with their first STEMI were prospectively randomized to either diagnostic ultrasound-guided high MI impulses during an intravenous Definity (Lantheus Medical Imaging, North Billerica, Massachusetts) infusion before, and following, emergent percutaneous coronary intervention (PCI), or to a control group that received PCI only (n = 50 in each group). A reference first STEMI group (n = 203) who arrived outside the randomization window was also analyzed. Angiographic recanalization before PCI, ST-segment resolution, infarct size by magnetic resonance imaging, and systolic function (LVEF) at 6 months were compared. RESULTS: ST-segment resolution occurred in 16 (32%) high MI PCI versus 2 (4%) PCI-only patients before PCI, and angiographic recanalization was 48% in high MI/PCI versus 20% in PCI only and 21% in the reference group (p < 0.001). Infarct size was reduced (29 ± 22 g high MI/PCI vs. 40 ± 20 g PCI only; p = 0.026). LVEF was not different between groups before treatment (44 ± 11% vs. 43 ± 10%), but increased immediately after PCI in the high MI/PCI group (p = 0.03), and remained higher at 6 months (p = 0.015). Need for implantable defibrillator (LVEF ≤30%) was reduced in the high MI/PCI group (5% vs. 18% PCI only; p = 0.045). CONCLUSIONS: Sonothrombolysis added to PCI improves recanalization rates and reduces infarct size, resulting in sustained improvements in systolic function after STEMI. (Therapeutic Use of Ultrasound in Acute Coronary Artery Disease; NCT02410330).

Validation of the 2014 European Society of Cardiology Sudden Cardiac Death Risk Prediction Model in Hypertrophic Cardiomyopathy in a Reference Center in South America.

Sudden cardiac death (SCD) is a common cause of death in hypertrophic cardiomyopathy (HC). Our aim was to conduct an external and independent validation in South America of the 2014 European Society of Cardiology (ESC) SCD risk prediction model to identify patients requiring an implantable cardioverter defibrillator. This study included 502 consecutive patients with HC followed from March, 1993 to December, 2014. A combined end point of SCD or appropriate implantable cardioverter defibrillator therapy was assessed. For the quantitative estimation of individual 5-year SCD risk, we used the formula: 1 - 0.998(exp(Prognostic index)). Our database also included the abnormal blood pressure response to exercise as a risk marker. We analyzed the 3 categories of 5-year risk proposed by the ESC: low risk (LR) <4%; intermediate risk (IR) ≥4% to <6%, and high risk (HR) ≥6%. The LR group included 387 patients (77%); the IR group 39 (8%); and the HR group 76 (15%). Fourteen patients (3%) had SCD/appropriate implantable cardioverter defibrillator therapy (LR: 0%; IR: 2 of 39 [5%]; and HR: 12 of 76 [16%]). In a receiver-operating characteristic curve, the new model proved to be an excellent predictor because the area under the curve for the estimated risk is 0.925 (statistical C: 0.925; 95% CI 0.8884 to 0.9539, p <0.0001). In conclusion, the SCD risk prediction model in HC proposed by the 2014 ESC guidelines was validated in our population and represents an improvement compared with previous approaches. A larger multicenter, independent and external validation of the model with long-term follow-up would be advisable.

Role of stress Echo on the Prognosis of Hypertrophic Cardiomyopathy / Rol del eco estrés en el pronóstico de la miocardiopatía hipertrófica

Background: Patients with hypertrophic cardiomyopathy (HCM) frequently present with confusing and misleading symptoms. In these instances stress tests may help to stratify the risk of future events. Objective: The purpose of this study was to assess the prognostic usefulness of the different variables obtained with exercise stress echo (ESE) in patients with HCM. Methods: A retrospective and observational study was performed in 110 patients evaluated with ESE. Patients were divided into 3 groups according to their left ventricular outflow tract obstruction level (LVOTO): 1) persistent LVOTO (peak instantaneous gradient at rest obtained by continuous Doppler = 30 mmHg), 2) latent LVOTO (gradient = 50 mmHg with exercise); and no LVOTO. Median follow-up was 2.7 years. The primary endpoint was the composite of death, sudden death, sustained ventricular tachycardia or hospitalization for heart failure. Results: Persistent LVOTO was present in 19.1% of cases, latent LVOTO in 31.8% and no LVOTO in 49.1%. Ventricular function, wall thicknesses and diameters were similar for the three groups. Poor prognostic variables were significantly higher for persistent LVOTO. The latent LVOTO group developed more symptoms, electrocardiographic changes and mitral regurgitation after exercise than the group without LVOTO, although it was not associated with a higher number of events.Variables that were associated with increased rate of events during follow-up were the presence of gradient = 30 mmHg at rest (p=0.07), electrocardiographic changes during the test (p=0.020) and the inverse relationship of METs (p=0.07). Conclusions: Patients with HCM who achieved a high exercise capacity, expressed as METs = 7, showed excellent mid- to long-term outcomes. LVOTO at rest and electrocardiographic changes during maximal stress exercise were associated with an increased number of events during follow-up.

Introducción: Los pacientes con miocardiopatía hipertrófica (MCH) presentan con frecuencia síntomas confusos y equívocos. En estas instancias, las pruebas de esfuerzo pueden ayudar a la estratificación de riesgo de eventos futuros. Objetivo: Evaluar el valor pronóstico de las diferentes variables obtenidas mediante el eco estrés con ejercicio (EEE) en pacientes con diagnóstico de MCH. Material y métodos: Estudio retrospectivo y observacional. Se evaluaron 110 pacientes mediante EEE, los cuales se dividieron según el grado de obstrucción a nivel del tracto de salida del ventrículo izquierdo (OTSVI) en: 1) OTSVI persistente (gradiente máximo instantáneo obtenido en reposo mediante Doppler continuo = 30 mm Hg), 2) OTSVI latente (gradiente = 50 mm Hg ante el ejercicio) y 3) sin OTSVI. La mediana de seguimiento fue de 2,7 años. Se definió punto final primario a la ocurrencia de muerte, muerte súbita, taquicardia ven-tricular sostenida o internación por insuficiencia cardíaca. Resultados: El 19,1% de los pacientes presentaron OTSVI persistente, el 31,8% OTSVI latente y el 49,1% no presentaban OTSVI. La función ventricular, los espesores parietales y los diámetros fueron similares para los tres grupos. Las variables de mal pronóstico fueron significativamente mayores para la OTSVI persistente. El grupo con OTSVI latente desarrolló más síntomas, alteraciones electrocardiográficas e insuficiencia mitral posejercicio que el grupo sin OTSVI, aunque no se asoció con un número mayor de eventos. Las variables que se asociaron con más eventos en el seguimiento fueron la presencia de gradiente = 30 mm Hg en reposo (p = 0,07), alteraciones electrocardiográficas durante la prueba (p = 0,020) y los MET en su relación inversa (p = 0,07). Conclusiones: Los pacientes con MCH que alcanzaron una alta capacidad de ejercicio, expresada como MET = 7, presentaron excelentes resultados a mediano-largo plazo. La OTSVI en reposo y los cambios del electrocardiograma durante el esfuerzo máximo se asociaron con más eventos en el seguimiento.

Doppler tisular y estratificación del riesgo en la miocardiopatía hipertrófica / Tissue Doppler and Risk Stratification in Hypertrophic Cardiomyopathy

Introducción El Doppler pulsado tisular ha demostrado beneficio en la detección temprana de la miocardiopatía hipertrófica y en el diagnóstico diferencial de esta con otras causas secundarias de hipertrofia. Objetivo Determinar el valor pronóstico de las velocidades miocárdicas sistólicas tisulares preservadas en pacientes con diagnóstico de miocardiopatía hipertrófica. Material y métodos Se incluyeron 146 pacientes con diagnóstico de miocardiopatía hipertrófica, los cuales fueron evaluados en forma prospectiva mediante un estudio de ecocardiograma Doppler. Se obtuvieron las velocidades sistólicas tisulares del promedio de las velocidades septales y laterales; se compararon los pacientes con velocidades miocárdicas sistólicas tisulares preservadas (Sa = 8 cm/seg; cuartil superior) con los que presentaban velocidades disminuidas. Se definió como punto final primario a la presencia de muerte súbita, accidente cerebrovascular, insuficiencia cardíaca o internación de causa cardiovascular en el seguimiento. Resultados El 29% (n = 43) presentó velocidades miocárdicas sistólicas tisulares preservadas en las imágenes del Doppler tisular, con más frecuencia de varones (76,7% vs. 53,4%; p = 0,009) y sin diferencias en la edad. Los diámetros ventriculares y los espesores fueron similares, en tanto que el área auricular fue significativamente menor (23,7 ± 6,7 cm² vs. 28,8 ± 8 cm²; p < 0,001). En el seguimiento (mediana de 2,7 años), el número de eventos aumentó significativamente a medida que disminuyeron las velocidades sistólicas en el Doppler pulsado tisular. Ningún paciente del grupo velocidades miocárdicas sistólicas tisulares preservadas presentó el punto final combinado, con diferencias significativas con respecto al grupo control (0% vs. 21,6%; p = 0,001) y un valor predictivo negativo del 100%. Conclusiones En nuestra población de pacientes portadores de miocardiopatía hipertrófica, la presencia de velocidades miocárdicas sistólicas tisulares preservadas en el Doppler pulsado tisular permitió identificar a una subpoblación de pacientes de riesgo bajo, con un escaso número de eventos en el seguimiento, con un valor predictivo negativo elevado.

Usefulness of Tissue Doppler Imaging to Identify Low Risk Patients with Diagnosis of Hypertrophic Cardiomyopathy Background Pulsed tissue Doppler imaging is a useful tool for the early detection of hypertrophic cardiomyopathy and the differential diagnosis of this disease from other secondary causes of hypertrophy. Objective The aim of the study was to determine the prognostic significance of preserved systolic tissue velocities in patients with diagnosis of hypertrophic cardiomyopathy. Methods One hundred and forty six patients with diagnosis of hypertrophic cardiomyopathy were prospectively included by means of a Doppler echocardiography study. Systolic tissue velocities were obtained from the averaged septal and lateral velocities. Patients with preserved systolic tissue velocities (Sa = 8 cm/s; upper quartile) were compared with those presenting decreased velocities. The primary endpoint was defined as the presence of sudden death, stroke, heart failure, or hospitalization for cardiovascular causes at follow up. Results Twenty nine percent of patients (n = 43) presented preserved systolic tissue velocities in the tissue Doppler images, mostly in men (76.7% vs. 53.4%, p = 0.009) and with no age differences. Ventricular diameter and thickness were similar between the two groups while the atrial area was significantly lower (23.7 ± 6.7 vs. 28.8 ± 8, p <0.01). At follow up (median of 2.7 years), the number of events increased significantly as systolic pulsed tissue Doppler velocities decreased. No patient from the group with preserved systolic tissue velocities presented the combined endpoint, with significant differences with respect to the control group (0% vs. 21.6%, p = 0.001), and a negative predictive value of 100%. Conclusions In our population with hypertrophic cardiomyopathy, the presence of preserved systolic tissue velocities in pulsed tissue Doppler imaging identified low-risk patients with a very low number of events at follow up and high negative predictive value.