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Background Current evidence suggests that Brugada syndrome (BrS), far from being a purely electrical condition, is associated with subtle mechanical abnormalities primarily affecting the right ventricle (RV). We aimed to characterize RV function in BrS and investigate the echocardiographic profile of patients with arrhythmic events, with a special focus on parameters of RV dyssynchrony using speckle-tracking echocardiography (STE). Methods An echocardiogram was performed in 71 BrS patients and 25 healthy controls. STE was performed to assess regional and global RV mechanics, including RV outflow tract shortening (RVOTS). RVOT contraction time was considered to calculate the modified RV mechanical dispersion (RVMDm). Arrhythmic events were prospectively evaluated in the BrS cohort. Results Compared to controls, BrS patients showed subtle contractile abnormalities, including impaired RV longitudinal strain, higher RV index of myocardial performance (RIMP) and lower RVOTS. BrS patients also exhibited a greater contraction delay between the lateral and the septal aspect of the RV. After a median follow-up of 7.3 year (IQR 5.2–10.8), 6 patients presented malignant arrhythmic events. RIMP >0.50, RVOTS 42 ms showed high sensitivity for the identification of BrS patients with arrhythmic events during follow-up. Conclusions Subtle RV mechanical abnormalities were present in BrS patients. RIMP and RVOTS, a novel STE-derived parameter, were found to be sensitive markers of arrhythmic events. Adding the RVOT contraction time to the analysis of RVMD may help identify patients at higher risk, reflecting the importance of the RVOT mechanical substrate in the assessment of the arrhythmic risk in BrS.

Cardiac amyloidosis (CA) is produced by amyloid fiber deposition in the myocardium. The most frequent forms are those caused by light chains (AL) and transthyretin (ATTR). Our objective was to describe the diagnosis, treatment and outcomes of CA in a specialized Spanish center.We included all patients diagnosed with CA in Hospital Universitario Puerta de Hierro Majadahonda from May 2008 to September 2018. We analyzed their clinical characteristics, outcomes, and survival.We included 180 patients with CA, of whom 64 (36%) had AL (50% men; mean age, 65±11 years) and 116 had ATTR (72% men; mean age 79±11 years; 18 with hereditary ATTR). The most common presentation was heart failure in both groups (81% in AL and 45% in ATTR, P.01). Other forms of presentation in ATTR patients were atrial arrhythmias (16%), conduction disorders (6%), and incidental finding (6%); 70 patients (40%), had a previous alternative cardiac diagnosis. Diagnosis was noninvasive in 75% of ATTR patients. Diagnostic delay was higher in ATTR (2.8±4.3 vs 0.6±0.7 years, P.001), but mortality was greater in AL patients (48% vs 32%, P=.028). Independent predictors of mortality were AL subtype (HR, 6.16; 95%CI, 1.56-24.30; P=.01), female sex (HR, 2.35; 95%CI, 1.24-4.46; P=.01), and NYHA functional class III-IV (HR, 2.07; 95%CI, 1.11-3.89; P=.02).CA is a clinical challenge, with wide variability in its presentation depending on the subtype, leading to diagnostic delay and high mortality. Improvements are needed in the early diagnosis and treatment of these patients.

Previous studies using conventional echocardiographic measurements have reported subclinical left ventricular (LV) diastolic abnormalities in patients with Marfan syndrome (MFS). Left atrial (LA) strain allows an accurate categorization of LV diastolic dysfunction. We aimed to characterize LV myocardial performance in a cohort of MFS patients using STE-derived measurements (LV and LA strain) along with conventional echocardiographic parameters. We studied 127 adult patients with MFS (no prior cardiac surgery or significant valvular regurgitation) and 38 healthy controls. We performed detailed echocardiograms and selected left atrial reservoir strain (LASr) as a surrogate of impaired relaxation. Additionally, we searched for possible determinants of LASr in patients with MFS, with a special focus on the elastic properties of the aorta. In spite of lower E-wave, septal and lateral e’ velocities and average E/e’ ratio in MFS patients, all participants had normal diastolic function according to current guidelines. MFS patients exhibited reduced LV global longitudinal strain (19.3 ± 2.6 vs 21.6 ± 2.1%, p

Learning objectives Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, affecting 1 in 500 individuals in the general population. In 60% of cases, HCM presents an autosomal-dominant trait due to mutations in genes encoding cardiac sarcomeric proteins, and the remaining aetiologies include a variety of clinical entities such as glycogen and lysosomal storage disorders, mitochondrial diseases or cardiac amyloidosis (CA), among others.1 In all cases, a systematised echocardiographic approach is essential to establish the diagnosis of HCM and to decide on specific therapeutic strategies. Moreover, echocardiography plays a key role in the evaluation and guidance of the different options to tackle obstructive HCM (HOCM). Indeed, recent improvements in survival and quality of life in HCM might be attributed to several factors, including the development of reliable imaging techniques. On top of this, echocardiography provides valuable information for the management of HCM regarding other aspects beyond left ventricular outflow tract obstruction (LVOTO). This includes sudden cardiac death (SCD) prediction, thromboembolic risk stratification and family screening. In this article, we review and focus on the current role of echocardiography in the diagnosis and management of HCM. ### Role of echocardiography in the diagnosis and evaluation of HCM A systematic echocardiographic approach is essential to establish the diagnosis of HCM and should include the following features (summarised in figure 1). Figure 1 Echocardiographic assessment of hypertrophic cardiomyopathy (HCM): key points. LVOTO, left ventricular outflow tract obstruction; SAM, systolic anterior motion; WT, wall thickness. ### Left ventricular hypertrophy evaluation In individuals with suspected …

Background Evolution of left and right ventricular (LV and RV) function after heart transplantation (HT) has not been well described. Our objective was to evaluate the evolution of echocardiographic parameters of both ventricles along the first 2 years after HT. Methods We followed 31 HT recipients with serial echocardiograms for up to 2 years. Echocardiograms with AR ≥2R were excluded. We analyzed LV global longitudinal strain (LV GLS) by speckle tracking in 12 segments in four- and two-chamber views and RV global longitudinal strain (RV GLS) in four-chamber view. Control group included 25 healthy volunteers. Results Even though LVEF was preserved, LV GLS was reduced early post-HT (−17.7 ± 3.0 in HT vs. −20.7 ± 2.8 in controls, P = 0.02), improving progressively until its complete normalization 2 years after HT (−20.0 ± 3.7 vs. −20.7 ± 2.8, P = 0.60). TAPSE was impaired in the early post-HT period and increased progressively (11.9 ± 2.9 mm at baseline vs. 19.0 ± 3.6 mm at 2 years, P < 0.001). RV GLS rose during follow-up as well (−17.4 ± 3.5 at baseline vs. −22.6 ± 3.3 at 2 years, P = 0.001), reaching normal values 1 year after HT. Conclusion In this series of HT recipients with uneventful postoperative course, LV and RV GLS values were significantly reduced early after HT and improved progressively until their complete normalization two and 1 year after HT, respectively. This is the first study to show a full recovery of LV and RV deformation parameters and offers “normal” strain values that, if confirmed in larger studies, could be useful for monitoring the evolution of HT recipients.

Cardiac Magnetic Resonance (CMR) imaging has recently become a very useful tool in the diagnosis of myocarditis. We describe a patient in whom acute myocarditis was presented as an acute myocardial infarction and had an atypical course with rapid normalization of ECG abnormalities. In this case CMR imaging was essential to confirm the diagnosis of myocarditis.

Myocarditis is a rare disease with variable clinical presentation and diverse electrocardiographic and echocardiographic features. Viral infection is the most common cause, but myocarditis can also be caused by bacterial infection. The most frequently involved bacterial agent is group A Streptococcus, which is also an etiologic agent of erysipelas. We present the case of a man aged 46 years with left-leg erysipelas who developed myocarditis. Cardiac magnetic resonance played an essential role in diagnosis. This case is, to our knowledge, the first description of an association between erysipelas and myocarditis.

About 30% of patients treated with cardiac resynchronization therapy (CRT) do not respond to treatment. The aim of this study was to identify clinical predictors of lack of improvement in patients receiving CRT. From 197 consecutive patients scheduled to receive CRT, 143 fulfilled the inclusion criteria. Mean age was 68 +/- 7 years and 79% were men. Heart failure was due to ischemic heart disease in 49 patients (34%). Mean QRS duration was 165 +/- 26 ms, and left ventricular ejection fraction was 27 +/- 7%. Nonresponder patients were defined as those who died of heart failure, underwent heart transplantation, or did not increase the distance walked in 6 minutes >10%. At 6-month follow-up, there were 28 nonresponders (20%). Among nonresponders, 2 patients received a heart transplantation and 9 patients died of heart failure. In logistic regression analysis, independent predictors of lack of response to CRT were ischemic heart disease (odds ratio [OR] 2.9, 95% confidence interval [CI] 1.2 to 7; p = 0.023), severe mitral regurgitation (OR 3.5, 95% CI 1.3 to 9; p = 0.014), and left ventricular end-diastolic diameter > or =75 mm (OR 3.1, 95% CI 1.1 to 8; p = 0.026). Patients with these 3 predictors had a probability response of 27%.

Transesophageal echocardiography is a semi-invasive diagnostic technique with a very low incidence of significant complications. Only two deaths related to the procedure have been reported in the literature. We present the case of a 46-year-old man with a right atrial mass, presumably a cardiac myxoma. During attempts at esophageal intubation for a transesophageal echocardiography procedure, the mass fragmented and dislodged, causing massive pulmonary embolism and death. We believe that to avoid this potential complication, great care must be taken in the evaluation of patients with a right intracardiac mass by means of transesophageal echocardiography.

A 65-year-old cardiac transplant recipient suffered rupture of the mitral valve apparatus during endomyocardial biopsy of the left ventricle. Severe mitral regurgitation resulted, and because of heart failure with progressive clinical deterioration the patient was finally subjected to mitral valve replacement. He had a favorable postoperative course and is now asymptomatic.

With the aim of assessing the effectiveness and safety of lovastatin in patients with hypercholesterolemia after heart transplantation, as well as the potential differences in the lipid-lowering effect of lovastatin between patients with or without pretransplant coronary artery disease (CAD), we studied 63 heart transplant patients who had serum total cholesterol250 mg/dl in spite of dietary therapy. Mean age of subjects was 47 +/- 2 years. Triple-drug immunosuppressive therapy consisted of cyclosporine, azathioprine, and steroids. Thirty-nine patients (62%) had pretransplant CAD and 24 (38%) did not. Pretreatment serum lipid levels were: total cholesterol, 302 +/- 32 mg/dl; low-density lipoprotein (LDL) cholesterol, 201 +/- 35 mg/dl; high-density lipoprotein (HDL) cholesterol, 60 +/- 19 mg/dl; triglycerides, 205 +/- 86 mg/dl; and total/HDL cholesterol ratio, 5.4 +/- 1.6. Patients received 10 to 40 mg/day of lovastatin (mean dose 17 +/- 6) for 13 +/- 4 months. There were no serious adverse events. At 3 months, lovastatin decreased total cholesterol by 15% (p0.001), LDL cholesterol by 21% (p0.001), triglycerides by 17% (p0.05), and total/HDL cholesterol ratio by 17% (p0.001), and increased HDL cholesterol by 3% (NS). Although lovastatin was effective in both patients with pretransplant CAD and non-CAD, analysis of its effect in each subgroup (CAD and non-CAD) revealed that its lipid-lowering effect was higher for non-CAD patients (-20% vs -12% for total cholesterol, and -27% vs -17% for LDL cholesterol, both p0.01).(ABSTRACT TRUNCATED AT 250 WORDS)