The Natural History of Atrial Functional Mitral Regurgitation.

BACKGROUND: The natural history of moderate/severe atrial functional mitral regurgitation (AFMR) is unknown. OBJECTIVES: The authors sought to study the incidence of left ventricular (LV) systolic dysfunction (LVSD), progression or regression of ≥mild-moderate AFMR, and impact on mortality. METHODS: Adults with left atrial (LA) volume index ≥40 mL/m2, ≥mild-moderate AFMR, and follow-up echocardiogram were followed for incident LVSD (ejection fraction <50% and ≥10% lower than baseline), progression of mild-moderate/moderate AFMR to severe, and persistent regression of AFMR to no/trivial. Relation of AFMR progression or regression as time-dependent covariates with all-cause mortality was studied. Incidence of LVSD was compared with patients with no/mild AFMR matched on age, sex, comorbidities and ejection fraction. Patients were followed until mitral intervention, myocardial infarction, or last follow-up. RESULTS: A total of 635 patients (median age 75 years, 51% female, 96% mild-moderate/moderate AFMR, 4% severe AFMR) were included. Over a median 2.2 years (Q1-Q3: 1.0-4.3 years), incidence rates per 100 person-years were 3.2 for LVSD (P = 0.52 vs patients with no/mild AFMR), 1.9 for progression of AFMR, and 3.9 for regression. Female sex and larger LA volume index were independently associated with progression, whereas younger age, male sex, absent atrial fibrillation, and higher LA emptying fraction were independently associated with regression. Neither AFMR progression nor regression was independently associated with mortality. Instead, independent risk factors for mortality included older age, concentric LV geometry, and higher estimated LV filling and pulmonary pressures. CONCLUSIONS: In patients with predominantly mild-moderate/moderate AFMR, regression of MR was more common than progression, but neither was associated with mortality. Instead, diastolic function abnormalities were more important. Over a median 2-year follow-up, LVSD risk was not increased.

Circulating miR-let7a levels predict future diagnosis of chronic thromboembolic pulmonary hypertension.

Distinct patterns of circulating microRNAs (miRNAs) were found to be involved in misguided thrombus resolution. Thus, we aimed to investigate dysregulated miRNA signatures during the acute phase of pulmonary embolism (PE) and test their diagnostic and predictive value for future diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). Microarray screening and subsequent validation in a large patient cohort (n = 177) identified three dysregulated miRNAs as potential biomarkers: circulating miR-29a and miR-720 were significantly upregulated and miR-let7a was significantly downregulated in plasma of patients with PE. In a second validation study equal expression patterns for miR-29a and miR-let7a regarding an acute event of recurrent venous thromboembolism (VTE) or deaths were found. MiR-let7a concentrations significantly correlated with echocardiographic and laboratory parameters indicating right ventricular (RV) dysfunction. Additionally, circulating miR-let7a levels were associated with diagnosis of CTEPH during follow-up. Regarding CTEPH diagnosis, ROC analysis illustrated an AUC of 0.767 (95% CI 0.54-0.99) for miR-let7a. Using logistic regression analysis, a calculated patient-cohort optimized miR-let7a cut-off value derived from ROC analysis of ≥ 11.92 was associated with a 12.8-fold increased risk for CTEPH. Therefore, miR-let7a might serve as a novel biomarker to identify patients with haemodynamic impairment and as a novel predictor for patients at risk for CTEPH.

A pilot study for risk stratification of ventricular tachyarrhythmia in hypertrophic cardiomyopathy with routine echocardiography parameters.

Ventricular tachyarrhythmia (VTA) are frequent arrhythmias in patients with hypertrophic cardiomyopathy (HCM). Representing a major risk factor for sudden cardiac death, Holter ECG at first clinical presentation appears insufficient. This study aims to investigate the ability of routinely obtained parameters associated with myocardial remodeling in stratifying for VTA in HCM. In this monocentric analysis, patients with HCM underwent 12-channel electrocardiography and echocardiography, including tissue doppler imaging. The study's primary endpoint was the documentation of non-sustained and sustained ventricular tachycardia-summarized as ventricular tachyarrhythmias (VTA) on Holter ECG or active devices. The occurrence of VTA was exploratory. Based on our collective, we developed a risk model regarding VTA. Of 140 HCM patients, 38 (27.1%) had an episode of VTA. Patients with VTA were likelier to have a history of atrial fibrillation (p < 0.001), a thicker interventricular septum (p < 0.001) and lower peak systolic mitral annular velocity (p < 0.001). The parameters were independently associated with endpoint in univariate and multivariate logistic regression. We created a logistic equation and calculated a cut-off value. The resulting ROC curve revealed a discriminative ability with AUC of 0.80 (sensitivity, 63%; specificity, 88%). Our risk model including these widely available parameters is able to distinguish low and high-risk of VTA in patients with HCM.

Late presentation of traumatic tricuspid valve chordal rupture and pericardial rupture with cardiac herniation: a case report.

BACKGROUND: Although chest trauma happens very often, accompanying tricuspid valve injuries occur rarely and may be manifested by scarce symptoms and signs. Pericardial rupture with cardiac herniation is even a bigger rarity. Transthoracic echocardiography plays a key role in the diagnosis of valve injuries but is of limited value in cardiac herniation. CASE PRESENTATION: We present the case of 58-year-old man who experienced severe chest trauma in a car accident. Symptoms of right heart failure occurred 10 years after the injury, due to the loss of tricuspid leaflet support caused by the rupture of tendinous chords with significant tricuspid regurgitation. Intraoperatively, old posttraumatic pericardial rupture into left pleura was also found, with partial cardiac herniation and pressure of the edge of pericardium on all left-sided coronary arteries simultaneously. The patient was successfully operated and is free of symptoms 4 years later. CONCLUSIONS: This case emphasizes the importance of timely diagnosis and underlines a mechanism that leads to delayed rupture of the tricuspid valve apparatus. Repeated echocardiography in all patients who experienced chest trauma could be of great importance. Also, given the limited value of echocardiography in posttraumatic pericardial rupture and cardiac herniation, cardiac computed tomography should be performed.

Right heart failure after durable left ventricular assist device implantation.

INTRODUCTION: Right heart failure (RHF) is a well-known complication after left ventricular assist device (LVAD) implantation and portends increased morbidity and mortality. Understanding the mechanisms and predictors of RHF in this clinical setting may offer ideas for early identification and aggressive management to minimize poor outcomes. A variety of medical therapies and mechanical circulatory support options are currently available for the management of post-LVAD RHF. AREAS COVERED: We reviewed the existing definitions of RHF including its potential mechanisms in the context of durable LVAD implantation and currently available medical and device therapies. We performed a literature search using PubMed (from 2010 to 2023). EXPERT OPINION: RHF remains a common complication after LVAD implantation. However, existing knowledge gaps limit clinicians' ability to adequately address its consequences. Early identification and management are crucial to reducing the risk of poor outcomes, but existing risk stratification tools perform poorly and have limited clinical applicability. This is an area ripe for investigation with the potential for major improvements in identification and targeted therapy in an effort to improve outcomes.

Outcomes of patients with elevated pulmonary artery systolic pressure on echocardiography due to chronic lung diseases.

BACKGROUND: Pulmonary hypertension is associated with increased mortality, and lung diseases are the second most common cause of pulmonary hypertension. We aimed to evaluate the prognostic value of echocardiography in low-middle income countries where right heart catheterization is difficult to perform. METHODS: This retrospective chart review study included adult patients hospitalized from June 2012 to May 2021, with a pulmonary artery systolic pressure (PASP) of ≥35 mmHg on echocardiography. The control arm consisted of patients with similar lung diseases who did not have an elevated PASP. RESULTS: The study and control arm consisted of 128 patients each, with both groups having similar lung diseases. Obesity hypoventilation syndrome was the most common etiology of elevated PASP (28.1 %), followed by pulmonary embolism (20.3 %). The overall 1-year mortality of the study cohort, after diagnosis of elevated PASP, was 20.3 %. The control cohort with normal PASP had a 1-year mortality of 4.7 %. In the study cohort, patients with bronchiectasis had the highest cause-specific 1-year mortality (45.5 %). In the normal PASP cohort, the highest cause-specific 1-year mortality was observed in patients with interstitial lung disease (13.0 %). One-year hospital readmission was observed in 46.9 % and 33.6 % of patients in the study and control arms, respectively. On multivariate analysis, increased odds of 1-year mortality were observed in patients with elevated PASP, patients with 1-year hospital readmission, and in patients with interstitial lung disease or bronchiectasis. CONCLUSION: Elevated PASP on echocardiography may be a prognostic factor for mortality in patients with chronic lung diseases.

Factors influencing left ventricular diastolic dysfunction on echocardiography of people living with HIV in Kunming, China.

BACKGROUND: The widespread use of antiretroviral therapy has prolonged the survival of people living with HIV (PLWH). Among these patients, co-existing cardiovascular diseases, particularly left ventricular diastolic dysfunction (LVDD), are receiving increasing attention. METHODS: We recruited 386 patients in the PLWH group and 386 sex- and age (± 3 years)-matched individuals in the HIV-negative group, and used logistic regression to determine the risk factors of LVDD. RESULTS: Compared to the HIV-negative group, PLWH had a significantly higher prevalence of smoking (p < .001), alcohol consumption (p < .001), hypertension (p = .002), diabetes (p = .020), and hyperlipidemia (p < .001) and a lower prevalence of body mass index (BMI) ≥ 24.0 kg/m2 (p < .001). The prevalence of LVDD on echocardiography was significantly higher in PLWH than in the HIV-negative group (25.9% vs 16.1%, p = .001). The multivariate analysis showed that non-youth (OR = 8.666; 95%CI = 4.310-17.459; p < .001), BMI ≥ 24.0 kg/m2 (OR = 1.992; 95% CI = 1.007-3.939; p = .048), hypertension (OR = 1.888; 95% CI = 1.044-3.415; p = .036), hyperlipidemia (OR = 1.911; 95% CI = 1.068-3.418; p = .029), and HIV infection (OR = 2.003; 95%CI = 1.341-2.992; p = .001) were risk factors for LVDD. CONCLUSION: The rate of echocardiographic abnormalities was higher in PLWH. LVDD was associated with non-youth, BMI ≥ 24.0 kg/m2, hypertension, hyperlipidemia, and living with HIV.

Association of Lung Ultrasound B-Lines with Left Ventricular Diastolic Function in Clinically Euvolemic Haemodialysis Patients.

INTRODUCTION: Left ventricular diastolic dysfunction (LVDD) frequently occurs in haemodialysis patients and is associated with adverse outcomes. Lung ultrasound (LUS) has been recently proposed for the quantification of extravascular lung water through assessment of B-lines. LUS findings and their relationship with LVDD in clinically euvolemic haemodialysis patients were investigated in this study. METHODS: Echocardiography and LUS examinations were performed on each patient. Multivariate linear regression and forward stepwise logistic regression were performed to determine the relationship between B-lines and LVDD. A receiver operating characteristic (ROC) curve with area under the curve (AUC) was calculated to determine the accuracy of B-lines for evaluating LVDD. RESULTS: A total of 119 patients were enrolled. The number of B-lines was statistically related to echocardiographic parameters (LAVI, LVEDVI, E/A, and E/e') of diastolic function, while the relationship between B-lines and LVEF disappeared after adjusting for potential confounding factors. Additionally, compared with the mild B-line group (B-lines: <14), the moderate (B-lines: 14-30) and severe B-line groups (B-lines: >30) were associated with an increased risk of LVDD (OR 24.344, 95% CI 4.854-122.084, p < 0.001, and OR 94.552, 95% CI 9.617-929.022, p < 0.001, respectively). Furthermore, the AUC of the ROC curve for B-lines predicting LVDD was 0.845, and the cut-off of B-lines was 14.5 (sensitivity 64.91%, specificity 93.55%). CONCLUSION: LUS B-lines were closely associated with left ventricular diastolic function in clinically euvolemic haemodialysis patients. Moreover, our findings suggested a B-line ≥14.5 as a reliable cut-off value for identifying patients with LVDD. LUS B-lines may be used as a novel indicator for evaluating LVDD.

Right ventricular free wall and four-chamber longitudinal strain in relation to incident heart failure in the general population.

AIMS: Right ventricular free wall (RVFWLS) and four-chamber longitudinal strain (RV4CLS) are associated with adverse events in various patient populations including patients with heart failure (HF). We sought to investigate the prognostic value of RVFWLS and RV4CLS for the development of incident HF in participants from the general population. METHODS AND RESULTS: Participants from the 5th Copenhagen City Heart Study (2011-2015) without known chronic ischaemic heart disease or HF at baseline were included. RVFWLS and RV4CLS were obtained using two-dimensional speckle-tracking echocardiography from the right ventricular (RV)-focused apical four-chamber view. The primary endpoint was incident HF. Among 2740 participants (mean age 54 ± 17 years, 42% male), 43 (1.6%) developed HF during a median follow-up of 5.5 years (IQR 4.5-6.3). Both RVFWLS and RV4CLS were associated with an increased risk of incident HF during follow-up independent of age, sex, hypertension, diabetes, body mass index and tricuspid annular plane systolic excursion (TAPSE), (HR 1.06, 95%CI 1.00-1.11, P = 0.034, per 1% absolute decrease and HR 1.14, 95%CI 1.05-1.23, P = 0.001, per 1% absolute decrease, respectively). Left ventricular ejection fraction (LVEF) modified the association between RV4CLS and incident HF (P for interaction = 0.016) such that RV4CLS was only of prognostic importance among those with LVEF < 55% (HR 1.21, 95%CI 1.11-1.33, P < 0.001 vs. HR 0.94, 95%CI 0.80-1.10, P = 0.43 in patients with LVEF ≥ 55%). CONCLUSION: In participants from the general population, both RVFWLS and RV4CLS were associated with a greater risk of incident HF independent of important baseline characteristics and TAPSE, and LVEF modified the relationship between RV4CLS and incident HF.

Association of abnormalities in electrocardiography and ultrasonic echocardiography with the occurrence of cardiovascular disease in patients with advanced chronic kidney disease.

BACKGROUND: In patients with chronic kidney disease (CKD), the incidence of cardiovascular disease (CVD) increases with disease progression. CVD screening tests in those with CKD were researched to determine whether abnormalities observed in electrocardiography (ECG) and ultrasonic echocardiography (UCG) were risk factors associated with the development of CVD. METHODS: This study included 604 patients with CKD G4 and G5, for whom both ECG and UCG were performed. They were divided into four groups: those without ECG- and UCG-indicated abnormalities (group A, n = 333), with only ECG abnormalities (group B, n = 106), with only UCG abnormalities (group C, n = 75), and with both ECG and UCG abnormalities (group D, n = 90). Multivariate analysis using Cox regression analysis of the occurrence of CVD was performed during a follow-up period. RESULTS: During the observation period, 124 patients had clinical events. Among them, 45 patients (13.5%) were in Group A, 25 patients (23.6%) in Group B, 19 patients (25.3%) in Group C, and 35 patients (38.9%) in Group D, respectively. CVD event occurrence was highest in Group D. The results of the multivariate analysis also showed that the CVD event rates were significantly higher in Group C (HR: 2.96, P = < .001) and D (HR: 4.22, P < .001) than in Group A. CONCLUSION: In patients with advanced CKD, there was a significant correlation of ECG and UCG abnormalities with CVD events. Additionally, those having both types of abnormalities may have a higher risk of coronary artery disease than other groups.

ECHOCARDIOGRAPHIC ASPECTS OF ASSESSMENT OF MITRAL INSUFFICIENCY IN PATIENTS WITH ACUTE MYOCARDIAL INFARCTION WITH REDUCED LEFT VENTRICULAR EJECTION FRACTION.

OBJECTIVE: The aim is to analyze patients with acute myocardial infarction with reduced ejection fraction in order to determine risk factors for mitral insufficiency. PATIENTS AND METHODS: Materials and methods: The study included 149 patients with acute myocardial infarction. Among the patients, there were 113 males (75.8%) and 36 females (24.2%). The age of the patients ranged from 43 to 86 years. Echocardiography was performed using a Phillips Epiq 7 machine. Patients were examined three times: upon admission to the hospital, after revascularization, and six months after discharge. Patients received transthoracic echocardiography, which was used to determine the presence and degree of mitral regurgitation. RESULTS: Results: According to the results of the study, it was found that the presence of concomitant somatic pathology worsens the course of an acute myocardial infarction, in turn increasing the likelihood of valvular pathology. An increase in left ventricular volume indicators, such as end-diastolic volume, end-systolic volume, end-diastolic index, and the index of contractile function (ejection fraction) contributes to the development of mitral valve insufficiency. CONCLUSION: Conclusions: The presence of mitral regurgitation in patients with acute myocardial infarction and reduced left ventricular ejection fraction worsens the course of the disease and negatively affects the prognosis.

A Clinical Decision Tool for Risk Stratifying Patients with Systemic Sclerosis-Related Pulmonary Hypertension.

We devised a scoring system to identify patients with systemic sclerosis (SSc) at risk for pulmonary hypertension (PH) and predict all-cause mortality. Using 7 variables obtained via pulmonary function testing, echocardiography, and computed tomographic chest imaging, we applied the score to a retrospective cohort of 117 patients with SSc. There were 60 (51.3%) who were diagnosed with PH by right heart catheterization. Using a scoring threshold ≥ 0, our decision tool predicted PH with a sensitivity, specificity, and accuracy of 0.87 (95% CI 0.75, 0.94), 0.74 (95% CI 0.60, 0.84), and 0.80 (95% CI 0.72, 0.87), respectively. When adjusted for age at PH diagnosis, sex, and receipt of pulmonary arterial vasodilators, each one-point score increase was associated with an adjusted HR of 1.19 (95% CI 1.05, 1.34) for all-cause mortality. With further validation in external cohorts, our simplified clinical decision tool may better streamline earlier detection of PH in SSc.

Sex-specific difference in cardiac function in patients with systemic sclerosis: association with cardiovascular outcomes.

BACKGROUND: Cardiovascular involvement is one of the leading causes of mortality in systemic sclerosis (SSc) and is reported to be higher in men as compared with women. However, the cause of this difference is largely unknown. The objective of this study was to assess sex differences in echocardiographic characteristics, including left ventricular global longitudinal strain (LV GLS), as a potential explanation of sex differences in outcomes. METHODS: A total of 746 patients with SSc from four centres, including 628 (84%, 54±13 years) women and 118 (16%, 55±15 years) men, were evaluated with standard and advanced echocardiographic examinations. The independent association of the echocardiographic parameters with the combined endpoint of cardiovascular events-hospitalisation/death was evaluated. RESULTS: Men and women with SSc showed significant differences in disease characteristics and cardiac function. After adjusting for the most important clinical characteristics, while LV ejection fraction and diastolic function were not significantly different anymore, men still presented with more impaired LV GLS as compared with women (-19% (IQR -20% to -17%) vs -21% (IQR: -22% to -19%), p<0.001). After a median follow-up of 48 months (IQR: 26-80), the combined endpoint occurred in 182 patients. Men with SSc experienced higher cumulative rates of cardiovascular events-hospitalisation/mortality (χ2=8.648; Log-rank=0.003), and sex differences were maintained after adjusting for clinical confounders, but neutralised when matching the groups for LV GLS. CONCLUSION: In patients with SSc, male sex is associated with worse cardiovascular outcomes even after adjusting for important clinical characteristics. LV GLS was more impaired in men as compared with women and potentially explains the sex difference in cardiovascular outcomes.

Assessment of Myocardial Function by Speckle Tracking Echocardiography in Patients with Acute Ischemic Stroke.

Background: Acute stroke is an important cause of morbidity and mortality. Myocardial injury is an important complication of acute cerebrovascular events. Neurogenic stress cardiomyopathy (NSC) is a condition of acute myocardial systolic dysfunction that can be observed after acute cerebrovascular events. Objective: In this study, we aimed to investigate the relationship between myocardial function assessed by two-dimensional speckle-tracking echocardiography and National Institutes of Health Stroke Scale (NIHSS) score in patients with acute ischemic stroke. Materials and Methods: This cross-sectional study screened 97 patients (males, 42; females, 55; 65 ± 16 years) with acute ischemic stroke. Around 17 patients were excluded and 80 patients were studied. Patients were divided into two groups based on the calculated NIHSS score (Group 1, NIHSS score <16; Group 2, NIHSS score ≥16). Demographic, clinical, and laboratory data for all patients were collected. Cardiac function was evaluated by two-dimensional speckle tracking echocardiography within 48 h of admission to the neurology care unit. Results: There were no significant differences in the demographic parameters of patients. The absolute value of global longitudinal systolic strain (GLS) was significantly higher in Group 1 patients than in Group 2 patients (21.4 ± 2.2 vs 15.9 ± 2.7, P = 0.0281). We found that thirteen patients (22%) had normal LVEF and abnormal LV GLS in Group 1 (P = 0.036). Eight patients (36%) had normal LVEF and abnormal LV GLS in Group 2 (P = 0.042). E/e', QT on ECG, and serum troponin levels were significantly higher in Group 2 patients than in Group 1 patients (P < 0.05). Conclusions: Our results suggest that GLS is associated with stroke severity on admission in patients with acute ischemic stroke. GLS is an indicator of myocardial deformation with a different from LVEF. GLS can detect early myocardial dysfunction despite preserved LVEF.

Cardioembolic Stroke in Sinus of Valsalva Aneurysm, A Case Report.

A case of cardioembolic stroke in a patient with sinus of Valsalva aneurysm. The patient presented with chief complaints of syncope, with slurring of speech and a history of deviation of angle of mouth to the right side, which resolved over one day. Examination revealed no focal neurological deficit. CT Brain revealed a lacunar infarct over the left frontal cortex. Echocardiography showed sinus of Valsalva aneurysm of the non-coronary cusp. Hence a diagnosis of cardioembolic stroke secondary to thrombus present in the sinus of Valsalva of the aneurysm was considered. This is a rare presentation of Sinus of Valsalva as a cardioembolic stroke.

Abnormal inter-ventricular diastolic mechanical delay in patients with ST-segment elevation myocardial infarction.

BACKGROUND: This study aimed to investigate the ventricular mechanical relaxation pattern and its clinical influence in patients with ST-segment elevation myocardial infarction (STEMI). METHODS: Echocardiography was performed to measure mitral and tricuspid diastolic opening times. Left ventricular diastolic mechanical delay (LVMDd) was defined as diastolic filling of the right ventricle earlier than that of the left ventricle, and right ventricular diastolic mechanical delay (RVMDd) was defined as the right ventricular diastolic filling later than left ventricular filling. RESULTS: Among 152 patients with STEMI, 100 (65.8%) had LVMDd, and 47 (30.9%) had RVMDd. In-hospital complications were significantly increased in patients with RVMDd (61.6% vs. 41.0%, P = 0.017). Those with RVMDd exhibited significantly lower left ventricular global longitudinal strain (11.7 ± 4.1% vs. 13.2 ± 4.0%, P = 0.035), global work index (913.8 ± 365.9 vs. 1098.9 ± 358.8 mmHg%, P = 0.005) and global constructive work (1218.6 ± 392.8 vs. 1393.7 ± 432.7 mmHg%, P = 0.021). Mitral deceleration time significantly decreased (127.4 ± 33.5 vs. 145.6 ± 41.7 ms, P = 0.012), and the ratio of early mitral inflow to early mitral annular velocity (E/E') significantly increased [13.0(11.0-20.0) vs. 11.9(9.3-14.3), P = 0.006] in the RVMDd group. Logistic regression analysis showed that age (odds ratio [OR]:0.920; P = 0.001), brain natriuretic peptide level (OR: 1.1002; P = 0.036) and mitral E/E' (OR: 1.187; P = 0.003) were independently associated with RVMDd. CONCLUSIONS: Delayed right ventricular filling is related to more severe left ventricular systolic and diastolic dysfunction in STEMI patients. More attention should be paid to patients with RVMDd to prevent adverse events during hospitalization.

Prognostic Performance of Right Ventricular Global Longitudinal Strain Measurements in Patients With Ebstein Anomaly.

BACKGROUND: There are limited data on the prognostic role of right ventricular global longitudinal strain (RVGLS) in patients with Ebstein anomaly. OBJECTIVES: This study sought to assess the relationship between RVGLS and mortality and to compare prognostic performance of RVGLS with conventional echocardiographic indices of right ventricular (RV) systolic function. METHODS: This study identified adults with Ebstein anomaly with echocardiographic assessment of RV systolic function (RVGLS, RV fractional area change [RVFAC], RV tissue Doppler systolic velocity [RV s'], and tricuspid annular plane systolic excursion [TAPSE]) from 2003 to 2020. For ease of presentation, RVGLS was modeled as absolute values (ie, without the negative sign). RESULTS: Of 620 patients (median age 37 years; men 261 [42%]), the mean absolute RVGLS, RVFAC, RV s', and TAPSE were 18% ± 5%, 32% ± 9%, 14 ± 6 cm/s, and 22 ± 8 mm, respectively. There were correlations between absolute RVGLS and RVFAC (r = 0.71; P < 0.001), between absolute RVGLS and RV s' (r = 0.41; P = 0.03), and between absolute RVGLS and TAPSE (r = 0.44; P = 0.002). Of 620 patients, 47 (8%) died during follow-up, and 34 of these deaths were cardiovascular. Absolute RVGLS was independently associated with all-cause mortality (adjusted HR: 0.94; 95% CI: 0.92-0.96 per unit increase) and cardiovascular mortality (adjusted HR: 0.92; 95% CI: 0.90-0.94 per unit increase). Absolute RVGLS had superior prognostic power (ie, ability to predict mortality) as compared with RVFAC, RV s', or TAPSE. CONCLUSIONS: These data support the use of RVGLS for risk stratification in Ebstein anomaly, and further studies are required to assess how interventions may affect different patients according to risk stratification.

Divergent Findings in the Diagnosis of ATTR-CM Using Common Cardiac Diagnostics and 99m Tc-DPD Scintigraphy.

ABSTRACT: A 77-year-old man presented with progressive deterioration of physical capacity after successful percutaneous coronary intervention of known chronic coronary syndrome. Transthoracic echocardiography revealed hypertrophy of the left ventricle, and electrocardiogram showed low QRS voltage in all leads. 99m Tc-DPD scintigraphy was conducted to differentiate etiology such as amyloidosis and revealed increased cardiac tracer uptake in the left (grade 3) and right ventricle. Immunofixation showed no signs of paraproteinemia or Bence-Jones proteinuria. Thus, biventricular involvement of ATTR-cardiomyopathy was identified by 99m Tc-DPD scintigraphy. This approach should be considered if hypertrophic phenotype is present in patients with persistent deterioration of physical capacity not attributable to coronary artery disease.

The portrait of a stranger: the hypereosinophilic syndrome with cardiac involvement.

INTRODUCTION: Hypereosinophilic syndrome is a rare clinical condition, and cardiac involvement confers a poor prognosis. Hypereosinophilic myocarditis is a medical emergency and targeted treatment should be started promptly even before a definitive diagnosis could be made. CASE PRESENTATION: A 27-year-old female patient is hospitalized for exertional dyspnea, chest pain, and fatigue for the past 2 weeks. She also describes left leg paresthesias. Clinical examination was in normal limits. ECG showed sinus tachycardia, QS pattern in V1-V4, and diffuse flattened T waves. Laboratory tests revealed increased inflammatory markers, hypereosinophilia, elevated cardiac enzymes, high NT-proBNP. Echocardiography revealed LV dysfunction (EF 31%), while cardiac MRI showed diffuse delayed enhancement with predominant subendocardial disposition. The electromyogram was suggestive of left tibial nerve neuropathy. We interpreted the case as eosinophilic myocarditis with an urgent requirement of therapy and initiated high-dose glucocorticoid therapy and the GDMT 4-pillar heart failure treatment. We excluded common infectious, myeloproliferative syndromes, and frequent associated autoimmune diseases. With prednisone, the eosinophil count rapidly normalized and we gradually tapered the dose by 5 mg per week, however continuing with heart failure therapy. At monthly follow-up visits, there was a significant clinical improvement, with normalization of the eosinophilic count, and a near-normalization of myocardial function. The only symptom that persisted was paresthesias linked to left tibial neuropathy. CONCLUSION: The surprisingly rapid and favorable course of the disease offers a high index of suspicion for a toxic or a reactive transitory etiology, however still unidentified. In our case, the cause of eosinophilia remained unknown, although we managed to narrow down the possible etiologies. A surprisingly good clinical response was obtained with non-specific treatment targeting mainly hyperosinophilic myocarditis.