Malignant Primary and Metastatic Cardiac Tumors: A Single-Center 27-Year Case Review.

BACKGROUND: Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022. METHODS: Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed. RESULTS: Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: the most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (interquartile range: 1.0-11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0-13.4) months (minimum of 7 days, maximum of 5 years). CONCLUSION: Diagnosis of metastatic cardiac tumors was more common than that of malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favorable outcome in carefully selected patients with sarcomas.

Global left ventricular myocardial work index and medium-term adverse cardiovascular events after ST-elevation myocardial infarction.

BACKGROUND: Left ventricular global longitudinal strain (GLS) has incremental prognostic value over ejection fraction (EF) in patients with ST-segment-elevation myocardial infarction (STEMI), but it is also load dependent. It has been recently demonstrated that Myocardial work (MW), integrating blood pressure with GLS, predicts long-term all-cause mortality. We aimed to further explore the prognostic value of MW for cardiovascular endpoints in patients with STEMI. METHODS AND RESULTS: Retrospective study of 200 consecutive patients admitted with a STEMI, mean age of 62 (SD 12) years, 79.5% males, that survived to discharge. Transthoracic echocardiography was performed before discharge (5 ± 3 days after admission). Mean follow-up was 790 days. The primary outcome was a composite of cardiovascular death, non-fatal myocardial infarction, and unplanned cardiovascular admission (ACE). During follow-up, 26 patients had a ACE. In univariable Cox regression analysis, male gender, body mass index, GRACE risk score and Global Work Index (GWI) were selected to the multivariable analysis, in which, only GWI (per 100 mmHg% decrease: hazard ratio estimate 1.19, 95% confidence interval 1.07-1.34, p-value = 0.002) remained independently associated with ACE, with effective reclassification of non-events. The best GWI cut-off to predict ACE was ≤1165 mmHg% (Log-rank, p = 0.034). CONCLUSIONS: LV GWI is independently associated with medium-term ACE. Nevertheless, prospective studies in a larger sample of patients are warranted to confirm this finding.

Evaluation of left atrial strain imaging and integrated backscatter as predictors of recurrence in patients with paroxysmal, persistent, and long-standing persistent atrial fibrillation undergoing catheter ablation.

BACKGROUND: Impaired left atrial (LA) strain predicts atrial fibrillation (AF) recurrence after catheter ablation (CA), but currently there is no cut-off to guide patient selection for CA. Integrated backscatter (IBS) is a promising tool for noninvasive quantification of myocardial fibrosis. The aim of this study was to compare LA strain and IBS between paroxysmal, persistent, and long-standing persistent AF and evaluate their association with AF recurrence after CA. METHODS: Analysis of consecutive patients with symptomatic paroxysmal and persistent AF who underwent CA. LA phasic strain, strain rate and IBS were assessed by two-dimensional speckle-tracking at baseline. RESULTS: We analyzed 78 patients, 31% with persistent AF (46% long-standing AF), 65% male, mean age 59 ± 14 years, who underwent CA and were followed-up for 12 months. AF recurrence occurred in 22 (28%) patients. LA phasic strain parameters were significantly impaired in patients with AF recurrence and were independent predictors of AF recurrence in a multivariable analysis. LA reservoir strain (LASr) < 18% predicted AF recurrence with 86% sensitivity and 71% specificity, with a higher predictive power compared to LA volume index (LAVI). LASr < 22% in paroxysmal AF and LASr < 12% in persistent AF correlated with AF recurrence. Increased IBS was a predictor of AF recurrence in patients with paroxysmal AF. CONCLUSION: LA phasic strain parameters were predictors of AF recurrence after CA, independently of LAVI and AF subtype. LASr < 18% showed a higher predictive power compared to LAVI. Further studies are needed to investigate the role of IBS as a predictor of AF recurrence.

Multimodality imaging of a cardiac angiosarcoma presenting with atrial flutter.

A 39-year-old female patient presents to the emergency department with palpitations and dyspnoea, with the admission electrocardiogram revealing atrial flutter. Step-by-step multimodality imaging techniques, including transthoracic echocardiography, computed tomography, cardiac magnetic resonance imaging, and intracardiac echocardiography-guided tissue biopsy, led to the final diagnosis of a right atrial angiosarcoma.

Sacubitril/Valsartan Improves Left Atrial and Ventricular Strain and Strain Rate in Patients with Heart Failure with Reduced Ejection Fraction.

BACKGROUND: Data on the impact of sacubitril/valsartan (SV) therapy on phasic left atrial (LA) and left ventricular (LV) strain in heart failure with reduced ejection fraction (HFrEF) are limited. The aim of this study was to evaluate changes in two-dimensional speckle tracking (2D-STE) parameters with SV therapy in HFrEF patients. METHODS: Prospective evaluation of HFrEF patients receiving optimized medical therapy. Two-dimensional speckle tracking (2D-STE) parameters were assessed at baseline and after 6 months of SV therapy. LA strain and strain rate (SR) in reservoir, conduit, and contraction phases were compared with LV longitudinal, radial, and circumferential strain and SR and stratified according to heart rhythm and HFrEF etiology. RESULTS: A total of 35 patients completed the 6-month follow-up, with a mean age of 59 ± 11 years, 40% in atrial fibrillation, 43% with ischemic etiology, and LVEF of 29 ± 6%. There were significant improvements in LA reservoir, conduit, and contractile strain and SR following SV therapy, particularly among patients in sinus rhythm. There were significant improvements in longitudinal, radial, and circumferential LV function indices. CONCLUSION: SV therapy in HFrEF was associated with improved longitudinal, radial, and circumferential function, particularly among patients in sinus rhythm. These findings can provide insights into the mechanisms underlying the improvement of cardiac function and help assess subclinical responses to the treatment.

Three-dimensional echocardiography for the evaluation of hypertrophic cardiomyopathy patients: relation to symptoms and exercise capacity.

Patients with hypertrophic cardiomyopathy may exhibit impaired functional capacity, associated with increased morbidity and mortality. Systolic function is one of the determinants of functional capacity. Early identification of systolic disfunction may identify patients at risk for adverse outcomes. Myocardial deformation parameters, derived from three-dimensional (3D) speckle-tracking echocardiography (3DSTE) are useful tools to assess left ventricular systolic function, and are often abnormal before a decline in ejection fraction is seen. The aim of this study was to evaluate the correlation between myocardial deformation parameters obtained by 3DSTE and functional capacity in patients with hypertrophic cardiomyopathy. Seventy-four hypertrophic cardiomyopathy adult patients were prospectively evaluated. All patients underwent a dedicated 2D and 3D echocardiographic examination and cardiopulmonary exercise testing (CPET). Values of 3D global radial (GRS), longitudinal (3DGLS) and circumferential strain (GCS) were overall reduced in our population: 99% (n = 73) of the patients had reduced GLS, 82% (n = 61) had reduced GRS and all patients had reduced GCS obtain by 3DSTE. Average peak VO2 was 21.01 (6.08) ml/Kg/min; 58% (n = 39) of the patients showed reduced exercise tolerance (predicted peak VO2 < 80%). The average VE/VCO2 slope was 29.0 (5.3) and 16% (n = 11) of the patients had impaired ventilatory efficiency (VE/VCO2 > 34). In multivariable analysis, 3D GLS (ß1 = 0.10, 95%CI: 0.03;0.23, p = 0.014), age (ß1 = -0.15, 95%CI: -0.23; -0.05, p = 0.002) and female gender (ß1 = -5.10, 95%CI: -7.7; -2.6, p < 0.01) were independently associated with peak VO2. No association was found between left ventricle ejection fraction obtain and peak VO2 (r = 0.161, p = 0.5). Impaired myocardial deformation parameters evaluated by 3DSTE were associated with worse functional capacity assessed by peak VO2.

Microvascular Dysfunction Is Associated With Impaired Myocardial Work in Obstructive and Nonobstructive Hypertrophic Cardiomyopathy: A Multimodality Study.

Background Two-dimensional speckle tracking echocardiography has been shown to correlate with microvascular dysfunction, a hallmark of hypertrophic cardiomyopathy (HCM). We hypothesized that there is an association between myocardial work and left ventricular ischemia, with incremental value to global longitudinal strain, in patients with HCM. Methods and Results We performed a prospective assessment of patients with HCM, undergoing 2-dimensional speckle tracking echocardiography and stress perfusion cardiac magnetic resonance. Results were stratified according to obstructive or nonobstructive HCM and the presence of significant replacement fibrosis (late gadolinium enhancement ≥15% of left ventricular mass). Seventy-five patients with HCM (63% men, age 55±15 years) were evaluated, 28% with obstructive HCM (mean gradient 89±60 mm Hg). Perfusion defects were found in 90.7%, involving 22.5±16.9% of left ventricular mass, and 38.7% had late gadolinium enhancement ≥15%. In a multivariable analysis, a lower global work index (r=-0.519, ß-estimate -10.822; P=0.001), lower global work efficiency (r=-0.379, ß-estimate -0.123; P=0.041), and impaired global constructive work (r=-0.532, ß-estimate -13.788; P<0.001) significantly correlated with ischemia. A segmental analysis supported these findings, albeit with lower correlation coefficients. A global work index cutoff ≤1755 mm Hg% was associated with hypoperfusion with a sensitivity of 88% and a specificity of 71%, while the best cutoff for global longitudinal strain (>-15.5%) had a sensitivity of 64% and a specificity of 57%. The association between myocardial work and perfusion defects was significant independently of late gadolinium enhancement ≥15% and obstructive HCM. Conclusions Impaired myocardial work was significantly correlated with the extent of ischemia in cardiac magnetic resonance, independently of the degree of left ventricular hypertrophy or fibrosis, with a higher predictive power than global longitudinal strain.

Impact of repaired aortic coarctation in left ventricular myocardial work.

AIMS: Myocardial strain analysis enables more precise assessment of cardiac performance but is relatively load dependent. New tools have been developed with afterload adjustment. Our objective was to assess myocardial work (MW) in patients with repaired aortic coarctation (rACo). METHODS: Prospective study of consecutive patients with rACo who underwent a routine transthoracic echocardiogram in 2018 and 2019 at our center. Patients with significant aortic valve disease, pacemaker, or other congenital heart diseases (except for mild bicuspid aortic valve disease) were excluded. Global longitudinal strain with two dimensional speckle tracking analysis and MW were obtained (GWI:Global Work Index; GCW: Global Constructive Work; GWW: Global Wasted Work; GWE: Global Work Efficiency). Blood pressure was measured in the patient's right arm. RESULTS: We included 42 patients in the analysis, mean age of 37±10 years, 38% males. In this group, 52% had hypertension and 64% had a concomitant bicuspid aortic valve. In comparison to previously published reference values, patients with rACo had significantly lower GWI (1807 vs. 1896 mmHg%) and GCW (2173 vs. 2232 mmHg%) (p<0.001), particularly in males. Systolic blood pressure is an independent predictor for GWI (ß=0.432) and for GCW (ß=0.534) and GLS an independent predictor of all MW parameters (ß>0.594). Neither age nor gender were independent predictors. CONCLUSIONS: In patients with rACo, there are some signs of left ventricular dysfunction with a reduction in GCW and GWI and with preserved GWE, despite normal ejection fraction and strain.

Late gadolinium enhancement in the left ventricular wall is associated with atrial fibrillation in patients with hypertrophic cardiomyopathy.

PURPOSE: Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to evaluate the relation between AF and left ventricular (LV) late gadolinium enhancement (LGE). METHODS: 55 patients with HCM were retrospectively included. Patients were divided in HCM with AF and HCM without AF. Baseline clinical, echocardiographic and cardiovascular magnetic resonance (CMR) characteristics were collected and compared between groups. RESULTS: In univariable analysis, the factors related to AF development were HCM risk score for sudden cardiac death (SCD) > 2.29% (p = 0.002), left atrium (LA) diameter > 42.5 mm (p = 0.014) and LGE in the mid anterior interventricular septum (IVS) (p = 0.021), basal inferior IVS (p = 0.012) and mid inferior IVS (p = 0.012). There were no differences in LV diastolic function and LA strain between groups. Independent predictors of AF were LA diameter (p = 0.022, HR 5.933) and LGE in mid inferior IVS (p = 0.45, HR 3.280). Combining LA diameter (> 42.5 mm or < 42.5 mm) and LGE in mid inferior IVS (present or absent) in a model with four groups showed a statistically significant difference between groups (p = 0.013 for the model). CONCLUSIONS: LGE in mid inferior IVS is an independent predictor for AF occurrence in patients with HCM. Combining both LGE in mid inferior IVS and enlarged LA improves prediction of AF and may be preferred for risk stratification.

Benefits of cardiac resynchronization therapy in "very dilated cardiomyopathy".

INTRODUCTION: Recent clinical trials have studied parameters that could predict response to cardiac resynchronization therapy (CRT) in patients with advanced heart failure. Left ventricular end-diastolic dimension (LVEDD) is regarded as a possible predictor of response to CRT. OBJECTIVE: To study the response to CRT in patients with very dilated cardiomyopathy, i.e. those at a more advanced stage of the pathology, analyzing both the responder rate and reverse remodeling in two groups of patients classified according to LVEDD. METHODS: We performed a retrospective analysis of 71 patients who underwent CRT (aged 62 +/- 11 years; 65% male; 93% in NYHA functional class > or = III; 31% with ischemic cardiomyopathy; left ventricular ejection fraction [LVEF] 25.6 +/- 6.8%; 32% in atrial fibrillation; QRS 176 +/- 31 ms). Twenty-two (31%) patients with LVEDD > or = 45 mm/m2 (49.2 +/- 3.5 mm/m2) were considered to have very dilated cardiomyopathy (Group A) and 49 patients had LVEDD > 37 mm/m2 and < 45 mm/m2 (39.4 +/- 3.8 mm/m2) (Group B). All patients were assessed by two-dimensional echocardiography at baseline and six months after CRT. The following parameters were analyzed: NYHA functional class, LVEF and LVEDD. Responders were defined clinically (improvement of > or = 1 NYHA class) and by echocardiography, with a minimum 15% increase over baseline LVEF combined with a reduction in LVEDD (reverse remodeling). RESULTS: There were no significant differences in baseline demographic characteristics between the two groups. At six-month followup, we observed an improvement in LVEF (delta 8.5 +/- 11.8%) and a reduction in LVEDD (delta 3.7 +/- 6.8 mm/m2), with fifty-seven (79%) patients being classified as clinical responders. The percentage of patients with reverse remodeling was similar in both groups (64% vs. 73%, p = NS), as were percentages of improved LVEF (delta 6.3 +/- 11% vs. delta 9.6 +/- 12%; p = NS) and decreased LVEDD (delta 3.7 +/- 5.5 mm/m2 vs. delta 3.7 +/- 7.4 mm/m2; p = NS). We found a higher percentage of clinical responders in patients with very dilated cardiomyopathy (96% vs. 72%, p < 0.05). CONCLUSION: In this study, a significant number of responders showed reverse remodeling after CRT. Although a higher percentage of patients with very dilated cardiomyopathy showed improvement in functional class, the extent of reverse remodeling was similar in both groups.

Primary angiosarcoma of the pericardium: case report and review of the literature.

Primary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Mean survival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient with localised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy and surgery.

Giant interatrial septal aneurysms mimicking quistic masses. Two cases with different therapeutic options.

With the recent technical improvement in echocardiography imaging (second harmonics) the number of interatrial septum aneurysms (ASA) increased and are easily recognized. We assist to an overdiagnosing number of cases and diagnostic criteria emerged to face this problem. In the great majority of the cases ASA are small and inoffensive, but as ASA is considered a risk factor for cardioembolism when associated with persistence of foramen oval (PFO), an examination by transesophageal echocardiography (TEE) for exclusion of PFO makes the sense and is a common testing in patients with cryptogenic stroke. Besides these frequent ASA, other forms exist; the authors describe two cases of uncommon and huge ASA, one mimicking a right atrial tumor and the other a quistic, hipoechoic mass. The first case was associated with mitral stenosis and was submitted to surgery and the second was closed with an Amplatzer occluder device usually used in atrial septal defect (ASD).

Noncompaction cardiomyopathy--a review of eight cases.

Left ventricular noncompaction is a genetic disorder that is thought to be related to an arrest in endomyocardial development. It is characterized by the presence of a prominent trabecular meshwork and deep recesses. In order to better characterize this recently described disorder, whose prognosis remains unclear, we review eight cases diagnosed at our hospital, describing their clinical, electrocardiographic and echocardiographic features as well as therapy and follow-up. We also discuss the most relevant data from the literature concerning pathogenesis, clinical presentation, diagnostic criteria, therapy and prognosis.

Echocardiographic variables predictive of appropriate therapies for ventricular tachyarrhythmia in patients undergoing combined cardiac resynchronization therapy.

INTRODUCTION: The significant risk of sudden arrhythmic death in patients with congestive heart failure and electromechanical ventricular dyssynchrony has led to increased use of combined cardiac resynchronization therapy defibrillator (CRT-D) devices. OBJECTIVES: To evaluate the echocardiographic variables in patients undergoing CRT-D that predict the occurrence of appropriate therapies (AT) for ventricular tachyarrhythmia. METHODS: We analyzed 38 consecutive patients (mean age 60 +/- 12 years, 63% male) with echocardiographic evaluation before and 6 months after CRT-D implantation. Patients with AT were identified in a mean follow-up of 471 +/- 323 days. A standard echocardiographic study was performed including tissue Doppler imaging (TDI). Responders were defined as patients with improvement in NYHA class of < or = 1 in the first six months, and reverse remodeling as a decrease in left ventricular end-systolic volume of < or = 15% and/or an increase in left ventricular ejection fraction of > 25%. RESULTS: The responder rate was 74%, and the reverse remodeling rate was 55%. AT occurred in 21% of patients, who presented with greater left ventricular end-diastolic internal diameter (LVEDD) before implantation (86 +/- 8 vs. 76 +/- 11 mm, p = 0.03) and at 6 months (81 +/- 8 vs. 72 +/- 14 mm, p = 0.08), and increased left ventricular end-systolic internal diameter (66 +/- 14 vs. 56 +/- 14 mm, p = 0.03) and lower ejection fraction (24 +/- 6 vs. 34 +/- 14%, p = 0.08) at 6 months. In the group with AT, the responder rate was lower (38 vs. 83%, p = 0.03), without significant differences in reverse remodeling (38% for the AT group vs. 60%, p = 0.426) or in the other variables. By univariate analysis, predictors of AT were LVEDD before implantation and E' after implantation. Age, gender, ischemic etiology, use of antiarrhythmic drugs, reverse remodeling and the other echocardiographic parameters did not predict AT. In multivariate logistic regression analysis, both LVEDD before implantation (OR 1.24, 95% CI 1.04-1.48, p = 0.019) and postimplantation E' (OR 0.27, 95% CI 0.09-0.76, p = 0.014) remained as independent predictors of AT. CONCLUSIONS: In patients undergoing CRT-D, episodes of ventricular tachyarrhythmia occur with high incidence, independently of echocardiographic response, with LVEDD before implantation and E' after implantation as the only independent predictors of AT in the medium-term. These results highlight the importance of combined devices with defibrillation capability.

Left atrial volume: an old echocardiographic measure with renewed prognostic significance: a study in patients with dilated cardiomyopathy.

UNLABELLED: Left atrial enlargement is common in many cardiac diseases; a major determinant of this enlargement is left ventricular diastolic pressure. Left atrial volume (LAV) has recently been linked to adverse prognosis. The objective of our study was to assess LAV as a prognostic marker in patients with dilated cardiomyopathy (DCM) and to correlate LAV with other known prognostic factors such as maximum oxygen consumption (VO2 max) and pro-B-type natriuretic peptide (pro-BNP). METHODS: Our population consisted of 35 patients with DCM followed regularly in our hospital and with mean ejection fraction (EF) of 31+/-9.6%. All patients underwent routine 2D Doppler echocardiographic examination, TDI was used to obtain early peak velocity of the mitral annulus, and LAV was calculated using Simpson's method of disks. Pro-BNP was measured and cardiopulmonary exercise testing was also carried out. Mean follow-up was 24+/-4 months and new hospital admission due to heart failure, cardiac transplantation and death were considered cardiac events. RESULTS: All two-dimensional (left ventricular dimensions and volumes and EF) and conventional Doppler echocardiographic parameters (mitral inflow) were analyzed; mean LAV was 78.6+/-33 ml. Age and gender had no influence on LAV. LAV (and LAV indexed to body surface area [BSA]) showed an inverse correlation with VO2max (r=-0.48, p=0.008) and with pro-BNP (r=0.43, p=0.02). With regard to prognosis, LAV and LAV/BSA were associated with cardiac events (r=0.4, p=0.02), like other classic prognostic markers (EF, left ventricular volumes, mitral regurgitation and E/E'). The cut-off values of LAV and LAV/BSA for cardiac events on ROC curve analysis were 70 ml and 38 ml/m2 respectively. CONCLUSIONS: LAV (LAV/BSA) has incremental prognostic value in patients with dilated cardiomyopathy and correlates with exercise tolerance and pro-BNP.

Cardiac resynchronization therapy--clinical and echocardiographic characteristics of responders and exceptional responders.

UNLABELLED: Cardiac resynchronization therapy (CRT) is a novel treatment for systolic heart failure and in successful cases reverse remodeling occurs with reduction of left ventricular (LV) dimensions and volumes. The term "responders" applies to all patients who improve in functional class and quality of life; however, some responders show exceptional improvement, including normalization of clinical and echocardiographic parameters (exceptional or super-responders). OBJECTIVE: The purpose of our study was to analyze responders (R) and super-responders (SR). METHODS: 36 patients were referred for CRT due to depressed left ventricular function (mean ejection fraction 26 +/- 6.9%) and QRS duration (QRSd) of 175 +/- 29.9 ms. All patients underwent complete 2D, Doppler, and tissue Doppler (TDI) echocardiography before and after CRT (performed 3-6 months after CRT implantation) and measurement of pro-BNP. Twelve were non-responders (nR--Group I). Of the 24 patients showing improvement in NYHA functional class (responders), 11 were classified as super-responders with ejection fraction > or =40% (Group II--13 patients [R] and Group III--11 patients [SR]). RESULTS: There were no differences between the three groups in QRSd or the following baseline echocardiographic parameters: LV dimensions and volumes, ejection fraction, TDI velocities and dyssynchrony parameters. The exception was left atrial (LA) dimensions (greater in group I) and mitral inflow, with a predominant restrictive pattern in Group I. Baseline maximum VO2 was lower in Group I compared to Group III (13 vs. 21 mm/kg/min, p < 0.0001). We compared the three groups during a mean follow-up of 15 +/- 8 months. Group II showed slight improvement of left ventricular function without statistical significance, only pro-BNP showing a significant reduction (p = 0.04). Comparing Groups I and III, there were differences in the super-responder group for pro-BNP and echocardiographic LV dimensions, volumes and ejection fraction, LA dimensions, pulmonary artery pressure and TDI velocities, with normalization of septal systolic velocity: Group III--5.13 cm/s vs. Group I--2.95 cm/s, p = 0.001. CONCLUSION: The degree of improvement in CRT patients is difficult to predict by baseline echocardiography and QRSd, only a restrictive mitral inflow pattern appearing to be a determining factor. Remodeling in super-responders is probably due to a structural process, with a trend towards normalization of ejection fraction and other systolic parameters.

Primary idiopathic chylopericardium.

Chylopericardium is a rare entity which is usually associated with trauma (thoracic duct lesions), neoplasia or filaria infection. Primary forms are even rarer and are caused by lymphatic circulation malformations. We present the case of a 52-year-old male patient with primary chylopericardium. We discuss the diagnostic approach, as well as the favorable clinical evolution with conservative management.

Short- and long-term outcome after alcohol septal ablation in obstructive hypertrophic cardiomyopathy: Experience of a reference center.

INTRODUCTION: In obstructive hypertrophic cardiomyopathy (HCM), alcohol septal ablation (ASA) can lead to gradient reduction and symptom improvement. We aimed to assess the efficacy and safety of ASA in a long-term outcome study. METHODS: We analyzed patients who underwent ASA over a seven-year period in a tertiary center. The primary echocardiographic endpoint was >50% reduction in left ventricular outflow tract (LVOT) gradient within a year of the procedure. The primary clinical endpoints were improvement in functional capacity and a combined endpoint of cardiac death and rehospitalization for cardiac cause. The follow-up period was 4.17±2.13 years. RESULTS: A total of 80 patients, mean age 63.9±12.3 years, 30.0% male, were analyzed. Baseline LVOT gradient was 96.3±34.6 mmHg and interventricular septal thickness was 21.6±3.1 mm. Minor complications were observed in 6.3% and major complications in 2.5%, and 8.8% received a permanent pacemaker. The primary echocardiographic endpoint was achieved by 85.7%. At three-month follow-up, LVOT gradient was 25.8±26.0 mmHg in the successful procedure group, compared to 69.2±35.6 mmHg in the other patients (p=0.001). At six months, LVOT gradient was 27.1±27.4 vs. 58.2±16.6 mmHg (p=0.024). Among 74 patients in NYHA class III/IV before the procedure, 57 (77%) improved to NHYA class I/II. The combined primary clinical endpoint (cardiac death and rehospitalization for cardiac cause) was observed in 27.5% (n=22). In the unsuccessful group, the combined endpoint was observed in 54.5%, compared to only 22.7% in the successful group. Only two patients died of cardiac causes. CONCLUSION: ASA is a safe procedure with a high success rate. Patients who achieved significant reductions in LVOT gradient suffered less cardiac death and rehospitalization for cardiac cause.

Response and outcomes of cardiac resynchronization therapy in patients with renal dysfunction.

PURPOSE: Renal dysfunction is often associated with chronic heart failure, leading to increased morbi-mortality. However, data regarding these patients after cardiac resynchronization therapy (CRT) is sparse. We sought to evaluate response and long-term mortality in patients with heart failure and renal dysfunction and assess renal improvement after CRT. METHODS: We analyzed 178 consecutive patients who underwent successful CRT device implantation (age 64 ± 11 years; 69% male; 92% in New York Heart Association (NYHA) functional class ≥ III; 34% with ischemic cardiomyopathy). Echocardiographic response was defined as ≥ 15% reduction in left ventricular end-systolic diameter and clinical response as a sustained improvement of at least one NYHA functional class. Renal dysfunction was defined as an estimated glomerular filtration rate (eGFR) below 60 mL/min/1.73 m2. RESULTS: Renal dysfunction was present in 34.7%. Renal dysfunction was not an independent predictor of echocardiographic response (OR 1.109, 95% CI 0.713-1.725, p 0.646) nor clinical response (OR 1.003; 95% CI 0.997-1.010; p 0.324). During follow-up (mean 55.2 ± 32 months), patients with eGFR < 60mL/min/1.73 m2 had higher overall mortality (HR 4.902, 95% CI 1.118-21.482, p 0.035). However, clinical response in patients with renal dysfunction was independently associated with better long-term survival (HR 0.236, 95% CI 0.073-0.767, p 0.016). Renal function was significantly improved in patients who respond to CRT (ΔeGFR + 5.5 mL/min/1.73 m2 at baseline vs. follow-up, p 0.049), while this was not evident in nonresponders. Improvements in eGFR of at least 10 mL/min/1.73 m2 were associated with improved survival in renal dysfunction patients (log-rank p 0.036). CONCLUSION: Renal dysfunction was associated with higher long-term mortality in CRT patients, though, it did not influence echocardiographic nor functional response. Despite worse overall prognosis, renal dysfunction patients who are responders showed long-term survival benefit and improvement in renal function following CRT.

Rupture of a submitral ventricular aneurysm into the left atrium diagnosed by transesophageal echocardiography.

Submitral ventricular aneurysm is a thoroughly studied pathology but is not well known due to its rarity. Clinically, it is manifested by symptoms and signs of heart failure, mitral regurgitation and/or ventricular arrhythmias, and may be associated with thromboembolic phenomena and myocardial ischemia due to compression of the coronary arteries by the aneurysm. A rare complication of this type of aneurysm is rupture into the left atrium. Transthoracic echocardiography plays an important role in the definitive diagnosis of this pathology, although the role of transesophageal echocardiography in the evaluation of these patients is less known. We report a case of a submitral ventricular aneurysm complicated by rupture into the left atrium, which was diagnosed by transesophageal echocardiography.