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.

Cardiotoxicity in breast cancer treatment: What about left ventricular diastolic function and left atrial function?

AIMS: Cardiotoxicity is a possible complication of cancer treatment, particularly with anthracyclines and anti-HER2 drugs. Systolic dysfunction has already been described. Diastolic dysfunction and left atrial function are less studied. We sought to analyze the impact of cardiotoxic treatments on left ventricular diastolic function and left atrial (LA) function. METHODS AND RESULTS: Retrospective study of 100 patients (all women, with a mean age of 54 ± 12 years) with three exams in the span of 1 year during treatment for breast cancer. Patients with previous cancer treatment, coronary artery disease, significant valvular disease, and atrial arrhythmias were excluded. Diastolic dysfunction was classified according to international guidelines and left atrial strain was analyzed by two-dimensional speckle tracking. In our sample, 74% received anthracyclines, 83% anti-HER2, and 76% radiation treatment. In the follow-up, 20% developed new or worsening diastolic dysfunction. Age was the only independent predictor (OR 1.93, 95% CI 1.04-3.58, P = .037). In left atrial function, only the contractile function was significantly reduced in 20.8% of the patients and age was also the only independent predictor, but with a protective effect (OR 0.51, 95% CI 0.28-0.91, P = .023). CONCLUSIONS: During breast cancer treatment, 20% of the patients develop new or worsening diastolic dysfunction, being age the main determinant, suggesting higher impact of chemotherapy in older patients. Contractile left atrial function is also compromised but, in this case, age seems to be protective. Our results support a stricter surveillance in older patients together to eventually adjust chemotherapy regimens.

Long-Term Follow Up After Successful Percutaneous Balloon Mitral Valvuloplasty.

BACKGROUND: Percutaneous balloon mitral valvuloplasty (PMV) is the cornerstone of rheumatic mitral stenosis treatment in suitable patients. Previous studies have reported low rates of technical failure and few major complications, with good long-term results after successful PMV. The study aim was to assess the very long-term outcome in patients after PMV performed at a single tertiary center. METHODS: Between 1991 and 2010, a total of 213 consecutive patients underwent PMV at the authors' center. The criteria used to define successful procedure were post-procedural mitral valve area ≥1.5 cm2 and mitral regurgitation less than grade III, without in-hospital major cardiac or cerebrovascular events. The primary endpoint evaluated during the follow up period was the occurrence of cardiovascular death and need for mitral reintervention (percutaneous or surgical). Kaplan-Meier curves were generated to determine event rates, and predictors of major cardiac events in patients with successful PMV were determined using Cox regression analysis. RESULTS: A total of 190 patients (89%) underwent a successful PMV; 88% of these are currently being followed up at the authors' center. During a mean follow up of 11.2 ± 7.3 years, at least one major adverse cardiac event occurred in 25.1% of patients (6.6% all-cause death, 6.6% repeated PMV, 21% required mitral valve surgery). Cumulative event-free survival at 20 years was 54.7 ± 6.3%. On univariate analysis, the echocardiographic score [hazard ratio (HR) = 1.25 (1.00-1.70), p <0.05], left atrial diameter [HR = 1.06 (1.01-1.11), p <0.05] and mean mitral valve gradient soon after the procedure [HR = 1.25 (1.02-1.55), p <0.05] were predictors of events. On multivariate analysis, the echocardiographic mitral valve score before PMV was the only independent predictor of primary outcome [HR=1.75 (1.16-2.64), p<0.01]. CONCLUSIONS: Up to 20 years after successful PMV, a sizeable proportion of patients remained event-free, which confirmed the late efficacy of PMV. Among the present patient cohort, echocardiographic score before PMV was the only independent predictor of long term events.

Modified continuity equation using left ventricular outflow tract three-dimensional imaging for aortic valve area estimation.

PURPOSE: Aortic valve area (AVA) is usually estimated by the continuity equation (CE) in which the left ventricular outflow tract (LVOT) area is calculated assuming a circular shape. This study aimed to compare measurements of LVOT area using standard 2D transthoracic echocardiography (2DTTE), 3D transesophageal echocardiography (3DTEE), and multidetector computed tomography (MDCT) and assess their relative impact on AVA estimated by the CE. METHODS AND RESULTS: We prospectively enrolled 60 patients with severe aortic stenosis (AS) referred for transcatheter aortic valve replacement (TAVR) who systematically underwent 2DTTE, 3DTEE, and MDCT. Mean LVOT areas obtained by 2DTTE (3.28±0.66 cm2 ) and 3DTEE (3.95±0.90 cm2 ) were significantly underestimated when compared to the mean MDCT LVOT area (4.31±0.99 cm2 ). LVOT was rather elliptical than round, with a mean eccentricity index of 1.47 (ratio of maximum to minimum LVOT diameters) assessed by MDCT. Mean TTE AVA estimated by the CE was 0.62±0.20 cm2 . Substitution of 2DTTE LVOT area by 3DTEE LVOT area in the CE resulted in AVA of 0.74±0.24 cm2 , while using MDCT LVOT area held an AVA of 0.80±0.24 cm2 . MDCT-derived AVA was similar to MDCT planimetric AVA and allowed 24% of patients to be reclassified from severe to moderate AS. CONCLUSIONS: 2DTTE and 3DTEE underestimate LVOT area when compared to MDCT with significant impact on AVA estimation. Assessment through MDCT fusion AVA may be of incremental value in patients with discrepant severity criteria for AS.

Bicuspid aortic valve outcomes.

BACKGROUND: Bicuspid aortic valve is the most common CHD. Its association with early valvular dysfunction, endocarditis, thoracic aorta dilatation, and aortic dissection is well established. OBJECTIVE: The aim of this study was to assess the incidence and predictors of cardiac events in adults with bicuspid aortic valve. METHODS: We carried out a retrospective analysis of cardiac outcomes in ambulatory adults with bicuspid aortic valve followed-up in a tertiary hospital centre. Outcomes were defined as follows: interventional - intervention on the aortic valve or thoracic aorta; medical - death, aortic dissection, aortic valve endocarditis, congestive heart failure, arrhythmias, or ischaemic heart disease requiring hospital admission; and a composite end point of both. Kaplan-Meier curves were generated to determine event rates, and predictors of cardiac events were determined by multivariate analysis. RESULTS: A total of 227 patients were followed-up over 13±9 years; 29% of patients developed severe aortic valve dysfunction and 12.3% reached ascending thoracic aorta dimensions above 45 mm. At least one cardiac outcome occurred in 38.8% of patients, with an incidence rate at 20 years of follow-up of 47±4%; 33% of patients were submitted to an aortic valve or thoracic aorta intervention. Survival 20 years after diagnosis was 94±2%. Independent predictors of the composite end point were baseline moderate-severe aortic valve dysfunction (hazard ratio, 3.19; 95% confidence interval, 1.35-7.54; p<0.01) and aortic valve leaflets calcification (hazard ratio, 4.72; 95% confidence interval, 1.91-11.64; p<0.005). CONCLUSIONS: In this study of bicuspid aortic valve, the long-term survival was excellent but with occurrence of frequent cardiovascular events. Baseline aortic valve calcification and dysfunction were the only independent predictors of events.

The value of right ventricular longitudinal strain in the evaluation of adult patients with repaired tetralogy of Fallot: a new tool for a contemporary challenge.

OBJECTIVE: The role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients. METHODS: Patients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI. Right ventricular longitudinal strain was evaluated in the four-chamber view using the standard semiautomatic method. RESULTS: In total, 42 patients were included (61% male, 32±8 years). The mean right ventricular longitudinal strain was -16.2±3.7%, and the right ventricular ejection fraction, measured by MRI, was 42.9±7.2%. Longitudinal strain showed linear correlation with tricuspid annular systolic excursion (r=-0.40) and right ventricular ejection fraction (r=-0.45) (all p<0.05), which in turn showed linear correlation with right ventricular fractional area change (r=0.50), pulmonary regurgitation colour length (r=0.35), right ventricular end-systolic volume (r=-0.60), and left ventricular ejection fraction (r=0.36) (all p<0.05). Longitudinal strain (ß=-0.72, 95% confidence interval -1.41, -0.15) and left ventricular ejection fraction (ß=0.39, 95% confidence interval 0.11, 0.67) were independently associated with right ventricular ejection fraction. The best threshold of longitudinal strain for predicting a right ventricular ejection fraction of <40% was -17.0%. CONCLUSIONS: Right ventricular longitudinal strain is a powerful method for evaluating patients with tetralogy of Fallot. It correlated with echocardiographic right ventricular function parameters and was independently associated with right ventricular ejection fraction derived by MRI.

New-Onset Hemolytic Anemia after Percutaneous Paravalvular Leak Closure.

BACKGROUND AND AIM OF THE STUDY: Despite being usually clinically silent, paravalvular leak can present with congestive heart failure (CHF) or haemolytic anemia. Here, the case is reported of a paravalvular leak presenting with CHF, complicated by new-onset severe hemolytic anemia after percutaneous closure with a large ventricular septal duct (VSD) occlude device. METHODS: A 57-year-old patient presented with infectious endocarditis of a native mitral valve with major mitral regurgitation and was submitted for mitral valvuloplasty. However, one month later failure of the valvuloplasty forced the need for mechanical prosthetic valve implantation. Early endocarditis of the mechanical valve with CHF was noted two months later and led to mechanical valve substitution. One year later the patient presented with a major paravalvular leak and CHF recurrence. Hence, percutaneous paravalvular leak closure was proposed. RESULTS: A 16-mm VSD occluder was used, and clinical and echocardiography success was noticed. However, new-onset hemolytic anemia with acute kidney injury forced surgical re-intervention, with the successful implantation of a third mechanical valve. CONCLUSIONS: Usually, percutaneous paravalvular leak closure is a safe and successful method to treat high-risk surgical patients. However, hemolytic anemia may develop after a technically successful procedure, forcing surgical intervention. Dedicated devices are needed to overcome this important safety issue. Video 1: Periprocedural acquisition of 3-D transesophageal echocardiography showing two AVP II in the left atrium after unsuccessful deployment. Video 2: Periprocedural imaging of a muscular ventricular septal duct (mVSD) occluder (16 mm), with successful closure of the paravalvular leak.

Contribution of Dobutamine Stress Echocardiography to the Diagnosis and Prognosis of Low-Flow/Low-Gradient Aortic Stenosis.

BACKGROUND: Aortic valve replacement (AVR) is the treatment of choice in patients with severe symptomatic aortic stenosis (AS). Patients with a low left ventricular ejection fraction (LVEF) represent a challenge for evaluation and therapeutic decision. Dobutamine stress echocardiography (DSE) allows the distinction to be made between fixed low-flow/low-gradient (LF/LG) AS and pseudosevere AS. METHODS: Between 2001 and 2014 a retrospective analysis was conducted of patients who underwent DSE to investigate severe AS. DSE was performed in 3- to 5-min steps up to a maximum dose of 20 µg/kg/min. Parameters evaluated at baseline and at each step of DSE included: left ventricular enddiastolic volume (LVEDV) and left ventricular endsystolic volume (LVESV), maximum gradient (MaxG), mean gradient (MG) and aortic valve area (AVA). AS was considered to be severe if the AVA at peak dose was ≤1 cm2. Patients were allocated to two groups according to their therapy: group 1 received only medical treatment, while group 2 underwent AVR. The average follow up was 51.5 ± 4.4 months. RESULTS: A total of 41 patients (28 males, 13 females; mean age 71.7 ± 8.3 years) was analyzed. Severe AS was diagnosed in 34 patients (83%). Baseline echocardiographic characteristics were: AVA 0.7 ± 0.2 cm2, MaxG 42.0 ± 9.1 mmHg, MG 25.6 ± 6.4 mmHg, LVEF 33.1 ± 8.4%, LVEDV 149.6 ± 44.5 ml, and LVESV 104.0 ± 42.6 ml. At peak DSE, AVA was 0.8 ± 0.2 cm2, MaxG 62.7 ± 18.2 mmHg, MG 38.1 ± 11.6 mmHg, LVEF 42.2 ± 9.9%, LVEDV 142.6 ± 43.85 ml, and LVESV 89.7 ± 37.4 ml. Nineteen patients were allocated to group 1, and 22 to group 2. In group 2, two patients underwent transcatheter aortic valve implantation (TAVI) and 20 had surgery. Mortality in group 1 was significantly higher than in group 2 (78.9% versus 27.3%). A Cox proportional hazard model analysis showed that no-intervention was the only predictor of mortality (unadjusted to age; hazard ratio (HR) 5.13, 95% confidence interval (CI) 1.96-13.44, p = 0.001; adjusted to age - HR 4.01, 95% CI 1.46-11.01, p = 0.007). CONCLUSIONS: LF/LG AS has a poor prognosis without intervention. DSE allows the lesion severity to be established. In the present study intervention was a predictor of survival during follow up.

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.

Permanent pacemaker implantation after alcoholic septal ablation induced complete heart block: Long-term impact.

INTRODUCTION AND OBJECTIVES: Patients with hypertrophic obstructive cardiomyopathy (HOCM) that remain symptomatic despite optimized medical therapy often undergo alcohol septal ablation (ASA). One of the most frequent complications is complete heart block (CHB), requiring a permanent pacemaker (PPM) in variable rates of up to 20% of patients. The long-term impact of PPM implantation in these patients remains unclear. This study aimed to evaluate the long-term clinical outcomes in patients who implant PPM after ASA. METHODS: Patients who underwent ASA at a tertiary center were consecutively and prospectively enrolled. Patients with previous PPM or implantable cardio-defibrillator were excluded from this analysis. Patients with and without PPM implantation after ASA were compared based on their baseline characteristics, procedure data and three-year primary endpoint of composite of all-cause mortality and hospitalization and secondary endpoint of composite of all-cause mortality and cardiac cause hospitalization. RESULTS: Between 2009 and 2019, 109 patients underwent ASA, 97 of whom were included in this analysis (68% female, mean age 65.2 years old). 16 patients (16.5%) required PPM implantation for CHB. In these patients, no vascular access, pacemaker pocket or pulmonary parenchyma complications were noted. The baseline characteristics of comorbidities, symptoms, echocardiographic and electrocardiographic findings were identical in the two groups, with higher mean age (70.6±10.0 years vs. 64.1±11.9 years) and lower beta-blocker therapy rate (56% vs. 84%) in the PPM group. Procedure-related data showed higher creatine kinase (CK) peaks in the PPM group (1692 U/L vs. 1243 U/L), with no significant difference in the alcohol dose. At three years after ASA procedure, there were no differences in the primary and secondary endpoints between the two groups. CONCLUSIONS: Permanent pacemaker after ASA induced CHB do not affect long term prognosis in hypertrophic obstructive cardiomyopathy patients.

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.

Giant pericardial cyst mimicking dextrocardia on chest X-ray.

Pericardial cysts are rare benign congenital malformations, usually small, asymptomatic and detected incidentally on chest X-ray as a mass located in the right costophrenic angle. Giant pericardial cysts are very uncommon and produce symptoms by compressing adjacent structures. In this report, the authors present a case of a symptomatic giant pericardial cyst incorrectly diagnosed as dextrocardia on chest X-ray.

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.

[Right atrial aneurysm after chest trauma: late presentation]. / Aneurisma da aurícula direita após traumatismo torácico: apresentação tardia.

We report the case of a 59-year-old patient, with a history of severe chest trauma with rib fractures at the age of 20 and recent onset of fatigue and palpitations, who was diagnosed with atrial tachycardia, which was converted pharmacologically. Cor triatriatum or Ebstein's anomaly were suspected on initial imaging studies (transthoracic echocardiography and MRI). Due to recurrence of arrhythmia, he subsequently underwent repeat echocardiographic evaluation, which established a diagnosis of right atrial aneurysm. The arrhythmia was converted electrically. The patient has remained asymptomatic during 18 months of follow-up, without arrhythmia recurrence, medicated with carvedilol (after an initial period with amiodarone) and warfarin.

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.

Myocardial Work Brings New Insights into Left Ventricular Remodelling in Cardio-Oncology Patients.

Serial transthoracic echocardiographic (TTE) assessment of 2D left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) are the gold standard screening methods for cancer therapeutics-related cardiac dysfunction (CTRCD). Non-invasive left ventricular (LV) pressure-strain loop (PSL) provides a novel method of quantifying myocardial work (MW) with potential advantages to evaluate the impact of cardiotoxic treatments on heart function. We prospectively assessed breast cancer female patients undergoing cancer therapy through serial monitoring by 2D and 3D TTE. Patients were evaluated at T0, T1 and T2 (before, 4-6 and 12-14 months after starting therapy, respectively). Through PSL analysis, MW indices were calculated. A total of 122 patients, with a mean age of 54.7 years, who received treatment with anthracyclines (77.0%) and anti-HER2 (75.4%) were included. During a mean follow-up of 14.9 ± 9.3 months, LVEF and GLS were significantly diminished, and 29.5% developed CTRCD. All MW indices were significantly reduced at T1 compared with baseline and tended to return to baseline values at T2. Global work index and global work efficiency showed a more pronounced variation in patients with CTRCD. The presence of more than one cardiovascular risk factor, obesity and baseline left atrium volume were predictors of changes in MW parameters. In conclusion, breast cancer treatment was associated with LV systolic dysfunction as assessed by MW, with its peak at 4-6 months and a partial recovery afterwards. Assessment of myocardial deformation parameters allows a more detailed characterization of cardiac remodelling and could enhance patient screening and selection for cardioprotective therapeutics.

Blunted coronary flow velocity reserve is associated with impairment in systolic function and functional capacity in hypertrophic cardiomyopathy.

BACKGROUND: Coronary microvascular dysfunction constitutes an important pathophysiological feature in hypertrophic cardiomyopathy (HCM). We aimed to assess the association between impaired coronary flow velocity reserve (CFVR) and ventricular systolic function and functional capacity. METHODS: Eighty-three patients with HCM were enrolled in this prospective cohort study. Patients underwent echocardiogram to evaluate ventricular performance and CFVR in the left anterior descending artery (LAD) and posterior descending artery (PD). Diastolic coronary flow velocity was measured in basal conditions and in hyperemia. CFVR was calculated as the ratio of hyperemic and basal peak diastolic flow velocities. Functional capacity was evaluated by cardiopulmonary exercise testing (CPET). The link between CFVR and biventricular systolic function and peak VO2 was studied. RESULTS: Age was 55.0(14.4)years, 50 patients (60%) were male; 59 patients (71%) had nonobstructive HCM. Mean CFVR LAD was 1.81(0.49) and CFVR PD was 1.73(0.55). Lower CFVR PD was associated with impaired global longitudinal strain (GLS) 2D (ß-estimate:-3.240,95%CI:-4.634;-1.846, p < 0.001), GLS 3D (ß-estimate:-2.559,95%CI:-3.932;-1.186, p < 0.001) and area strain (ß-estimate:-3.044,95%CI:-5.373;-0.716, p = 0.011). Lower values of CFVR PD related to worse global work index (ß-estimate:267.824,95%CI:75.964;459.683, p = 0.007), global constructive work (ß-estimate:217.300,95%CI:38.750;395.850, p = 0.018) and global work efficiency (ß-estimate:5.656,95%CI:2.229;9.084, p = 0.002). Impaired CFVR LAD (ß-estimate:2.826, 95%CI:0.913;4.739, p = 0.004) and CFVR PD (ß-estimate:2.801,95%CI:0.657;4.945, p = 0.011) were associated with lower TAPSE. Lower values of CFVR LAD (ß-estimate:2.580, 95%CI:0.169;4.991, p = 0.036) and CFVR PD (ß-estimate:3.163, 95%CI: 0.721;5.606, p = 0.012) were associated with worse peak VO2. CONCLUSION: Lower CFVR was associated with impairment in biventricular systolic function parameters and functional capacity assessed by pVO2.

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.