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.

Infective endocarditis in a cohort of adult CHD patients.

BACKGROUND: CHD increases the risk of infective endocarditis due to the substrate of prosthetic materials and residual lesions. However, lesion-specific and mortality risks data are lacking. We sought to analyse clinical course and mortality of infective endocarditis in a cohort of adult CHD. METHODS: Retrospective analysis of all cases of proven and probable infective endocarditis (Duke's criteria) followed in our adult CHD clinic between 1970 and August, 2021. Epidemiological, clinical and imaging data were analysed. Predictors of surgical treatment and mortality were assessed using regression analysis. RESULTS: During a mean follow-up of 15.8 ± 10.9 years, 96 patients had 105 infective endocarditis episodes, half with previous cardiac surgery (corrective or palliative). The most frequent diagnoses were: ventricular septal defect, bicuspid aortic valve, Tetralogy of Fallot and pulmonary atresia. The site of infection was identified by echocardiography in 82 episodes (91%), most frequently in aortic (n = 27), tricuspid (n = 15), and mitral (n = 13) valves. Blood cultures were positive in 79% of cases, being streptococci (n = 29) and staphylococci (n = 23) the predominant pathogens. Surgery was necessary in 40% and the in-hospital mortality was 10.5%, associated with heart failure (p < 0.001; OR 13.5) and a non-surgical approach (p = 0.003; OR 5.06). CONCLUSIONS: In an adult CHD cohort, infective endocarditis was more frequent in patients with ventricular septal defect and bicuspid aortic valves, which contradicts the current guidelines that excludes them from prophylaxis. Surgical treatment is often required and mortality remains substantial. Prevention of this serious complication should be one of the major tasks in the care of adults with CHD.

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.

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.

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.

Left ventricular global longitudinal strain is associated with filling pressure and cardiac output in an outpatient setting: Insights from CardioMEMS™.

INTRODUCTION AND OBJECTIVES: Left ventricular global longitudinal strain (LVGLS) is an indicator of myocardial function in patients with heart failure with reduced ejection fraction (HFrEF) and preserved ejection fraction (HFpEF). Nevertheless, it is not clear whether LVGLS correlates with filling pressures and cardiac output (CO) in an ambulatory setting. We aimed to assess whether LVGLS is associated with invasive pulmonary artery pressures (PAP) and CO in outpatients using the invasive remote monitoring CardioMEMS™ system. METHODS: This single-center, prospective observational study included patients with HFrEF undergoing remote monitoring using the CardioMEMS™ system, between January 2020 and December 2022. Repeated transthoracic echocardiography (TTE) studies were performed in each patient and invasive hemodynamic data were obtained during the TTE studies using the CardioMEMS™ system. Univariate and multivariate models were used to assess the potential association between LVGLS and invasive PAP and CO. RESULTS: Twelve patients were included and 46 TTE studies were analyzed. LVGLS was correlated with diastolic (d) PAP (r=0.403, p=0.041) and CO (r=-0.426, p=0.039) in the univariate analysis. In multivariate models, LVGLS was an independent predictor of dPAP and CO, but not mean PAP or systolic PAP. The variation of LVGLS between TTE studies was correlated with the variation of dPAP during follow-up (r=0.60, p=0.017). CONCLUSIONS: In a cohort of HFrEF patients under invasive hemodynamic remote monitoring, LVGLS was independently associated with invasive filling pressures and CO, in an outpatient setting. These findings reinforce the value of LVGLS for the management of outpatients with HFrEF.

Understanding cardiac myxoma recurrence: a case report.

Recurrence of cardiac myxoma is a rare condition, observed in about 3% of patients in sporadic cases, although it is more frequent in familial ones. Several mechanisms have been proposed to explain such recurrence, and the importance of increased vascularization as a facilitating feature is the subject of debate. The authors report the case of a non-familial right atrial myxoma, unusual for both its histopathology and 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.

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.

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.

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.

Long term follow-up of Marfan Syndrome - experience of an adult congenital heart disease centre.

BACKGROUND: Marfan Syndrome (MFS) is one of the most common connective tissue disorders. The aim of this study was to characterize an adult population with MFS and evaluate its long-term prognosis. METHODS: A retrospective analysis of adult patients with MFS followed up during the past 40 years in a tertiary congenital heart disease outpatient clinic was performed. Survival analysis was performed according to different parameters, and survival curves were compared using the log-rank test. RESULTS: A total of 62 MFS patients were followed up for a mean period of 12 years (47% male; mean age, 39 years). The baseline mean aortic root diameter (ARD) at the Valsalva sinus was 42.4 ± 10.3 mm, with 15% of patients having moderate-to-severe aortic regurgitation and seven patients with acute aortic syndrome. The Bentall procedure was the most commonly performed surgical technique, and five patients required re-operation. Of the 17 pregnancies, 29% developed fetal complications; however, there was no maternal morbidity or mortality. A total of ten deaths occurred at a mean age of 52 years. Patients with an ARD ≤ 45 mm had a significantly lower all-cause mortality rate than patients with 45 < ARD ≤ 50 mm or with ARD > 50 mm (P = 0.004 and P < 0.001, respectively). Heart failure symptoms were associated with a worse outcome (P = 0.041), while the presence of extracardiac involvement had a protective effect (P < 0.001). CONCLUSION: MFS-related aortopathy is associated with high morbidity rates. In the overall population, an ARD > 45 mm at the time of diagnosis was associated with higher mortality during follow-up.

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.

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.