Biventricular Function Parameters Research Articles

Multiparametric cardiac magnetic resonance in patients with thalassemia intermedia: new insights from the E-MIOT network.

In a relatively large cohort of thalassemia intermedia (TI) patients, we systematically investigated myocardial iron overload (MIO), function, and replacement fibrosis using cardiac magnetic resonance (CMR), we assessed the clinical determinants of global heart T2* values, and we explored the association between multiparametric CMR findings and cardiac complications. We considered 254 beta-TI patients (43.14 ± 13.69years, 138 females) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia project. MIO was quantified by T2* technique and biventricular function and atrial areas by cine images. Macroscopic myocardial fibrosis was detected by late gadolinium enhancement technique. Compared to never/sporadically transfused patients, regularly transfused (RT)-TI patients exhibited significantly lower global heart T2* values, biventricular end-diastolic volume indexes, left ventricular mass index, and cardiac index. In RT-TI patients, age and serum ferritin levels were the strongest predictors of global heart T2* values. Independently from the transfusional state, cardiac T2* values were not associated with biventricular function. Of the 103 (40.6%) patients in whom the contrast medium was administrated, 27 (26.2%) had replacement myocardial fibrosis. Age, sex distribution, cardiac iron, and biventricular function parameters were comparable between patients without and without replacement myocardial fibrosis. Twenty-five (9.8%) patients had a history of cardiac complications (heart failure and arrhythmias). Increased age and replacement myocardial fibrosis emerged as significant risk markers for cardiac complications. In TI, regular transfusions are associated with less pronounced cardiac remodeling but increase the risk of MIO. Replacement myocardial fibrosis is a frequent finding associated with cardiac complications.

Impact of genotype on multi-organ iron and complications in patients with non-transfusion-dependent β-thalassemia intermedia.

We evaluated the impact of the genotype on clinical and hematochemical features, hepatic and cardiac iron levels, and endocrine, hepatic, and cardiovascular complications in non-transfusion-dependent (NTD) β-thalassemia intermedia (TI) patients. Sixty patients (39.09 ± 11.11years, 29 females) consecutively enrolled in the Myocardial Iron Overload in Thalassemia project underwent Magnetic Resonance Imaging to quantify iron overload, biventricular function parameters, and atrial areas and to detect replacement myocardial fibrosis. Three groups of patients were identified: homozygous β+ (N = 18), heterozygous β0β+ (N = 22), and homozygous β0 (N = 20). The groups were homogeneous for sex, age, splenectomy, hematochemical parameters, chelation therapy, and iron levels. The homozygous β° genotype was associated with significantly higher biventricular end-diastolic and end-systolic volume indexes and bi-atrial area indexes. No difference was detected in biventricular ejection fractions or myocardial fibrosis. Extramedullary hematopoiesis and leg ulcers were significantly more frequent in the homozygous β° group compared to the homozygous β+ group. No association was detected between genotype and liver cirrhosis, hypogonadism, hypothyroidism, osteoporosis, heart failure, arrhythmias, and pulmonary hypertension. Heart remodelling related to a high cardiac output state cardiomyopathy, extramedullary hematopoiesis, and leg ulcers were more pronounced in patients with the homozygous β° genotype compared to the other genotypes analyzed. The knowledge of the genotype can assist in the clinical management of NTD β-TI patients.

Echocardiographic assessment of pediatric heart transplantation: A single-center experience in China.

Pediatric heart transplant (HT) has become the standard of care for end-stage heart failure in children worldwide. Serial echocardiographic evaluations of graft anatomy and function during follow-up are crucial for post-HT management. However, evolution of cardiac structure and function after pediatric HT has not been well described, especially during first year post-HT. This study aimed to characterize the evolution of cardiac structure and function after pediatric HT and investigate the correlation between biventricular function with adverse clinical outcomes. A single-center retrospective study of echocardiographic data obtained among 99 pediatric HT patients was conducted. Comprehensive echocardiographic examination was performed in all patients at 1-, 3-, 6-, 9- and 12-months post-HT. We obtained structural, functional and hemodynamic parameters from both left- and right-side heart, such as left ventricular stroke volume (LVSV), left ventricular ejection fraction (LVEF), right ventricular fractional area change (RVFAC), etc. The cardiac evolution of pediatric HT patients during first post-HT year was described and compared between different time points. We also explored the correlation between cardiac function and major adverse transplant events (MATEs). 1) Evolution of left heart parameters: left atrial length, mitral E velocity, E/A ratio, LVSV and LVEF significantly increased while mitral A velocity significantly decreased over the first year after HT (P<.05). Compared with 1 month after HT, interventricular septum (IVS) and left ventricular posterior wall (LVPW) decreased at 3 months but increased afterwards. (2) Evolution of right heart parameters: right ventricular base diameter and mid-diameter; right ventricular length diameter, tricuspid E velocity, E/A ratio, tricuspid annular velocity e' at free wall, and RVFAC increased, while tricuspid A velocity decreased over the first year after HT (P<.05). (3) Univariate logistic regression model suggests that biventricular function parameters at 1-year post-HT (LVEF, RVFAC, tricuspid annular plane systolic excursion and tricuspid lateral annular systolic velocity) were associated with MATEs. Gradual improvement of LV and RV function was seen in pediatric HT patients within the first year. Biventricular function parameters associated with MATEs. The results of this study pave way for designing larger and longer follow-up of this population, potentially aiming at using multiparameter echocardiographic prediction of adverse events.

The Prognostic Value of One-Year Changes in Biventricular Mechanics for Three-Year Survival in Patients with Precapillary Pulmonary Hypertension: A Cardiovascular Magnetic Resonance Feature Tracking Study.

Background and Objectives: The management of patients with pulmonary hypertension (PH) poses a considerable challenge. While baseline cardiac magnetic resonance imaging (cMRI) indices are recognized for survival prognosis in PH, the prognostic value of one-year changes in biventricular mechanics, especially as assessed using feature tracking (FT) technology, remains underexplored. This study aims to assess the predictive value of one-year change in cMRI-derived biventricular function and mechanics parameters, along with N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and six-minute walking test (6MWT) results for three-year mortality in precapillary PH patients. Materials and Methods: In this retrospective study, 36 patients diagnosed with precapillary pulmonary hypertension (mPAP 55.0 [46.3-70.5] mmHg, pulmonary capillary wedge pressure 10.0 [6.0-11.0] mmHg) were included. Baseline and one-year follow-up cMRI assessments, clinical data, and NT-proBNP levels were analyzed. FT technology was utilized to assess biventricular strain parameters. Patients were categorized into survival and non-survival groups based on three-year outcomes. Statistical analyses, including univariate logistic regression and Cox regression, were performed to identify predictive parameters. Results: The observed three-year survival rate was 83.3%. Baseline right ventricle (RV) ejection fraction (EF) was significantly higher in the survival group compared to non-survivors (41.0 [33.75-47.25]% vs. 28.0 [23.5-36.3]%, p = 0.044), and values of ≤32.5% were linked to a 20-fold increase in mortality risk. RV septum longitudinal strain (LS) and RV global LS exhibited significant improvement over a one-year period in the survival group compared to the non-survival group (-1.2 [-6.4-1.6]% vs. 4.9 [1.5-6.7]%, p = 0.038 and -3.1 [-9.1-2.6]% vs. 4.5 [-2.1-8.5]%, p = 0.048, respectively). Declines in RV septum LS by ≥2.95% and in RV GLS by ≥3.60% were associated with a 25-fold and 8-fold increase in mortality risk, respectively. Conclusions: The decrease in right ventricular septal and global longitudinal strain over a one-year period demonstrates a significant predictive value and an association with an increased three-year mortality risk in patients with precapillary PH.

Low Vitamin D Levels Are Associated with Increased Cardiac Iron Uptake in Beta-Thalassemia Major.

We evaluated the association of vitamin D and parathormone (PTH) levels with cardiac iron and function in beta-thalassemia major (β-TM) patients. Two-hundred and seventy-eight TM patients (39.04 ± 8.58 years, 56.8% females) underwent magnetic resonance imaging for the assessment of iron overload (T2* technique), biventricular function parameters (cine images), and replacement myocardial fibrosis (late gadolinium enhancement technique). Vitamin D levels were deficient (<20 ng/dL) in 107 (38.5%) patients, insufficient (20-30 ng/dL) in 96 (34.5%) patients, and sufficient (≥30 ng/dL) in 75 (27.0%) patients. Deficient vitamin D patients had a significantly higher frequency of myocardial iron overload (MIO; global heart T2* < 20 ms) than patients with sufficient and insufficient vitamin D levels and a significantly higher left ventricular end-diastolic volume index and mass index than patients with sufficient vitamin D levels. PTH was not associated with cardiac iron, function, or fibrosis. In the multivariate regression analysis, vitamin D, serum ferritin, and pancreatic iron levels were the strongest predictors of global heart T2* values. In receiver operating characteristic curve analysis, a vitamin D level ≤ 17.3 ng/dL predicted MIO with a sensitivity of 81.5% and a specificity of 75.3% (p < 0.0001). In TM, the periodic and regular assessment of vitamin D levels can be beneficial for the prevention of cardiac iron accumulation and subsequent overt dysfunction.

Abstract 15401: Artificial Intelligence-Enabled Exercise-CMR for Evaluation of Cardiac Functional Reserve in Heart Failure

Introduction: Exercise unmasks pathophysiological markers in heart failure (HF), not evident at rest. Exercise CMR (Ex-CMR) has the potential to offer an accurate and reproducible assessment of cardiac functional reserve in HF. Objective: To study the cardiac functional reserve in HF using an artificial intelligence (AI) enabled exercise CMR imaging protocol. Method: We prospectively recruited patients with an established clinical diagnosis of HF and healthy adults. Subjects were imaged at rest and immediately post-exercise using a supine ergometer positioned on the CMR scan. The imaging protocol included free-breathing, real-time, highly accelerated cine short-axis images covering the entire LV. Cine images were collected with a spatial resolution of 1.9 х 1.9 mm 2 and temporal resolution of 38.8 ms using a 14.8-fold acceleration using an in-house inline deep learning reconstruction. Pre- and post-exercise biventricular volumetric and functional parameters and their corresponding percentage changes were calculated. Results: The study included 77 subjects, consisting of 37 HF patients (24 males; 60 ± 12 years) [HFpEF, n=13; HFmrEF, n=5; HFimpEF, n=13; and HFrEF, n=6], and 40 healthy adult (20 males; 45 ± 16 years). Changes in LV end-diastolic and end-systolic volume among subgroups revealed distinct clusters (Fig 1). These findings corresponded to a blunted to a modest rise in stroke volume (SV) observed in HFpEF and HFmrEF patients, respectively, while HFimpEF and HFrEF patients exhibited a mildly to excessively higher SV rise compared to other subgroups (Fig 1). HFpEF and HFmrEF showed no improvement in SV index beyond 10 ml/m 2 . Patients with known post capillary pulmonary hypertension demonstrated an inadequate right ventricle response in preload recruitment, contributing to a blunted increase in RV SV (Fig 1). Conclusion: Our AI-enabled, high-accelerated Ex-CMR imaging protocol reveals a distinctive biventricular functional reserve response in HF.

From Compressed-Sensing to Deep Learning MR: Comparative Biventricular Cardiac Function Analysis in a Patient Cohort.

Conventional segmented, retrospectively gated cine (Conv-cine) is challenged in patients with breath-hold difficulties. Compressed sensing (CS) has shown values in cine imaging but generally requires long reconstruction time. Recent artificial intelligence (AI) has demonstrated potential in fast cine imaging. To compare CS-cine and AI-cine with Conv-cine in quantitative biventricular functions, image quality, and reconstruction time. Prospective human studies. 70 patients (age, 39 ± 15 years, 54.3% male). 3T; balanced steady state free precession gradient echo sequences. Biventricular functional parameters of CS-, AI-, and Conv-cine were measured by two radiologists independently and compared. The scan and reconstruction time were recorded. Subjective scores of image quality were compared by three radiologists. Paired t-test and two related-samples Wilcoxon sign test were used to compare biventricular functional parameters between CS-, AI-, and Conv-cine. Intraclass correlation coefficient (ICC), Bland-Altman analysis, and Kendall's W method were applied to evaluate agreement of biventricular functional parameters and image quality of these three sequences. A P-value <0.05 was considered statistically significant, and standardized mean difference (SMD) < 0. 100 was considered no significant difference. Compared to Conv-cine, no statistically significant differences were identified in CS- and AI-cine function results (all P > 0.05), except for very small differences in left ventricle end-diastole volumes of 2.5 mL (SMD = 0.082) and 4.1 mL (SMD = 0.096), respectively. Bland-Altman scatter plots revealed that biventricular function results were mostly distributed within the 95% confidence interval. All parameters had acceptable to excellent interobserver agreements (ICC: 0.748-0.989). Compared with Conv-cine (84 ± 13 sec), both CS (14 ± 2 sec) and AI (15 ± 2 sec) techniques reduced scan time. Compared with CS-cine (304 ± 17 sec), AI-cine (24 ± 4 sec) reduced reconstruction time. CS-cine demonstrated significantly lower quality scores than Conv-cine, while AI-cine demonstrated similar scores (P = 0.634). CS- and AI-cine can achieve whole-heart cardiac cine imaging in a single breath-hold. Both CS- and AI-cine have the potential to supplement the gold standard Conv-cine in studying biventricular functions and benefit patients having difficulties with breath-holds. 1 TECHNICAL EFFICACY STAGE: 1.

Surgical Outcomes of Repairing Ebstein Anomaly: A Single-Center Experience

Background: Ebstein anomaly (EA) is a rare congenital disorder of the tricuspid valve (TV), accounting for about one percent of congenital heart defects. Considering highly variable anatomy, diverse corrective surgical methods are currently utilized. Cone reconstruction provides a near anatomic tricuspid restoration method that has triggered enthusiasm for early repair over recent years. Objectives: In the current study, we aimed to evaluate the clinical and echocardiographic features and outcomes of patients who underwent surgical correction of EA in our referral center. Methods: In this study, we reviewed the medical records of 35 patients with EA who underwent cone repair or TV replacement and had accessible echocardiographic data before and at least one year following the corrective surgery. The patients were evaluated for residual tricuspid regurgitation (TR) severity, right ventricular (RV) size, RV fractional area change (FAC), left ventricular end-systolic diameter (LVESD), left ventricular end-diastolic diameter (LVEDD), and 2D left ventricular ejection fraction (LVEF) before and after the operation. Results: Mean age of the patients was 29.49 ± 9.13 years, and 54.3% of them were female. Twenty-one (60%) patients underwent cone repair, and 14 were subjected to TV replacement. Seven (20%) patients required reoperation; redo TV repair was conducted in three patients (8.6%); two (5.7%) patients underwent biologic TV replacement; mechanical TV replacement was performed in one patient (2.9%) who had degenerative biologic TVs, and one patient (2.9%) was subjected to redo-operation due to mechanical TV malfunction. Positive changes in the means of RV-FAC and 2D-LVEF were statistically significant in all patients irrespective of the type of surgery (P &lt; 0.001), but changes in the means of RV size (P = 0.38) and LVESD (P = 0.302) were not statistically significant. There was a significant increase in functional RV size in both groups of patients who underwent repair (P = 0.063) or replacement (P = 0.02). Conclusions: The present study revealed satisfactory post-op results for cone TV repair in patients with EA, evidenced by reduced TR severity and improved biventricular functional parameters. Assessing the durability of the outcomes of cone TV repair needs longer-term follow-ups.

Decreased ventricular systolic function in chemotherapy-naive patients with acute myeloid leukemia: a three-dimensional speckle-tracking echocardiography study.

The relationship between acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL) and cardiac function is not well established. This study aimed to evaluate whether AML patients exist early myocardial damages prior to chemotherapy and to investigate its association with cardiovascular biomarkers. Conventional echocardiography and three-dimensional speckle-tracking strain analysis were performed prospectively in 72 acute leukemia (AL) patients before any chemotherapy therapy (of whom 44 were AML patients, 28 ALL patients). The results were compared with those from 58 control group matched for age and gender. There were no significant differences in conventional biventricular systolic function parameters between AL patients and controls. The left ventricular global longitudinal strain (LVGLS) and right ventricular free wall longitudinal strain (RVFWLS) were significantly lower in AL patients (-23.0 ± 1.4% vs. -24.1 ± 1.3% and -27.9 ± 7.1% vs. -33.0 ± 4.6%, respectively, P < 0.001 for all). Compared with ALL patients, AML patients had lower LVGLS and RVFWLS (-22.7 ± 1.3% vs. -23.5 ± 1.6% and -26.2 ± 7.6% vs. -30.4 ± 5.5%, respectively, P < 0.05 for all). LVGLS was lower in ALL patients compared with controls (-23.5 ± 1.6% vs. -24.7 ± 1.4%, P < 0.05), however, there was no difference in right ventricular systolic function parameters between the two groups. LVGLS in AL patients was independently correlated with left ventricular ejection fraction (LVEF) and the absolute number of circulating lymphocytes. Our findings suggest that baseline myocardial systolic function is lower in AL patients than controls. AML patients had lower baseline LVGLS and RVFWLS than controls and ALL patients. The decreased LVGLS is correlated with LVEF and the absolute number of circulating lymphocytes.

Prognostic Significance of Cardiac Magnetic Resonance in Left Atrial and Biventricular Strain Analysis during the Follow-Up of Suspected Myocarditis.

To assess the variation in left atrial (LA) and biventricular strain and its prognostic value in the course of suspected myocarditis, this retrospective study included 55 patients with clinically suspected myocarditis who underwent cardiac magnetic resonance (CMR) examinations at baseline and follow-up periods. Cine images were used for feature tracking analysis. Paired Student's t test, McNemar's test, and Cox proportional hazard regression were used for statistical analysis. The LA total emptying fraction was the only functional index that showed a statistically significant improvement. The initial LA peak's late negative strain rate (SRa) was the only parameter with a significant predictive power of major adverse cardiac events under univariable (hazard ratio [HR] 2.396, 95% confidence interval [CI] 1.044-5.498, p = 0.039) and multivariable Cox survival analysis when adjusted by LA strain parameters (HR 5.072, 95% CI 1.478-17.404, p = 0.010), LA strain and functional parameters (HR 7.197, 95% CI 1.679-30.846, p = 0.008), and LA and biventricular strain and functional parameters (HR 10.389, 95% CI 2.250-47.977, p = 0.003). Thus, our findings indicate that CMR strain is useful for monitoring LA and ventricular function in suspected myocarditis, that LA function may recover preceding ventricular function changes, and that LA strain may serve as an incremental tool to predict adverse outcomes.

Prognostic value of multiparametric cardiac magnetic resonance in sickle cell patients.

The aim of this multicenter study was to prospectively assess the predictive value of multiparametric cardiac magnetic resonance (CMR) for cardiovascular complications in sickle cell disease (SCD) patients. Among all patients with hemoglobinopathies consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network, we selected 102 SCD patients (34.38 ± 12.67years, 49 females). Myocardial iron overload (MIO) was measured by the multislice multiecho T2* technique. Atrial dimensions and biventricular function parameters were quantified by cine images. Late gadolinium enhancement (LGE) images were acquired to detect focal myocardial fibrosis. At baseline CMR, only two patients had significant MIO (global heart T2* < 20ms). During a mean follow-up of 63.01 ± 24.95months, 11 cardiovascular events (10.8%) were registered: 3 pulmonary hypertension, 2 supraventricular arrhythmias, 1 heart failure, 1 death for heart failure, 1 pulmonary embolism, 1 peripheral vascular disease, 1 transient ischemic attack, and 1 death after acute chest syndrome. In the multivariate analysis, the independent CMR predictors of cardiovascular events were left ventricular (LV) ejection fraction (hazard ratio-HR = 0.88; p = 0.025) and right ventricular (RV) mass index (HR = 1.09; p = 0.047). According to the receiver-operating characteristic curve analysis for adverse events, an LV ejection fraction < 58.9% and an RV mass index > 31g/m2 were optimal cut-off values. Reduced left ventricular ejection fraction and increased right ventricular mass index showed a significant prognostic value in patients with SCD. Our data seem to suggest that CMR may be added as a screening tool for identifying SCD patients at high risk for cardiopulmonary and vascular diseases.

Comprehensive Evaluation of Left Ventricle Dysfunction by a New Computed Tomography Scanner: The E-PLURIBUS Study

BackgroundAlthough cardiac magnetic resonance (CMR) is considered the gold standard for myocardial fibrosis detection, cardiac computed tomography (CCT) is emerging as a promising alternative. ObjectivesThe purpose of this study was to assess feasibility and diagnostic accuracy of a comprehensive functional and anatomical evaluation with CCT as compared with CMR in patients with newly diagnosed left ventricular dysfunction (LVD). MethodsA total of 128 consecutive patients with newly diagnosed LVD were screened. Based on the exclusion criteria, 28 cases were excluded. CCT was performed within 10 days from CMR. Biventricular volumes and ejection fraction, and presence and pattern of delayed enhancement (DE), were determined, along with evaluation of coronary arteries among patients undergoing invasive angiography in the 6 months after CCT. ResultsSix cases were excluded because of claustrophobia at CMR. Among the 94 patients who formed the study population, the concordance between CCT and CMR in suggesting the cause of the LVD was high (94.7%, 89/94 patients) in the overall population and was 100% for identifying ischemic cardiomyopathy. The CCT diagnostic rate for DE assessment was also high (96.7%, 1,544/1,598 territories) and similar to that of CMR (97.4%; P = 0.345, CCT vs CMR). Moreover, CCT showed high diagnostic accuracy in the detection of DE (94.8%, 95% CI: 93.6%-95.8%) in a territory-based analysis. Biventricular volumes and function parameters as measured by CCT and CMR were similar, without significant differences with the exception of a modest difference in RV volume. CCT was confirmed to be accurate for assessing arterial coronary circulation. The mean radiation exposure of the whole CCT was 7.78 ± 2.53 mSv (0.84 ± 0.24 mSv for DE). ConclusionsCCT performed with low-dose whole-heart coverage scanner and high-concentration contrast agent appears an effective noninvasive tool for a comprehensive assessment of patients with newly diagnosed LVD.

End-Systolic Eccentricity Index Obtained by Enhanced Computed Tomography Is a Predictor of Pulmonary Vascular Resistance in Patients with Chronic Thromboembolic Pulmonary Hypertension.

The usefulness of the parameters of biventricular function simultaneously measured using enhanced multi-detector computed tomography (MDCT) pulmonary angiography in patients with chronic thromboembolic pulmonary hypertension (CTEPH) has not been clarified. This study aimed to verify the correlation between left and right ventricular (RV) parameters and pulmonary vascular resistance (PVR). Patients who underwent enhanced MDCT before diagnostic right heart catheterization at Nagoya University Hospital between October 2014 and April 2021 were enrolled. The correlation of biventricular function and volume parameters with PVR was assessed. Eighty patients were retrospectively analyzed. Patients’ mean age was 65 ± 13 years, mean PVR was 9.1 (range, 6.1–11.3) Wood units, and mean end-systolic eccentricity index (esEI) was 1.76 ± 0.50. RV end-systolic volume (ESV) (p = 0.007), RV cardiac output (CO) (p < 0.001), RV ejection fraction (p < 0.001), LV end-diastolic volume (EDV) (p < 0.001), left ventricular (LV) ESV (p = 0.006), LVCO (p < 0.001), end-diastolic EI (p < 0.001), and esEI (p < 0.001) were significantly correlated with PVR. The LVEDV (p = 0.001) and esEI (p < 0.009) were independent predictors of PVR. Systolic pulmonary arterial pressure (PAP) (p < 0.001), diastolic PAP (p < 0.001), mean PAP (p < 0.001), right atrial pressure (p < 0.023), and PVR (p < 0.001) were significantly higher in the high esEI group than in the low esEI group. The esEI was a simple predictor of CTEPH severity.

Biventricular Myocardial Strain Analysis in Patients with Pulmonary Arterial Hypertension Using Cardiac Magnetic Resonance Tissue-Tracking Technology.

: ObjectivesTo evaluate both left and right ventricular (LV and RV) function in patients with pulmonary arterial hypertension (PAH) using cardiac magnetic resonance tissue-tracking (CMR-TT) technology and explore its clinical value. Methods: A total of 79 participants (including 47 patients with PAH and 32 healthy controls) underwent cardiac magnetic resonance imaging (CMRI) with a short-axis balanced steady-state free precession (SSFP) sequence. The biventricular cardiac function parameters and strain parameters were obtained by postprocessing with CVI42 software. A comparative analysis was performed between the LV and RV strain parameters in all PAH patients and in PAH patients with reduced or preserved cardiac function. Results: The results showed preferable repeatability of CMR-TT in analyzing the global radial strain (GRS), circumferential strain (GCS), and longitudinal strain (GLS) of the left and right ventricles in the PAH group. The GRS, GCS, and GLS of the left and right ventricles except for LV GRS (LVGRS) of PAH patients were significantly lower than those of healthy controls (p < 0.05 for all). The GRS and GCS of the left and right ventricles showed a moderate correlation in the PAH group (r = 0.323, p = 0.02; r = 0.301, p = 0.04, respectively). PAH patients with preserved RV function (n = 9) showed significantly decreased global and segmental RS, CS, and LS of the right ventricles than healthy controls (p < 0.05 for all), except for basal RVGCS (RVGCS-b, p = 0.996). Only the LVGLS was significantly different between the PAH patients with preserved LV function (n = 32) and the healthy controls (−14.23 ± 3.01% vs. −16.79 ± 2.86%, p < 0.01). Conclusions: As a nonradioactive and noninvasive technique, CMR-TT has preferable feasibility and repeatability in quantitatively evaluating LV and RV strain parameters in PAH patients and can be used to effectively detect early biventricular myocardial damage in patients with PAH.

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

BackgroundCoronary 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. MethodsEighty-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. ResultsAge 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 (β-estimate:-3.240,95%CI:-4.634;-1.846, p < 0.001), GLS 3D (β-estimate:-2.559,95%CI:-3.932;-1.186, p < 0.001) and area strain (β-estimate:-3.044,95%CI:-5.373;-0.716, p = 0.011). Lower values of CFVR PD related to worse global work index (β-estimate:267.824,95%CI:75.964;459.683, p = 0.007), global constructive work (β-estimate:217.300,95%CI:38.750;395.850, p = 0.018) and global work efficiency (β-estimate:5.656,95%CI:2.229;9.084, p = 0.002). Impaired CFVR LAD (β-estimate:2.826, 95%CI:0.913;4.739, p = 0.004) and CFVR PD (β-estimate:2.801,95%CI:0.657;4.945, p = 0.011) were associated with lower TAPSE. Lower values of CFVR LAD (β-estimate:2.580, 95%CI:0.169;4.991, p = 0.036) and CFVR PD (β-estimate:3.163, 95%CI: 0.721;5.606, p = 0.012) were associated with worse peak VO2. ConclusionLower CFVR was associated with impairment in biventricular systolic function parameters and functional capacity assessed by pVO2.

Link between Genotype and Multi-Organ Iron and Complications in Children with Transfusion-Dependent Thalassemia.

We evaluated the impact of the genotype on hepatic, pancreatic and myocardial iron content, and on hepatic, cardiac and endocrine complications in children with transfusion-dependent β-thalassemia (β-TDT). We considered 68 β-TDT patients (11.98 ± 3.67 years, 51.5% females) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network. Iron overload was quantified by T2* technique and biventricular function by cine images. Replacement myocardial fibrosis was evaluated by late gadolinium enhancement technique. Three groups of patients were identified: homozygous β+ (N = 19), compound heterozygous β0β+ (N = 24), and homozygous β0 (N = 25). The homozygous β0 group showed significantly lower global heart and pancreas T2* values than the homozygous β+ group. Compared to patients with homozygous β+ genotype, β0β+ as well as β0β0 patients were more likely to have pancreatic iron overload (odds ratio = 6.53 and 10.08, respectively). No difference was detected in biventricular function parameters and frequency of replacement fibrosis. No patient had cirrhosis/fibrosis, diabetes or heart failure, and the frequency of endocrinopathies was comparable among the groups. In pediatric β-TDT patients, there is an association between genotype and cardiac and pancreatic iron overload. The knowledge of patients’ genotype can be valuable in predicting some patients’ phenotypic features and in helping the clinical management of β-TDT patients.

Biventricular mechanical pattern of the athlete"s heart: comprehensive characterization using 3D echocardiography

Abstract Funding Acknowledgements Type of funding sources: None. Regular, intense exercise results in complex morphological and functional cardiac remodeling, commonly referred to as the athlete"s heart. While left ventricular (LV) adaptation is thoroughly studied, data are scarce concerning the right ventricular (RV) mechanical changes and their continuum with exercise performance. Accordingly, our aim was to characterize biventricular morphology and function and its relation to sex, age and sport classes in a large cohort of elite athletes using 3D echocardiography. Four hundred and twenty-two elite, competitive athletes (male/female: 295/127, adult/adolescent: 207/215) from the 4 major sport classes (mixed type n = 293; endurance n = 88; power n = 33; skill n = 8) and healthy, sedentary volunteers (n = 55) were enrolled. 3D transthoracic echocardiographic datasets were acquired to quantify LV and RV end-diastolic volumes (EDVi), and ejection fractions (EF). In order to characterize biventricular mechanical parameters, LV and RV global longitudinal (GLS) and global circumferential strains (GCS) were measured using dedicated softwares. Additionally, all subjects underwent cardiopulmonary exercise testing in order to determine peak oxygen uptake (VO2/kg). Athletes had significantly higher LV and RV EDVi compared with controls, whereas male athletes had larger volumes than female athletes, and adult athletes had also larger LV EDVi than adolescent athletes (all p &amp;lt; 0.05). However, RV EDVi was similar between the two age groups. Endurance athletes had significantly larger RV EDVi compared with the other sport classes (ANOVA p &amp;lt; 0.05). Concerning biventricular functional parameters, athletes had significantly lower resting LV and RV EF (athletes vs. controls; LVEF: 57 ± 4 vs. 61 ± 5%; RVEF: 55 ± 5 vs. 59 ± 5%; p &amp;lt; 0.001) as well as LV GLS (-19.2 ± 2.3 vs. -21.2 ± 2.0%), LV GCS (-27.7 ± 3.0 vs. -31.0 ± 3.5%), and RV GCS (-20.9 ± 4.4 vs. -24.5 ± 4.5%; all p &amp;lt; 0.001) compared with controls. In contrast, RV GLS (-21.8 ± 3.4 vs. -22.2 ± 3.6%) did not differ between athletes and controls. The exercise-induced relative decrease in LV GLS (9.5 ± 10.7%) and LV GCS (10.7 ± 9.8%) was similar, however, the decrement in RV GCS (14.8 ± 17.8%) was disproportionately larger compared with RV GLS (1.7 ± 15.4%, p &amp;lt; 0.01) in the athlete population. By multivariable linear regression analysis among echocardiographic parameters using ordinary least squares, RVEDVi was found to be the strongest and independent predictor of VO2/kg, followed by RV GCS and LV EDVi. Regular physical exercise results in significant and specific changes in LV and RV geometry and function. Resting LV mechanics of the athlete"s heart is characterized by a balanced decrement in GLS and GCS, however, in the RV the circumferential shortening decreases disproportionately compared with the longitudinal shortening. Moreover, this mechanical pattern is associated with better exercise capacity, which emphasizes the importance of the RV in determining athletic performance.

Normal CMR Bi-Atrial and Biventricular Reference Values in Sickle Cell Disease Patients without Heart Damage

Background. Cardiac function indices in patients with hemoglobinopathies are different from those in healthy population, mainly due to chronic anemia. Normal reference values specific for SCD patients are not available by CMR.Aim. We aimed to define the normal cut-off value in SCD patients for bi-atrial and biventricular cardiac magnetic resonance (CMR) parameters.Methods. We considered forty-eight adult SCD patients with no known risk factors or cardiac disease, normal electrocardiogram, no macroscopic myocardial fibrosis, and all cardiac segments with T2*≥20 ms, consecutively enrolled in the MIOT network (Myocardial iron overload in thalassemia). SCD patients were compared with ninety-six healthy controls and 96 thalassemia major (TM) patients without cardiac damage, both matched for age and gender. Nine pediatric SCD patients were also analysed in comparison with 9 TM patients and 9 healthy subjects matched for age and gender. Cine images were acquired to quantify biventricular function parameters: LV and RV end-diastolic volume (EDV), end-systolic volume (ESV) and stroke volume (SV) were normalized for body surface area (EDVI, ESVI, SVI), as well as biventricular mass and atrial areas. Myocardial iron overload was assessed by segmental T2* technique. Late gadolinium enhancement (LGE) images were acquired for evaluation of macroscopic myocardial fibrosis.Results. In all three groups males showed higher biventricular volumes and mass indexes than females. SCD male patients had significantly higher LVEDVI (p<0.0001), LVESVI (p=0.010), LVSVI (p=0.003), cardiac index (p=0.002), LV and RV mass index (p=0.008 and p=0.001, respectively) and left and right atrial areas (p<0.001 and p=0.011) than healthy subjects. No significant differences were found in RVEDVI, EVESVI and biventricular EF. Compared to healthy volunteers, females with SCD showed a larger LVEDVI (p=0.020), LVSVI (p=0.039), RV mass index (p=0.002) and left atrial area (p=0.008).SCD and TM patients showed comparable values of bi-atrial and biventricular volumes and function. When compared to TM, SCD patients showed a larger LV (p<0.001) and RV mass index (p=0.001) in male group and a larger RV mass index (p=0.001) in female group. Table 1 shows the cut-offs for bi-atrial and biventricular MR parameters for adult SCD patients by gender.No significant differences in MR parameters were found among the pediatric groups.Conclusions. Normal reference ranges of bi-atrial and biventricular MR parameters for adult males and females SCD patients were established. The use of these reference values will prevent possible misdiagnosis of cardiomyopathy in patients with SCD. [Display omitted] DisclosuresPepe: Chiesi Farmaceutici S.p.A: Other: no profit support; Bayer S.p.A.: Other: no profit support. Quarta: Sanofi - Genzyme: Membership on an entity's Board of Directors or advisory committees, Other: collaboration relationships for Advisory boards, Webinar events, editorial projects; Speaker at conferences; Blue Bird Bio: Other: collaboration relationships for Advisory boards, Webinar events, editorial projects; Celgene: Other: collaboration relationships for Advisory boards, Webinar events, editorial projects; Novartis: Membership on an entity's Board of Directors or advisory committees, Other: collaboration relationships for Advisory boards, Webinar events, editorial projects; Speaker at conferences; Takeda: Other: collaboration relationships for Advisory boards, Webinar events, editorial projects; speaker at conferences; Bristol Meyer Squibb: Membership on an entity's Board of Directors or advisory committees, Other: Speaker at conferences. Maggio: Bluebird Bio: Membership on an entity's Board of Directors or advisory committees; Celgene Corp: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees.

Correlation Between Quantitative Uptake of 99mTC-DPD and Echocardiographic Parameters in Cardiac ATTR: A Novel Follow-Up Strategy.

Aims: There has been a paradigm shift in diagnosis of cardiac transthyretin amyloidosis (ATTR) with non-invasive techniques including technetium-99m 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) bone scintigraphy. We evaluated structural and functional biventricular alterations by transthoracic echocardiography (TTE) and determined the correlation with 99mTc-DPD tracer uptake in ATTR.Materials and Methods: ATTR patients (wild-type, hereditary or asymptomatic transthyretin [TTR] variant carriers) with 99mTc-DPD and TTE were selected; 99mTc-DPD uptake was analyzed quantitatively. TTE assessment of left ventricle (LV) and right ventricle (RV) parameters was performed.Results: Forty ATTR patients (wild-type n = 17; hereditary ATTR and TTR variant carriers n = 23; median age 68.8 ± 22 years) were included. TTE parameters displaying good correlation with 99mTc-DPD tracer uptake included LV average wall thickness (r = 0.837), LV indexed mass (LVMI; r = 0.802), RV wall thickness (r = 0.610), average e' (r = −0.830), E/e' ratio (r = 0.786), LV global longitudinal strain (GLS; r = 0.714) and RV GLS (r = 0.632; p < 0.001 for all). Hereditary ATTR and TTR variant carriers without cardiac tracer uptake had normal echocardiographic parameters. Receiver operating characteristic curves demonstrated strong diagnostic accuracies for structural (LV wall thickness, LVMI and RV wall thickness; area under the curve (AUC) of 0.96 for all) and functional (LV and RV GLS; AUC of 0.86 and 0.88, respectively) parameters.Conclusion: Good correlations between TTE biventricular structural and functional parameters were demonstrated with quantitative 99mTc-DPD uptake. Echocardiography may potentially assume a significant role in longitudinal follow-up for monitoring disease progression and for evaluating treatment response.

Cardiovascular Magnetic Resonance in Peripartum Cardiomyopathy: Comparison with Idiopathic Dilated Cardiomyopathy.

Background: Peripartum (PPCM) and dilated (DCM) cardiomyopathies are distinct forms of cardiac disease that share certain aspects in clinical presentation. Aim: We hypothesized that different cardiac structural changes underlie PPCM and DCM, and we aimed to investigate them with cardiovascular magnetic resonance (CMR). Methods: We included 21 PPCM patients (30.5 ± 5.9 years) and 30 female DCM patients (41.5 ± 16.8 years) matched for left ventricular ejection fraction. Biventricular and biatrial volumetric and functional parameters were assessed along with ventricular and atrial strain indices based on feature-tracking techniques. The presence of late gadolinium enhancement (LGE) was also assessed. Results: In PPCM, the left ventricular (LV) stroke volume index was lower (p = 0.04), right atrial (RA) minimal and pre-systolic volumes were higher (p < 0.01 and p = 0.02, respectively), and the total RA ejection fraction was lower (p = 0.02) in comparison to DCM. Moreover, in PPCM, the LV global longitudinal strain (p = 0.03), global circumferential strain rate (p = 0.04), and global longitudinal strain rate (p < 0.01) were less impaired than in DCM. Both PPCM and DCM patients with LGE had more dilated ventricles and more impaired LV and left atrial function than in PPCM and DCM patients without LGE. Conclusions: Subtle differences appear on CMR between PPCM and DCM. Most importantly, the RA is larger and more impaired, and LV global longitudinal strain is less reduced in PPCM than in DCM. Furthermore, similarly to DCM, PPCM patients with LGE have more dilated and impaired ventricles than patients without LGE.