In this report, we present a rare case of severe mitral regurgitation due to isolated mitral valve chordal rupture without valve leaflet prolapse in a patient with Fabry cardiomyopathy. This finding could be due to subvalvular apparatus storage of glycosphingolipids rather than fibro-elastic deficiency, underlying how close cardiological follow-up of Fabry patients must be comprehensive and not only focused on left ventricular hypertrophy and arrhythmias. We feel that our finding could be of interest for the readership of your Journal.

Transthoracic and transesophageal imaging of the 30-year-old patient who presented with palpitations and shortness of breath revealed flow from the aorta to the right atrium. Aortic-right atrial fistula, which is a rare anomaly, was evaluated. It was considered congenital, as there was no acquired cause. Since the patient was symptomatic, it was closed percutaneously with the Amplatzer Duct Occluder II device.

Echocardiography is commonly utilized in patients with acute respiratory distress syndrome (ARDS) for assessment of cardiac function, volume status, and the potential development of acute cor pulmonale. In severe ARDS, prone positioning is frequently used, which imposes technical challenges during transthoracic echocardiography (TTE) image acquisition. Moreover, prone positioning can affect cardiopulmonary function in ways that are reflected on the echocardiographic findings in this position. Historically, a transesophageal approach was recommended when a patient is prone, with few studies reporting utility of TTE in this setting. However, recent publications have begun to address this knowledge gap. This review explores recent literature addressing the use of TTE in prone patients with ARDS, with a special focus on the cardiopulmonary effects of proning and potential solutions to the technical difficulties that arise in this position.

Background: Early cardiovascular impairment in obstructive sleep apnea (OSA) patients is often overlooked, leading to irreversible outcome. Left ventricular (LV) global longitudinal strain (GLS) derived from automated function imaging (AFI) echocardiography provides a fast tool to assess global longitudinal function. We therefore aimed to compare the feasibility and reproducibility of AFI with mitral annulus plane systolic excursion (MAPSE) as obesity is common in OSA. Methods: A comprehensive echocardiographic examination was done in 186 consecutive patients having polysomnography for suspected OSA in this prospective study. MAPSE was measured by using M-mode. AFI was derived by offline analysis of three long-axis views that semi-automatically detects LV endocardial boundary, which is adjusted manually as necessary. Variability of AFI and MAPSE were compared among the different subgroups and further tested in BMI subgroups. Results: Despite a relatively high obesity rate (42.9%), AFI was feasible in 94% (175/186) patients and MAPSE could be recorded in all patients. Although more segments were measured with AFI it showed excellent correlation (r=0.882) superior to MAPSE (r=0.819) between the expert and beginner. Intra- and inter- observer variability of AFI were comparable with MAPSE in Bland-Altman analysis, 5.5% and 6.5% for AFI, 6.2% and 8.8% for MAPSE, respectively. In repeated measurements, AFI showed higher intra-class correlation (ICC=0.95) than MAPSE (ICC=0.87). Furthermore, analysis showed that AFI was feasible even in more obese patients (BMI≥28kg/m2). Conclusions: Even in obese patients with OSA, AFI-GLS is feasible and more reliable for less expert operators than MAPSE for detecting LV longitudinal dysfunction.

Background: Because transcatheter closure of patent foramen ovale (PFO) has become effective for preventing cryptogenic stroke (CS), it is necessary to determine high-risk PFO associated with CS. This study aimed to clarify the importance of direct right-to-left (RL) shunt through the PFO for identifying high-risk PFO. Methods: We analyzed 137 patients with and without CS who were confirmed to have PFO. The timing of RL shunt through the PFO was evaluated by cardiac cycles after right atrium (RA) opacification on saline contrast transesophageal echocardiography. Direct RL shunt was defined as microbubbles crossing the PFO before and at the same time of RA opacification. Results: Cardiac cycles of microbubbles crossing the PFO were shorter in patients with CS than in those without CS (2.0 ± 2.2 vs. 0.5 ± 1.1, P < .01). Direct RL shunt was more frequently observed in patients with CS than in those without CS (77% vs. 29%, P < .01), with a sensitivity of 79% and a specificity of 71% for the association with CS. Multivariate analysis revealed that direct RL shunt was related to atrial septal aneurysm and low-angle PFO. Regarding functional features of PFO, the detection rate of CS was 50% for large RL shunt alone, and was increased to 83% when direct RL shunt was added. Conclusion: Direct RL shunt was associated with CS and had the incremental value in detecting PFO associated with CS for large RL shunt. The timing of RL shunt can be valuable for identifying high-risk PFO.

Background: Myocarditis have variable clinical presentation, evolution and prognosis. Aim of our study was to evaluate the value of speckle tracking echocardiography and cardiac magnetic resonance (CMR) in the prediction of ventricular arrhythmias and other cardiovascular adverse events in patients with acute myocarditis, at hospital admission. Methods: 70 patients (62 M, 8 F; mean age 31,3±13,2) with myocarditis and preserved left ventricle ejection fraction (LVEF) were enrolled. Electrocardiogram (ECG), continuous ECG monitoring, echocardiography with measurement of global longitudinal strain of the left ventricle (GLS), mechanical dispersion (MD) and CMR with quantitative measurement of delayed enhancement (DE) were performed. Adverse events were assessed (arrhythmias, heart failure, cardiogenic shock, syncope) during in-hospital stay. Results: We found a significant greater amount of DE mass in patients with cardiac arrhythmias (p = 0,01), but not of edema (p = 0,57). GLS was significantly impaired in patients with ventricular arrhythmias (p = 0,04), conversely MD was not significantly prolonged in this setting (p = 0,16). GLS > - 19.2% (sens 100%, specif 55,7%) and a DE mass >9,9 gr (sens 100%; specif. 58,6%) had the best sensitivity and specificity to identify patients with cardiac arrhythmias. Compared to GLS, DE mass showed a stronger association with ventricular arrhythmias (p < 0,001). Conclusions: in our study DE mass and GLS were associated with ventricular arrhythmias in patients with acute myocarditis and preserved LVEF. DE showed the stronger association with the occurrence of ventricular arrhythmias.

Spontaneous pseudoaneurysm of the aortic arch is an exceptionally rare and potentially life-threatening condition.Diagnosis of pseudoaneurysms of the aortic arch mainly depends on imaging examinations.Contrast-enhanced echocardiography, as a non-invasive, bed-side and radiation-free tool, plays an important role in the rapid diagnosis and postoperative follow-up of pseudoaneurysms of the aortic arch.

Rheumatic heart disease is the most common cause of mitral valve stenosis. Left atrial appendage thrombus is associated with mitral stenosis, but in rare cases the thrombus can extend to the left atrial cavity. We present a case of a severe rheumatic mitral stenosis and associated large left atrial thrombus, with embolic sequelae.

Double Inlet Left Ventricle through a common AV valve is a rare type of functionally univentricular heart. We report a fetus with double inlet left ventricle with discordant ventriculo-arterial connection and pulmonary atresia. The anatomy was readily apparent on three dimensional rendering by spatio-temporal imaging correlation technique(STIC)

Background: Concern exists regarding adequacy of visualization of stress echocardiograms performed without intravenous contrast in persons with Class III obesity (body mass index ≥ 40 kg/m2). Methods: Dobutamine stress echocardiography (DSE) was performed on 128 candidates for bariatric surgery with class III obesity without chest pain or pre-existent coronary artery disease (CAD). DSE without intravenous contrast was initially performed on 62 patients with class III obesity, then was subsequently was performed with intravenous contrast on 66 patients with class III obesity. Left ventricular (LV) regional wall motion was assessed at baseline and peak stress using the 16-segment model. Results: In the intravenous contrast group 1046 of 1056 LV segments studied (99.1%) were well-visualized and interpretable at baseline and 1044 of 1056 LV segments studied (98.9%) were well-visualized and interpretable at peak stress. In the non-contrast group 905 of 992 segments studied (91.2%) were well-visualized and interpretable at baseline and 886 of 992 segments studied (89.3%) were well-visualized and interpretable at peak stress. A significantly greater number of LV segments were well-visualized and interpretable in the intravenous contrast group than in the group compared to the non-contrast group, at baseline and at peak stress (p < 0.00001 for both). DSE was positive for ischemia in 1 patient. All patients underwent bariatric surgery without cardiovascular complications. Six months after surgery, all patients were alive; none developed cardiovascular events. Conclusion: The use of intravenous contrast during DSE significantly improves visualization and interpretability of LV segments in patients with class III obesity.

Objectives: Systolic and diastolic dysfunctions are related to adverse clinical outcomes in patients with sinus rhythm. The aim of this study was to clarify the prognostic significance of systolic and diastolic dysfunctions in patients with chronic persistent atrial fibrillation (AF). Methods: We evaluated data for 114 consecutive patients with chronic AF who underwent measurement of LVEDP at our hospital between 1 March 2011 and 31 December 2014. In total, 114 consecutive patients with chronic AF were divided into two groups according to the left ventricular ejection fraction (LVEF): LVEF < 50 (reduced ejection fraction, REF group) and LVEF ≥50 (preserved EF, PEF group). The PEF group was further divided into two subgroups according to the left ventricular end-diastolic filling pressure (LVEDP): LVEDP >15 mmHg and LVEDP ≤ 15 mmHg. The 3-year clinical outcomes were compared between the PEF and REF groups and the LVEDP ≥15 mmHg and LVEDP <15 mmHg groups. Results: During the 3-year follow-up period, the rate of heart failure (HF) hospitalisation and incidence of AF with rapid ventricular rhythm (RVR) were higher in the REF group than in the PEF group. Multivariate analysis revealed that REF was the only significant predictor of HF hospitalisation (hazard ratio, 4.71; 95% confidence interval, 1.48–15.02; p=0.009). Conclusions: Our observations during a mid-term follow-up period revealed that systolic dysfunction could be an important predictor of HF hospitalisation in patients with AF. However, elevated LVEDP may not be associated with mid-term adverse clinical outcomes in patients without systolic dysfunction.

Primary cardiac tumors are extremely rare.Most primary tumors are benign,and malignant tumors comprise about 15%1.Angiosarcoma is the most common type of primary cardiac malignant tumors.Compared with the left atrium or ventricle,the tumor prefer to occur in the right atrium or ventricle,especially the right atrium.In this case report,we present the case of a 32-year-old female with cardiac angiosarcoma primary to the right atrial appendage(RAA).

Background: The aim of our study was to characterize echocardiographic changes during pregnancy in women with known LVOT obstruction or AS compared to the healthy pregnancy controls, and to assess the relationship with pregnancy outcomes. Methods: We retrospectively studied 34 pregnant patients with congenital LVOT obstruction or AS with healthy age-matched pregnant controls. Patients with other significant valvular lesions, structural heart disease (LVEF <40%), or prior valve surgery were excluded. All LVOTO/AS patients underwent a minimum of 2 consecutive echocardiograms between 1 year pre-conception up to 1 year postpartum, with at least 2 studies during the pregnancy. Comprehensive echocardiographic evaluation was performed including speckle-tracking LV global longitudinal strain. Results: A total of 83 echocardiograms from the study group and 34 echocardiograms from the control group were evaluated. Over the range of LVOTO/AS, a significantly greater increase in the AV gradients and LV and LA volumes were observed as compared with the controls. In the sub-group of LVOTO/AS pregnant women with > moderate (n=8) vs.

Purpose: Cardiac resynchronization therapy (CRT) has a positive effect on the improvement of functional mitral regurgitation in patients with heart failure with reduced ejection fraction. However geometric changes in the mitral valve apparatus, subvalvular structures and their contribution to the improvement of mitral regurgitation after CRT have not been clearly defined. The aim of our study was to evaluate the geometric parameters of mitral valve apparatus measured with 3Dimensional (3D) transesophageal echocardiography (TEE) before CRT implantation and to determine the parameters predicting the improvement of mitral regurgitation after CRT. Methods: In this prospective study thirty patients with moderate or severe mitral regurgitation with low EF heart failure planned for CRT implantation and had an indication for TEE were included. Effective regurgitant orifice (ERO) and regurgitant volume (RV) measurements were performed before CRT implantation. Detailed quantitative measurements of mitral valve were done from recorded images by 3D TEE. ERO, RV measurements were repeated to evaluate mitral regurgitation at the end of 3rd month. Results: There were no significant changes in left ventricular EF and left ventricular diameters at third month follow-up, whereas ERO and RV values were decreased. posterior leaflet angle was found higher in non-responder group compared to responder group. (28,93 ± 8,41 vs 41,25 ± 10,90, p = 0,006). Conclusion: Heart failure patients with moderate or severe functional mitral regurgitation who underwent CRT implantation were found lower posterior leaflet angle which was measured by 3D TEE in the patient group whose mitral regurgitation improved after CRT.

Left atrial intramural hematoma (LAIH) is an uncommon entity for which a timely diagnosis is critical for decision making. Cardiac surgical or catheter-based procedures are potential causing factors. Though cardiac computerized tomography and magnetic resonance are highly accurate diagnostic modalities, their role is limited by the lack of widespread availability. The present clinical case illustrates the diagnostic features of LAIH that can be obtained using echocardiography at the bedside in critically ill patients. We report a case of LAIH, that followed a catheter ablation procedure and was complicated by cardiac and cerebral ischemia. Cardiac surgical management was required.

In case of valvular infective endocarditis, the infection is mainly localized on the flow side of the valves or at damaged valvular endothelium. We describe a rare case of an aortic valvular inefctive endocarditis with an aortic-valve vegetation situated on the aortic side of the valve. We believe this is the first description of this unusual localization of vegetations in a native aortic valve.

Pulmonary artery and pulmonary valve sarcoma are malignant and very rare vascular tumors with aggressive clinical course and very poor outcomes. Patients affected by coronavirus disease of 2019 (COVID-19) are at higher risk for thromboembolism complication. we describe young woman with history of corona virus pneumonia and progressive dyspnea, hemodynamic disturbance, edema with initial evaluation and clinical diagnosis of pulmonary thromboembolism. But further imaging study and pathology demonstrated, Giant sarcoma of pulmonary valve, obstructing pulmonary valve and extending to right ventricular outflow tract and main of pulmonary artery.

BACKGROUND. Multiple Doppler Echocardiography (DE) algorithms have been proposed to estimate mean pulmonary artery pressure (PAPM) and assess pulmonary hypertension (PH) likelihood. We assessed the accuracy of 4 different DE approaches to estimate PAPM in patients with heart failure (HF) undergoing near-simultaneous right heart catheterization (RHC), and compared their diagnostic performance to identify PH with recommendation-advised tricuspid regurgitation peak velocity (TRVmax). METHODS. PAPM was retrospectively assessed in 112 HF patients employing 4 previously validated DE algorithms. Association and agreement with invasive PAPM were assessed. Diagnostic performance of DE methods vs. TRVmax=2.8m/sec to identify invasive PAPM ≥ 25mmHg were compared. RESULTS. All DE algorithms demonstrated reasonable association (r = 0.41 to 0.65; p<0.001) and good agreement with invasive PAPM, with relatively lower mean bias and higher precision observed in algorithms that included TRVmax or velocity time integral. All methods demonstrated strong ability (AUC=0.70-0.80; p<0.001) to identify PH but did not outperform TRVmax (AUC=0.84; p<0.001). Echocardiographic estimates of right atrial pressure were considered in 3 of 4 DE algorithms and falsely elevated in as many as 30% of patients. CONCLUSIONS. Echocardiographic estimates of PAPM demonstrate reasonable accuracy to represent invasive PAPM and strong ability to identify PH in HF. However, even the best performing algorithm did not outperform recommendation-advised TRVmax. The additional value of echocardiographic estimates of right atrial pressure may need to be re-evaluated.