A previously healthy 68-year-old woman presented to the outpatient clinic with a 2-month history of palpitation. Physical examination and laboratory findings were unremarkable. Her electrocardiogram showed sinus tachycardia with a heart rate of 115 beats/m. Transthoracic echocardiography (TTE) showed a normal ejection fraction with a huge mass in the left atrium (Figure 1a). Transesophageal echocardiography (TEE) and cardiac magnetic resonance imaging (MRI) were performed for further evaluation. TEE revealed a hyperechogenic, well-demarcated mass in the left atrium, that was attached to the interatrial septum and adjacent to the left pulmonary veins (Figure 1b, 1c,1d). Cardiac MRI revealed a heterogeneous left atrial mass located on the fossa ovalis, 58x52x54 mm in size and markedly hyperintense on a STIR sequence (Figure 1e). These findings were suggestive of a benign cardiac tumour such as myxoma or hemangioma. A decision for surgery was made and coronary angiography was performed which showed that the branch of the circumflex artery supplied and surrounded the mass in the form of a net (Figure 1e). The patient underwent complete excision of the mass (Figure 2a, 2b). Histopathological examination revealed a nested architecture of epitelioid cells, the nests are round or oval in shape and invested by an fibrovascular stroma. Tumor cells had centrally and eccentrically located round nuclei and cytoplasm ranging from finely granular to eosiniphilic. At immunohistochemical staining, the nests were positive for chromogranin A, negative for cytokeratin (Figure 2c, 2d, 2e, 2f). A diagnosis of paraganglioma was made. After an uneventful postoperative course, she was discharged home on postoperative day 6. Cardiac paraganglioma is a very rare neuroendocrine tumour and accounts for less than 1% of primary cardiac tumours (1,2). Approximately 10% of paragangliomas may be malignant, complete surgical resection remains the first-line treatment (3).

Abernethy malformation, also known as congenital extrahepatic shunt, is a rare anomaly characterized by partial or complete diversion of the portal blood into the systemic venous circulation. The clinical manifestations of Abernethy malformation during childhood include neonatal cholestasis, failure to thrive, mental retardation, and other congenital defects. We report a case of Abernethy malformation Type Ⅱ in a 9-year-old boy whose left ventricle was slightly enlarged because of several major aortopulmonary collateral arteries but normal laboratory examinations five years earlier. The characteristics of congenital heart disease in patients with Abernethy malformation are discussed. We propose that enlargement of the left ventricular with systemic-pulmonary collateral circulation should raise the suspicion of Abernethy malformation.

Background: In atrial fibrillation patients undergoing left atrial appendage occlusion with a Watchman device, surveillance imaging with a transesophageal echocardiogram (TEE) is typically performed at 45 days and 1 year to evaluate for device-related thrombus (DRT) and peri-device leak (PDL) before cessation of oral anticoagulation. The incidence of these complications is relatively low, and the ideal timing and duration of surveillance is unknown. We sought to evaluate the incidence of DRT and PDL after Watchman placement at 45 days and 1 year to determine the necessity of surveillance TEEs. Methods: We retrospectively analyzed 361 patients who received a Watchman device between January 2016 and January 2020. Baseline clinical and echocardiographic data, post-procedure antithrombotic therapy and surveillance echocardiographic data were collected from the NCDR LAAO Registry. Nested backward variable elimination regression was performed to derive independent predictors of the composite outcome of DRT and PDL. Results: A total of 286 patients who had post-procedure TEEs were included in the analysis. At 45 days, 9 patients had DRT (3.2%) and 44 patients had PDL (15.0%). At 1 year, 5 patients had DRT (5.6%) and 8 patients had PDL (8.9%). All DRT at 45 days was treated with continued anticoagulation while no change in protocol occurred with PDL. All DRT at 1 year occurred in new patients without prior thrombus. A history of prior transient ischemic attack (TIA) and thromboembolism were significantly associated with DRT or PDL at 1 year. Conclusions: We identified several patients with device-related complications at 45 days and 1 year despite appropriate device sizing and adequate use of antithrombotic therapy. The incidence of DRT increased from 45 days to 1 year and occurred in patients without prior thrombus. These findings highlight the importance of surveillance imaging and suggest the potential need for extended surveillance in select patients.

Prosthetic valve endocarditis with mechanical complications causing pulmonary edema is fatal, therefore it needs to be diagnosed early and should be treated surgically in emergency setting. Transesophageal echocardiogram is crucial for recognizing the mechanical complications, which can be encountered on daily practice, but the coexistence of complications occurring on different mechanism is rather uncommon. Herein, we report a 21-year-old gentleman presenting with acute heart failure, whose imaging tests showed a combination of dehiscence of mechanical aortic valve prosthesis, aortic dissection, pseudoaneurysm and hematoma causing right ventricular collapse.

57-years old man presented with exertional dyspnea. An early systolic murmur was heard over the aortic areas 2D and 3D Echocardiography revealed unicuspid , unicommissural aortic valve (UAV) with a characteristic “teardrop” lateral orifice (Figure A) and moderate valve stenosis (3D planimetered aortic valve area (AVA) is 1.1cm2) (Figure B) Continuous wave Doppler across aortic valve (AV) showed high peak and mean systolic gradients of 85 and 60mmHg respectively.(Figure C). 2D /3D Transesophageal Echocardiography (TOE) revealed a subaortic ridge attached to the posterior annulus (Arrow) (Figure D) Further En-face viewing of the aortic valve from the left ventricular outflow tract (LVOT) perspective showed a shelf-like ridge extending from the commissure to the cusp (Arrow) (Figure E) Zoomed mode of the aortic- LVOT junction confirmed the presence of the subaortic ridge seen attached to the posterior aortic annulus near the commissural opening (Figure F) The patient was referred for surgical consultation .. Unicupid aortic valve (UAV) is a rare congenital anomaly that has.2 subtypes ; unicomissural and acommissural subtypes. Both can present with variable degrees of the aortic stenosis (AS) and/or aortic valve regurgitation (AR).UAV has more early, accelerated and severe valvular degeneration in addition to smaller orifice in comparison with bicuspid and tricuspid aortic valve. Echocardiography is the gold standard for diagnosis and evaluation of the AV morphology and function and the associated disorders such as ventricular septal defect , aortopathy and subaortic obstruction.. Surgical aortic valve replacement (AVR) and repair of the associated anomalies are the most common treatment modality .

Objectives: Echocardiographic assessment of Left ventricular systolic function is traditionally being performed by estimation of fractional shortening and Ejection fraction. Speckle tracking echocardiography (STE) is a promising tool for assessment of myocardial function. The aim of this study is to evaluate the global longitudinal strain (GLS) using 2D-STE in healthy neonates to establish normal reference ranges. Method: it is a retrospective study through an analysis of transthoracic echocardiogram of normal healthy neonates. We enrolled all neonates in our institution from 1 st January 2021 to 28 th February 2021. 2-D STE was used to assess left ventricular global longitudinal strain from the apical views. Results: 185 neonates were enrolled. Mean value for left ventricle GLS (%) was -19.9 ± 1.2, GLS-derived ejection fraction (%) was 60.0 ± 2.7; while the left ventricle ejection fraction by biplane Simpson’s method (%) was 61.0 ± 3. There is a good positive correlation between the Left Ventricle EF by biplane Simpson’s method and EF by 2-D STE, which was statistically significant ( r = 0.294,  n = 102,  p=0.003). Apical 4-chamber longitudinal strain and strain derived EF is significantly correlated with GLS and bi-plan EF respectively. Conclusion: 2-STE is feasible technique for analyzing newborn myocardial systolic function. The normal range of GLS in neonates is not much different than reported for the pediatric. There is a good positive correlation between the Left Ventricle EF by 2-D STE and EF by biplane method.

Background Two-dimensional (2D) strain imaging has become an important tool in assessing subclinical myocardial dysfunction in children. However, there are no pediatric reference values for vendor independent strain software. The aim of this study was to estimate 2D strain values in a cohort of healthy children using Tomtec cardiac performance analysis (CPA), a vendor independent software. Methods Transthoracic echocardiograms of healthy pediatric outpatients (0-18yrs) were retrospectively analyzed from the Vanderbilt Pediatric Heart Institute using CPA. Cardiac assessment included global longitudinal strain (GLS), global longitudinal strain rate (GLSR), global circumferential strain (GCS), global circumferential strain rate (GCSR). Mean strain values with standard deviation (SD) are reported. The Wilcoxon rank sum test, linear regression and one-way analysis of variance were used to assess differences among the various groups. Results Among 142 children analyzed, 79 (56%) were male, and the median age was 5.5 (range, 0-18) years. The mean (SD) strain values were GLS -19.3 ± 3.4, GLSR -1.1 ± 0.22; GCS -24.7 ± 4.3, GCSR -1.5 ± 0.28. Age accounted for <8% of variation in GLS, GCS and GSCR. However, for GLSR, there was a statistically significant difference between younger and older age groups with higher GLSR in the younger age group. Age accounted for ~25% of variation in GLSR (R 2 = 0.25, P < 0.001). There were no significant differences in strain based on sex. Conclusion We report normal reference values in healthy children by age for strain using CPA. These values are necessary for the interpretation of 2D strain imaging for both clinical care and research.

Background: Three-dimensional echocardiography (3DE) is an emerging method for volumetric cardiac measurements; however, few vendor-neutral analysis packages exist. Ventripoint Medical System Plus (VMS3.0+) proprietary software utilizes a validated MRI database of normal ventricular and atrial morphologies to calculate chamber volumes. This study aimed to compare left ventricular (LV) and atrial (LA) volumes obtained using VMS3.0+ to Tomtec echocardiography analysis software. Methods: Healthy controls (n=98) aged 0 to 18 years were prospectively recruited and 3D DICOM datasets focused on the LV and LA acquired. LV and LA volumes and ejection fractions were measured using TomTec Image Arena 3D LV analysis package and using VMS3.0+. Pearson correlation coefficients, Bland-Altman’s plots and intraclass coefficients (ICC) were calculated, along with analysis time. Results: There was a very good correlation between VMS and Tomtec LV systolic (r 2 = 0.88, ICC 0.89 [95% CI 0.81,0.94]), and diastolic (r 2 = 0.88, ICC 0.90 [95% CI 0.77,0.95]) volumes, and between VMS and Tomtec LA diastolic (r 2 =0.75, ICC 0.89 [95% CI 0.81,0.93]) and systolic (r 2 =0.88, ICC 0.91 [95% CI 0.78,0.96]) volumes on linear regression models. Natural log transformations eliminated heteroscedasticity, and power transformations provided best fit. The time (mins) to analyze volumes using VMS were less than using Tomtec (LV VMS 2.3±0.5, Tomtec 3.3±0.8, p<0.001; LA: VMS 1.9±0.4, Tomtec 3.4±1.0, p<0.001). Conclusions: There was very good correlation between knowledge-based (VMS3.0+) and 3D (Tomtec) algorithms when measuring 3D echocardiography derived LA and LV volumes in pediatric patients. VMS was slightly faster than Tomtec in analyzing volumetric measurements.

COVID-19 related MIS-C (Multisystem inflammatory syndrome in children) can present with cardiovascular complications like shock, arrhythmias, pericardial effusion, and coronary artery dilatation. The majority of MIS-C associated coronary artery abnormalities are dilation or small aneurysms which are transient and resolve in a few weeks[[1, 2]](#ref-0001). We present here a case of a 3-month-old child who was noted to have giant aneurysms of her coronary arteries (LAD and RCA) twenty-six days after testing positive for COVID-19. She was treated with IVIG, infliximab, and glucocorticoids along with aspirin, clopidogrel and enoxaparin. She did not show any signs of coronary ischemia or cardiac dysfunction but continued to have persistent giant coronary artery aneurysms involving the LAD (z-score ~35) and RCA (z-score ~30) [Fig. 1]. This study emphasizes the importance of early detection and aggressive management of MIS-C to prevent potentially life-threatening consequences.

Objectives In stress echocardiography (SE), dipyridamole (DIP) and dynamic stress (ExSE) are reported as safer than dobutamine stress (DSE). We investigated whether commonly used stressors cause myocardial injury, measured by high sensitivity troponin T (hsTnT). Methods 135 patients (DSE n=46, ExsE n=46, DIP n=43) with negative SE were studied. Exclusion criteria were known ischemic heart disease (IHD), baseline wall motion abnormalities, left ventricle systolic dysfunction/regional wall motion abnormalities, septum/posterior wall ≥13 mm, diabetes, baseline hsTnT level ≥14 ng/L, baseline blood pressure ≥160/100 mmHg, peak pulmonary pressure ≥45mmHg, eGFR <1mL/s/1.73m2, more than mild to moderate valvular disease and dobutamine side effects. HsTnT was measured before and 180 minutes after the test. Results All patients had low pre-test probability of IHD. HsTnT increased in DSE, less so in ExSE, and unchanged in DIP group [9.4 (1.5–58.6), 1.1 (-0.9–15.7), -0.1 (-1.4–2.1) ng/L, p<0.001]. In DSE, hsTnT change was associated with peak dobutamine dose (r=0.30, p= 0.045), test length (r=0.43, p=0.003) and atropine use (p<0001). In ExSE, hsTnT rise was more likely in females (p=0.012) and elderly (>65 years) (r=0.32, p=0.03), no association was found between atropine use (p=0.786) or test length and hsTnT rise (r=0.10, p=0.530). Conclusions DSE is associated with myocardial injury in patients with negative SE, no injury was observed in DIP and only mild one in ExSE. Whether myocardial injury is causative of the higher reported adverse event rates in DSE remains to be determined.

Background: Accessory mitral valve tissue (AMVT) is an extremely rare causes left ventricular outflow tract (LVOT) obstruction and is usually incidentally detected in childhood. It is often associated with other cardiac and vascular congenital malformations. Case Presentation: In this case, we present a 15-year-old girl was diagnosed with AMVT by transesophageal echocardiography, resulting in LVOT obstruction during systole. Interestingly enough, the patient’s accessory mitral valve remained undetected for years until he became symptomatic for wide ASD. Successful closure of the ASD with resection of the AMVT was performed with a transaortic approach. The patient was hemodynamically stable postoperatively. There were no abnormalities in the mitral valves and LVOT. Conclusion: It was also unusual to see AMVT with ASD instead of other frequently associated other congenital anomalies. Accessory mitral valve should be considered a rare but important cause of left ventricular outflow tract obstruction in childhood.

[Abstract] Objective To review the imaging characteristics and evaluate the diagnostic value of echocardiography for fetal congenitally unguarded tricuspid valve orifice (CUTVO). Methods Doppler echocardiography was performed and the images were compared with operative and necropsy findings in ten fetuses with CUTVO. The aim of the study was to summarize the characteristics of fetal echocardiography and analyze the causes of missed diagnoses and misdiagnoses.  Results There were six cases with complete absence and four cases with partial absence of the tricuspid leaflet. In seven of ten cases the pregnancy was terminated. In six cases CUTVO was confirmed by autopsy after induced labor, while one case had no autopsy. After birth, one case died due to severe illness. The two remaining cases survived with an atrial septal defect and patent ductus arteriosus on postpartum ultrasonic scans. These cases underwent surgical treatment resulting in less moderate tricuspid regurgitation. Among all cases, four were misdiagnosed and diagnosis for CUVTO missed, but CUVTO was demonstrated after induced labor. CUTVO ultrasonographic characteristics consist of the atrioventricular connection with normal arteries and the tricuspid valve device partially or completely absent. The annulus of the tricuspid valve can be describe as “empty” in the apical 4-chamber view, Doppler evaluation shows to-and-fro flow across the tricuspid orifice with low velocity and two-way spectrum. Conclusion diagnosis and differential diagnosis of CUTVO by fetal echocardiography has important clinical value.

A 22-year male presented with complaints of dyspnea. Multimodality imaging revealed a polypoidal right atrial mass with sub-massive pulmonary embolism. The patient underwent urgent surgery. The pathological examination confirmed it as cardiac myxoma. Cardiac myxoma, a most common primary cardiac tumor, is commonly found in the left atrium. The right atrium is an uncommon site and the usual mode of presentation is the tumor or thrombus embolisation to the pulmonary circulation.

Right atrial appendage aneurysms (RAAAs) are extremely rare in cardiac anomaly. According to the literature, no more than 25 cases have been reported so far, among which only 3 cases were children. Here, we reported an infant with a giant RAAA and severe symptoms. The RAAA was diagnosed by echocardiography and surgically resected under cardiopulmonary bypass. The role of transesophageal echocardiography was very important during the aneurysm resection surgery, which helped surgeons to plan surgical procedures during the surgery and evaluate the surgical effect postoperatively.

Ruptured abdominal aortic aneurysms are associated with high mortality rates and require prompt diagnosis with subsequent intervention. CT scan is considered the gold-standard for diagnosis, however, in the acute setting ultrasound may be a reasonable diagnostic test for certain patients. We report a case that demonstrates the utility of bedside ultrasound of rAAA for a patient in extremis. Also, we provide a brief review of literature for the diagnosis of rAAA with ultrasound.

We describe an adult patient who presented with purulent pericarditis in whom two-dimensional transthoracic echocardiography demonstrated a marked decrease in the area of the right ventricular wall together with the overlying fibrin following intrapericardial administration of a fibrinolytic agent. Documentation of this decrease by measurements performed and illustrated on two-dimensional images have not been reported previously in an adult patient with purulent pericarditis, to the best of our knowledge.

Introduction: Cardiac point-of-care ultrasound (c-POCUS) is an increasingly implemented diagnostic tool with the potential to guide clinical management. We sought to characterize and analyze the existing c-POCUS literature with a focus on the temporal trends and differences across specialties. Methods: A literature search for c-POCUS and related terms was conducted using Ovid (MEDLINE and Embase) and Web of Science databases through 2020. Eligible publications were classified by publication type and topic, author specialty, geographical region of senior author, and journal specialty. Results: The initial search produced 1761 potential publications. A strict definition of c-POCUS yielded a final total of 574 cardiac POCUS manuscripts. A yearly increase in c-POCUS publications was observed. Nearly half of publications were original research (48.8%) followed by case report or series (22.8%). Most publications had an emergency medicine senior author (37.5%), followed by cardiology (20.2%), anesthesiology (12.2%), and critical care (12.2%). The proportion authored by emergency medicine and cardiologists has decreased over time while those by anesthesiology and critical care has generally increased, particularly over the last decade. First authorship demonstrated a similar trend. Articles were published at similar numbers in emergency medicine journals (23.0%) and cardiology journals (19.9%). Conclusion: The annual number of c-POCUS publications has steadily increased over time reflecting the increased recognition and utilization of c-POCUS. This study can help inform clinicians of the current state of c-POCUS and augment the discussion surrounding barriers to continued adoption across all specialties.

Objective: Predictors for post-operative reverse remodeling in patients with severe aortic regurgitation (AR) and reduced left ventricular ejection fraction (LVEF) are unknown. We performed low-dose dobutamine stress echocardiography (DSE) in patients with severe AR and reduced LVEF to evaluate the relationship between contractile reserve (CR) and reverse remodeling after surgery. Methods: In 31 patients with chronic severe AR and reduced LVEF (LVEF < 50%), we performed pre-operative DSE, assessed CR and examined whether changes in preoperative DSE were associated with improvement of post-operative LVEF after aortic valve surgery. Results: The pre-operative echocardiographic findings were as follows: left ventricular (LV) end-diastolic dimension: 67 ± 10 mm, LV end-systolic dimension: 52 ± 13 mm and LVEF: 42% ± 8%. All patients underwent aortic valve surgery. Patients with pre-operative LVEF of >45% exhibited a significant increase in LVEF; however, patients with pre-operative LVEF of <45% showed no significant change. When we examined the results of DSE performed in patients with pre-operative LVEF of <45%, ΔLVEF of ≥6% (with CR) during DSE was related to an improvement in post-operative LVEF; ΔLVEF of ≥6% during DSE predicted an improvement in post-operative LVEF, with a sensitivity and specificity of 80% and 85%, respectively. Conclusions: DSE may be a helpful tool for predicting post-operative reverse remodeling in patients with severe AR and moderately reduced LVEF.