Extensive cardiovascular involvement in a young boy with Gaucher's disease: a case report.

Background: Lysosomal storage diseases (LSDs) are rare, progressive, multi-organ disorders caused by inherited enzyme deficiencies. Gaucher's disease (GD) is the most prevalent form of LSDs. Case summary: A 19-year-old Caucasian male presented with exertional dyspnoea. Physical examination revealed a Grade III/VI systolic diamond murmur at the heart base and a Grade IV/VI systolic murmur at the apex. Electrocardiogram showed signs of left ventricular hypertrophy (LVH). Trans-thoracic echocardiography (TTE) and trans-oesophageal echocardiography (TEE) demonstrated moderate LVH, severe aortic valve stenosis, severe supra-valvular aortic stenosis, and moderate mitral stenosis with severe degenerative mitral valve regurgitation. Bone marrow biopsy and aspiration confirmed the presence of characteristic Gaucher's cells. The patient underwent the Bentall procedure and mitral valve replacement and was discharged in good condition. Discussion: Gaucher's disease exhibits three clinical phenotypes, and cardiovascular involvement is commonly seen in GD Type III. Valvular calcification and ascending aorta involvement are frequent cardiovascular manifestations. Although severe valvular heart involvement is rare in GD, cardiac valve surgery has shown favourable outcomes in previous studies and our case.

Transcatheter closure of a ruptured sinus of valsalva: a systematic review of the literature.

Background: Ruptured sinus of Valsalva (RSOV) is a rare pathology, and current data regarding its symptoms, anatomy, associated pathologies, and appropriate therapeutic approaches are scarce. Transcatheter closure (TCC) has been performed in multiple cases; however, the information on its success rate and complications is limited. Methods: Two independent reviewers performed an advanced search based on inclusion criteria on Scopus, PubMed, and Embase from January 1985 through July 2022. The main search terms were "Sinus of Valsalva", "Rupture/Aneurysm", and "Transcatheter/Catheter/Device". Results: Totally, 1,017 relevant articles from the 3 databases were retrieved. After the final review and appraisal, 94 articles describing 407 patients who underwent the TCC of RSOV were included. Males comprised 65% of the studied patients, and the average age was 34.5 years. The total success rate of TCC was 95.6%. Forty-nine patients (12%) developed complications, the most significant of which were sustained residual shunts in 7 patients (1.7%), substantial new onset or progression of aortic insufficiency in 6 (1.5%), and RSOV recurrence in 6 (1.5%). Post-interventional mortality was reported in only 2 patients (0.5%). Conclusions: The present study is the first systematic review of available data regarding the TCC of RSOV principally comprising case series and case reports. Although TCC seems a good option, precise patient selection is mandatory.

Transcatheter closure of ruptured sinus of Valsalva: A report of first five cases in Tehran Heart Center, Iran.

Ruptured sinus of Valsalva (RSOV) is a potentially lethal lesion requiring prompt intervention. Transcatheter Closure of RSOV is a novel alternative to the standard treatment, which is open-heart surgery. In this case series, we have reported our center's first five cases of RSOV patients undergoing Transcatheter Closure.

Left atrial function evaluation by 2D speckle-tracking echocardiography in patients with vasovagal syncope.

OBJECTIVES: Evidence indicates left atrial (LA) involvement in vasovagal syncope (VVS). The LA regulates left ventricular filling during the cardiac cycle. We aimed to assess LA function in patients with VVS by 2D speckle-tracking echocardiography. METHODS: Sixty-nine consecutive patients with VVS were recruited. Based on the head-up tilt test (HUTT) results, the study population was divided into two groups: HUTT+ (n = 45) and HUTT- (n = 24). Fifty-one consecutive subjects were enrolled as the control group. LA myocardial deformation parameters were measured by 2D speckle-tracking echocardiography to evaluate LA function. RESULTS: Maximal, minimal, and pre-P LA volumes were lower in patients with VVS. Strain and strain rate during reservoir, conduit, and contraction phases in VVS patients with HUTT+ or HUTT- were not statistically significantly different from those in the control group. Additionally, the volumetric parameters of LA function showed no difference in statistical significance between the three study groups. CONCLUSIONS: While LA phasic function was not different between the two groups of VVS patients with HUTT+ or HUTT- and the control group, LA size during the three LA phases was smaller in patients with VVS.

Correlation between Heart rate recovery and Left Atrial phasic functions evaluated by 2D speckle-tracking Echocardiography after Acute Myocardial infarction.

BACKGROUND: Heart rate recovery (HRR) in the exercise test is the index of cardiac autonomic system function and sympathovagal balance impaired in patients with myocardial infarction (MI). An instance is left atrial (LA) phasic function, which is impaired in such patients. In this study, we investigated the role of HRR in predicting LA phasic functions in patients with MI. METHODS: The present study recruited 144 consecutive patients with ST-elevation MI. A symptom-limited exercise test was performed about 5 weeks after MI, with echocardiography conducted just before the exercise test. The patients were divided into abnormal and normal HRR at 60 s (HRR60) and again into abnormal and normal HRR at 120 s (HRR120) after the exercise test. LA phasic functions, evaluated by 2D speckle-tracking echocardiography, were compared between the 2 groups. RESULTS: Patients with abnormal HRR120 had lower LA strain values and strain rates during the reservoir, conduit, and contraction phases, while those with abnormal HRR60 had lower LA strain values and strain rates during the reservoir and conduit phases. The differences were lost after adjustments for possible confounders, except for LA strain and strain rate during the conduit phase, in patients with abnormal HRR120. CONCLUSIONS: Abnormal HRR120 in the exercise test can independently predict decreased LA conduit function in patients with ST-elevation MI.

Evaluation of cardiac chamber functions by speckle-tracking echocardiography in the presence of coronary artery flow disturbances other than stenosis: A review.

Conditions other than stenosis also disturb the coronary flow. Such conditions include the coronary slow flow phenomenon, coronary artery ectasia, and coronary artery tortuosity. Evidence exists regarding myocardial dysfunction in these conditions. In this review, we present studies that have used speckle-tracking echocardiography to determine whether coronary flow disturbances are accompanied by myocardial dysfunction. Additionally, we seek to show the gaps in knowledge concerning this issue and the dimensions that future studies should consider.

Case report: Multimodal imaging diagnosis of a giant coronary artery fistula: A report of two cases.

Being a very rare cardiac disease, most cases of coronary artery fistula (CAF) are genetic. Complications such as coronary steal syndrome, myocardial infarction, heart failure, or tamponade can manifest following the abnormal communication that the fistula creates between the coronary arteries and cardiac chambers or major vessels and the subsequent shunt. Most CAFs are small and asymptomatic, making diagnosis difficult. In symptomatic patients, the initial diagnostic workup is generally made with chest radiography and electrocardiography. Other imaging modalities have also been suggested to improve diagnostic accuracy. Cardiac catheterization and coronary angiography are currently the gold standard for diagnosis and planning the intervention, as they can recognize the quantum of the shunt as well as complications of a fistulous track (e.g., aneurysm formation, thrombus, leak, and the number of openings to the receiving chamber/vessel); however, this invasive method may be associated with risk. Herein, we report two patients with giant CAFs, one from the left circumflex artery to the coronary sinus and the other to the superior vena cava. Moreover, we describe how multimodal imaging, including two- and three-dimensional transesophageal echocardiography, coronary cineangiography, coronary computed tomography angiography, and enhanced chest computed tomography, can facilitate diagnosis and estimate the disease course in such patients. We believe that using multimodal imaging cannot only help the initial diagnosis regarding the presence of a CAF and the accurate anatomical site of the fistula in the patient but can also help predict the disease course and choose the most suitable treatment modality. Therefore, we suggest multimodal imaging be done to diagnose patients suspected of CAF. However, invasive cineangiography should be necessarily followed, regardless of whether an intervention is planned or not.

Catheter-Directed Thrombolysis vs Anticoagulation in Patients With Acute Intermediate-High-risk Pulmonary Embolism: The CANARY Randomized Clinical Trial.

Importance: The optimal treatment of intermediate-high-risk pulmonary embolism (PE) remains unknown. Objective: To assess the effect of conventional catheter-directed thrombolysis (cCDT) plus anticoagulation vs anticoagulation monotherapy in improving echocardiographic measures of right ventricle (RV) to left ventricle (LV) ratio in acute intermediate-high-risk PE. Design, Setting, and Participants: The Catheter-Directed Thrombolysis vs Anticoagulation in Patients with Acute Intermediate-High-Risk Pulmonary Embolism (CANARY) trial was an open-label, randomized clinical trial of patients with intermediate-high-risk PE, conducted in 2 large cardiovascular centers in Tehran, Iran, between December 22, 2018, through February 2, 2020. Interventions: Patients were randomly assigned to cCDT (alteplase, 0.5 mg/catheter/h for 24 hours) plus heparin vs anticoagulation monotherapy. Main Outcomes and Measures: The proportion of patients with a 3-month echocardiographic RV/LV ratio greater than 0.9, assessed by a core laboratory, was the primary outcome. The proportion of patients with an RV/LV ratio greater than 0.9 at 72 hours after randomization and the 3-month all-cause mortality were among secondary outcomes. Major bleeding (Bleeding Academic Research Consortium type 3 or 5) was the main safety outcome. A clinical events committee, masked to the treatment assignment, adjudicated clinical outcomes. Results: The study was prematurely stopped due to the COVID-19 pandemic after recruiting 94 patients (mean [SD] age, 58.4 [2.5] years; 27 women [29%]), of whom 85 patients completed the 3-month echocardiographic follow-up. Overall, 2 of 46 patients (4.3%) in the cCDT group and 5 of 39 patients (12.8%) in the anticoagulation monotherapy group met the primary outcome (odds ratio [OR], 0.31; 95% CI, 0.06-1.69; P = .24). The median (IQR) 3-month RV/LV ratio was significantly lower with cCDT (0.7 [0.6-0.7]) than with anticoagulation (0.8 [0.7-0.9); P = .01). An RV/LV ratio greater than 0.9 at 72 hours after randomization was observed in fewer patients treated with cCDT (13 of 48 [27.0%]) than anticoagulation (24 of 46 [52.1%]; OR, 0.34; 95% CI, 0.14-0.80; P = .01). Fewer patients assigned to cCDT experienced a 3-month composite of death or RV/LV greater than 0.9 (2 of 48 [4.3%] vs 8 of 46 [17.3%]; OR, 0.20; 95% CI, 0.04-1.03; P = .048). One case of nonfatal major gastrointestinal bleeding occurred in the cCDT group. Conclusions and Relevance: This prematurely terminated randomized clinical trial of patients with intermediate-high-risk PE was hypothesis-generating for improvement in some efficacy outcomes and acceptable rate of major bleeding for cCDT compared with anticoagulation monotherapy and provided support for a definitive clinical outcomes trial. Trial Registration: ClinicalTrials.gov Identifier: NCT05172115.

Repositioning a Displaced Right Ventricular Pacing Lead via Percutaneous Approach in Three Patients.

Lead-related complications compose a noticeable share of device-related complications. Pacemaker lead perforation is a recognized complication of lead implantation, particularly with active fixation leads, and should be considered in postoperative lead malfunction cases. We herewith present 3 challenging cases with ventricular pacemaker lead dispositioning who were successfully treated via percutaneous access.

Automatic cardiac evaluations using a deep video object segmentation network.

BACKGROUND: Accurate cardiac volume and function assessment have valuable and significant diagnostic implications for patients suffering from ventricular dysfunction and cardiovascular disease. This study has focused on finding a reliable assistant to help physicians have more reliable and accurate cardiac measurements using a deep neural network. EchoRCNN is a semi-automated neural network for echocardiography sequence segmentation using a combination of mask region-based convolutional neural network image segmentation structure with reference-guided mask propagation video object segmentation network. RESULTS: The proposed method accurately segments the left and right ventricle regions in four-chamber view echocardiography series with a dice similarity coefficient of 94.03% and 94.97%, respectively. Further post-processing procedures on the segmented left and right ventricle regions resulted in a mean absolute error of 3.13% and 2.03% for ejection fraction and fractional area change parameters, respectively. CONCLUSION: This study has achieved excellent performance on the left and right ventricle segmentation, leading to more accurate estimations of vital cardiac parameters such as ejection fraction and fractional area change parameters in the left and right ventricle functionalities, respectively. The results represent that our method can predict an assured, accurate, and reliable cardiac function diagnosis in clinical screenings.

Evaluation of the right atrial phasic functions in patients with anterior ST-elevation myocardial infarction: a 2D speckle-tracking echocardiography study.

BACKGROUND: Evidence suggests that changes in left ventricular systolic and diastolic functions may affect right atrial (RA) phasic functions. We aimed to evaluate RA phasic functions in the presence of anterior ST-elevation myocardial infarction (ASTEMI) as an acute event and to compare the findings with those in a control group. METHODS: We recruited 92 consecutive ASTEMI patients without accompanying significant stenosis in the proximal and middle parts of the right coronary artery and 31 control subjects, matched for age, sex, diabetes, and hypertension. RA phasic functions were evaluated concerning their longitudinal 2D speckle-tracking echocardiography-derived markers. The ASTEMI group was followed up for all-cause mortality or reinfarction. RESULTS: In the ASTEMI group, RA strain was reduced during the reservoir (33.2% ± 4.3% vs 30.5% ± 8.1%; P = 0.021) and conduit (16% [12-18%] vs 14% [9-17%]; P = 0.048) phases. The other longitudinal 2D speckle-tracking echocardiography-derived markers of RA phasic functions were not different between the 2 groups. RA strain and strain rate during the contraction phase were predictive of all-cause mortality or reinfarction (hazard ratio = 0.80; P = 0.024 and hazard ratio = 0.39; P = 0.026, respectively). CONCLUSIONS: Based on 2D speckle-tracking echocardiography, in the ASTEMI group, compared with the control group, RA reservoir and conduit functions were reduced, while RA contraction function was preserved. RA contraction function was predictive of all-cause mortality or reinfarction during the follow-up period.

A systematic review and meta-analysis of the normal reference value of the longitudinal left atrial strain by three dimensional speckle tracking echocardiography.

The normal reference value of the global longitudinal left atrial strain during the reservoir phase (LASr) by 3D speckle-tracking echocardiography (3DSTE) is needed to define the abnormal and normal spectra and to compare and interpret the obtained values. The present study is a meta-analysis of 3DSTE-derived normal reference value of the longitudinal LASr and an attempt to determine probable contributing factors in the variations of reported ranges. The databases of PubMed, Scopus, and Embase were searched for the following keywordS: "Left atrial/left atrium" and "strain/speckle/deformation" and "three-dimensional/3-dimensional/three dimensional/3 dimensional/three dimension/3 dimension/three-dimension/3-dimension/3D/3-D". The studies selected included those on adult healthy subjects without cardiovascular risk factors. A random-effect model was used to calculate the global 3DSTE-derived longitudinal LASr, and meta-regression was applied to determine inter-study heterogeneity. Our search yielded 316 adult subjects from 5 studies. The mean value of the global 3DSTE-derived longitudinal LASr was 27.5% (95% CI, 25.2-29.8%). There was significant heterogeneity between the studies. The meta-regression analysis revealed the publication year, the heart rate, and systolic and diastolic blood pressure as the sources of heterogeneity. The current meta-analysis determined a normal reference value of the global 3DSTE-derived longitudinal LASr of 27.5% (95% CI, 25.2-29.8%). The heterogeneity between studies may be explained by the publication year, the heart rate, and systolic and diastolic blood pressure.

Effect of minocycline on the left ventricular function following ST-elevation myocardial infarction treated by primary percutaneous coronary intervention.

BACKGROUND: Cardiac remodeling following myocardial infarction is a pathological process. We aimed to examine the effect of early short-term minocycline on the left ventricular function following ST-elevation myocardial infarction treated by the primary percutaneous coronary intervention. METHODS: In this double-blind, randomized controlled trial, data of 73 patients STEMI patients who were candidates for primary PCI were enrolled. Patients were then randomized to receive minocycline 50 mg orally, followed by 50 mg once a day for 5 days or a placebo with the same schedule. Measurement of serum matrix metalloproteinase-9 (MMP-9) and 2-dimensional speckle tracking echocardiography was performed at baseline and between 4 and 6 months after discharge. Then the demographic, clinical, echocardiographic, and angiographic data, as well as the levels of MMP-9, were compared between the study groups. RESULTS: There was no statistically significant difference between the study groups regarding the baseline characteristics. Serum levels of MMP-9 did not change following the intervention within each group and were not significantly different between the groups after follow-up. In the follow-up echocardiography, we also did not observe any difference between the two groups CONCLUSION: In this study, we did not observe any effect of minocycline on cardiac remodeling based on 2-dimensional speckle tracking echocardiography and MMP-9 levels. TRIAL REGISTRATION: Iranian Registry of Clinical Trials IRCT201411188698N15 . Registered on 22 June 2015, prospectively.

Right-sided Electrocardiogram in Patients With Acute Pulmonary Embolism.

BACKGROUND: Acute pulmonary embolism (APE) is an emergent condition. The diagnostic tools are CT angiography and echocardiography. However, all of these modalities can be used under stable and nonemergent conditions. As electrocardiographic (ECG) studies are more feasible under emergent situations, Objective: we aimed to study right-sided ECG (RS-ECG) in patients with APE with further analysis regarding echocardiographic findings and pulmonary embolism severity index (PESI). METHODS: Right-sided and standard electrocardiogram were obtained from 143 patients with confirmed APE. T-wave inversion, ST segment elevation (STE), and QS pattern in RS-ECG have been considered as abnormal changes. RESULTS: T-wave inversion, STE, and QS pattern were found in 78.3%, 42%, and 39.9% of patients, respectively. 88.1% of patients showed at least one of these abnormalities in their RS-ECGs. Sensitivity of RS-ECG for APE was 0.88, which was higher than sensitivity of standard ECG (0.79). Patients with STE in right precordial leads, had increased right to left ventricular (RV/LV) diameter ratio (P = 0.021) and had higher PESI score (P = 0.000). Moreover, STE in RS-ECG was an independent predictor for PESI score in patients with moderate (odds ratio 4.05; 95% confidence interval [CI], 1.37-11.96; P < 0.05) and high 30-day mortality risk (odds ratio 8.42; 95% CI, 2.08-33.93; P < 0.05). CONCLUSIONS: Abnormal changes in RS-ECG were associated with an increase in RV/LV diameter ratio and higher PESI score, which may be indicated poor prognosis in patients with APE. The most clinically useful ECG finding was STE which was associated with increased RV/LV diameter ratio and PESI score.

Determinants of the Volumetric Markers of Left Atrial Contraction Function in Coronary Artery Disease: A Cross-sectional Study.

BACKGROUND: A body of research advocates the prognostic role and usefulness of the volumetric markers of left atrial (LA) phasic functions in the diagnosis of LA dysfunction. We aimed to determine the independent determinants of the volumetric markers of LA contraction function in candidates for coronary artery bypass graft (CABG) surgery. METHODS: This cross-sectional study enrolled 516 candidates for CABG. The biplane maximal, minimal, and pre-P volumes of the LA were measured with two-dimensional echocardiography, and LA active emptying fraction was calculated. The standardized correlation coefficient for the correlation between each factor and LA active emptying fraction was calculated by using univariate and backward multivariable regression analyses. RESULTS: The multivariable regression analysis demonstrated that the heart rate (ß = 0.15; p = 0.001), S (ß = 0.09; p = 0.036), E/e' ratio (ß = -0.11; p = 0.014), left ventricle (LV) ejection fraction (ß = 0.15; p = 0.001), and LA enlargement (ß = -0.19; p < 0.001) were the independent determinants of LA active emptying fraction. CONCLUSIONS: The independent determinants of LA contraction function were the heart rate, S, LV ejection fraction, LA enlargement, and E/e' ratio in candidates for CABG surgery.

Intramyocardial echo-free space with a turbulent flow within, suggestive of an aneurysmal coronary artery.

Coronary artery aneurysms are well known, and intramyocardial coronary artery aneurysms comprise a rare type of this condition. This case image presents an incidentally detected intramyocardial aneurysm in the left anterior descending artery of a 32-year-old man with atypical chest pain evaluated by multimodality imaging. The presence of an intramyocardial echo-free space may flag up this condition.

The evaluation of right ventricular function in patients with diabetes mellitus and significant stenosis at the proximal portion of the right coronary artery.

PURPOSE: Previous studies have indicated that right ventricular (RV) function is damaged in diabetes mellitus (DM); however, it is not clear whether in the presence of chronic ischemia, RV function is different between patients with and without DM (DM + and DM - , respectively). METHODS: This cross-sectional study enrolled 90 consecutive candidates for coronary artery bypass graft surgery and allocated them to 3 groups: 24 DM - patients with the absence of stenosis of more than 50% in the proximal and mid parts of the right coronary artery (the DM - RCA - group [control]), 33 DM - patients with the presence of significant stenosis (> 70%) in the proximal part of RCA (the DM - RCA + group), and 33 DM + patients with RCA + (the DM + RCA + group). RV function was evaluated based on longitudinal deformation markers, measured via the 2D speckle-tracking echocardiographic examination of right ventricular free wall (RVFW). RESULTS: The systolic strain value, systolic strain rate, and late diastolic strain rate of RVFW were not statistically significantly different between the three groups. Our adjusted post hoc analysis showed that the early diastolic strain rate of RVFW in the DM + RCA + group was lower than that in the DM - RCA + and DM - RCA - groups (1.5 s-1 ± 0.4 vs 1.7 s-1 ± 0.5 vs 1.7 s-1 ± 0.4). CONCLUSIONS: Diastolic function in the presence of DM was impaired irrespective of RCA - or RCA + . Additionally, RCA + had no effect on systolic and diastolic RV functions at rest in our DM - patients.