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Key Clinical Message: In this study, we introduced one of the rarest concomitants of the absence of left pulmonary artery (LPA), which was seen in our patient along with patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Abstract: Unilateral absence of pulmonary artery (UAPA) is a congenital heart disease in association with other abnormalities such as tetralogy of Fallot and septal defects or isolated in 30% of cases and occurs in the right lung in two thirds of cases. Our case is a 33-year-old man who was hospitalized with symptoms of cough, shortness of breath, and hemoptysis. The echocardiography revealed a large ventricular septal defect, absent left pulmonary artery, and severe pulmonary hypertension (PH) along with patent ductus arteriosus. These findings were confirmed by CT angiography. This association has rarely been found in past studies. Due to PH and pulmonary infection, the patient was treated with intravenous prostaglandin and antibiotics. However, in cases of timely diagnosis and treatment of UAPA, fatal complications such as pulmonary hypertension, morbidity, and mortality are reduced. This case emphasizes the importance of awareness of this abnormality and its associated anomalies to enable early diagnosis and treatment.
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Key Clinical Message: This case report presents an uncommon variant of Ebstein anomaly, where all three tricuspid valve leaflets exhibited apical displacement-a rare finding. It illustrates the complexities in diagnosing and managing such atypical presentations, with successful surgical correction through annuloplasty. The report adds valuable insights to the limited literature on this congenital heart disease. Abstract: Ebstein anomaly (EA), a rare congenital heart disorder, presents with diverse clinical spectrums. This case report explores a distinctive manifestation of EA, where all three tricuspid valve (TV) leaflets exhibited apical displacement, highlighting a novel aspect in the presentation of this condition. A 44-year-old woman, under long-term medical surveillance for EA, showcased an atypical clinical trajectory marked by the apical displacement of all TV leaflets, which is uncommon in EA. Despite a predominantly asymptomatic course, recent exacerbation of symptoms prompted further evaluation. Diagnostic modalities, including echocardiography and cardiac magnetic resonance imaging, revealed severe tricuspid regurgitation concomitant with unprecedented apical displacement of the anterior, septal, and posterior tricuspid leaflets. The displacement of the anterior leaflet was contrary to typical embryonic valvular formation expectations, indicating a unique presentation within EA. The patient underwent annuloplasty surgery, which successfully rectified the anomalous TV architecture. Postoperative evaluation demonstrated mild residual tricuspid regurgitation, and the patient was discharged in stable condition. This case underscores the variability in EA presentations and accentuates the significance of tailored surgical interventions. The observation of apical displacement involving all TV leaflets adds a unique dimension to the existing EA literature, reinforcing the need for careful diagnosis and personalized treatment approaches.
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BACKGROUND: Obesity is a known risk factor for atherosclerosis and cardiac disease. HYPOTHESIS: This study evaluated the effect of significant weight loss following bariatric surgery on myocardial deformation indices and right ventricular size (RV). This was a prospective cohort study. Morbid obese patients scheduled for bariatric surgery from July 2017 to February 2018 at Firoozgar Hospital were included in our study and referred for transthoracic echocardiography at Rajaie Cardiovascular Medical and Research Center. RESULTS: Thirty-four patients entered the study. The absolute value of global longitudinal strain (GLS) at baseline, 3, and 6 months after surgery was 17.42 ± 2.94%, 18.24 ± 3.09%, and 19.52 ± 2.78%, respectively, with a statistically significant difference from baseline to after six months (P value < 0.001). The absolute value of global circumferential strain (GCS) at baseline, 3, and 6 months after surgery was 20.14 ± 4.22%, 23.32 ± 4.66%, and 24.53 ± 4.52%, respectively, with statistically significant changes (P value < 0.001) from baseline to three months and from baseline to six months and no significant difference from three months to six months. A significant decrease was reported in mechanical dispersion of circumferential strain (38.05 ± 23.81-23.37 ± 20.86 ms, P value = 0.006) 6 months after surgery. Right ventricular size three- and six-month post-surgery showed a significant decrease relative to baseline echocardiography. CONCLUSIONS: Bariatric surgery could enhance cardiac function, as proven by 2D speckle echocardiography. Changes in RV size may be related to weight loss and should be considered when assessing patients who have undergone bariatric surgery.
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Background and Aims: ß-Thalassemia patients may have cardiac complications due to iron overload, which puts them at higher risk of cardiac complications induced by coronavirus disease 2019 (COVID-19) compared with the normal population. The present study aims to evaluate early cardiovascular complications following iron overload in ß-thalassemia patients who had early recovery from COVID-19 by cardiac magnetic resonance imaging (MRI) and feature-tracking technique. Methods: Thirty-two confirmed COVID-19-recovered ß-thalassemia cases were evaluated within 3 weeks to 3 months after a positive reverse-transcriptase polymerase chain reaction COVID-19 test. Both the heart and liver of all patients were examined using cardiac MRI. Results: We analyzed 32 patients with mean age of 32.84 ± 6.45 years at baseline. Left ventricular global strain values were significantly associated with myocardial T2*. A cut-off value of -15.08% for global longitudinal strain (GLS) with sensitivity and specificity of 90% and 61.1% (p = 0.017), 32.33% for global radial strain (GRS) with sensitivity and specificity of 80% and 94.4% (p = 0.001) and -16.21 for global circumferential strain (GCS), with sensitivity and specificity of 80% and 89.9% (p = 0.013) may indicate cardiac iron overload. Conclusion: GLS, GRS, and GCS were significantly decreased in patients with myocardial T2* <20 ms (iron overload), while no significant change was observed in the right and left ventricular ejection fraction (RV- and LVEF). Cardiac MRI feature-tracking may be helpful in the early detection of cardiac complications resulting from iron overload in ß-thalassemia patients who had early recovery from COVID-19.
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Heart failure caused by iron deposits in the myocardium is the primary cause of mortality in beta-thalassemia major patients. Cardiac magnetic resonance imaging (CMRI) T2* is the primary screening technique used to detect myocardial iron overload, but inherently bears some limitations. In this study, we aimed to differentiate beta-thalassemia major patients with myocardial iron overload from those without myocardial iron overload (detected by T2*CMRI) based on radiomic features extracted from echocardiography images and machine learning (ML) in patients with normal left ventricular ejection fraction (LVEF > 55%) in echocardiography. Out of 91 cases, 44 patients with thalassemia major with normal LVEF (> 55%) and T2* ≤ 20 ms and 47 people with LVEF > 55% and T2* > 20 ms as the control group were included in the study. Radiomic features were extracted for each end-systolic (ES) and end-diastolic (ED) image. Then, three feature selection (FS) methods and six different classifiers were used. The models were evaluated using various metrics, including the area under the ROC curve (AUC), accuracy (ACC), sensitivity (SEN), and specificity (SPE). Maximum relevance-minimum redundancy-eXtreme gradient boosting (MRMR-XGB) (AUC = 0.73, ACC = 0.73, SPE = 0.73, SEN = 0.73), ANOVA-MLP (AUC = 0.69, ACC = 0.69, SPE = 0.56, SEN = 0.83), and recursive feature elimination-K-nearest neighbors (RFE-KNN) (AUC = 0.65, ACC = 0.65, SPE = 0.64, SEN = 0.65) were the best models in ED, ES, and ED&ES datasets. Using radiomic features extracted from echocardiographic images and ML, it is feasible to predict cardiac problems caused by iron overload.
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Sobrecarga de Ferro , Talassemia , Disfunção Ventricular Esquerda , Talassemia beta , Humanos , Talassemia beta/complicações , Talassemia beta/diagnóstico por imagem , Volume Sistólico , Função Ventricular Esquerda , Talassemia/complicações , Talassemia/diagnóstico por imagem , Miocárdio , Ecocardiografia/métodos , Sobrecarga de Ferro/complicações , Sobrecarga de Ferro/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/complicaçõesRESUMO
Background: Thalassemia is the most common inherited anemia in worldwide. Heart failure is the most common cause of mortality and morbidity in patients with major and intermedia ß-thalassemia. This study aimed to evaluate the effect of oral administration of L-arginine on the improvement of systolic Pulmonary Artery Pressure (PAP) and cardiac function in patients with major and intermedia ß-thalassemia. Methods: This randomized clinical trial was done on 88 patients with ß-thalassemia admitted to Ali Asghar Hospital, Tehran, Iran between 2020 and 2021. Echocardiography was performed for all the patients before the intervention. Afterwards, the patients were randomly divided into two groups of placebo and L-arginine. The patients underwent echocardiography after eight weeks and were compared with respect to the results. Results: The mean blood transfusion interval was 20.21 d in the placebo group and 17.14 d in the L-arginine group (P=0.082). The results revealed no significant difference between the two groups regarding the mean levels of Hemoglobin (Hb) and ferritin, frequency of splenectomy. However, the mean PAP significantly decreased from 32.88 to 26.02 in the L-arginine group (P=0.009), but did not change in the placebo group. Nonetheless, no significant change was observed in the mean Ejection Fraction (EF) before and after L-arginine administration. Conclusion: L-arginine administration prevented the increase of PAP and was effective in preventing cardiovascular disorders including increased systolic PAP in patients with major and intermedia B-thalassemia. However, the results have to be confirmed in further studies with larger sample sizes.
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Accounting for 1.5% of thoracic trauma, blunt thoracic aortic injury (BTAI) is a rare disease with a high mortality rate that nowadays is treated mostly via thoracic endovascular aortic repair (TEVAR). Personalised computational models based on fluid-solid interaction (FSI) principals not only support clinical researchers in studying virtual therapy response, but also are capable of predicting eventual outcomes. The present work studies the variation of key haemodynamic parameters in a clinical case of BTAI after successful TEVAR, using a two-way FSI model. The three-dimensional (3D) patient-specific geometries of the patient were coupled with three-element Windkessel model for both prior and post intervention cases, forcing a correct prediction of blood flow over each section. Results showed significant improvement in velocity and pressure distribution after stenting. High oscillatory, low magnitude shear (HOLMES) regions require careful examination in future follow-ups, since thrombus formation was confirmed in some previously clinically reported cases of BTAI treated with TEVAR. The strength of swirling flows along aorta was also damped after stent deployment. Highlighting the importance of haemodynamic parameters in case-specific therapies. In future studies, compromising motion of aortic wall due to excessive cost of FSI simulations can be considered and should be based on the objectives of studies to achieve a more clinical-friendly patient-specific CFD model.
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Alarminas , Hemodinâmica , Humanos , Aorta , Correção Endovascular de Aneurisma , Movimento (Física)RESUMO
BACKGROUND: Although cardiac magnetic resonance (CMR) is the most reliable tool for assessment of CIO in patients with thalassemia, it is not always readily available. Recent studies have explored the potential of GLS as an alternative for diagnosis of CIO. We aimed to investigate the efficacy of global longitudinal strain (GLS) for detection of cardiac iron level (CIO). METHODS: We searched SCOPUS, MEDLINE, and Embase to identify the studies which used GLS for assessment of CIO. We searched for individual participant data (IPD) in eligible studies to perform ROC curve analysis. CMR with a T2* cut-off value of 20 ms was considered as the gold standard. A meta-analysis was performed and the risk of bias was assessed using the JBI Checklist. RESULTS: A total of 14 studies with 789 thalassemia patients (310 and 430 with and without CIO respectively and 49 with undetermined condition) were considered eligible for meta-analysis. IPDs of 405 participants were available. GLS was significantly lower in patients with CIO (-17.5 ± 2.7%) compared to those without CIO (-19.9 ± 2.3%; WMD = 1.6%, 95% CI = [0.76-2.4], p = 0.001, I2 = 77.1%) and to normal population (-20.61 ± 2.26%; WMD = 2.2%, 95% CI = [0.91-3.5], p = 0.001, I2 = 83.9%). A GLS < -19.5% could predict CIO with 92.8% sensitivity and 34.63% specificity (AUC = 0.659, 95% CI = [0.6-0.72], p-value < 0.0001). A GLS value < -6% has 100% positive predictive and ≥ -24.5% has 100% negative predictive values for detection of CIO. CONCLUSIONS: According to our study, GLS is a strong predictor of CIO and when CMR is not available, it may be a useful screening method for identification of CIO in thalassemia patients.
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Sobrecarga de Ferro , Talassemia , Coração , Humanos , Sobrecarga de Ferro/diagnóstico por imagem , Sobrecarga de Ferro/etiologia , Imageamento por Ressonância Magnética/métodos , Imagem Cinética por Ressonância Magnética/métodos , Estudos Observacionais como Assunto , Valor Preditivo dos Testes , Talassemia/complicações , Talassemia/diagnóstico , Função Ventricular EsquerdaRESUMO
Carcinoid heart disease is a rare condition that occurs in less than half of patients with carcinoid syndrome. The disease mainly affects right-sided heart valves; however, in 5%-10%, it can also involve left-sided valves. This case illustrates the most complicated form of the disease involving four heart valves.
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Background: Structural heart disease (SHD) has great impacts on healthcare systems, creating further public health concerns. Proper data are scant regarding the magnitude of the affected population by SHD. Objectives: This study aimed to determine the prevalence of SHD among children and adolescents in an Iranian population. Methods: In this population-based study, a multistage cluster-random sampling was used to choose schools from the Tehran urban area. All students were examined using a handheld Vscan device by echocardiographer, and the results were concurrently supervised and interpreted by cardiologists. All the major findings were reevaluated in hospital clinics. Results: Of 15,130 students (6-18 years, 52.2% boys) who were examined, the prevalence of individuals with congenital heart disease (CHD) and cardiomyopathy was 152 (10.046 per 1,000 persons) and 9 (0.595 per 1,000 persons), respectively. The prevalence of definite and borderline rheumatic heart disease (RHD) was 30 (2 per 1,000 persons) and 113 (7.5 per 1,000 persons), correspondingly. Non-rheumatic valvular heart disease (VHD) was also detected in 465 (30.7 per 1,000 persons) students. Of all the pathologies, only 39 (25.6%) cases with CHD and 1 (0.007%) cases with RHD had already been diagnosed. Parental consanguinity was the strongest predictor of CHD and SHD (odds ratio [OR]: 1.907, 95% CI, 1.358 to 2.680; P < 0.001 and OR, 1.855, 95% CI, 1.334 to 2.579; P < 0.001, respectively). The female sex (OR, 1.262, 95% CI, 1.013 to 1.573; P = 0.038) and fathers' low literacy (OR, 1.872, 95% CI, 1.068 to 3.281; P = 0.029) were the strongest predictors of non-rheumatic VHD and RHD, correspondingly. Conclusions: The implementation of echocardiographic examinations for detecting SHD among young population is feasible which detected SHD prevalence in our population comparable to previous reports. Further studies are required to delineate its economic aspects for community-based screening.
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Cardiopatias Congênitas , Cardiopatia Reumática , Adolescente , Criança , Ecocardiografia/métodos , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Irã (Geográfico)/epidemiologia , Masculino , Programas de Rastreamento/métodos , Prevalência , Cardiopatia Reumática/epidemiologia , Instituições AcadêmicasRESUMO
BACKGROUND: Patients with moderate-to-severe mitral stenosis (MS) have bee excluded from all major randomized controlled trials (RCTs) comparing non-vitamin K antagonist oral anticoagulants (NOACs) with warfarin in patients with atrial fibrillation (AF). METHODS AND RESULTS: In this pilot RCT, 40 patients were randomized to rivaroxaban 20 mg daily or warfarin. No patients experienced symptomatic ischemic strokes and systemic embolic events (the primary composite study outcome) during a 12-month follow-up. No major bleeding was reported. During the follow-up, 18.2% of patients in both groups showed echocardiographic signs of increased thrombogenicity in the left atrial appendage. The rate of silent cerebral ischemia was 13.3% in the rivaroxaban group and 17.6% in the warfarin group at brain magnetic resonance imaging. CONCLUSION: Our results suggest acceptable efficacy and safety for rivaroxaban in patients with AF and moderate-to-severe MS and are encouraging for larger RCTs in this so far neglected setting (NCT03926156).
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Fibrilação Atrial , Estenose da Valva Mitral , Acidente Vascular Cerebral , Administração Oral , Animais , Anticoagulantes/efeitos adversos , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico por imagem , Fibrilação Atrial/tratamento farmacológico , Humanos , Estenose da Valva Mitral/diagnóstico por imagem , Estenose da Valva Mitral/tratamento farmacológico , Projetos Piloto , Rivaroxabana/uso terapêutico , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controle , Resultado do Tratamento , Varfarina/uso terapêuticoRESUMO
AIMS: Thrombolysis is an alternative to surgery for mechanical prosthetic valve thrombosis (MPVT). Randomized clinical trials have yet to test the safety and efficacy of a proposed ultraslow thrombolytic infusion regimen. METHODS AND RESULTS: This single-centre, open-label, pilot randomized clinical trial randomized adult patients with acute obstructive MPVT to an ultraslow thrombolytic regimen [25 mg of recombinant tissue-type plasminogen activator (rtPA) infused in 25 h] and a fast thrombolytic regimen (50 mg of rtPA infused in 6 h). If thrombolysis failed, a repeated dose of 25 mg of rtPA for 6 h was administered in both groups up to a cumulative dose of 150 mg or the occurrence of a complication. The primary outcome was a complete MPVT resolution (>75% fall in the obstructive gradient by transthoracic echocardiography, <10° limitation in opening and closing valve motion angles by fluoroscopy, and symptom improvement). The key safety outcome was a Bleeding Academic Research Consortium type III or V major bleeding. Overall, 120 patients, including 63 (52.5%) women, at a mean age of 36.3 ± 15.3 years, were randomized. Complete thrombolysis success was achieved in 51 patients (85.0%) in the ultraslow-regimen group and 47 patients (78.3%) in the fast-regimen group [odds ratio 1.58; 95% confidence interval (CI) 0.25-1.63; P = 0.34]. One case of transient ischaemic attack and three cases of intracranial haemorrhage (absolute risk difference -6.6%; 95%CI -12% -0.3%; P = 0.07) were observed only in the fast-regimen group. CONCLUSION: The ultraslow thrombolytic regimen conferred a high thrombosis resolution rate without major complications. Such findings should be replicated in more adequately powered trials.
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Doenças das Valvas Cardíacas , Próteses Valvulares Cardíacas , Trombose , Adulto , Feminino , Fibrinolíticos/efeitos adversos , Doenças das Valvas Cardíacas/complicações , Próteses Valvulares Cardíacas/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Terapia Trombolítica/efeitos adversos , Trombose/diagnóstico , Trombose/tratamento farmacológico , Trombose/etiologia , Ativador de Plasminogênio Tecidual/efeitos adversos , Adulto JovemRESUMO
Primary cardiac lymphoma (PCL) is a rare primary cardiac neoplasm with a relatively poor prognosis despite confinement to the heart and/or pericardium. We report a 54-year-old man who had presented with complete heart block for which he had undergone permanent pacemaker implantation, and six months later he was referred to us for evaluation of progressive exertional dyspnea. Multislice spiral computed tomography angiography of the heart and major vasculature showed infiltrative tumoral lesion that has involved interatrial septum, right atrium, left atrium, and left ventricle with invasion into the pulmonary artery. Tissue samples were taken using uniportal video-assisted thoracoscopic surgery, and the histologic examination revealed diffuse large B-cell lymphoma. Chemoimmunotherapy was effective in inducing tumor regression and the patient was still in remission during the next six months after treatment.
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Neoplasias Cardíacas , Linfoma Difuso de Grandes Células B , Adulto , Dispneia , Átrios do Coração/patologia , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/etiologia , Bloqueio Cardíaco/terapia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/terapia , Humanos , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/terapia , Masculino , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
A 23-year-old woman with history of tetralogy of Fallot presented with new hypertension and constitutional symptoms first diagnosed as coartation of aorta based on primary imaging but that turned out to be Takayasu arteritis after more evaluation by laboratory data and complementary imaging modalities .
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OBJECTIVES: We sought to determine the cardiac magnetic resonance (CMR) indicators of intermediately to highly probable pulmonary hypertension (IHpPH) in patients with thalassemia referred for myocardial iron overload assessments to prevent further cardiac complications. METHODS: The study population consisted of 152 patients with thalassemia (major or intermedia) (49.3% women, mean age = 33 ± 10.1 years) who underwent non-contrast CMR and echocardiographic examinations on the same day. Functional, T2*, and global strain parameters via a feature-tracking method were extracted from CMR. The probability of PH was defined based on the tricuspid regurgitation velocity and echocardiographic parameters. The catheterization-derived hemodynamic data of patients with moderate to high probable PH was registered. RESULTS: Twenty-two (14.5%) patients suffered from IHpPH. The multivariate logistic regression analysis revealed that the right ventricular end-systolic volume index (RVESVI) was the strongest of all the CMR parameters for the prediction of IHpPH (OR: 1.044, 95% CI: 1.021-1.067). The other powerful IHpPH predictor was age (OR: 1.066, 95% CI: 1.009-1.126). A cutoff point of greater than 47 ml for RVESVI (AUC: .801, 95% CI: .728-.861) was found to predict IHpPH with 73.91% sensitivity and 70.31% specificity. The single most robust CMR-derived strain parameter for IHpPH prediction was the right ventricular global longitudinal strain (OR: .887, 95% CI: .818-.961). A p value of less than 0.05 was considered significant. CONCLUSIONS: Both CMR functional and global strain parameters were strong predictors of IHpPH in our patients with thalassemia.
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Hipertensão Pulmonar , Talassemia beta , Adulto , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico por imagem , Imageamento por Ressonância Magnética , Imagem Cinética por Ressonância Magnética , Masculino , Valor Preditivo dos Testes , Adulto JovemRESUMO
Ventricular septal rupture (VSR) is an ominous mechanical complication of acute myocardial infarction (MI) accompanied with a poor prognosis. Transcatheter closure (TCC) of VSR has been proposed as an alternative approach for surgery. This study presents a 79-year-old man with diabetes mellitus, hypertension, dyslipidemia, and chronic stable angina with 3-vessel coronary artery disease, who had refused a coronary artery bypass graft. He complained of orthopnea and dyspnea of New York Heart Association (NYHA) function class III after the recent neglected inferior MI. Transthoracic echocardiography revealed moderate left ventricular (LV) systolic dysfunction, true aneurysm formation at the base of the inferoseptal wall, as well as a large-sized (12 mm) VSR at the posterior aspect of basal inferoseptal segment with significant left to right shunt and a peak systolic gradient of 50 mm Hg at the VSR site. Given the high risk profile for surgery and patient refusal, he was a candidate for TCC of VSR and staged multi-vessel percutaneous coronary intervention (PCI). A 30 mm Figulla atrial septal defect (ASD) occluder device was chosen and successfully deployed at the VSR site with minimal residual shunt. A month later, successful multi-vessel PCI was performed with good procedural and clinical outcomes on the 6-month follow-up.
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Aneurisma Cardíaco/complicações , Intervenção Coronária Percutânea/métodos , Dispositivo para Oclusão Septal , Ruptura do Septo Ventricular/cirurgia , Idoso , Doença da Artéria Coronariana/cirurgia , Ecocardiografia , Aneurisma Cardíaco/diagnóstico por imagem , Humanos , Masculino , Infarto do Miocárdio/complicações , Ruptura do Septo Ventricular/diagnóstico por imagemRESUMO
Background: The frequency of left ventricular diastolic dysfunction (DD) is overestimated by earlier recommendations. We compared the 2009 and 2016 guidelines regarding the detection of DD and explored the potential of adding left atrial (LA) strain to the current guideline. Methods: Consecutive patients with heart failure were enrolled. All the patients were examined using 2-dimensional speckle-tracking echocardiography (2D-STE) and tissue Doppler imaging. DD was evaluated in terms of E/e', e' velocity, E, A, tricuspid regurgitation velocity, LA volume, and LA strain. Results: This study evaluated 147 patients (101 males, 68.7%) at a mean age of 54.73±14.42 years. LA strain decreased with increasing grades of DD in both guidelines. The rate of reclassification between the 2 guidelines was 41%. The detection rate of normal diastolic function increased after the implementation of the 2016 guideline. LA strain discriminated individuals with normal diastolic function from those with DD more accurately than did LA volume index (area under the curve [AUC] =0.816 vs AUC=0.759, respectively). When LA strain <23% was incorporated into the 2016 guideline, 2 out of 4 patients with indeterminate diastolic function were reclassified as normal and 2 patients as grade I DD. The rate of reclassification was 4.1% after the addition of LA strain to the current guideline (κ=0.939, P<0.001). Conclusion: This study showed that the current guideline detected lower rates of DD than did the earlier recommendations. Furthermore, the incorporation of LA strain into the current guideline resulted in lower rates of indeterminate diastolic function.
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BACKGROUND: Iron accumulation leads to increased susceptibility to cardiovascular diseases in thalassemia major (TM) patients. Depressed heart rate variability (HRV) and development of arrhythmia are among the manifestations of subclinical cardiac involvement in TM cases. Determination of subclinical cardiac involvement is essential for preventive measures. Thus, we aimed to evaluate the best method for identification of subclinical cardiac dysfunction in TM cases. MATERIALS AND METHODS: In this prospective study, 45 TM and 45 non-TM cases, who were referred for cardiac evaluation, were enrolled. Exclusion criteria included non-sinus rhythm and overt cardiac disease. TM cases underwent cardiac MRI, electrocardiography (ECG), and Holter monitoring. TM cases were divided into two groups of normal versus iron overload with a cardiac T2* of more or less than 20 ms, respectively. The non-TM cases underwent only ECG and Holter monitoring. RESULTS: We observed no significant difference regarding HRV between normal versus iron overload TM and non-TM cases. Higher rates of premature atrial complex, low limb voltage, low atrial rhythm, as well as minimum and average HR with lower QRS duration and PR interval were detected in TM versus non-TM cases (p value <0.05). CONCLUSIONS: We observed a higher prevalence of low limb voltage and low atrial rhythm in TM cases versus non-TM cases. Indeed, the role of fragmented QRS (fQRS) for subclinical detection of cardiac disease in TM cases is still so controversial and needs more evaluation. Application of HRV and fQRS in this regard may need to be performed at the right time point after initiation of blood transfusion, but this needs to be determined.
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AIMS: Hypokinetic non-dilated cardiomyopathy (HNDC), which is determined by impaired left ventricular (LV) systolic function despite normal LV size, has been categorized as a subgroup of dilated cardiomyopathy (DCM) spectrum. Lack of data regarding advanced echocardiographic data in this population motivated us to design the present study in order to assess LV myocardial deformation properties of HNDC patients against the ones with dilated left ventricle. METHODS AND RESULTS: Thirty-one HNDC patients and 23 DCM patients were enrolled in the study consecutively. Myocardial deformation parameters including global longitudinal strain, global circumferential strain, LV basal and apical rotation, LV twist, and LV mechanical dispersion were obtained with the use of two-dimensional speckle tracking-based methods in all patients. Left cardiac chamber volume was also measured using three-dimensional HeartModel application. Patients with enlarged left ventricle tend to have lower LV ejection fraction. Comparing with HNDC group, DCM patients showed worse global circumferential strain (coefficient ± standard error 3.59 ± 0.94, P < 0.001) and LV mechanical dispersion (coefficient ± standard error 16.46 ± 7.09, P = 0.02) after regression analysis, while neither the global longitudinal strain nor the LV twist was not significantly different between two study population. CONCLUSIONS: Left ventricular enlargement has a substantial effect on the circumferential strain and mechanical dispersion more than other deformation parameters that may play a role in the assumed poor prognosis of heart failure patients with dilated left ventricle.