The automatic localization of the left ventricle(LV)in short-axis magnetic resonance(MR)images is a required step to process cardiac images using convolutional neural networks for the extraction of a region of interes...The automatic localization of the left ventricle(LV)in short-axis magnetic resonance(MR)images is a required step to process cardiac images using convolutional neural networks for the extraction of a region of interest(ROI).The precise extraction of the LV’s ROI from cardiac MRI images is crucial for detecting heart disorders via cardiac segmentation or registration.Nevertheless,this task appears to be intricate due to the diversities in the size and shape of the LV and the scattering of surrounding tissues across different slices.Thus,this study proposed a region-based convolutional network(Faster R-CNN)for the LV localization from short-axis cardiac MRI images using a region proposal network(RPN)integrated with deep feature classification and regression.Themodel was trained using images with corresponding bounding boxes(labels)around the LV,and various experiments were applied to select the appropriate layers and set the suitable hyper-parameters.The experimental findings showthat the proposed modelwas adequate,with accuracy,precision,recall,and F1 score values of 0.91,0.94,0.95,and 0.95,respectively.This model also allows the cropping of the detected area of LV,which is vital in reducing the computational cost and time during segmentation and classification procedures.Therefore,itwould be an ideal model and clinically applicable for diagnosing cardiac diseases.展开更多
Background: The differential diagnosis for intracavitary cardiac masses is limited, typically including vegetations, tumors, or thrombi. Cardiac thrombi can often mimic cardiac tumors on imaging, creating a diagnostic...Background: The differential diagnosis for intracavitary cardiac masses is limited, typically including vegetations, tumors, or thrombi. Cardiac thrombi can often mimic cardiac tumors on imaging, creating a diagnostic challenge. Primary cardiac tumors are rare and usually benign, whereas most cardiac tumors result from metastases, commonly originating from malignancies in the breast, lung, or melanoma. Aim: This report highlights the importance of distinguishing various cardiac masses based on clinical presentations, clinical courses, and radiological features. Case Presentation: We describe two cases of cardiac masses with unique and diverse clinical features. Each case posed significant diagnostic challenges due to their distinct presentations and clinical progressions. Conclusion: These cases underscore the importance of considering both benign and metastatic origins in the differential diagnosis of intracavitary cardiac masses. Accurate differentiation between thrombi and tumors is crucial for appropriate management and treatment.展开更多
BACKGROUND Li-Fraumeni syndrome(LFS)is a rare autosomal dominant cancer-predisposing syndrome,which can manifest as a polymorphic spectrum of malignancies.LFS is associated with an early onset in life,with the majorit...BACKGROUND Li-Fraumeni syndrome(LFS)is a rare autosomal dominant cancer-predisposing syndrome,which can manifest as a polymorphic spectrum of malignancies.LFS is associated with an early onset in life,with the majority of cases occurring prior to the age of 46.Notwithstanding the infrequency of primary cardiac tumors,it behooves clinicians to remain vigilant in considering the differential diagnosis of such tumors in LFS patients who present with a cardiac mass.This is due to the markedly elevated risk for malignancy in this particular population,far surpassing that of the general populace.CASE SUMMARY Herein,we present a case of a 30-year-old female with LFS who was found to have a tricuspid valve leaflet mass.CONCLUSION This case exemplifies valuable learning points in the diagnostic approach for this exceptionally rare patient population.展开更多
Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosy...Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosylceramide (Gb3). This leads to cellular dysfunction in various organs, with cardiovascular compromise being the major cause of morbidity and mortality. This study aimed to provide a comprehensive overview of FD focusing on its genetic, epidemiological, clinical, diagnostic, and therapeutic aspects. This study explored the genetic mutations associated with FD, its epidemiology, clinical phenotypes, cardiac manifestations, diagnostic approaches, and current treatment options. Background: FD is caused by mutations in GLA on the X chromosome, with over 1000 identified variants. Neonatal screening and specific studies have shown an increased incidence of FD. The clinical presentation varies between classic and late phenotypes, with cardiac involvement being a major concern, particularly in late-onset FD. Purpose: This study aimed to summarize the current knowledge on FD, emphasizing cardiac involvement, diagnostic modalities, and treatment options. Methods: A literature review of relevant studies on FD, including genetics, epidemiology, clinical presentation, diagnostic methods, and treatment options, was conducted. Results: Cardiac manifestations of FD included left ventricular hypertrophy (LVH), heart failure, arrhythmias, and sudden death. Diagnostic approaches such as electrocardiography, echocardiography, and cardiac magnetic resonance imaging play crucial roles in the early detection and monitoring of cardiac involvement. Enzyme replacement therapy (ERT) and emerging treatments have shown promise in managing FD, although challenges remain. Conclusions: FD remains a challenging condition in cardiology, with under-diagnosis being a concern. Early detection and specific therapy are essential to improve patient outcomes. Echocardiography and cardiac MRI are valuable tools for diagnosis and follow-up. Despite the advances in treatment, accessibility remains an issue. More research is needed to deepen our understanding of FD and to improve therapeutic strategies.展开更多
It is widely accepted that the heart current source can be reduced into a current multipole. By adopting three linear inverse methods, the cardiac magnetic imaging is achieved in this article based on the current mult...It is widely accepted that the heart current source can be reduced into a current multipole. By adopting three linear inverse methods, the cardiac magnetic imaging is achieved in this article based on the current multipole model expanded to the first order terms. This magnetic imaging is realized in a reconstruction plane in the centre of human heart, where the current dipole array is employed to represent realistic cardiac current distribution. The current multipole as testing source generates magnetic fields in the measuring plane, serving as inputs of cardiac magnetic inverse problem. In the heart-torso model constructed by boundary element method, the current multipole magnetic field distribution is compared with that in the homogeneous infinite space, and also with the single current dipole magnetic field distribution. Then the minimum-norm least-squares (MNLS) method, the optimal weighted pseudoinverse method (OWPIM), and the optimal constrained linear inverse method (OCLIM) are selected as the algorithms for inverse computation based on current multipole model innovatively, and the imaging effects of these three inverse methods are compared. Besides, two reconstructing parameters, residual and mean residual, are also discussed, and their trends under MNLS, OWPIM and OCLIM each as a function of SNR are obtained and compared.展开更多
This paper discusses the forward and inverse problem for cardiac magnetic fields and electric potentials. A torso-heart model established by boundary element method (BEM) is used for studying the distributions of ca...This paper discusses the forward and inverse problem for cardiac magnetic fields and electric potentials. A torso-heart model established by boundary element method (BEM) is used for studying the distributions of cardiac magnetic fields and electric potentials. Because node-to-node and triangle-to-triangle BEM can lead to discrepant field distributions, their properties and influences are compared. Then based on constructed torso-heart model and supposed current source functional model-current dipole array, the magnetic and electric imaging by optimal constrained linear inverse method are applied at the same time. Through figure and reconstructing parameter comparison, though the magnetic current dipole array imaging possesses better reconstructing effect, however node-to-node BEM and triangleto-triangle BEM make little difference to magnetic and electric imaging.展开更多
Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies.Echocardiography remains the first-line imaging tool, and when d...Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies.Echocardiography remains the first-line imaging tool, and when disease is suspected on echocardiography, cardiac magnetic resonance imaging and nuclear imaging play critical roles in the non-invasive diagnosis and evaluation of cardiac amyloidosis. Advances in multi-modality cardiac imaging allowing earlier diagnosis and initiation of novel therapies have significantly improved the outcomes in these patients. Cardiac imaging also plays important roles in the risk stratification of patients presenting with cardiac amyloidosis. In the current review, we provide a clinical and imaging focused update, and importantly outline the imaging protocols, diagnostic and prognostic utility of multimodality cardiac imaging in the assessment of cardiac amyloidosis.展开更多
Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years.Different types of amyloidosis can affect the heart.Transthyretin a...Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years.Different types of amyloidosis can affect the heart.Transthyretin amyloidosis and light chain amyloidosis are the two most common types of cardiac amyloidosis.These entities have a poor prognosis,so accurate diagnostic techniques are imperative for determining an early therapeutic approach.Recent advances in cardiac imaging and diagnostic strategies show that these tools are safe and can avoid the use of invasive diagnostic techniques to histological confirmation,such as endomyocardial biopsy.We performed a review on the diagnostic and prognostic implications of different cardiac imaging techniques in cardiac amyloidosis.We mainly focus on reviewing echocardiography,cardiac magnetic resonance,computed tomography and nuclear imaging techniques and the different safety measurements that can be done with each of them.展开更多
Non-invasive cardiac imaging has explored enormous advances in the last few decades.In particular,hybrid imaging represents the fusion of information from multiple imaging modalities,allowing to provide a more compreh...Non-invasive cardiac imaging has explored enormous advances in the last few decades.In particular,hybrid imaging represents the fusion of information from multiple imaging modalities,allowing to provide a more comprehensive dataset compared to traditional imaging techniques in patients with cardiovascular diseases.The complementary anatomical,functional and molecular information provided by hybrid systems are able to simplify the evaluation procedure of various pathologies in a routine clinical setting.The diagnostic capability of hybrid imaging modalities can be further enhanced by introducing novel and specific imaging biomarkers.The aim of this review is to cover the most recent advancements in radiotracers development for SPECT/CT,PET/CT,and PET/MRI for cardiovascular diseases.展开更多
There is a growing evidence of cardiovascular complications in coronavirusdisease 2019 (COVID-19) patients. As evidence accumulated of COVID-19 mediatedinflammatory effects on the myocardium, substantial attention has...There is a growing evidence of cardiovascular complications in coronavirusdisease 2019 (COVID-19) patients. As evidence accumulated of COVID-19 mediatedinflammatory effects on the myocardium, substantial attention has beendirected towards cardiovascular imaging modalities that facilitate this diagnosis.Cardiac magnetic resonance imaging (CMRI) is the gold standard for thedetection of structural and functional myocardial alterations and its role inidentifying patients with COVID-19 mediated cardiac injury is growing. Despiteits utility in the diagnosis of myocardial injury in this population, CMRI’s impacton patient management is still evolving. This review provides a framework for theuse of CMRI in diagnosis and management of COVID-19 patients from theperspective of a cardiologist. We review the role of CMRI in the management ofboth the acutely and remotely COVID-19 infected patient. We discuss patientselection for this imaging modality;T1, T2, and late gadolinium enhancementimaging techniques;and previously described CMRI findings in other cardiomyopathieswith potential implications in COVID-19 recovered patients.展开更多
Despite optimal interventional and medical therapy, ischemic heart disease is still an important cause of morbidity and mortality worldwide. Although not included in standard of care rehabilitation, stem cell therapy(...Despite optimal interventional and medical therapy, ischemic heart disease is still an important cause of morbidity and mortality worldwide. Although not included in standard of care rehabilitation, stem cell therapy(SCT) could be a solution for prompting cardiac regeneration. Multiple studies have been published from the beginning of SCT until now, but overall no unanimous conclusion could be drawn in part due to the lack of appropriate endpoints. In order to appreciate the impact of SCT, multiple markers from different categories should be considered: Structural, biological, functional, physiological, but also major adverse cardiac events or quality of life. Imaging end-points are among the most used-especially left ventricle ejection fraction(LVEF) measured through different methods. Other imaging parameters are infarct size, myocardial viability and perfusion. The impact of SCT on all of the aforementioned end-points is controversial and debatable. 2 D-echocardiography is widely exploited, but new approaches such as tissue Doppler, strain/strain rate or 3 D-echocardiography are more accurate, especially since the latter one is comparable with the MRI gold standard estimation of LVEF. Apart from the objective parameters, there are also patient-centered evaluations to reveal the benefits of SCT, such as quality of life and performance status, the most valuable from the patient point of view. Emerging parameters investigating molecular pathways such as non-coding RNAs or inflammation cytokines have a high potential as prognostic factors. Due to the disadvantages of current techniques, new imaging methods with labelled cells tracked along their lifetime seem promising, but until now only pre-clinical trials have been conducted in humans. Overall, SCT is characterized by high heterogeneity not only in preparation, administration and type of cells, but also in quantification of therapy effects.展开更多
There is increasing evidence on the utility of cardiac computed tomography(CCT)in infective endocarditis(IE)to investigate the valvular pathology,the extracardiac manifestations of IE and pre-operative planning.CCT ca...There is increasing evidence on the utility of cardiac computed tomography(CCT)in infective endocarditis(IE)to investigate the valvular pathology,the extracardiac manifestations of IE and pre-operative planning.CCT can assist in the diagnosis of perivalvular complications,such as pseudoaneurysms and abscesses,and can help identify embolic events to the lungs or systemic vasculature.CCT has also been shown to be beneficial in the pre-operative planning of patients by delineating the coronary artery anatomy and the major cardiovascular structures in relation to the sternum.Finally,hybrid nuclear/computed tomography techniques have been shown to increase the diagnostic accuracy in prosthetic valve endocarditis.This manuscript aims to provide a contemporary update of the existing evidence base for the use of CCT in IE.展开更多
BACKGROUND We report a patient who was diagnosed with toxic myopericarditis secondary to hydrocarbon abuse using cardiac magnetic resonance imaging(CMR).CASE SUMMARY A 25-year-old male presented to emergency departmen...BACKGROUND We report a patient who was diagnosed with toxic myopericarditis secondary to hydrocarbon abuse using cardiac magnetic resonance imaging(CMR).CASE SUMMARY A 25-year-old male presented to emergency department with chest pain for 3 d.Patient also reported sniffing hydrocarbon containing inhalant for the last 1 year.Labs showed elevated troponin and electrocardiography was suggestive of acute pericarditis.Echocardiogram showed left ventricular(LV)ejection fraction(EF)of 40%.Given patient’s troponin elevation and reduced EF,cardiac catheterization was performed which showed normal coronaries.CMR was performed for myocardial infarction with non-obstructive coronary arteries evaluation.CMR showed borderline LV function with edema in mid and apical LV suggestive of myocarditis.CONCLUSION CMR can be used to diagnose toxic myopericarditis secondary to hydrocarbon abuse.展开更多
Cardiac magnetic resonance imaging(MRI)is an evolving technology,proving to be a highly accurate tool for quantitative assessment.Most recently,it has been increasingly used in the diagnostic and prognostic evaluation...Cardiac magnetic resonance imaging(MRI)is an evolving technology,proving to be a highly accurate tool for quantitative assessment.Most recently,it has been increasingly used in the diagnostic and prognostic evaluation of conditions involving an elevation in troponin or troponinemia.Although an elevation in troponin is a nonspecific marker of myocardial tissue damage,it is a frequently ordered investigation leaving many patients without a specific diagnosis.Fortunately,the advent of newer cardiac MRI protocols can provide additional information.In this review,we discuss several conditions associated with an elevation in troponin such as myocardial infarction,myocarditis,Takotsubo cardiomyopathy,coronavirus disease 2019 related cardiac dysfunction and athlete’s heart syndrome.展开更多
Objective:To investigate the correlation of cardiac magnetic resonance imaging(MRI)in the assessment of myocardial activity in patients with myocardial infarction and the outcome of cardiac function after PCI.Methods:...Objective:To investigate the correlation of cardiac magnetic resonance imaging(MRI)in the assessment of myocardial activity in patients with myocardial infarction and the outcome of cardiac function after PCI.Methods:30 patients with myocardial infarction(MI)who had complete clinical and imaging data from July 2016 to December 2018 after PCI were analyzed retrospectively.MR and echocardiogram were performed before and 6 months after PCI,and the parameters related to left ventricular function were measured by post-processing software of MRI workstation.The left ventricular transmural degree of CMR late gadolinium enhancement was compared with the left ventricular wall motion degree of 6-month echocardiography as a standard to judge the viable myocardium.Result:There were 193 left ventricular segmental abnormalities in 30 cases,including 121 viable myocardium and 72 non viable myocardium in CMR-LGE before operation.Six months after PCI,echocardiography showed that 125 of 193 abnormal segments of left ventricle detected by CMR-LGE before PCI were viable myocardium and 68 were non viable myocaridium.The sensitivity and specificity of CMR-LGE to determinate of viable myocardium were 92.0%and 91.1%respectively.The larger the non-viable myocardial area of the left ventricular wall,the worse the recovery of wall motion ability,and there was a negative correlation between them(r=0.416,P<0.05).The first-pass perfusion time in CMR-LEG region was significantly longer than that in normal myocardial region(4.85(+)1.51)s and(3.79(+)1.73)s,respectively.The difference was statistically significant(t=5.191,P<0.05).Conclusion:MRI can evaluate the myocardial activity of myocardial infarction,reflect the range of viable myocardium,and provide imaging basis for clinical treatment and prognosis.展开更多
Left ventricular isolated hypoplasia is a seldom-described cardiac abnormality.Right ventricular hypoplasia is usually associated with congenital anomalies of the pulmonary or the tricuspid valve,whereas biventricular...Left ventricular isolated hypoplasia is a seldom-described cardiac abnormality.Right ventricular hypoplasia is usually associated with congenital anomalies of the pulmonary or the tricuspid valve,whereas biventricular isolated apical hypoplasia has never been described.We report the case of a 48-year-old man with no history of known cardiac disease who was found to have a complex cardiac abnormality characterized by:1)Deficiency of the myocardium within the biventricular apex with adipose tissue infiltration;2)Truncated right ventricle because of an absent trabecular portion of the inflow tract;3)Truncated and spherical left ventricular apex;4)Origin of the mitral papillary muscle in the flattened anterior left ventricular apex.Multimodality imaging was performed to delineate the morphological and functional characteristics of this cardiomyopathy fully.To the best of our knowledge,this is the first description of a new cardiac abnormality characterized by the hypoplasia of the apical region of both ventricles in the absence of valvular or coronary artery disease.展开更多
BACKGROUND Isolated left ventricular apical hypoplasia(ILVAH),also known as truncated left ventricle(LV),is a very unusual cardiomyopathy.It is characterised by a truncated,spherical,and non-apex forming LV.The true a...BACKGROUND Isolated left ventricular apical hypoplasia(ILVAH),also known as truncated left ventricle(LV),is a very unusual cardiomyopathy.It is characterised by a truncated,spherical,and non-apex forming LV.The true apex is occupied by the right ventricle.Due to the rarity of the disease,just a few case reports and limited case series have been published in the field.AIM To analysing the so far 37 reported ILVAH cases worldwide.METHODS The electronic databases PubMed and Scopus were investigated from their establishment up to December 13,2022.RESULTS The majority of cases reported occurred in males(52.7%).Mean age at diagnosis was 26.1±19.6 years.More than a third of the patients were asymptomatic(35.1%).The most usual clinical presentation was breathlessness(40.5%).The most commonly detected electrocardiogram changes were T wave abnormalities(29.7%)and right axis deviation with poor R wave progression(24.3%).Atrial fibrillation/flutter was detected in 24.3%.Echocardiography was performed in 97.3%of cases and cardiac MRI in 91.9%of cases.Ejection fraction was reduced in more than a half of patients(56.7%).An associated congenital heart disease was found in 16.2%.Heart failure therapy was administered in 35.1%of patients.The outcome was favorable in the vast majority of patients,with just one death.CONCLUSION ILVAH is a multifaceted entity with a so far unpredictable course,ranging from benign until the elderly to sudden death during adolescence.展开更多
Artificial intelligence(AI)is the process of having a computational program that can perform tasks of human intelligence by mimicking human thought processes.AI is a rapidly evolving transdisciplinary field which inte...Artificial intelligence(AI)is the process of having a computational program that can perform tasks of human intelligence by mimicking human thought processes.AI is a rapidly evolving transdisciplinary field which integrates many elements to develop algorithms that aim to simulate human intuition,decision-making,and object recognition.The overarching aims of AI in cardiovascular medicine are threefold:To optimize patient care,improve efficiency,and improve clinical outcomes.In cardiology,there has been a growth in the potential sources of new patient data,as well as advances in investigations and therapies,which position the field well to uniquely benefit from AI.In this editorial,we highlight some of the main research priorities currently and where the next steps are heading us.展开更多
Background:Ventricular crypts are quite a common finding during cardiac imaging,but their etiology is unclear.A possible final result of a spontaneous ventricular septal defect closure has been supposed but never inve...Background:Ventricular crypts are quite a common finding during cardiac imaging,but their etiology is unclear.A possible final result of a spontaneous ventricular septal defect closure has been supposed but never investigated in earlier studies.Method:From January 1997 to December 2020,all newborns diagnosed to have a ventricular septal defect were prospectively entered in our database and those with an isolated defect were included in the study.Ventricular septal defects were classified into four types:perimembranous,trabecular muscular,inlet and outlet.A long-term follow up was performed in order to visualize the possible residual formation of a septal myocardial crypt.Results:A total of 376 isolated ventricular septal defects(314 muscular and 54 perimembranous,4 inlet,4 outlet)were detected.Follow up ranged from 1 to 23 years and showed that,among muscular type,a spontaneous closure occurred in 284(91%),26 did not close(8,28%),2 required surgical intervention(0,63%),3 were lost at follow up(0,95%).During this period,after spontaneous defect closure closure,20 crypts were found(6,4%).Conclusion:This study shows that a muscular ventricular septal defect may evolve in the 6.4%of cases in a residual septal crypt.Although septal crypts occur more frequently in patients affected by hypertrophic and hypertensive cardiomyopathy,they may also represent the evolution of a spontaneous closure of a muscular interventricular defect.展开更多
基金supported by the Ministry of Higher Education(MOHE)through the Fundamental Research Grant Scheme(FRGS)(FRGS/1/2020/TK0/UTHM/02/16)the Universiti Tun Hussein Onn Malaysia(UTHM)through an FRGS Research Grant(Vot K304).
文摘The automatic localization of the left ventricle(LV)in short-axis magnetic resonance(MR)images is a required step to process cardiac images using convolutional neural networks for the extraction of a region of interest(ROI).The precise extraction of the LV’s ROI from cardiac MRI images is crucial for detecting heart disorders via cardiac segmentation or registration.Nevertheless,this task appears to be intricate due to the diversities in the size and shape of the LV and the scattering of surrounding tissues across different slices.Thus,this study proposed a region-based convolutional network(Faster R-CNN)for the LV localization from short-axis cardiac MRI images using a region proposal network(RPN)integrated with deep feature classification and regression.Themodel was trained using images with corresponding bounding boxes(labels)around the LV,and various experiments were applied to select the appropriate layers and set the suitable hyper-parameters.The experimental findings showthat the proposed modelwas adequate,with accuracy,precision,recall,and F1 score values of 0.91,0.94,0.95,and 0.95,respectively.This model also allows the cropping of the detected area of LV,which is vital in reducing the computational cost and time during segmentation and classification procedures.Therefore,itwould be an ideal model and clinically applicable for diagnosing cardiac diseases.
文摘Background: The differential diagnosis for intracavitary cardiac masses is limited, typically including vegetations, tumors, or thrombi. Cardiac thrombi can often mimic cardiac tumors on imaging, creating a diagnostic challenge. Primary cardiac tumors are rare and usually benign, whereas most cardiac tumors result from metastases, commonly originating from malignancies in the breast, lung, or melanoma. Aim: This report highlights the importance of distinguishing various cardiac masses based on clinical presentations, clinical courses, and radiological features. Case Presentation: We describe two cases of cardiac masses with unique and diverse clinical features. Each case posed significant diagnostic challenges due to their distinct presentations and clinical progressions. Conclusion: These cases underscore the importance of considering both benign and metastatic origins in the differential diagnosis of intracavitary cardiac masses. Accurate differentiation between thrombi and tumors is crucial for appropriate management and treatment.
文摘BACKGROUND Li-Fraumeni syndrome(LFS)is a rare autosomal dominant cancer-predisposing syndrome,which can manifest as a polymorphic spectrum of malignancies.LFS is associated with an early onset in life,with the majority of cases occurring prior to the age of 46.Notwithstanding the infrequency of primary cardiac tumors,it behooves clinicians to remain vigilant in considering the differential diagnosis of such tumors in LFS patients who present with a cardiac mass.This is due to the markedly elevated risk for malignancy in this particular population,far surpassing that of the general populace.CASE SUMMARY Herein,we present a case of a 30-year-old female with LFS who was found to have a tricuspid valve leaflet mass.CONCLUSION This case exemplifies valuable learning points in the diagnostic approach for this exceptionally rare patient population.
文摘Fabry disease (FD) is a rare X-linked lysosomal accumulation disorder caused by a deficiency in the enzyme alpha-galactosidase A (Gal A), resulting in excessive storage of glycosphingolipids, particularly globotriaosylceramide (Gb3). This leads to cellular dysfunction in various organs, with cardiovascular compromise being the major cause of morbidity and mortality. This study aimed to provide a comprehensive overview of FD focusing on its genetic, epidemiological, clinical, diagnostic, and therapeutic aspects. This study explored the genetic mutations associated with FD, its epidemiology, clinical phenotypes, cardiac manifestations, diagnostic approaches, and current treatment options. Background: FD is caused by mutations in GLA on the X chromosome, with over 1000 identified variants. Neonatal screening and specific studies have shown an increased incidence of FD. The clinical presentation varies between classic and late phenotypes, with cardiac involvement being a major concern, particularly in late-onset FD. Purpose: This study aimed to summarize the current knowledge on FD, emphasizing cardiac involvement, diagnostic modalities, and treatment options. Methods: A literature review of relevant studies on FD, including genetics, epidemiology, clinical presentation, diagnostic methods, and treatment options, was conducted. Results: Cardiac manifestations of FD included left ventricular hypertrophy (LVH), heart failure, arrhythmias, and sudden death. Diagnostic approaches such as electrocardiography, echocardiography, and cardiac magnetic resonance imaging play crucial roles in the early detection and monitoring of cardiac involvement. Enzyme replacement therapy (ERT) and emerging treatments have shown promise in managing FD, although challenges remain. Conclusions: FD remains a challenging condition in cardiology, with under-diagnosis being a concern. Early detection and specific therapy are essential to improve patient outcomes. Echocardiography and cardiac MRI are valuable tools for diagnosis and follow-up. Despite the advances in treatment, accessibility remains an issue. More research is needed to deepen our understanding of FD and to improve therapeutic strategies.
基金Project supported by the State Key Development Program for Basic Research of China(Grant No.2006CB601007)the National Natural Science Foundation of China(Grant No.10674006)the National High Technology Research and Development Program of China(Grant No.2007AA03Z238)
文摘It is widely accepted that the heart current source can be reduced into a current multipole. By adopting three linear inverse methods, the cardiac magnetic imaging is achieved in this article based on the current multipole model expanded to the first order terms. This magnetic imaging is realized in a reconstruction plane in the centre of human heart, where the current dipole array is employed to represent realistic cardiac current distribution. The current multipole as testing source generates magnetic fields in the measuring plane, serving as inputs of cardiac magnetic inverse problem. In the heart-torso model constructed by boundary element method, the current multipole magnetic field distribution is compared with that in the homogeneous infinite space, and also with the single current dipole magnetic field distribution. Then the minimum-norm least-squares (MNLS) method, the optimal weighted pseudoinverse method (OWPIM), and the optimal constrained linear inverse method (OCLIM) are selected as the algorithms for inverse computation based on current multipole model innovatively, and the imaging effects of these three inverse methods are compared. Besides, two reconstructing parameters, residual and mean residual, are also discussed, and their trends under MNLS, OWPIM and OCLIM each as a function of SNR are obtained and compared.
基金Project supported by the State Key Development Program for Basic Research of China (Grant No. 2006CB601007)the National Natural Science Foundation of China (Grant No. 10674006)the National High Technology Research and Development Program of China (Grant No. 2007AA03Z238)
文摘This paper discusses the forward and inverse problem for cardiac magnetic fields and electric potentials. A torso-heart model established by boundary element method (BEM) is used for studying the distributions of cardiac magnetic fields and electric potentials. Because node-to-node and triangle-to-triangle BEM can lead to discrepant field distributions, their properties and influences are compared. Then based on constructed torso-heart model and supposed current source functional model-current dipole array, the magnetic and electric imaging by optimal constrained linear inverse method are applied at the same time. Through figure and reconstructing parameter comparison, though the magnetic current dipole array imaging possesses better reconstructing effect, however node-to-node BEM and triangleto-triangle BEM make little difference to magnetic and electric imaging.
文摘Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies.Echocardiography remains the first-line imaging tool, and when disease is suspected on echocardiography, cardiac magnetic resonance imaging and nuclear imaging play critical roles in the non-invasive diagnosis and evaluation of cardiac amyloidosis. Advances in multi-modality cardiac imaging allowing earlier diagnosis and initiation of novel therapies have significantly improved the outcomes in these patients. Cardiac imaging also plays important roles in the risk stratification of patients presenting with cardiac amyloidosis. In the current review, we provide a clinical and imaging focused update, and importantly outline the imaging protocols, diagnostic and prognostic utility of multimodality cardiac imaging in the assessment of cardiac amyloidosis.
文摘Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years.Different types of amyloidosis can affect the heart.Transthyretin amyloidosis and light chain amyloidosis are the two most common types of cardiac amyloidosis.These entities have a poor prognosis,so accurate diagnostic techniques are imperative for determining an early therapeutic approach.Recent advances in cardiac imaging and diagnostic strategies show that these tools are safe and can avoid the use of invasive diagnostic techniques to histological confirmation,such as endomyocardial biopsy.We performed a review on the diagnostic and prognostic implications of different cardiac imaging techniques in cardiac amyloidosis.We mainly focus on reviewing echocardiography,cardiac magnetic resonance,computed tomography and nuclear imaging techniques and the different safety measurements that can be done with each of them.
文摘Non-invasive cardiac imaging has explored enormous advances in the last few decades.In particular,hybrid imaging represents the fusion of information from multiple imaging modalities,allowing to provide a more comprehensive dataset compared to traditional imaging techniques in patients with cardiovascular diseases.The complementary anatomical,functional and molecular information provided by hybrid systems are able to simplify the evaluation procedure of various pathologies in a routine clinical setting.The diagnostic capability of hybrid imaging modalities can be further enhanced by introducing novel and specific imaging biomarkers.The aim of this review is to cover the most recent advancements in radiotracers development for SPECT/CT,PET/CT,and PET/MRI for cardiovascular diseases.
文摘There is a growing evidence of cardiovascular complications in coronavirusdisease 2019 (COVID-19) patients. As evidence accumulated of COVID-19 mediatedinflammatory effects on the myocardium, substantial attention has beendirected towards cardiovascular imaging modalities that facilitate this diagnosis.Cardiac magnetic resonance imaging (CMRI) is the gold standard for thedetection of structural and functional myocardial alterations and its role inidentifying patients with COVID-19 mediated cardiac injury is growing. Despiteits utility in the diagnosis of myocardial injury in this population, CMRI’s impacton patient management is still evolving. This review provides a framework for theuse of CMRI in diagnosis and management of COVID-19 patients from theperspective of a cardiologist. We review the role of CMRI in the management ofboth the acutely and remotely COVID-19 infected patient. We discuss patientselection for this imaging modality;T1, T2, and late gadolinium enhancementimaging techniques;and previously described CMRI findings in other cardiomyopathieswith potential implications in COVID-19 recovered patients.
文摘Despite optimal interventional and medical therapy, ischemic heart disease is still an important cause of morbidity and mortality worldwide. Although not included in standard of care rehabilitation, stem cell therapy(SCT) could be a solution for prompting cardiac regeneration. Multiple studies have been published from the beginning of SCT until now, but overall no unanimous conclusion could be drawn in part due to the lack of appropriate endpoints. In order to appreciate the impact of SCT, multiple markers from different categories should be considered: Structural, biological, functional, physiological, but also major adverse cardiac events or quality of life. Imaging end-points are among the most used-especially left ventricle ejection fraction(LVEF) measured through different methods. Other imaging parameters are infarct size, myocardial viability and perfusion. The impact of SCT on all of the aforementioned end-points is controversial and debatable. 2 D-echocardiography is widely exploited, but new approaches such as tissue Doppler, strain/strain rate or 3 D-echocardiography are more accurate, especially since the latter one is comparable with the MRI gold standard estimation of LVEF. Apart from the objective parameters, there are also patient-centered evaluations to reveal the benefits of SCT, such as quality of life and performance status, the most valuable from the patient point of view. Emerging parameters investigating molecular pathways such as non-coding RNAs or inflammation cytokines have a high potential as prognostic factors. Due to the disadvantages of current techniques, new imaging methods with labelled cells tracked along their lifetime seem promising, but until now only pre-clinical trials have been conducted in humans. Overall, SCT is characterized by high heterogeneity not only in preparation, administration and type of cells, but also in quantification of therapy effects.
文摘There is increasing evidence on the utility of cardiac computed tomography(CCT)in infective endocarditis(IE)to investigate the valvular pathology,the extracardiac manifestations of IE and pre-operative planning.CCT can assist in the diagnosis of perivalvular complications,such as pseudoaneurysms and abscesses,and can help identify embolic events to the lungs or systemic vasculature.CCT has also been shown to be beneficial in the pre-operative planning of patients by delineating the coronary artery anatomy and the major cardiovascular structures in relation to the sternum.Finally,hybrid nuclear/computed tomography techniques have been shown to increase the diagnostic accuracy in prosthetic valve endocarditis.This manuscript aims to provide a contemporary update of the existing evidence base for the use of CCT in IE.
文摘BACKGROUND We report a patient who was diagnosed with toxic myopericarditis secondary to hydrocarbon abuse using cardiac magnetic resonance imaging(CMR).CASE SUMMARY A 25-year-old male presented to emergency department with chest pain for 3 d.Patient also reported sniffing hydrocarbon containing inhalant for the last 1 year.Labs showed elevated troponin and electrocardiography was suggestive of acute pericarditis.Echocardiogram showed left ventricular(LV)ejection fraction(EF)of 40%.Given patient’s troponin elevation and reduced EF,cardiac catheterization was performed which showed normal coronaries.CMR was performed for myocardial infarction with non-obstructive coronary arteries evaluation.CMR showed borderline LV function with edema in mid and apical LV suggestive of myocarditis.CONCLUSION CMR can be used to diagnose toxic myopericarditis secondary to hydrocarbon abuse.
文摘Cardiac magnetic resonance imaging(MRI)is an evolving technology,proving to be a highly accurate tool for quantitative assessment.Most recently,it has been increasingly used in the diagnostic and prognostic evaluation of conditions involving an elevation in troponin or troponinemia.Although an elevation in troponin is a nonspecific marker of myocardial tissue damage,it is a frequently ordered investigation leaving many patients without a specific diagnosis.Fortunately,the advent of newer cardiac MRI protocols can provide additional information.In this review,we discuss several conditions associated with an elevation in troponin such as myocardial infarction,myocarditis,Takotsubo cardiomyopathy,coronavirus disease 2019 related cardiac dysfunction and athlete’s heart syndrome.
基金Hainan key research and development plan science and technology project funding(No.ZDYF2018166)Hainan key science and technology project Sanya supporting fund project support(No.2019PT107)Sanya medical science and technology innovation project(No.2019YW01)
文摘Objective:To investigate the correlation of cardiac magnetic resonance imaging(MRI)in the assessment of myocardial activity in patients with myocardial infarction and the outcome of cardiac function after PCI.Methods:30 patients with myocardial infarction(MI)who had complete clinical and imaging data from July 2016 to December 2018 after PCI were analyzed retrospectively.MR and echocardiogram were performed before and 6 months after PCI,and the parameters related to left ventricular function were measured by post-processing software of MRI workstation.The left ventricular transmural degree of CMR late gadolinium enhancement was compared with the left ventricular wall motion degree of 6-month echocardiography as a standard to judge the viable myocardium.Result:There were 193 left ventricular segmental abnormalities in 30 cases,including 121 viable myocardium and 72 non viable myocardium in CMR-LGE before operation.Six months after PCI,echocardiography showed that 125 of 193 abnormal segments of left ventricle detected by CMR-LGE before PCI were viable myocardium and 68 were non viable myocaridium.The sensitivity and specificity of CMR-LGE to determinate of viable myocardium were 92.0%and 91.1%respectively.The larger the non-viable myocardial area of the left ventricular wall,the worse the recovery of wall motion ability,and there was a negative correlation between them(r=0.416,P<0.05).The first-pass perfusion time in CMR-LEG region was significantly longer than that in normal myocardial region(4.85(+)1.51)s and(3.79(+)1.73)s,respectively.The difference was statistically significant(t=5.191,P<0.05).Conclusion:MRI can evaluate the myocardial activity of myocardial infarction,reflect the range of viable myocardium,and provide imaging basis for clinical treatment and prognosis.
文摘Left ventricular isolated hypoplasia is a seldom-described cardiac abnormality.Right ventricular hypoplasia is usually associated with congenital anomalies of the pulmonary or the tricuspid valve,whereas biventricular isolated apical hypoplasia has never been described.We report the case of a 48-year-old man with no history of known cardiac disease who was found to have a complex cardiac abnormality characterized by:1)Deficiency of the myocardium within the biventricular apex with adipose tissue infiltration;2)Truncated right ventricle because of an absent trabecular portion of the inflow tract;3)Truncated and spherical left ventricular apex;4)Origin of the mitral papillary muscle in the flattened anterior left ventricular apex.Multimodality imaging was performed to delineate the morphological and functional characteristics of this cardiomyopathy fully.To the best of our knowledge,this is the first description of a new cardiac abnormality characterized by the hypoplasia of the apical region of both ventricles in the absence of valvular or coronary artery disease.
文摘BACKGROUND Isolated left ventricular apical hypoplasia(ILVAH),also known as truncated left ventricle(LV),is a very unusual cardiomyopathy.It is characterised by a truncated,spherical,and non-apex forming LV.The true apex is occupied by the right ventricle.Due to the rarity of the disease,just a few case reports and limited case series have been published in the field.AIM To analysing the so far 37 reported ILVAH cases worldwide.METHODS The electronic databases PubMed and Scopus were investigated from their establishment up to December 13,2022.RESULTS The majority of cases reported occurred in males(52.7%).Mean age at diagnosis was 26.1±19.6 years.More than a third of the patients were asymptomatic(35.1%).The most usual clinical presentation was breathlessness(40.5%).The most commonly detected electrocardiogram changes were T wave abnormalities(29.7%)and right axis deviation with poor R wave progression(24.3%).Atrial fibrillation/flutter was detected in 24.3%.Echocardiography was performed in 97.3%of cases and cardiac MRI in 91.9%of cases.Ejection fraction was reduced in more than a half of patients(56.7%).An associated congenital heart disease was found in 16.2%.Heart failure therapy was administered in 35.1%of patients.The outcome was favorable in the vast majority of patients,with just one death.CONCLUSION ILVAH is a multifaceted entity with a so far unpredictable course,ranging from benign until the elderly to sudden death during adolescence.
文摘Artificial intelligence(AI)is the process of having a computational program that can perform tasks of human intelligence by mimicking human thought processes.AI is a rapidly evolving transdisciplinary field which integrates many elements to develop algorithms that aim to simulate human intuition,decision-making,and object recognition.The overarching aims of AI in cardiovascular medicine are threefold:To optimize patient care,improve efficiency,and improve clinical outcomes.In cardiology,there has been a growth in the potential sources of new patient data,as well as advances in investigations and therapies,which position the field well to uniquely benefit from AI.In this editorial,we highlight some of the main research priorities currently and where the next steps are heading us.
文摘Background:Ventricular crypts are quite a common finding during cardiac imaging,but their etiology is unclear.A possible final result of a spontaneous ventricular septal defect closure has been supposed but never investigated in earlier studies.Method:From January 1997 to December 2020,all newborns diagnosed to have a ventricular septal defect were prospectively entered in our database and those with an isolated defect were included in the study.Ventricular septal defects were classified into four types:perimembranous,trabecular muscular,inlet and outlet.A long-term follow up was performed in order to visualize the possible residual formation of a septal myocardial crypt.Results:A total of 376 isolated ventricular septal defects(314 muscular and 54 perimembranous,4 inlet,4 outlet)were detected.Follow up ranged from 1 to 23 years and showed that,among muscular type,a spontaneous closure occurred in 284(91%),26 did not close(8,28%),2 required surgical intervention(0,63%),3 were lost at follow up(0,95%).During this period,after spontaneous defect closure closure,20 crypts were found(6,4%).Conclusion:This study shows that a muscular ventricular septal defect may evolve in the 6.4%of cases in a residual septal crypt.Although septal crypts occur more frequently in patients affected by hypertrophic and hypertensive cardiomyopathy,they may also represent the evolution of a spontaneous closure of a muscular interventricular defect.