Background:Since 2015,the pulmonary artery banding(PAB),following the Giessen protocol,has treated endstage heart failure in selected infants with preserved right ventricular function,acting as a bridge to transplant ...Background:Since 2015,the pulmonary artery banding(PAB),following the Giessen protocol,has treated endstage heart failure in selected infants with preserved right ventricular function,acting as a bridge to transplant or recovery,as a result of ventricular-ventricular interaction.Objectives:To elucidate whether PAB is a feasible and reproducible procedure in a rodent model of pharmacologically induced dilated cardiomyopathy(DCM)and to evaluate PAB-induced ventricular rehabilitation.Methods:We used 49 Sprague-Dawley rats divided into four groups:a sham surgery control group,a healthy animal group undergoing PAB,a doxorubicin(DOX)-treated control group,and a DOX+PAB-treated group.All underwent echocardiographic,histological,and molecular analyses.Results:Preliminary results showed high mortality in rats with DOX-induced DCM,with contractile dysfunction confirmed by 2D echocardiography.Signs of damage were detected through transmission electron microscopy,but not via standard histological/molecular tests.PAB after DOX improved contractile function,enhancing ejection fraction(p=0.01)and fractional shortening(p=0.03).Conclusion:The DOX-induced DCM model,while reproducible,may not reflect DCM’s true pathology.High mortality and individual variability limited the study.Further research is needed to find alternative models with lower mortality and to explore the PAB-induced molecular signaling pathways and cardiac proliferation potential.展开更多
BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is ...BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.展开更多
Left ventricular contractility was measured using Doppler echocardiography in 37 pa-tients with rheumatic mitral valve disease.Myocardial pathological lesions were studied quanti-tatively by means of the image analysi...Left ventricular contractility was measured using Doppler echocardiography in 37 pa-tients with rheumatic mitral valve disease.Myocardial pathological lesions were studied quanti-tatively by means of the image analysis system and light microscopic examination.The resultsshowed that attenuated cardiac function is due to pathological change of the mitral valve andpathological lesions of the left ventrieular myocardium including increased interstitial fibrosis,thickened intramyocardial arterial wall leading to concentric narrowing of the lumen,hyper-trophic myocardial fiber and endocardial proliferation.Since mitral valve replacement can notcorrect the irreversible myocardial pathological lesions,perioperative protection of the my-ocardium should be emphasized,and intraoperative injury to the myocardium should be mini-mized as far as possible.展开更多
Sudden cardiac death (SCD) remains a leading mode of death in western countries.Since SCD can be the first and last clinical presentation of the underlying disease,autopsy could be the only medical examination availab...Sudden cardiac death (SCD) remains a leading mode of death in western countries.Since SCD can be the first and last clinical presentation of the underlying disease,autopsy could be the only medical examination available for early diagnosis and it should be performed according to the guidelines of the Association for European Cardiovascular Pathology.Although the vast majority of SCD are due to coronary artery disease,non-ischemic causes of SCD do exist and are prevalent in young people with structural (i.e.arrhythmogenic,hypertrophic and inflammatory cardiomyopathy) and non-structural (ion channel diseases)cardiomyopathies,accounting for up to one half of cases.A standardized autopsy protocol,in combination with blood sampling to ensure feasibility of postmortem molecular testing if needed,is mandatory.The pathologist is called to provide the correct diagnosis and to advice the relatives on the need of a cascade clinical and genetic screening in the presence of a heredo-familial disease.展开更多
Cardiovascular disease represents the main cause of death in developed countries.Cardiovascular disease frequently may account for premature fatal outcomes,even in the apparently healthy young, appearing in the form o...Cardiovascular disease represents the main cause of death in developed countries.Cardiovascular disease frequently may account for premature fatal outcomes,even in the apparently healthy young, appearing in the form of sudden and unexpected death [1]. Sudden death (SD) is defined according to commonly accepted criteria in witnessed cases as follows.SD is a natural death that occurs within 6h of the beginning of symptoms in an apparently healthy subject or in one whose disease is not so severe that a fatal outcome would be expected [2].In cases of unwitnessed death, which is a common situation in forensic practice, this definition requires that the deceased was last seen alive and functioning normally 24 h before being found dead [3].展开更多
基金funded by BIRD201443-2020by a Grant from“Associazione Un Cuore Un Mondo Padova-ONLUS”.
文摘Background:Since 2015,the pulmonary artery banding(PAB),following the Giessen protocol,has treated endstage heart failure in selected infants with preserved right ventricular function,acting as a bridge to transplant or recovery,as a result of ventricular-ventricular interaction.Objectives:To elucidate whether PAB is a feasible and reproducible procedure in a rodent model of pharmacologically induced dilated cardiomyopathy(DCM)and to evaluate PAB-induced ventricular rehabilitation.Methods:We used 49 Sprague-Dawley rats divided into four groups:a sham surgery control group,a healthy animal group undergoing PAB,a doxorubicin(DOX)-treated control group,and a DOX+PAB-treated group.All underwent echocardiographic,histological,and molecular analyses.Results:Preliminary results showed high mortality in rats with DOX-induced DCM,with contractile dysfunction confirmed by 2D echocardiography.Signs of damage were detected through transmission electron microscopy,but not via standard histological/molecular tests.PAB after DOX improved contractile function,enhancing ejection fraction(p=0.01)and fractional shortening(p=0.03).Conclusion:The DOX-induced DCM model,while reproducible,may not reflect DCM’s true pathology.High mortality and individual variability limited the study.Further research is needed to find alternative models with lower mortality and to explore the PAB-induced molecular signaling pathways and cardiac proliferation potential.
文摘BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.
基金Supported by the Youth Foundation of the General Logistics Department of PLA
文摘Left ventricular contractility was measured using Doppler echocardiography in 37 pa-tients with rheumatic mitral valve disease.Myocardial pathological lesions were studied quanti-tatively by means of the image analysis system and light microscopic examination.The resultsshowed that attenuated cardiac function is due to pathological change of the mitral valve andpathological lesions of the left ventrieular myocardium including increased interstitial fibrosis,thickened intramyocardial arterial wall leading to concentric narrowing of the lumen,hyper-trophic myocardial fiber and endocardial proliferation.Since mitral valve replacement can notcorrect the irreversible myocardial pathological lesions,perioperative protection of the my-ocardium should be emphasized,and intraoperative injury to the myocardium should be mini-mized as far as possible.
基金This work was supported by the Registry for cardiac cerebro-vascular Pathology,Veneto RegionMinistry of Education,University and Research[grant number PRIN 2015ZLNETW]Ministry of Health[grant number RF-2016-02363774].
文摘Sudden cardiac death (SCD) remains a leading mode of death in western countries.Since SCD can be the first and last clinical presentation of the underlying disease,autopsy could be the only medical examination available for early diagnosis and it should be performed according to the guidelines of the Association for European Cardiovascular Pathology.Although the vast majority of SCD are due to coronary artery disease,non-ischemic causes of SCD do exist and are prevalent in young people with structural (i.e.arrhythmogenic,hypertrophic and inflammatory cardiomyopathy) and non-structural (ion channel diseases)cardiomyopathies,accounting for up to one half of cases.A standardized autopsy protocol,in combination with blood sampling to ensure feasibility of postmortem molecular testing if needed,is mandatory.The pathologist is called to provide the correct diagnosis and to advice the relatives on the need of a cascade clinical and genetic screening in the presence of a heredo-familial disease.
文摘Cardiovascular disease represents the main cause of death in developed countries.Cardiovascular disease frequently may account for premature fatal outcomes,even in the apparently healthy young, appearing in the form of sudden and unexpected death [1]. Sudden death (SD) is defined according to commonly accepted criteria in witnessed cases as follows.SD is a natural death that occurs within 6h of the beginning of symptoms in an apparently healthy subject or in one whose disease is not so severe that a fatal outcome would be expected [2].In cases of unwitnessed death, which is a common situation in forensic practice, this definition requires that the deceased was last seen alive and functioning normally 24 h before being found dead [3].
