Pub Date : 2016-10-30DOI: 10.20418/JRCD.VOL2NO8.262
P. Rubis
{"title":"Report from the European Society of Cardiology Congress 2016 in Rome","authors":"P. Rubis","doi":"10.20418/JRCD.VOL2NO8.262","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO8.262","url":null,"abstract":"","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"11 1","pages":"275-276"},"PeriodicalIF":0.0,"publicationDate":"2016-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86946872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-09-30DOI: 10.20418/JRCD.VOL2NO8.246
O. Kruszelnicka, M. Chmiela, M. Raźny, Bernadeta Chyrchel, Jolanta Świerszcz, A. Surdacki, J. Nessler
Primary systemic amyloidosis (AL amyloidosis) is the most common subtype of amyloidosis in developed countries. Amyloid fibrils deposition results from an abnormal secondary structure of immunoglobulin light chains produced by a plasma cell clone. The most common accompanying plasma cell dyscrasia is monoclonal gammopathy of undetermined significance, while multiple myeloma coexists in only 10–15% of patients. The kidneys and the heart are the most frequently affected organs. Patients usually present with concentric left ventricular concentric thickening displaying a restrictive filling pattern with well-preserved systolic function. Clinical suspicion of AL amyloidosis should be raised in older adults and elderly patients with diastolic heart failure accompanied by heavy proteinuria, upon detection of thick-walled heart on echocardiography with low-voltage QRS on ECG (“red-flags” for amyloidosis”), in non-diabetic subjects with peripheral neuropathy or autonomic neuropathy, commonly with severe postural hypotension. Classical clinical stigmata, i.e. periorbital purpura, macroglossia, carpal tunnel syndrome, are not frequent (10–20%) but can guide diagnosis. In the presence of typical echocardiographic features recommended diagnostic steps include identification of monoclonal gammopathy (serum and urine immunofixation and serum free light-chain kappa to lambda ratio) and confirmation of amyloid deposition, preferentially in a non-cardiac tissue such as periumbilical fat or minor salivary glands. Bone marrow biopsy, serum calcium assay and skeletal survey are mandatory to exclude multiple myeloma. Prognosis is mainly dependent on cardiac involvement, being determined by cardiac biomarkers and the difference between involved and uninvolved light chains. Intensive chemotherapy is the therapy of choice in intermediate-risk patients with AL amyloidosis. An improved overall survival was reported in patients with a complete haematological response and an adequate cardiac response, especially a fall in circulating levels of B-type natriuretic peptides. JRCD 2016; 2 (8): 245–253
{"title":"Cardiac AL Amyloidosis (RCD code: III-3A.2a)","authors":"O. Kruszelnicka, M. Chmiela, M. Raźny, Bernadeta Chyrchel, Jolanta Świerszcz, A. Surdacki, J. Nessler","doi":"10.20418/JRCD.VOL2NO8.246","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO8.246","url":null,"abstract":"Primary systemic amyloidosis (AL amyloidosis) is the most common subtype of amyloidosis in developed countries. Amyloid fibrils deposition results from an abnormal secondary structure of immunoglobulin light chains produced by a plasma cell clone. The most common accompanying plasma cell dyscrasia is monoclonal gammopathy of undetermined significance, while multiple myeloma coexists in only 10–15% of patients. The kidneys and the heart are the most frequently affected organs. Patients usually present with concentric left ventricular concentric thickening displaying a restrictive filling pattern with well-preserved systolic function. Clinical suspicion of AL amyloidosis should be raised in older adults and elderly patients with diastolic heart failure accompanied by heavy proteinuria, upon detection of thick-walled heart on echocardiography with low-voltage QRS on ECG (“red-flags” for amyloidosis”), in non-diabetic subjects with peripheral neuropathy or autonomic neuropathy, commonly with severe postural hypotension. Classical clinical stigmata, i.e. periorbital purpura, macroglossia, carpal tunnel syndrome, are not frequent (10–20%) but can guide diagnosis. In the presence of typical echocardiographic features recommended diagnostic steps include identification of monoclonal gammopathy (serum and urine immunofixation and serum free light-chain kappa to lambda ratio) and confirmation of amyloid deposition, preferentially in a non-cardiac tissue such as periumbilical fat or minor salivary glands. Bone marrow biopsy, serum calcium assay and skeletal survey are mandatory to exclude multiple myeloma. Prognosis is mainly dependent on cardiac involvement, being determined by cardiac biomarkers and the difference between involved and uninvolved light chains. Intensive chemotherapy is the therapy of choice in intermediate-risk patients with AL amyloidosis. An improved overall survival was reported in patients with a complete haematological response and an adequate cardiac response, especially a fall in circulating levels of B-type natriuretic peptides. JRCD 2016; 2 (8): 245–253","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"138 1","pages":"245-253"},"PeriodicalIF":0.0,"publicationDate":"2016-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77506122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-09-30DOI: 10.20418/JRCD.VOL2NO8.254
M. Nowakowska, A. Mossakowska, L. Chrzanowski, Simiera, D. Kasprzak
Pulmonary arterial hypertension associated with congenital heart disease is included in clinical group 1 pulmonary arterial hypertension. Eisenmenger’s syndrome develops over time as a result of large intra- and extra-cardiac arterial and venous blood communication. The pulmonary vascular resistance (PVR) increases and thus the systemic-to pulmonary blood flow reverses direction, producing a pulmonary-to-systemic shunt (Eisenmenger’s syndrome). Bosentan, an oral endothelin receptor antagonist A and B, is recommended in monotherapy and drug combination therapy in this group of patients. Liver toxic reactions occur in about 10% of treated patients but severe hepatotoxicity is rare. We present a clinical case of a patient with Eisenmenger’s syndrome due to large ventricular septal defect. The patient was for many years successfully treated with bosentan and subsequently developed drug-induced hepatitis. JRCD 2016; 2 (8): 259–262
{"title":"Macitentan therapy for bosentan hepatic intolerance in Eisenmenger’s syndrome patient. (RCD code: II‑1A.4d)","authors":"M. Nowakowska, A. Mossakowska, L. Chrzanowski, Simiera, D. Kasprzak","doi":"10.20418/JRCD.VOL2NO8.254","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO8.254","url":null,"abstract":"Pulmonary arterial hypertension associated with congenital heart disease is included in clinical group 1 pulmonary arterial hypertension. Eisenmenger’s syndrome develops over time as a result of large intra- and extra-cardiac arterial and venous blood communication. The pulmonary vascular resistance (PVR) increases and thus the systemic-to pulmonary blood flow reverses direction, producing a pulmonary-to-systemic shunt (Eisenmenger’s syndrome). Bosentan, an oral endothelin receptor antagonist A and B, is recommended in monotherapy and drug combination therapy in this group of patients. Liver toxic reactions occur in about 10% of treated patients but severe hepatotoxicity is rare. We present a clinical case of a patient with Eisenmenger’s syndrome due to large ventricular septal defect. The patient was for many years successfully treated with bosentan and subsequently developed drug-induced hepatitis. JRCD 2016; 2 (8): 259–262","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"15 1","pages":"259-262"},"PeriodicalIF":0.0,"publicationDate":"2016-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85330113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-09-30DOI: 10.20418/JRCD.VOL2NO8.238
Monika Smaś‑Suska, P. Weryński, N. Dłużniewska, M. Olszowska, P. Podolec, L. Tomkiewicz-Pajak
Background: The aim of Fontan procedure is to restore a balance between pulmonary and systemic circulation and improve or nearly normalize arterial saturation in patients with functionally univentricular heart. Nevertheless, due to the lack of subpulmonary pump, the circulatory system becomes haemodynamically less efficient, what can lead to the impairment of exercise capacity. Aim: The aim of the study was to investigate exercise capacity by means of cardiopulmonary exercise test and pulmonary function tests, of 37 adult Fontan patients with regard to the time passed from the index procedure. The patients were divided into 3 groups: group I – up to 15 years post procedure; group II – 16–20 years, group III – more than 20 years after Fontan procedure). Results: 37 Fontan patients (mean age was 24.4 ±5.7 years, 40% of women) were enrolled in the study. The mean postoperative time was 19.4 ±5.1 (13–30) years. Mean, peak oxygen consumption (VO2peak) was 22.7 ±7.1 ml/kg/min (64.2 ±18.5% of predicted value). According to postoperative time a significant increase of ventilatory equivalent of carbon dioxide (VE/VCO2) (p = 0.033) and significant decrease of forced expiratory volume in 1 s/ vital capacity (FEV1%VC) between group 2 and 3 (p = 0.026) were observed. Additionally, the age of the patients correlated negatively with and heart rate (HR) (r = -0.360, p <0.05) and peak oxygen consumption (VO2peak) (r = -0,337, p < 0.05). Moreover, age at Fontan operation and time after Fontan procedure was related to ventilator equivalent of oxygen (VE/VO2) (r = -0,343, p <0.05, and r = 0.393, p <0.05). Single ventricle ejection fraction (SVEF) and atrioventricular regurgitation degree did not corresponded with cardiopulmonary exercise test (CPET) values. Conclusions: Results highlight the complex problem of diminished exercise capacity of Fontan patients depending on the time passed from the procedure. Exercise tolerance deteriorates in time: VE/VCO2 increases, FEV1/VC markedly lowers in a group of Fontan patients at 20 years follow-up. JRCD 2016; 2 (8): 254–258
背景:Fontan手术的目的是恢复肺循环和体循环之间的平衡，改善或接近正常的动脉饱和度。然而，由于缺乏肺下泵，循环系统的血液动力学效率降低，这可能导致运动能力的损害。目的:本研究的目的是通过心肺运动试验和肺功能试验，探讨37例成年Fontan患者的运动能力，以及从指标程序经过的时间。患者分为3组:第一组(术后15年);II组- 16-20岁，III组-方丹手术后20岁以上)。结果:37例Fontan患者(平均年龄24.4±5.7岁，女性占40%)入组研究。术后平均时间19.4±5.1(13-30)年。平均峰值耗氧量(VO2peak)为22.7±7.1 ml/kg/min，为预测值的64.2±18.5%。按术后时间分，2、3组通气当量二氧化碳(VE/VCO2)显著升高(p = 0.033)， 1 s/肺活量用力呼气量(FEV1%VC)显著降低(p = 0.026)。患者年龄与心率(HR) (r = -0.360, p <0.05)、最大耗氧量(VO2peak) (r = -0,337, p <0.05)呈负相关。此外，Fontan手术年龄和术后时间与呼吸机耗氧量(VE/VO2)相关(r = -0,343, p <0.05, r = 0.393, p <0.05)。单心室射血分数(SVEF)和房室反流程度与心肺运动试验(CPET)值不一致。结论:结果突出了Fontan患者运动能力下降的复杂问题，这取决于手术后的时间。运动耐量随时间的推移而恶化:20年随访时，Fontan患者组VE/VCO2升高，FEV1/VC显著降低。JRCD 2016;2 (8): 254-258
{"title":"Exercise capacity in adult patients after Fontan procedure. (RCD code: IV‑5B.1)","authors":"Monika Smaś‑Suska, P. Weryński, N. Dłużniewska, M. Olszowska, P. Podolec, L. Tomkiewicz-Pajak","doi":"10.20418/JRCD.VOL2NO8.238","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO8.238","url":null,"abstract":"Background: The aim of Fontan procedure is to restore a balance between pulmonary and systemic circulation and improve or nearly normalize arterial saturation in patients with functionally univentricular heart. Nevertheless, due to the lack of subpulmonary pump, the circulatory system becomes haemodynamically less efficient, what can lead to the impairment of exercise capacity. Aim: The aim of the study was to investigate exercise capacity by means of cardiopulmonary exercise test and pulmonary function tests, of 37 adult Fontan patients with regard to the time passed from the index procedure. The patients were divided into 3 groups: group I – up to 15 years post procedure; group II – 16–20 years, group III – more than 20 years after Fontan procedure). Results: 37 Fontan patients (mean age was 24.4 ±5.7 years, 40% of women) were enrolled in the study. The mean postoperative time was 19.4 ±5.1 (13–30) years. Mean, peak oxygen consumption (VO2peak) was 22.7 ±7.1 ml/kg/min (64.2 ±18.5% of predicted value). According to postoperative time a significant increase of ventilatory equivalent of carbon dioxide (VE/VCO2) (p = 0.033) and significant decrease of forced expiratory volume in 1 s/ vital capacity (FEV1%VC) between group 2 and 3 (p = 0.026) were observed. Additionally, the age of the patients correlated negatively with and heart rate (HR) (r = -0.360, p <0.05) and peak oxygen consumption (VO2peak) (r = -0,337, p < 0.05). Moreover, age at Fontan operation and time after Fontan procedure was related to ventilator equivalent of oxygen (VE/VO2) (r = -0,343, p <0.05, and r = 0.393, p <0.05). Single ventricle ejection fraction (SVEF) and atrioventricular regurgitation degree did not corresponded with cardiopulmonary exercise test (CPET) values. Conclusions: Results highlight the complex problem of diminished exercise capacity of Fontan patients depending on the time passed from the procedure. Exercise tolerance deteriorates in time: VE/VCO2 increases, FEV1/VC markedly lowers in a group of Fontan patients at 20 years follow-up. JRCD 2016; 2 (8): 254–258","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"7 1","pages":"254-258"},"PeriodicalIF":0.0,"publicationDate":"2016-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75536916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-09-30DOI: 10.20418/jrcd.vol2no8.258
Sebastian Gurba, Bernadetta Deręgowska, Krzysztof Terpin, W. Guz, Jerzy Kuźnia, A. Przybylski
Pulmonary cement embolism is a relatively new medical issue. The cement used in orthopedics is not even mentioned in the 2014 European Society of Cardiology (ESC) guidelines on the diagnosis and management of acute pulmonary embolism, however it will be a more frequent occurrence as minimally-invasive orthopedic procedures become more commonplace. An 81-year-old female was admitted to the emergency room with syncope. She had reported similar episodes of loss of consciousness and mild dyspnea for a few years prior to hospital admission. In 2008 the patient had undergone percutaneous vertebroplasty due to vertebral compression fractures. Her chest radiograph revealed multiple calcifications along the pulmonary vessels. Pulmonary computed tomography angiography confirmed pulmonary cement embolism. The presence of coexisting thrombus in one of the branches of the pulmonary artery was also revealed. The patient was administered enoxaparin subcutaneously and discharged. After 3 weeks repeat echocardiography showed a slight reduction in the dimensions of the pulmonary artery and its branches. Our case demonstrates that pulmonary cement embolism after percutaneous vertebroplasty may coexist with thrombotic embolus. Screening chest radiography after procedures using medical cement should be considered. Long term anticoagulation seems to be appropriate after pulmonary cement embolism. JRCD 2016; 2 (8): 266–269
{"title":"Pulmonary cement embolism after percutaneous vertebroplasty. (RCD code: II‑1C.0)","authors":"Sebastian Gurba, Bernadetta Deręgowska, Krzysztof Terpin, W. Guz, Jerzy Kuźnia, A. Przybylski","doi":"10.20418/jrcd.vol2no8.258","DOIUrl":"https://doi.org/10.20418/jrcd.vol2no8.258","url":null,"abstract":"Pulmonary cement embolism is a relatively new medical issue. The cement used in orthopedics is not even mentioned in the 2014 European Society of Cardiology (ESC) guidelines on the diagnosis and management of acute pulmonary embolism, however it will be a more frequent occurrence as minimally-invasive orthopedic procedures become more commonplace. An 81-year-old female was admitted to the emergency room with syncope. She had reported similar episodes of loss of consciousness and mild dyspnea for a few years prior to hospital admission. In 2008 the patient had undergone percutaneous vertebroplasty due to vertebral compression fractures. Her chest radiograph revealed multiple calcifications along the pulmonary vessels. Pulmonary computed tomography angiography confirmed pulmonary cement embolism. The presence of coexisting thrombus in one of the branches of the pulmonary artery was also revealed. The patient was administered enoxaparin subcutaneously and discharged. After 3 weeks repeat echocardiography showed a slight reduction in the dimensions of the pulmonary artery and its branches. Our case demonstrates that pulmonary cement embolism after percutaneous vertebroplasty may coexist with thrombotic embolus. Screening chest radiography after procedures using medical cement should be considered. Long term anticoagulation seems to be appropriate after pulmonary cement embolism. JRCD 2016; 2 (8): 266–269","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"112 1","pages":"266-269"},"PeriodicalIF":0.0,"publicationDate":"2016-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76244189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-09-30DOI: 10.20418/JRCD.VOL2NO8.237
Jacek Kuźma, A. Rudziński, W. Król, Beata Załuska-Pitak, Jolanta Oko‑Łagan, E. Siara, B. Pietrucha
We report a case of a 15 year old boy with a cyst attached to the anterior leaflet of mitral valve. At the age of 6 he was submitted for a cardiac evaluation due to a heart murmur. On physical exam a soft systolic murmur with loudness of 2–3/6 in Levine scale was found without symptoms of congestion. Electrocardiogram and chest X ray were within normal range. Cardiac echo study (ECHO) revealed a single balloon-like anomaly with small compartments inside. The tumor was localized on the ventricular surface of the anterior leaflet of mitral valve. Initially inflow and outflow tracts in colour and pulse wave Doppler were normal without any disturbances. During follow up the patient was asymptomatic without episodes of syncope, congestive heart failure or thromboembolic episodes. He was not treated pharmacologically. At the age of 15, in a subsequent ECHO the size of the cyst was up to 20 mm. The inflow was normal without signs of stenosis, while the left ventricle outflow tract revealed mild dynamic stenosis at the end of systole with maximum pressure gradient of 15 mmHg. The child was qualified for surgery, however the parents did not agree for operation due to the high risk of the procedure and high likelihood of a need for mitral prosthesis implantation requiring lifelong anticoagulation. JRCD 2016; 2 (8): 263–265
{"title":"Blood cyst attached to the anterior leaflet of the mitral valve. (RCD code: VI‑1A.0)","authors":"Jacek Kuźma, A. Rudziński, W. Król, Beata Załuska-Pitak, Jolanta Oko‑Łagan, E. Siara, B. Pietrucha","doi":"10.20418/JRCD.VOL2NO8.237","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO8.237","url":null,"abstract":"We report a case of a 15 year old boy with a cyst attached to the anterior leaflet of mitral valve. At the age of 6 he was submitted for a cardiac evaluation due to a heart murmur. On physical exam a soft systolic murmur with loudness of 2–3/6 in Levine scale was found without symptoms of congestion. Electrocardiogram and chest X ray were within normal range. Cardiac echo study (ECHO) revealed a single balloon-like anomaly with small compartments inside. The tumor was localized on the ventricular surface of the anterior leaflet of mitral valve. Initially inflow and outflow tracts in colour and pulse wave Doppler were normal without any disturbances. During follow up the patient was asymptomatic without episodes of syncope, congestive heart failure or thromboembolic episodes. He was not treated pharmacologically. At the age of 15, in a subsequent ECHO the size of the cyst was up to 20 mm. The inflow was normal without signs of stenosis, while the left ventricle outflow tract revealed mild dynamic stenosis at the end of systole with maximum pressure gradient of 15 mmHg. The child was qualified for surgery, however the parents did not agree for operation due to the high risk of the procedure and high likelihood of a need for mitral prosthesis implantation requiring lifelong anticoagulation. JRCD 2016; 2 (8): 263–265","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"98 1","pages":"263-265"},"PeriodicalIF":0.0,"publicationDate":"2016-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76491894","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-09-30DOI: 10.20418/JRCD.VOL2NO8.263
P. Podolec
His Holiness Pope Francis, European Society of Cardiology Congress, Rome, Italy 31 st August 2016 Dear Readers, Unlike to previous Editorials, let’s start with the last article in this new issue of the Journal – Report form the European Society of Cardiology Congress that took place in the last days of August in the everlasting city of Rome. Just a few days ago, we witnessed an unprecedented event in the whole history of not only European but world cardiology. It was that His Holiness Pope Francis, who is known for extraordinary gestures, attended Fiera di Roma – the venue where the annual European Society of Cardiology Congress has been held for several days. In the very emotional and yet sensible words, Pope Francis expressed His admiration for all health‑care professionals for all their devotion for the sick and poor. Moreover, Pope Francis addressed particularly scientists with their ongoing quest for truth. Along the same line of thinking, Pope Francis reinforced the ultimate purpose of science that should be an improvement of care delivered to every human‑being. Those wise words shall accompany us with all our daily struggle! Coming back to the current issue of the Journal, there is a Review article on cardiac amyloidosis and Original article about exercise capacity in patients after Fontan operation. Moreover, as usual the middle part is composed of four exceptional clinical cases presented and commented by the managing teams. The Journal ends with the report from the annual European Society of Cardiology Congress that took place in August in Rome, Italy. (...)
{"title":"“The sciences alone, however, whether natural or physical, are not sufficient to understand the mystery contained within each person”","authors":"P. Podolec","doi":"10.20418/JRCD.VOL2NO8.263","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO8.263","url":null,"abstract":"His Holiness Pope Francis, European Society of Cardiology Congress, Rome, Italy 31 st August 2016 Dear Readers, Unlike to previous Editorials, let’s start with the last article in this new issue of the Journal – Report form the European Society of Cardiology Congress that took place in the last days of August in the everlasting city of Rome. Just a few days ago, we witnessed an unprecedented event in the whole history of not only European but world cardiology. It was that His Holiness Pope Francis, who is known for extraordinary gestures, attended Fiera di Roma – the venue where the annual European Society of Cardiology Congress has been held for several days. In the very emotional and yet sensible words, Pope Francis expressed His admiration for all health‑care professionals for all their devotion for the sick and poor. Moreover, Pope Francis addressed particularly scientists with their ongoing quest for truth. Along the same line of thinking, Pope Francis reinforced the ultimate purpose of science that should be an improvement of care delivered to every human‑being. Those wise words shall accompany us with all our daily struggle! Coming back to the current issue of the Journal, there is a Review article on cardiac amyloidosis and Original article about exercise capacity in patients after Fontan operation. Moreover, as usual the middle part is composed of four exceptional clinical cases presented and commented by the managing teams. The Journal ends with the report from the annual European Society of Cardiology Congress that took place in August in Rome, Italy. (...)","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"3 1","pages":"243-244"},"PeriodicalIF":0.0,"publicationDate":"2016-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82395084","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-07-01DOI: 10.20418/JRCD.VOL2NO7.249
P. Podolec
And yet again, summer solstice is just behind the cor‑ ner. As the time passes so quickly, do we really have a chance to use it profitability? One way is to study hard and be up‑to‑date. Then we know new and com‑ plex syndromes or fancy and expensive drugs. But is this really what it is about? Spending less and less time with the patients or on patients’ records, surly we miss a great bulk of knowledge. Knowledge, that is just in front of us and yet we close our eyes and quickly turn on computers and internet in the quest for the holy grail. Is this right thing to do? Well, patients may think of us as very smart when we constantly use incom‑ prehensible medical jargon. But, occasionally we may jump into conclusions, that what was just described in other side of the globe, was ready to grasp in front of our noses. Thoughtful observation and thinking is as good as ground‑breaking science. And for our patients may be even better.
{"title":"For one mistake made for not knowing, ten mista kes are made for not looking","authors":"P. Podolec","doi":"10.20418/JRCD.VOL2NO7.249","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO7.249","url":null,"abstract":"And yet again, summer solstice is just behind the cor‑ ner. As the time passes so quickly, do we really have a chance to use it profitability? One way is to study hard and be up‑to‑date. Then we know new and com‑ plex syndromes or fancy and expensive drugs. But is this really what it is about? Spending less and less time with the patients or on patients’ records, surly we miss a great bulk of knowledge. Knowledge, that is just in front of us and yet we close our eyes and quickly turn on computers and internet in the quest for the holy grail. Is this right thing to do? Well, patients may think of us as very smart when we constantly use incom‑ prehensible medical jargon. But, occasionally we may jump into conclusions, that what was just described in other side of the globe, was ready to grasp in front of our noses. Thoughtful observation and thinking is as good as ground‑breaking science. And for our patients may be even better.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"17 1","pages":"207-208"},"PeriodicalIF":0.0,"publicationDate":"2016-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80198340","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2016-07-01DOI: 10.20418/jrcd.vol2no7.250
P. Rubis
Having visited several European Society of Cardiology (ESC) Congresses, this time the plan was to explore famous American College of Cardiology (ACC) annual Congress. For a long time American way of making science dominated over not only Europe but the rest of the world. Is it still holds true? Probably the answer is not simple, nevertheless, the glimpse of true American perspective can be tasted in real only on the American soil. The 65th ACC Congress was taken place between 2nd till 4th April in Chicago, Illinois at the gargantuan McCormick Convection Centre located just at the bank of magnificent Michigan lake. The first striking difference was the weather! When in Poland and rest of Europe, the spring was in full swing, the north-west part of the USA was griped in the frosty cold with an average temperature of minus five. Not to mention the icy winds for which Chicago is famous and called the windy-city. This is not an exaggeration, please do believe me! But now a little about the Congress itself.
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Pub Date : 2016-06-30DOI: 10.20418/JRCD.VOL2NO7.227
D. Stettner-Leonkiewicz, A. Tomaszewski, A. Wysokiński, J. Stążka, M. Czajkowski, M. Tomaszewski
Myxomas are one of the most common cardiac tumors. In 70–80% of cases they are located in the left atrium, 10–20% in the right atrium and in less than 10% in ventricles. We report a case of a 60-year-old patient after myocardial infarction of the posterior-inferior-lateral wall and subsequent percutaneous coronary interventions, who presented with symptoms of unstable angina. Based on standard criteria including cardiac enzymes acute coronary syndrome was excluded. Transthoracic echocardiogram showed hypokinesis of the lateral wall, normal left ventricular ejection fraction of 56%, left ventricular hypertrophy, enlarged heart chambers and moderate mitral regurgitation. Additional two masses were visualized - one of 3.1 x 1.4 cm size growing from interatrial septum in the area of fossa ovalis in the left atrium, second of 1.2 x 0.4 cm connected to the chordae tendinae in the left ventricle. On transthoracic echocardiography a distal part of the left atrial structure spontaneously fell into the left ventricle hitting the tendinae. Successful surgical removal of both masses followed by pathological evaluation confirmed the diagnosis of myxomas.
黏液瘤是最常见的心脏肿瘤之一。在70-80%的病例中，它们位于左心房，10-20%位于右心房，不到10%位于心室。我们报告了一例60岁的患者后下侧壁心肌梗死和随后的经皮冠状动脉介入治疗，谁提出了不稳定心绞痛的症状。根据包括心酶在内的标准标准，排除急性冠状动脉综合征。经胸超声心动图显示外侧壁运动不足，左室射血分数正常56%，左室肥厚，心室增大，二尖瓣中度返流。另外两个肿块可见-一个3.1 x 1.4 cm大小的肿块生长在左心房卵圆窝区域的房间隔上，第二个1.2 x 0.4 cm大小的肿块连接到左心室的腱索。经胸超声心动图显示左心房结构的远端部分自发地落入左心室撞击腱。手术成功切除了两个肿块，病理评估证实了黏液瘤的诊断。
{"title":"Spontaneous implantation of a left atrial myxoma into the left ventricle (RCD code: VI‑1A.1)","authors":"D. Stettner-Leonkiewicz, A. Tomaszewski, A. Wysokiński, J. Stążka, M. Czajkowski, M. Tomaszewski","doi":"10.20418/JRCD.VOL2NO7.227","DOIUrl":"https://doi.org/10.20418/JRCD.VOL2NO7.227","url":null,"abstract":"Myxomas are one of the most common cardiac tumors. In 70–80% of cases they are located in the left atrium, 10–20% in the right atrium and in less than 10% in ventricles. We report a case of a 60-year-old patient after myocardial infarction of the posterior-inferior-lateral wall and subsequent percutaneous coronary interventions, who presented with symptoms of unstable angina. Based on standard criteria including cardiac enzymes acute coronary syndrome was excluded. Transthoracic echocardiogram showed hypokinesis of the lateral wall, normal left ventricular ejection fraction of 56%, left ventricular hypertrophy, enlarged heart chambers and moderate mitral regurgitation. Additional two masses were visualized - one of 3.1 x 1.4 cm size growing from interatrial septum in the area of fossa ovalis in the left atrium, second of 1.2 x 0.4 cm connected to the chordae tendinae in the left ventricle. On transthoracic echocardiography a distal part of the left atrial structure spontaneously fell into the left ventricle hitting the tendinae. Successful surgical removal of both masses followed by pathological evaluation confirmed the diagnosis of myxomas.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"4 1","pages":"225-227"},"PeriodicalIF":0.0,"publicationDate":"2016-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82908925","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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