Pub Date : 2017-10-16DOI: 10.20418/JRCD.VOL3NO4.302
Paweł Rubiś
Brugada Syndrome (BrS) is traditionally considered a primary channelopathy, most commonly due to reduced inward sodium current with an increased risk of syncope and sudden cardiac death (SCD). According to the recent guidelines, BrS is diag‐ nosed in patients with ST‐segment elevation with type 1 morphol‐ ogy among the right precordial leads V1 and V2, occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs. Moreover, BrS is diagnosed in patients with type 2 or type 3 ST‐segment elevation in ≥1 lead among the right precordial leads when a provocative drug test with intravenous administration of Class I antiarrhyth‐ mic drugs induces a type I ECG morphology. Risk stratification of SCD is the most important aspect of the concise management of those patients. Importantly, once considered pure arrhyth‐ mic syndrome, nowadays, there is growing understanding that some structural abnormalities may be present in BrS. Therefore, imaging is actively investigated in this field. Brugada syndrome can be considered as a rare disease, with the prevalence of 1 in 1000 to 1 in 10 000, being more frequent in south‐east Asia than in the western countries. Thus, this topic has been already explored in the Journal of Rare Cardiovascular Disease (see references be‐ low). The purpose of this Review is to update the Readers with the main developments in this entity.
{"title":"Brugada syndrome: 2017 update. (RCD code: V-1A.1)","authors":"Paweł Rubiś","doi":"10.20418/JRCD.VOL3NO4.302","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO4.302","url":null,"abstract":"Brugada Syndrome (BrS) is traditionally considered a primary channelopathy, most commonly due to reduced inward sodium current with an increased risk of syncope and sudden cardiac death (SCD). According to the recent guidelines, BrS is diag‐ nosed in patients with ST‐segment elevation with type 1 morphol‐ ogy among the right precordial leads V1 and V2, occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs. Moreover, BrS is diagnosed in patients with type 2 or type 3 ST‐segment elevation in ≥1 lead among the right precordial leads when a provocative drug test with intravenous administration of Class I antiarrhyth‐ mic drugs induces a type I ECG morphology. Risk stratification of SCD is the most important aspect of the concise management of those patients. Importantly, once considered pure arrhyth‐ mic syndrome, nowadays, there is growing understanding that some structural abnormalities may be present in BrS. Therefore, imaging is actively investigated in this field. Brugada syndrome can be considered as a rare disease, with the prevalence of 1 in 1000 to 1 in 10 000, being more frequent in south‐east Asia than in the western countries. Thus, this topic has been already explored in the Journal of Rare Cardiovascular Disease (see references be‐ low). The purpose of this Review is to update the Readers with the main developments in this entity.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"4 1","pages":"108-109"},"PeriodicalIF":0.0,"publicationDate":"2017-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78972142","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 : 2017-10-16DOI: 10.20418/JRCD.VOL3NO4.295
H. Matsubara, P. Blaszczak, P. Podolec, G. Kopeć
Pulmonary arterial hypertension is a disease characterized by poor prognosis despite treatment. Even in a modern era of pharmaco- therapy there is a strong need to further improve survival of patients. The current therapeutic strategies do not offer a real break-through in terms of reducing mortality. In a search for better efficacy of treatment we discuss a strategy based on lowering pulmonary artery pressure as much as possible with epoprostenol in monotherapy or in combination. Epoprostenol with bosentan is an effective tool in achieving this goal. A rapid up-titration regardless of maximum epoprostenol dose achieved gives additional long-term benefit. Practical issues related to such modality of treatment are also discussed. JRCD 2017; 3 (4): 110–115
{"title":"Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‐1A.1)","authors":"H. Matsubara, P. Blaszczak, P. Podolec, G. Kopeć","doi":"10.20418/JRCD.VOL3NO4.295","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO4.295","url":null,"abstract":"Pulmonary arterial hypertension is a disease characterized by poor prognosis despite treatment. Even in a modern era of pharmaco- therapy there is a strong need to further improve survival of patients. The current therapeutic strategies do not offer a real break-through in terms of reducing mortality. In a search for better efficacy of treatment we discuss a strategy based on lowering pulmonary artery pressure as much as possible with epoprostenol in monotherapy or in combination. Epoprostenol with bosentan is an effective tool in achieving this goal. A rapid up-titration regardless of maximum epoprostenol dose achieved gives additional long-term benefit. Practical issues related to such modality of treatment are also discussed. JRCD 2017; 3 (4): 110–115","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"103 1","pages":"110-115"},"PeriodicalIF":0.0,"publicationDate":"2017-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80847564","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 : 2017-10-16DOI: 10.20418/JRCD.VOL3NO4.299
B. Hussain, Fateh Ali Tipoo Sultan, Z. Jamil
Objectives This study is an effort to bridge the gap regarding the data of prevalence of coronary anomalies in South Asian countries by utilizing coronary CT angiography. Methods This is a cross sectional, descriptive study done at Aga Khan University Hospital Karachi, Pakistan. All adult patients who underwent coronary CT angiography from 1-1-2005 to 1-11-2016 were included in the study. Results Among 897 patients undergoing CCTA, 36(4.01%)patients were identified as having coronary artery anomalies(CAA). The mean age of patients was 48.3±11.9 years. Male to female ratio was 3:1. Among patients with coronary anomalies, the most common indication for CCTA was screening for coronary artery disease(58.3%). 33 patients(3.67%) showed coronary origin and course anomalies, whereas coronary artery fistula were identified in 3 patients(0.3% of cases). The most common anomaly identified was the anomalous origin of coronary artery from opposite coronary sinus with anomalous course(1.4%) with anomalous origin of RCA from the left coronary sinus with an inter-arterial course seen in 9 patients. Single coronary artery was seen in three cases(0.3%), in all these cases the single coronary artery originated from the right coronary cusp. In one patient (0.1%) dual LAD system was identified. Coronary artery fistulas were seen in 3 patients (0.3%).In two cases(0.2%) fistulous connection of LAD with pulmonary artery was seen, while fistula from LAD to coronary sinus was identified in one patient. Conclusions This study highlights the fact that the prevalence of coronary anomalies on CCTA in this region is similar to the reported data from rest of the world.
{"title":"The prevalence of coronary artery anomalies on CT scan; Experience from a tertiary care center in Pakistan (RCD code: I-1C.1)","authors":"B. Hussain, Fateh Ali Tipoo Sultan, Z. Jamil","doi":"10.20418/JRCD.VOL3NO4.299","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO4.299","url":null,"abstract":"Objectives This study is an effort to bridge the gap regarding the data of prevalence of coronary anomalies in South Asian countries by utilizing coronary CT angiography. Methods This is a cross sectional, descriptive study done at Aga Khan University Hospital Karachi, Pakistan. All adult patients who underwent coronary CT angiography from 1-1-2005 to 1-11-2016 were included in the study. Results Among 897 patients undergoing CCTA, 36(4.01%)patients were identified as having coronary artery anomalies(CAA). The mean age of patients was 48.3±11.9 years. Male to female ratio was 3:1. Among patients with coronary anomalies, the most common indication for CCTA was screening for coronary artery disease(58.3%). 33 patients(3.67%) showed coronary origin and course anomalies, whereas coronary artery fistula were identified in 3 patients(0.3% of cases). The most common anomaly identified was the anomalous origin of coronary artery from opposite coronary sinus with anomalous course(1.4%) with anomalous origin of RCA from the left coronary sinus with an inter-arterial course seen in 9 patients. Single coronary artery was seen in three cases(0.3%), in all these cases the single coronary artery originated from the right coronary cusp. In one patient (0.1%) dual LAD system was identified. Coronary artery fistulas were seen in 3 patients (0.3%).In two cases(0.2%) fistulous connection of LAD with pulmonary artery was seen, while fistula from LAD to coronary sinus was identified in one patient. Conclusions This study highlights the fact that the prevalence of coronary anomalies on CCTA in this region is similar to the reported data from rest of the world.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"8 1","pages":"116-121"},"PeriodicalIF":0.0,"publicationDate":"2017-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81915798","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 : 2017-10-16DOI: 10.20418/JRCD.VOL3NO4.290
B. Hussain, Fateh Ali Tipoo Sultan, F. Mookadam
Hematological malignancies are protean in their presentations. Primary cardiac tumors are exceedingly rare, and primary cardiac leukemia presenting as an isolated mass is even rare. Here, we present a case of a young man who presented with one month history of fever and shortness of breath. His examination revealed signs of right sided heart failure. On workup he was found to have a mass in the heart on CT scan of the chest, his transthoracic echocardiography showed an isolated right atrioventricular groove mass. Surgical biopsy confirmed it to be an extremely rare presentation of Pre B Acute Lymphoblastic leukemia(ALL). Though the patient underwent surgical excision of the lesion yet, he developed bone marrow leukemic involvement confirmed by bone marrow biopsy and died of overwhelming sepsis secondary to pancytopenia. This case highlights that hematological malignancies can present as cardiac masses and should be considered during workup of patients presenting with chronic fever and right sided heart failure.
{"title":"An uncommon cause of right heart failure: primary cardiac pre-B cell acute lymphoblastic leukemia (RCD code: VI-2C.1).","authors":"B. Hussain, Fateh Ali Tipoo Sultan, F. Mookadam","doi":"10.20418/JRCD.VOL3NO4.290","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO4.290","url":null,"abstract":"Hematological malignancies are protean in their presentations. Primary cardiac tumors are exceedingly rare, and primary cardiac leukemia presenting as an isolated mass is even rare. Here, we present a case of a young man who presented with one month history of fever and shortness of breath. His examination revealed signs of right sided heart failure. On workup he was found to have a mass in the heart on CT scan of the chest, his transthoracic echocardiography showed an isolated right atrioventricular groove mass. Surgical biopsy confirmed it to be an extremely rare presentation of Pre B Acute Lymphoblastic leukemia(ALL). Though the patient underwent surgical excision of the lesion yet, he developed bone marrow leukemic involvement confirmed by bone marrow biopsy and died of overwhelming sepsis secondary to pancytopenia. This case highlights that hematological malignancies can present as cardiac masses and should be considered during workup of patients presenting with chronic fever and right sided heart failure.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"73 1","pages":"137-142"},"PeriodicalIF":0.0,"publicationDate":"2017-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73726031","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 : 2017-10-16DOI: 10.20418/JRCD.VOL3NO4.271
Klaudia Knap, Leszek Drabik, J. Błaut-Jurkowska, Michal Pacia, Maciej Krupińsk, M. Urbańczyk‑Zawadzka, W. Płazak
Complications of myocardial infarction (MI) such as left ventricular aneurysm, chronic ischaemic mitral regurgitation (CIMR) and heart failure are associated with a poor prognosis. The management of complex defects is challenging and requires an individualized strategy. We describe a case of 44-year-old male with multiple complications of MI including heart failure with reduced ejection fraction, left ventricular aneurysm and severe CIMR. We present complex approach, aimed at correcting various components of ischemic cardiomyopathy: relieving ischemia, reducing left ventricular wall tension, reducing volume overload and pulmonary hypertension by mitral valve surgery and left ventricular reconstruction (also known as the Dor procedure). In this case report, we describe the crucial role of echocardiography and magnetic resonance imaging before surgery.
{"title":"A successful treatment of ischemic cardiomyopathy associated with left ventricular aneurysm and chronic ischaemic mitral regurgitation (RCD code: III-1B.9.o).","authors":"Klaudia Knap, Leszek Drabik, J. Błaut-Jurkowska, Michal Pacia, Maciej Krupińsk, M. Urbańczyk‑Zawadzka, W. Płazak","doi":"10.20418/JRCD.VOL3NO4.271","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO4.271","url":null,"abstract":"Complications of myocardial infarction (MI) such as left ventricular aneurysm, chronic ischaemic mitral regurgitation (CIMR) and heart failure are associated with a poor prognosis. The management of complex defects is challenging and requires an individualized strategy. We describe a case of 44-year-old male with multiple complications of MI including heart failure with reduced ejection fraction, left ventricular aneurysm and severe CIMR. We present complex approach, aimed at correcting various components of ischemic cardiomyopathy: relieving ischemia, reducing left ventricular wall tension, reducing volume overload and pulmonary hypertension by mitral valve surgery and left ventricular reconstruction (also known as the Dor procedure). In this case report, we describe the crucial role of echocardiography and magnetic resonance imaging before surgery.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"66 2 1","pages":"122-128"},"PeriodicalIF":0.0,"publicationDate":"2017-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89869850","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 : 2017-07-05DOI: 10.20418/jrcd.vol3no3.297
P. Podolec
This June, here in Krakow we witness two important anniversaries. First, our hospital, now called after Pope John Paul II, is already 100 years old. Secondly, 20 years have already passed since the visit on the hospital’ grounds of our great patron – Pope John Paul II, still remembered in Krakow as Cardinal Karol Wojtyla. This was beautiful sunny and hot day in June 1997 when His Holiness blessed the hospital and said simple but remarkable words – “Let serve!”. Nothing more to add as this is exactly what it is all about. In the days when words do not mean much, it is of utmost importance to realize why and for whom we work. Despite numerous and absurd externally imposed regulations, despite growing financial debts of hospitals and outpatients clinics, despite all the “smart” people who constantly talk rather than do things, despite and despite … we can go on forever. Let’s be clear if we lose this compass, no matter how skilled and educated we are, this will be the beginning of our decline. We are lucky here in Krakow as this famous quote is engraved in the hospital’ lobby but in fact it should be engraved in everybody’s mind.
{"title":"“Let (the hospital) serve!”. Pope John Paul II. Visit at the John Paul II Hospital in Krakow, 9 June 1997","authors":"P. Podolec","doi":"10.20418/jrcd.vol3no3.297","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no3.297","url":null,"abstract":"This June, here in Krakow we witness two important anniversaries. First, our hospital, now called after Pope John Paul II, is already 100 years old. Secondly, 20 years have already passed since the visit on the hospital’ grounds of our great patron – Pope John Paul II, still remembered in Krakow as Cardinal Karol Wojtyla. This was beautiful sunny and hot day in June 1997 when His Holiness blessed the hospital and said simple but remarkable words – “Let serve!”. Nothing more to add as this is exactly what it is all about. In the days when words do not mean much, it is of utmost importance to realize why and for whom we work. Despite numerous and absurd externally imposed regulations, despite growing financial debts of hospitals and outpatients clinics, despite all the “smart” people who constantly talk rather than do things, despite and despite … we can go on forever. Let’s be clear if we lose this compass, no matter how skilled and educated we are, this will be the beginning of our decline. We are lucky here in Krakow as this famous quote is engraved in the hospital’ lobby but in fact it should be engraved in everybody’s mind.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"16 1","pages":"71-72"},"PeriodicalIF":0.0,"publicationDate":"2017-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86019541","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 : 2017-07-05DOI: 10.20418/jrcd.vol3no3.292
P. Rubis
{"title":"Report from the 66th American College of Cardiology Congress 2016 in Washington, DC, USA","authors":"P. Rubis","doi":"10.20418/jrcd.vol3no3.292","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no3.292","url":null,"abstract":"","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"84 1","pages":"101-102"},"PeriodicalIF":0.0,"publicationDate":"2017-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82371704","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 : 2017-07-05DOI: 10.20418/JRCD.VOL3NO3.283
O. Barabash, Y. Ivaniv, I. Ivaniv, Oksana Predzemirska
We present a case report of a 45‑year old man, who was admitted to medical center due to dyspnea and cough. Right bundle branch block was detected on electrocardiography. Transthoracic echocardiography demonstrated coronary sinus dilatation, enlargement of right‑side chambers, moderate to severe pulmonary hypertension and atrial septal defect with left‑to‑right shunt. Coronary sinus atrial septal defect was diagnosed by transesophageal echocardiography and computed tomography. Surgical repair of the defect was performed. Atrial flutter occurred in early postoperative period and was successfully treated by electrostimulation. Coronary sinus atrial septal defect in adults is a rare congenital heart disease. Transesophageal echocardiography and computed tomography are imaging modalities of choice in such cases. Surgical treatment is needed to prevent further complications. JRCD 2017; 3 (3): 86–88
{"title":"Coronary sinus atrial septal defect in adult (RCD code: IV‑2B.1)","authors":"O. Barabash, Y. Ivaniv, I. Ivaniv, Oksana Predzemirska","doi":"10.20418/JRCD.VOL3NO3.283","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO3.283","url":null,"abstract":"We present a case report of a 45‑year old man, who was admitted to medical center due to dyspnea and cough. Right bundle branch block was detected on electrocardiography. Transthoracic echocardiography demonstrated coronary sinus dilatation, enlargement of right‑side chambers, moderate to severe pulmonary hypertension and atrial septal defect with left‑to‑right shunt. Coronary sinus atrial septal defect was diagnosed by transesophageal echocardiography and computed tomography. Surgical repair of the defect was performed. Atrial flutter occurred in early postoperative period and was successfully treated by electrostimulation. Coronary sinus atrial septal defect in adults is a rare congenital heart disease. Transesophageal echocardiography and computed tomography are imaging modalities of choice in such cases. Surgical treatment is needed to prevent further complications. JRCD 2017; 3 (3): 86–88","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"34 1","pages":"86-88"},"PeriodicalIF":0.0,"publicationDate":"2017-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82705359","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 : 2017-07-05DOI: 10.20418/JRCD.VOL3NO3.266
Justyna Błaut-Jurkowska, M. Kaznica-Wiatr, Agnieszka Żygadło, L. Tomkiewicz-Pajak, P. Podolec, M. Olszowska
Sarcoidosis is a systemic inflammatory disease characterized by the presence of noncaseating granulomas. Etiology of the disease remains unknown. From 3.7% to 54.9% patients with extra‑cardiac sarcoidosis have asymptomatic heart involvement. Conduction abnormalities, arrhythmias and congestive heart failure are the most common clinical manifestations of cardiac sarcoidosis (CS). The aim of the study was to evaluate the type and frequency of electrocardiographic abnormalities in patients with pulmonary sarcoidosis and to compare differences in the occurrence of electrocardiographic changes between patients diagnosed with CS and patients without confirmation of CS. Materials and methods : 49 patients (18 women, 31 men), mean age 45.6 ±12.2 years with biopsy‑proven pulmonary sarcoidosis were enrolled in the study. The patients were divided into two groups. Group 1 consisted of 12 patients diagnosed with CS, and Group 2 of 37 patients without diagnosis of CS. 12‑lead baseline electrocardiogram (ECG) was recorded for all participants. Results : 89.8% patients with pulmonary sarcoidosis had abnormal ECG. The most common ECG abnormalities were ST‑T changes observed in 79.6%. Conduction abnormalities were present in 26.5% of patients. 22.45% patients had left axis deviation. Rhythm abnormalities were recorded in 20.4% of all analyzed ECGs. ECGs of 8.16% of patients met criteria of hypertrophy. There was a trend towards more frequent prevalence of some ECG changes in patients with CS than patients without CS. However, these differences were not statistically significant. Conclusions : We observed a trend towards more frequent prevalence of some ECG abnormalities in a group of patients with CS than in patients without CS. However, these differences were not statistically significant. ECG abnormalities in patients with pulmonary sarcoidosis require further diagnostics. JRCD 2017; 3 (3): 81–85
{"title":"Electrocardiographic abnormalities in patients with pulmonary sarcoidosis (RCD code: III)","authors":"Justyna Błaut-Jurkowska, M. Kaznica-Wiatr, Agnieszka Żygadło, L. Tomkiewicz-Pajak, P. Podolec, M. Olszowska","doi":"10.20418/JRCD.VOL3NO3.266","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO3.266","url":null,"abstract":"Sarcoidosis is a systemic inflammatory disease characterized by the presence of noncaseating granulomas. Etiology of the disease remains unknown. From 3.7% to 54.9% patients with extra‑cardiac sarcoidosis have asymptomatic heart involvement. Conduction abnormalities, arrhythmias and congestive heart failure are the most common clinical manifestations of cardiac sarcoidosis (CS). The aim of the study was to evaluate the type and frequency of electrocardiographic abnormalities in patients with pulmonary sarcoidosis and to compare differences in the occurrence of electrocardiographic changes between patients diagnosed with CS and patients without confirmation of CS. Materials and methods : 49 patients (18 women, 31 men), mean age 45.6 ±12.2 years with biopsy‑proven pulmonary sarcoidosis were enrolled in the study. The patients were divided into two groups. Group 1 consisted of 12 patients diagnosed with CS, and Group 2 of 37 patients without diagnosis of CS. 12‑lead baseline electrocardiogram (ECG) was recorded for all participants. Results : 89.8% patients with pulmonary sarcoidosis had abnormal ECG. The most common ECG abnormalities were ST‑T changes observed in 79.6%. Conduction abnormalities were present in 26.5% of patients. 22.45% patients had left axis deviation. Rhythm abnormalities were recorded in 20.4% of all analyzed ECGs. ECGs of 8.16% of patients met criteria of hypertrophy. There was a trend towards more frequent prevalence of some ECG changes in patients with CS than patients without CS. However, these differences were not statistically significant. Conclusions : We observed a trend towards more frequent prevalence of some ECG abnormalities in a group of patients with CS than in patients without CS. However, these differences were not statistically significant. ECG abnormalities in patients with pulmonary sarcoidosis require further diagnostics. JRCD 2017; 3 (3): 81–85","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"36 1","pages":"81-85"},"PeriodicalIF":0.0,"publicationDate":"2017-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86583138","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 : 2017-07-05DOI: 10.20418/JRCD.VOL3NO3.291
P. Matusik, Joanna Pudło, A. Rydlewska, J. Podolec, J. Lelakowski, P. Podolec
Brugada syndrome (BrS) is a cardiac channelopathy associated with ventricular arrhythmias and sudden cardiac death. Diagnosis of BrS is based on type 1 BrS electrocardiogram (ECG) pattern (coved pattern) presence, observed spontaneously or after provocation test. The worldwide prevalence of BrS ECG patterns is estimated to reach 0.4% and strongly depends on the population studied. BrS results from various genetic mutations of sodium, calcium and potassium channels and/or associated proteins affecting ion currents. SCN5A mutations are the most prevalent in BrS. Pathogenesis of BrS is explained by the depolarization theory, the repolarization theory and the neural crest theory, which seem to be complimentary, at least partially. This review summarizes current diagnostic criteria of BrS and epidemiology of BrS ECG patterns. We also discuss the recent understanding of BrS pathophysiology and the role of genetic testing in BrS. JRCD 2017; 3 (3): 73–80.
{"title":"Brugada syndrome: current diagnostics, epidemiology, genetic data and novel mechanisms (RCD code: V‑1A.1)","authors":"P. Matusik, Joanna Pudło, A. Rydlewska, J. Podolec, J. Lelakowski, P. Podolec","doi":"10.20418/JRCD.VOL3NO3.291","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO3.291","url":null,"abstract":"Brugada syndrome (BrS) is a cardiac channelopathy associated with ventricular arrhythmias and sudden cardiac death. Diagnosis of BrS is based on type 1 BrS electrocardiogram (ECG) pattern (coved pattern) presence, observed spontaneously or after provocation test. The worldwide prevalence of BrS ECG patterns is estimated to reach 0.4% and strongly depends on the population studied. BrS results from various genetic mutations of sodium, calcium and potassium channels and/or associated proteins affecting ion currents. SCN5A mutations are the most prevalent in BrS. Pathogenesis of BrS is explained by the depolarization theory, the repolarization theory and the neural crest theory, which seem to be complimentary, at least partially. This review summarizes current diagnostic criteria of BrS and epidemiology of BrS ECG patterns. We also discuss the recent understanding of BrS pathophysiology and the role of genetic testing in BrS. JRCD 2017; 3 (3): 73–80.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"50 1","pages":"73-80"},"PeriodicalIF":0.0,"publicationDate":"2017-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79947035","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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