Pub Date : 2017-03-31DOI: 10.20418/JRCD.VOL3NO2.260
J. Błaut-Jurkowska, M. Kaznica-Wiatr, Klaudia Knap, L. Tomkiewicz-Pajak, P. Podolec, M. Olszowska
Sarcoidosis is a systemic, granulomatous disease of unknown etiology. Cardiac involvement in the course of sarcoidosis occurs in 5% of patients. However, about 25% of patient with systemic/pulmonary sarcoidosis might present with asymptomatic myocardial injury. The main manifestations of cardiac sarcoidosis are conduction abnormalities, ventricular arrhythmias and heart failure. Diagnosis of cardiac sarcoidosis remains a challenge for physicians. Treatment should be introduced at the time of diagnosis to prevent potentially lethal progression of cardiac disease. Steroids are the first drugs of choice. Additional anti‑sarcoidosis agents are used as alternatives or to reduce the steroids dose. Some patients require implantation of a cardioverter‑defibrillator for the prevention of sudden cardiac death. Patients with refractory ventricular tachyarrhythmia or severe, intractable heart failure unresponsive to optimal pharmacotherapy, require heart transplantation. Prognosis in cardiac sarcoidosis is highly variable, dependent on location and severity of heart involvement. This article reviews current diagnostic and therapeutic recommendations for cardiac sarcoidosis. JRCD 2017; 3 (2): 37–43
{"title":"Cardiac sarcoidosis – management and prognosis (RCD code: III‑3A.3)","authors":"J. Błaut-Jurkowska, M. Kaznica-Wiatr, Klaudia Knap, L. Tomkiewicz-Pajak, P. Podolec, M. Olszowska","doi":"10.20418/JRCD.VOL3NO2.260","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO2.260","url":null,"abstract":"Sarcoidosis is a systemic, granulomatous disease of unknown etiology. Cardiac involvement in the course of sarcoidosis occurs in 5% of patients. However, about 25% of patient with systemic/pulmonary sarcoidosis might present with asymptomatic myocardial injury. The main manifestations of cardiac sarcoidosis are conduction abnormalities, ventricular arrhythmias and heart failure. Diagnosis of cardiac sarcoidosis remains a challenge for physicians. Treatment should be introduced at the time of diagnosis to prevent potentially lethal progression of cardiac disease. Steroids are the first drugs of choice. Additional anti‑sarcoidosis agents are used as alternatives or to reduce the steroids dose. Some patients require implantation of a cardioverter‑defibrillator for the prevention of sudden cardiac death. Patients with refractory ventricular tachyarrhythmia or severe, intractable heart failure unresponsive to optimal pharmacotherapy, require heart transplantation. Prognosis in cardiac sarcoidosis is highly variable, dependent on location and severity of heart involvement. This article reviews current diagnostic and therapeutic recommendations for cardiac sarcoidosis. JRCD 2017; 3 (2): 37–43","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"31 1","pages":"37-43"},"PeriodicalIF":0.0,"publicationDate":"2017-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87846374","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 : 2016-12-30DOI: 10.20418/jrcd.vol3no1.264
K. Holcman, P. Rubis, S. Wiśniowska-Śmiałek, A. Leśniak‑Sobelga, M. Hlawaty, M. Kostkiewicz, P. Podolec
Hemochromatosis is a disease resulting from excessive deposition of iron in parenchymal tissues. The most common form of this disease is associated with the homozygous p.Cys282Tyr mutation of the HFE gene. It leads to multisystemic disease including iron overload cardiomyopathy. Heterozygotes usually do not express a hemochromatosis phenotype, however there are known cases of iron overload in this group of patients. We present a case of 48-year old man, with family history of hereditary hemochromatosis, p.Cys282Tyr mutation carrier, who was admitted to cardiology department due to persistent atrial fibrillation episode. Laboratory tests revealed transferrin serum iron saturation value of 47,8% with other parameters of iron metabolism within the reference range. Transthoracic echocardiographic study showed image consistent with hypertrophic cardiomyopathy. Sinus rhythm was successfully restored by synchronized electrical cardioversion. Based on cardiovascular magnetic resonance imaging cardiac iron overload cardiomyopathy was ruled out. He was discharged home in good general condition without symptoms. JRCD 2016; 3 (1): 24–27
{"title":"Hypertrophic cardiomyopathy or hereditary hemochromatosis? (RCD code: III‑2B.3.o)","authors":"K. Holcman, P. Rubis, S. Wiśniowska-Śmiałek, A. Leśniak‑Sobelga, M. Hlawaty, M. Kostkiewicz, P. Podolec","doi":"10.20418/jrcd.vol3no1.264","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no1.264","url":null,"abstract":"Hemochromatosis is a disease resulting from excessive deposition of iron in parenchymal tissues. The most common form of this disease is associated with the homozygous p.Cys282Tyr mutation of the HFE gene. It leads to multisystemic disease including iron overload cardiomyopathy. Heterozygotes usually do not express a hemochromatosis phenotype, however there are known cases of iron overload in this group of patients. We present a case of 48-year old man, with family history of hereditary hemochromatosis, p.Cys282Tyr mutation carrier, who was admitted to cardiology department due to persistent atrial fibrillation episode. Laboratory tests revealed transferrin serum iron saturation value of 47,8% with other parameters of iron metabolism within the reference range. Transthoracic echocardiographic study showed image consistent with hypertrophic cardiomyopathy. Sinus rhythm was successfully restored by synchronized electrical cardioversion. Based on cardiovascular magnetic resonance imaging cardiac iron overload cardiomyopathy was ruled out. He was discharged home in good general condition without symptoms. JRCD 2016; 3 (1): 24–27","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"13 1","pages":"24-27"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86112886","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 : 2016-12-30DOI: 10.20418/JRCD.VOL3NO1.273
Monika Smaś‑Suska, L. Tomkiewicz-Pajak, M. Olszowska, P. Podolec
The presented case report illustrates a rare and unexpected cause of tachycardia and exercise tolerance reduction in a young patient with noncoronary sinus of Valsalva aneurysm rupture. The patient was referred to our hospital due to complaints of palpitation, dyspnoea and severe reduction of exercise tolerance of acute onset. Transthoracic echocardiography revealed a shunt between aorta and right atrium, moderate aortic and tricuspid regurgitation and signs of hyperkinetic circulation. Transesophageal echocardiogram confirmed the presence of ruptured aneurysm. The patient was consulted by a local multidisciplinary Heart Team and referred for cardiac surgery, after which he recovered without any complications. JRCD 2016; 3 (1): 17–19
{"title":"Ruptured aneurysm of sinus of Valsalva – an unexpected cause of tachycardia and severe dyspnea in a young patient (RCD code: I‑1B.0)","authors":"Monika Smaś‑Suska, L. Tomkiewicz-Pajak, M. Olszowska, P. Podolec","doi":"10.20418/JRCD.VOL3NO1.273","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO1.273","url":null,"abstract":"The presented case report illustrates a rare and unexpected cause of tachycardia and exercise tolerance reduction in a young patient with noncoronary sinus of Valsalva aneurysm rupture. The patient was referred to our hospital due to complaints of palpitation, dyspnoea and severe reduction of exercise tolerance of acute onset. Transthoracic echocardiography revealed a shunt between aorta and right atrium, moderate aortic and tricuspid regurgitation and signs of hyperkinetic circulation. Transesophageal echocardiogram confirmed the presence of ruptured aneurysm. The patient was consulted by a local multidisciplinary Heart Team and referred for cardiac surgery, after which he recovered without any complications. JRCD 2016; 3 (1): 17–19","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"236 1","pages":"17-19"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74692662","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 : 2016-12-30DOI: 10.20418/JRCD.VOL3NO1.261
Piotr Liszniański, Piotr Nowak, P. Stryjewski
The authors report a case of a 58-year-old man with coronary artery disease and left ventricular aneurysm following inferior wall infarction, who was implanted with a single chamber cardioverter-defibrillator (ICD) for primary prevention of sudden cardiac death. The patient presented with complaints including multiple ICD shocks due to recurrent ventricular tachycardia (VT) resistant to the implemented treatment, decreased quality of life and high level of anxiety associated with multiple hospitalizations. Pharmacological treatment was unsuccessful and led to drug-induced bradycardia. Due to developed pacemaker syndrome the patient underwent ICD upgrade to a dual-chamber device followed by radiofrequency ablation. Despite a number of interventions, VT did not completely disappear. A slight decrease in the number of ICD shocks was observed. After adjustment of the pacing lower rate up to 75 bpm, ventricular arrhythmias were reduced and eventually retreated. In the six months follow-up period no VT was recorded. The authors discuss the current recommendations for VT treatment with the indications for re-ablation or aneurysmectomy. JRCD 2016; 3 (1): 20–23
{"title":"Patient with multiple ICD interventions (RCD code: V‑2A.O)","authors":"Piotr Liszniański, Piotr Nowak, P. Stryjewski","doi":"10.20418/JRCD.VOL3NO1.261","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO1.261","url":null,"abstract":"The authors report a case of a 58-year-old man with coronary artery disease and left ventricular aneurysm following inferior wall infarction, who was implanted with a single chamber cardioverter-defibrillator (ICD) for primary prevention of sudden cardiac death. The patient presented with complaints including multiple ICD shocks due to recurrent ventricular tachycardia (VT) resistant to the implemented treatment, decreased quality of life and high level of anxiety associated with multiple hospitalizations. Pharmacological treatment was unsuccessful and led to drug-induced bradycardia. Due to developed pacemaker syndrome the patient underwent ICD upgrade to a dual-chamber device followed by radiofrequency ablation. Despite a number of interventions, VT did not completely disappear. A slight decrease in the number of ICD shocks was observed. After adjustment of the pacing lower rate up to 75 bpm, ventricular arrhythmias were reduced and eventually retreated. In the six months follow-up period no VT was recorded. The authors discuss the current recommendations for VT treatment with the indications for re-ablation or aneurysmectomy. JRCD 2016; 3 (1): 20–23","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"51 1","pages":"20-23"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85799361","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 : 2016-12-30DOI: 10.20418/JRCD.VOL3NO1.272
P. Podolec
Saint Mother Teresa of Calcutta Dear Readers, Before we go any further, try to ponder the quote once said by the Saint Teresa of Calcutta. She was by far one of the most entitled person in the world to say such an unpleasant truth. Certainly, for her entire life she had been working in extreme conditions in rural and underdeveloped areas in India. In the lands where bowl of hot soup, slice of whole‑meal bread and clean bed sheets were unobtainable and priceless commodities. But is that all? No, she perfectly knew that above all, recognition of every human‑being is the crucial thing. Those poor who were cared by her and her convention, could easily understand they are not treated as applicants but as subjects. This difference is of paramount importance and especially applies to the contemporary medicine. In the era of wealth, insurance, procedures, reimbursements, etc., we no longer spend a minute to see the patient in front us as a whole. Some would immediately start to think what is the best arterial access (either femoral or radial), some would argue that magnetic resonance is superior to echocardiography, others would say what guidelines recommend… It is a bit sad, indeed! In most cases, we really do not know what the true patient’s needs are. As a poor excuse, we focus our attention on tiny fragments of patient’s body and boldly claim that we cured him. Too often we try to fix one small body part (sometimes with too much risks and costs both from the patients and health ser‑ vice). How naive it is! Then we send the patient home, as quickly as possible, and are surprised to learn that the patient is not feeling so great in the follow‑up visits. Why is that? One might say, he needs one more procedure and it may be true but in the majority of cases, the patient needs our recognition, he wants us to see him as a human not as a diseased valve, atherosclerotic vessel or infarcted myocardium. Sometimes the hon‑ est conversation is much better that repeated (painful) examinations or procedures. Obviously, we cannot change our habits in a day but maybe it is a time think once more about the patient as a subject not an applicant.In this last issue of the Journal in 2016, majority of articles is dedicated to inherited cardiac disease. We start with an interesting Review on ophthalmic manifestations in Kearns‑Sayres syndrome. Further, there is the Original paper on long‑term observation following Ross operation. Obeying our policy, the middle part is composed of four clinical cases of rare cardiovascular diseases that are presented in details and commented by the managing teams. In line with the foreword, this issue ends with the report from the joint meeting of patients with pulmonary hypertension and their families with managing physicians. (...)
{"title":"One of the greatest disease is to be nobody to anybody","authors":"P. Podolec","doi":"10.20418/JRCD.VOL3NO1.272","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO1.272","url":null,"abstract":"Saint Mother Teresa of Calcutta Dear Readers, Before we go any further, try to ponder the quote once said by the Saint Teresa of Calcutta. She was by far one of the most entitled person in the world to say such an unpleasant truth. Certainly, for her entire life she had been working in extreme conditions in rural and underdeveloped areas in India. In the lands where bowl of hot soup, slice of whole‑meal bread and clean bed sheets were unobtainable and priceless commodities. But is that all? No, she perfectly knew that above all, recognition of every human‑being is the crucial thing. Those poor who were cared by her and her convention, could easily understand they are not treated as applicants but as subjects. This difference is of paramount importance and especially applies to the contemporary medicine. In the era of wealth, insurance, procedures, reimbursements, etc., we no longer spend a minute to see the patient in front us as a whole. Some would immediately start to think what is the best arterial access (either femoral or radial), some would argue that magnetic resonance is superior to echocardiography, others would say what guidelines recommend… It is a bit sad, indeed! In most cases, we really do not know what the true patient’s needs are. As a poor excuse, we focus our attention on tiny fragments of patient’s body and boldly claim that we cured him. Too often we try to fix one small body part (sometimes with too much risks and costs both from the patients and health ser‑ vice). How naive it is! Then we send the patient home, as quickly as possible, and are surprised to learn that the patient is not feeling so great in the follow‑up visits. Why is that? One might say, he needs one more procedure and it may be true but in the majority of cases, the patient needs our recognition, he wants us to see him as a human not as a diseased valve, atherosclerotic vessel or infarcted myocardium. Sometimes the hon‑ est conversation is much better that repeated (painful) examinations or procedures. Obviously, we cannot change our habits in a day but maybe it is a time think once more about the patient as a subject not an applicant.In this last issue of the Journal in 2016, majority of articles is dedicated to inherited cardiac disease. We start with an interesting Review on ophthalmic manifestations in Kearns‑Sayres syndrome. Further, there is the Original paper on long‑term observation following Ross operation. Obeying our policy, the middle part is composed of four clinical cases of rare cardiovascular diseases that are presented in details and commented by the managing teams. In line with the foreword, this issue ends with the report from the joint meeting of patients with pulmonary hypertension and their families with managing physicians. (...)","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"35 1","pages":"3-4"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77302937","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 : 2016-12-30DOI: 10.20418/jrcd.vol3no1.257
M. Szwabowicz, J. Miszczyk
Kearns-Sayre Syndrome is a multisystemic mitochondrial cytopathy characterized by specific ophthalmic signs, cardiac conduction disturbances with endocrine, musculoskeletal and central nervous system involvement. As a highly heterogeneous condition, establishing an accurate diagnosis of this disorder can often be seriously delayed. It usually occurs before the age of 20 with ocular symptoms at first. Cardiac manifestations include progressive degeneration of the conduction tissue, leading to different types of conduction disturbances, which in many cases are responsible for significant decrease of life expectancy. There is currently no causative therapy available for Kearns-Sayre Syndrome patients. Several interventions including ophthalmic or neurological may be necessary in order to improve the quality of life, however improving prognosis in this group of patients impose prompt recognitions of those, who require early pacemaker implantation. Therefore, the aim of this article is to review the current knowledge about Kearns-Sayre Syndrome in light of the most typical ophthalmic findings, which can handily be detected by cardiologists and applied to accelerate accurate diagnosis and elaborate the most appropriate therapeutic strategies. JRCD 2016; 3 (1): 5–8
{"title":"Ophthalmic manifestations suggesting Kearns--Sayre Syndrome among young adults with cardiac conduction defects (RCD code: III-1A.5a)","authors":"M. Szwabowicz, J. Miszczyk","doi":"10.20418/jrcd.vol3no1.257","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no1.257","url":null,"abstract":"Kearns-Sayre Syndrome is a multisystemic mitochondrial cytopathy characterized by specific ophthalmic signs, cardiac conduction disturbances with endocrine, musculoskeletal and central nervous system involvement. As a highly heterogeneous condition, establishing an accurate diagnosis of this disorder can often be seriously delayed. It usually occurs before the age of 20 with ocular symptoms at first. Cardiac manifestations include progressive degeneration of the conduction tissue, leading to different types of conduction disturbances, which in many cases are responsible for significant decrease of life expectancy. There is currently no causative therapy available for Kearns-Sayre Syndrome patients. Several interventions including ophthalmic or neurological may be necessary in order to improve the quality of life, however improving prognosis in this group of patients impose prompt recognitions of those, who require early pacemaker implantation. Therefore, the aim of this article is to review the current knowledge about Kearns-Sayre Syndrome in light of the most typical ophthalmic findings, which can handily be detected by cardiologists and applied to accelerate accurate diagnosis and elaborate the most appropriate therapeutic strategies. JRCD 2016; 3 (1): 5–8","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"19 1","pages":"5-8"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87699284","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 : 2016-12-30DOI: 10.20418/JRCD.VOL3NO1.274
J. Stępniewski
Annual Meeting of the Pulmonary Hypertension Patients and their Friends organized by Pulmonary Hypertension Association (PHA) of Poland was held on June 11, 2016 at the Department of Cardiac and Vascular Diseases of the John Paul II Hospital in Kra‑ kow, a referral pulmonary hypertension (PH) Centre. The Meet‑ ing attracted a great number of PH patients with their families and caregivers. More than 130 participants were hosted by the Di‑ rector of the John Paul II Hospital – dr Anna Prokop‑Staszecka, the Head of the Department of Cardiac and Vascular Diseases – prof. Piotr Podolec, the Coordinator of the PH Programme – assoc. prof. Grzegorz Kopec, and the President of PHA Poland – Mrs Alicja Morze, to spend a pleasant afternoon together and talk about lights and shadows of being a PH patient or a relative or a caregiver of such. (...)
{"title":"Highlights from the Polish Pulmonary Hypertension Patients and their Friends Meeting in Krakow","authors":"J. Stępniewski","doi":"10.20418/JRCD.VOL3NO1.274","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO1.274","url":null,"abstract":"Annual Meeting of the Pulmonary Hypertension Patients and their Friends organized by Pulmonary Hypertension Association (PHA) of Poland was held on June 11, 2016 at the Department of Cardiac and Vascular Diseases of the John Paul II Hospital in Kra‑ kow, a referral pulmonary hypertension (PH) Centre. The Meet‑ ing attracted a great number of PH patients with their families and caregivers. More than 130 participants were hosted by the Di‑ rector of the John Paul II Hospital – dr Anna Prokop‑Staszecka, the Head of the Department of Cardiac and Vascular Diseases – prof. Piotr Podolec, the Coordinator of the PH Programme – assoc. prof. Grzegorz Kopec, and the President of PHA Poland – Mrs Alicja Morze, to spend a pleasant afternoon together and talk about lights and shadows of being a PH patient or a relative or a caregiver of such. (...)","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"24 1","pages":"29-30"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88952948","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 : 2016-12-30DOI: 10.20418/JRCD.VOL3NO1.265
Aleksandra Lenart-Migdalska, Monika Smaś‑Suska, Klaudia Knap, M. Kaznica-Wiatr, M. Olszowska, P. Podolec, L. Tomkiewicz-Pajak
Aims: The aim of this study was to analyze the incidence of long-term complications observed during follow-up of patients who had undergone the Ross procedure in childhood. Methods: The study engaged a cohort of 9 patients, all of whom were between 19 to 32 years old. Patients had been in the care of the Centre for Rare Cardiovascular Diseases since the age of 18. Clinical and echocardiographic data were collected from the follow-up period. Results: 78% of patients had preserved global systolic function of the left ventricle, and 56% had dilatation of the ascending aorta. Due to the aneurysm of the ascending aorta one patient required the Bentall de Bono procedure. Another patient underwent a reoperation because of endocarditis of the pulmonary homograft with severe aortic and pulmonary regurgitation. 1/3 of the studied patients were being considered for a reoperation. 23% of patients developed severe pulmonary valve regurgitation, 33% moderate. 67% of patients developed mild to moderate pulmonary valve stenosis. Most patients were in NYHA class I-II. Conclusion: Late complications are frequent in this group of patients, and hence they require surveillance in specialized Centers for Grown-up Congenital Heart Diseases. JRCD 2016; 3 (1): 9–13
{"title":"Long term follow-up after the Ross procedure (RCD code: IV-5A.O)","authors":"Aleksandra Lenart-Migdalska, Monika Smaś‑Suska, Klaudia Knap, M. Kaznica-Wiatr, M. Olszowska, P. Podolec, L. Tomkiewicz-Pajak","doi":"10.20418/JRCD.VOL3NO1.265","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO1.265","url":null,"abstract":"Aims: The aim of this study was to analyze the incidence of long-term complications observed during follow-up of patients who had undergone the Ross procedure in childhood. Methods: The study engaged a cohort of 9 patients, all of whom were between 19 to 32 years old. Patients had been in the care of the Centre for Rare Cardiovascular Diseases since the age of 18. Clinical and echocardiographic data were collected from the follow-up period. Results: 78% of patients had preserved global systolic function of the left ventricle, and 56% had dilatation of the ascending aorta. Due to the aneurysm of the ascending aorta one patient required the Bentall de Bono procedure. Another patient underwent a reoperation because of endocarditis of the pulmonary homograft with severe aortic and pulmonary regurgitation. 1/3 of the studied patients were being considered for a reoperation. 23% of patients developed severe pulmonary valve regurgitation, 33% moderate. 67% of patients developed mild to moderate pulmonary valve stenosis. Most patients were in NYHA class I-II. Conclusion: Late complications are frequent in this group of patients, and hence they require surveillance in specialized Centers for Grown-up Congenital Heart Diseases. JRCD 2016; 3 (1): 9–13","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"9 1","pages":"9-13"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90742605","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 : 2016-12-30DOI: 10.20418/JRCD.VOL3NO1.236
Jacek Kuźma, A. Rudziński, G. Kopeć, P. Weryński, Dorota Palczewska, D. Porada, M. Rączka, M. Pitak
Development of irreversible pulmonary hypertension in D-transposition of the great arteries (D-TGA) with ventricular septal defect (VSD) is a well-known phenomenon. Coexistence of left ventricular outflow tract obstruction (LVOTO) should theoretically protect the patient against pulmonary arterial hypertension (PAH). Application of the Blalock-Taussig shunt (B-TS) temporarily improves condition of the patient, but may contribute to irreversible PAH requiring palliative complex therapy. We present a case of a 18 year-old female with congenital complex heart defect: D-TGA with inflow VSD and subpulmonary stenosis, Eisenmenger syndrome, who was treated with several subsequent palliative cardiosurgical operations, including B-TS and Senning procedure and eventually, combined PAH-specific therapy, what resulted in stabilization of clinical status. JRCD 2016; 3 (1): 14–16
{"title":"Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II‑1A.4d)","authors":"Jacek Kuźma, A. Rudziński, G. Kopeć, P. Weryński, Dorota Palczewska, D. Porada, M. Rączka, M. Pitak","doi":"10.20418/JRCD.VOL3NO1.236","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO1.236","url":null,"abstract":"Development of irreversible pulmonary hypertension in D-transposition of the great arteries (D-TGA) with ventricular septal defect (VSD) is a well-known phenomenon. Coexistence of left ventricular outflow tract obstruction (LVOTO) should theoretically protect the patient against pulmonary arterial hypertension (PAH). Application of the Blalock-Taussig shunt (B-TS) temporarily improves condition of the patient, but may contribute to irreversible PAH requiring palliative complex therapy. We present a case of a 18 year-old female with congenital complex heart defect: D-TGA with inflow VSD and subpulmonary stenosis, Eisenmenger syndrome, who was treated with several subsequent palliative cardiosurgical operations, including B-TS and Senning procedure and eventually, combined PAH-specific therapy, what resulted in stabilization of clinical status. JRCD 2016; 3 (1): 14–16","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"15 1","pages":"14-16"},"PeriodicalIF":0.0,"publicationDate":"2016-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87068795","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 : 2016-10-30DOI: 10.20418/JRCD.VOL2NO8.248
A. Sarnecka, A. Tyrka, G. Kopeć, P. Podolec
Pulmonary atresia with ventricular septal defect (PA + VSD) is a cyanotic congenital heart disease, also classified as Tetralogy of Fallot with pulmonary atresia. PA + VSD accounts for about 1–2% of congenital heart defects. The intracardiac anatomy is similar to tetralogy of Fallot but there is no direct communication between the right ventricle and pulmonary arteries. Major problems with surgical treatment are related to complexity of the pulmonary vascular bed. We report a case of a 50-year-old woman with congenital heart disease who was admitted to our Centre in July 2012. Congenital heart disease was first diagnosed at the age of 28 and at that time it was classified as a pulmonary valve atresia with ventricular septal defect with right-to-left shunt and common arterial trunk. Diagnostics performed in our Centre confirmed complicated anatomy of vessels in the chest, especially narrow and hypoplastic major aortopulmonary collateral arteries arising from descending aorta and left subclavian artery. The congenital heart disease was reclassified as a pulmonary atresia with ventricular septal defect. JRCD 2016; 2 (8): 270–274
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