Pub Date : 2018-06-25DOI: 10.20418/jrcd.vol3no7.326
Damian R. Maciejewski, P. Paluszek, Roman Machnik, Łukasz M. Tekieli, M. Trystuła, M. Kazibudzki, R. Musiał, J. Legutko, P. Pienia̧žek
We present the case of a 79‑year‑old woman with critical right internal carotid artery stenosis in whom carotid artery stenting was performed via a right radial artery approach with the use of a novel integrated embolic protection technology- Paladin System® (Contego Medical). JRCD 2018; 3 (7): 239–242
{"title":"Transradial carotid artery stenting with the use of a novel integrated embolic protection technology (RCD code: I‑1B.O)","authors":"Damian R. Maciejewski, P. Paluszek, Roman Machnik, Łukasz M. Tekieli, M. Trystuła, M. Kazibudzki, R. Musiał, J. Legutko, P. Pienia̧žek","doi":"10.20418/jrcd.vol3no7.326","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no7.326","url":null,"abstract":"We present the case of a 79‑year‑old woman with critical right internal carotid artery stenosis in whom carotid artery stenting was performed via a right radial artery approach with the use of a novel integrated embolic protection technology- Paladin System® (Contego Medical). JRCD 2018; 3 (7): 239–242","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"77 1","pages":"239"},"PeriodicalIF":0.0,"publicationDate":"2018-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83694845","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 : 2018-06-25DOI: 10.20418/JRCD.VOL3NO7.322
E. Lewicka, A. Dąbrowska-Kugacka, M. Chmara, B. Wasąg
Pulmonary arterial hypertension (PAH) is a rare disease with a high mortality and complex pathomechanism. Recent studies suggest an important role of genetic factors in the development of PAH. It was shown that patients with BMPR2 mutations present a disease at an earlier age and have more severe haemodynamics at diagnosis. Interestingly, in this disorder lifelong penetrance is estimated to be only about 20% and the mechanism of this phenomenon remains unknown. More recent studies focused on the mutational analysis of genes involved in TGFs signaling pathway in patients with PAH. The results of these studies are very promising but they still need to be confirmed. Moreover, data on the impact of identified mutations on the clinical course, PAH-specific treatment and prognosis in PAH is required. In Poland a multi-center study is planned to include patients from referral centers for pulmonary hypertension with diagnosed idiopathic, hereditary and drug-induced PAH or of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH). The aim of the study is to perform an extended molecular analysis for better understanding the molecular basis of pathogenesis of PAH, incomplete penetrance and to create an algorithm of molecular diagnosis of PAH patients. On the basis of the nationwide registry of patients with PAH treated in referral centers in Poland the effect of the detected mutations on the clinical course, the effect of PAH-specific treatment and the prognosis in PAH will be assessed.
{"title":"Genetics and genetic testing in pulmonary arterial hypertension (RCD code: II-1A.2)","authors":"E. Lewicka, A. Dąbrowska-Kugacka, M. Chmara, B. Wasąg","doi":"10.20418/JRCD.VOL3NO7.322","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO7.322","url":null,"abstract":"Pulmonary arterial hypertension (PAH) is a rare disease with a high mortality and complex pathomechanism. Recent studies suggest an important role of genetic factors in the development of PAH. It was shown that patients with BMPR2 mutations present a disease at an earlier age and have more severe haemodynamics at diagnosis. Interestingly, in this disorder lifelong penetrance is estimated to be only about 20% and the mechanism of this phenomenon remains unknown. More recent studies focused on the mutational analysis of genes involved in TGFs signaling pathway in patients with PAH. The results of these studies are very promising but they still need to be confirmed. Moreover, data on the impact of identified mutations on the clinical course, PAH-specific treatment and prognosis in PAH is required. In Poland a multi-center study is planned to include patients from referral centers for pulmonary hypertension with diagnosed idiopathic, hereditary and drug-induced PAH or of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH). The aim of the study is to perform an extended molecular analysis for better understanding the molecular basis of pathogenesis of PAH, incomplete penetrance and to create an algorithm of molecular diagnosis of PAH patients. On the basis of the nationwide registry of patients with PAH treated in referral centers in Poland the effect of the detected mutations on the clinical course, the effect of PAH-specific treatment and the prognosis in PAH will be assessed.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"25 1","pages":"226"},"PeriodicalIF":0.0,"publicationDate":"2018-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87383253","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 : 2018-06-25DOI: 10.20418/JRCD.VOL3NO7.324
Mateusz Wisniowski, P. Wańczura, Wojciech Stecko
We present the case of a patient with a large coronary fistula.Coronarography of the patient admitted to the Cardiology Department with STE‑ACS revealed a large fistula from the circumflex branch of the left coronary artery to the right atrium. The patient underwent successful percutaneous embolisation with three vascular coils. Currently he is under close ambulatory observation. JRCD 2018; 3 (7): 243–245.
{"title":"Coronary fistula to the right atrium (RCD code: I‑1C.4)","authors":"Mateusz Wisniowski, P. Wańczura, Wojciech Stecko","doi":"10.20418/JRCD.VOL3NO7.324","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO7.324","url":null,"abstract":"We present the case of a patient with a large coronary fistula.Coronarography of the patient admitted to the Cardiology Department with STE‑ACS revealed a large fistula from the circumflex branch of the left coronary artery to the right atrium. The patient underwent successful percutaneous embolisation with three vascular coils. Currently he is under close ambulatory observation. JRCD 2018; 3 (7): 243–245.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"82 1","pages":"243"},"PeriodicalIF":0.0,"publicationDate":"2018-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75001615","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 : 2018-04-13DOI: 10.20418/JRCD.VOL3NO6.320
Sylwia Iwańczyk, T. Mularek-Kubzdela
Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Due to the narrowing of the blood vessels in the lungs, pul- monary vascular resistance and pulmonary pressures increase. This results in reduced cardiac output, right heart failure and ultimately in death. Pulmonary hypertension, particularly PAH, is an independent risk factor for both peri-operative complications and post-operative mortality. The peri-operative management of patients with PAH is particularly challenging and requires a multidisciplinary approach. Appropriate patient preparation requires an accurate assessment of the severity of pulmonary hypertension, comorbidities and the type of surgery to be performed. Additionally, patients with PAH are more likely to develop an infection, particularly of the respiratory system. Infections are important risk factors for disease exacerbation, often affecting prognosis. For this reason, there is a need for effective prophylactic, diagnostic and rapid therapeutic strategies in PAH patients admitted with suspected infection. While pregnancy is not con- sidered a disease, it is associated with a significant mortality and morbidity risk in patients with PAH and therefore it is contraindicated in this group. Thus, the proper education of patients and effective contraception are necessary in order to minimize health risks. If a woman decides to maintain her pregnancy, careful monitoring, specific treatment optimization and close co-operation with an obstetrician are needed. The proper assessment of the severity of PAH and the optimization of specific treatments are crucial to improve the prognosis of PAH patients in all high-risk conditions. Additionally, the early diagnosis of high-risk conditions allows for early intensive treatment or control, which should be performed at a PAH treatment referral centre. JRCD 2018; 3 (6): 194–198
{"title":"Pulmonary arterial hypertension: management in specific medical conditions (RCD code: II‑1A.1)","authors":"Sylwia Iwańczyk, T. Mularek-Kubzdela","doi":"10.20418/JRCD.VOL3NO6.320","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO6.320","url":null,"abstract":"Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Due to the narrowing of the blood vessels in the lungs, pul- monary vascular resistance and pulmonary pressures increase. This results in reduced cardiac output, right heart failure and ultimately in death. Pulmonary hypertension, particularly PAH, is an independent risk factor for both peri-operative complications and post-operative mortality. The peri-operative management of patients with PAH is particularly challenging and requires a multidisciplinary approach. Appropriate patient preparation requires an accurate assessment of the severity of pulmonary hypertension, comorbidities and the type of surgery to be performed. Additionally, patients with PAH are more likely to develop an infection, particularly of the respiratory system. Infections are important risk factors for disease exacerbation, often affecting prognosis. For this reason, there is a need for effective prophylactic, diagnostic and rapid therapeutic strategies in PAH patients admitted with suspected infection. While pregnancy is not con- sidered a disease, it is associated with a significant mortality and morbidity risk in patients with PAH and therefore it is contraindicated in this group. Thus, the proper education of patients and effective contraception are necessary in order to minimize health risks. If a woman decides to maintain her pregnancy, careful monitoring, specific treatment optimization and close co-operation with an obstetrician are needed. The proper assessment of the severity of PAH and the optimization of specific treatments are crucial to improve the prognosis of PAH patients in all high-risk conditions. Additionally, the early diagnosis of high-risk conditions allows for early intensive treatment or control, which should be performed at a PAH treatment referral centre. JRCD 2018; 3 (6): 194–198","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"91 1","pages":"193-197"},"PeriodicalIF":0.0,"publicationDate":"2018-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73532737","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 : 2018-04-13DOI: 10.20418/JRCD.VOL3NO6.300
Fateh Ali Tipoo Sultan, B. Hussain, F. Rasheed
Budd‑Chiari syndrome (BCS) is a rare clinical disorder which is caused by obstruction of the major hepatic veins or suprahepatic portion of the inferior vena cava. Either thrombosis or a mechanical venous obstruction may be present. BCS has been shown to be associated with a number of diseases including polycythaemia vera, myeloproliferative disorders, malignancy or congenital web of the gastro‑intestinal tract. Right‑sided cardiac tumours, although rarely reported as a cause for BCS, are generally considered as a surgically curable cause for this otherwise potentially lethal clinical condition. JRCD 2018; 3 (6): 219–221.
{"title":"Right atrial myxoma in a patient with Budd‑Chiari syndrome (RCD code: VI‑1A.1)","authors":"Fateh Ali Tipoo Sultan, B. Hussain, F. Rasheed","doi":"10.20418/JRCD.VOL3NO6.300","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO6.300","url":null,"abstract":"Budd‑Chiari syndrome (BCS) is a rare clinical disorder which is caused by obstruction of the major hepatic veins or suprahepatic portion of the inferior vena cava. Either thrombosis or a mechanical venous obstruction may be present. BCS has been shown to be associated with a number of diseases including polycythaemia vera, myeloproliferative disorders, malignancy or congenital web of the gastro‑intestinal tract. Right‑sided cardiac tumours, although rarely reported as a cause for BCS, are generally considered as a surgically curable cause for this otherwise potentially lethal clinical condition. JRCD 2018; 3 (6): 219–221.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"49 1","pages":"218-220"},"PeriodicalIF":0.0,"publicationDate":"2018-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81109200","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 : 2018-04-13DOI: 10.20418/jrcd.vol3no6.315
W. Serra, G. Pastorini
Treatment of Fabry disease has improved since the introduction of enzyme replacement therapy (ERT). Two preparations of the recombinant enzyme α‑galactosidase A are available: agalsidase alfa and agalsidase beta. We aim to report on a disease improvement after switching therapy from agalsidase alfa to agalsidase beta in a patient with Fabry cardiomyopathy. We present a case of a 60‑year‑old male with chronic renal failure and hypothyroidism, diagnosed with Fabry disease in 2010. We investigated clinical changes in this patient during the 12‑months follow‑up. At the time of diagnosis, transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) showed widespread transmural inferolateral late enhancement (LE), poor ejection fraction and severe left ventricular hypertrophy. Despite of initiation of ERT with agalsidase alfa, clinical status of the patient did not improve. A shift to agalsidase beta was made, what resulted in marked betterment. Effectiveness of ERT on Fabry cardiomyopathy primarily depends on the stage of disease at baseline, since ERT may provide limited benefits in patients with evidence of fibrosis in CMR. Switch from agalsidase alfa to agalsidase beta may be needed in some patients, who do not improve on the first‑line therapy. JRCD 2018; 3 (6): 210–213
{"title":"Biventricular cardiomyopathy improvement by shifting therapy from agalsidase alfa to agalsidase beta in Anderson‑Fabry Disease (RCD code: III‑3B.2)","authors":"W. Serra, G. Pastorini","doi":"10.20418/jrcd.vol3no6.315","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no6.315","url":null,"abstract":"Treatment of Fabry disease has improved since the introduction of enzyme replacement therapy (ERT). Two preparations of the recombinant enzyme α‑galactosidase A are available: agalsidase alfa and agalsidase beta. We aim to report on a disease improvement after switching therapy from agalsidase alfa to agalsidase beta in a patient with Fabry cardiomyopathy. We present a case of a 60‑year‑old male with chronic renal failure and hypothyroidism, diagnosed with Fabry disease in 2010. We investigated clinical changes in this patient during the 12‑months follow‑up. At the time of diagnosis, transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) showed widespread transmural inferolateral late enhancement (LE), poor ejection fraction and severe left ventricular hypertrophy. Despite of initiation of ERT with agalsidase alfa, clinical status of the patient did not improve. A shift to agalsidase beta was made, what resulted in marked betterment. Effectiveness of ERT on Fabry cardiomyopathy primarily depends on the stage of disease at baseline, since ERT may provide limited benefits in patients with evidence of fibrosis in CMR. Switch from agalsidase alfa to agalsidase beta may be needed in some patients, who do not improve on the first‑line therapy. JRCD 2018; 3 (6): 210–213","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"47 1","pages":"209-212"},"PeriodicalIF":0.0,"publicationDate":"2018-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76874406","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 : 2018-04-13DOI: 10.20418/jrcd.vol3no6.304
B. Hussain, N. Basir
Dobutamine stress echo (DSE) allows for a relatively safe and accurate assessment of ischaemia in patients, who are unable to exercise. Side effects of DSE include nausea, vomiting, and in certain cases, excessive rise and fall in blood pressures. However, in the setting of an intrinsic catecholamine surge, dobutamine stress testing can lead to transient ventricular ballooning. Here, we report a case of a 66‑year‑old female who developed chest pain with ST‑segment elevation on electrocardiogram (ECG) along with wall motion abnormalities seen on transthoracic echocardiogram (TTE) during dobutamine stress testing. Her urgent coronary angiogram showed non‑obstructive coronaries with systolic mid‑anterolateral and antero‑septal wall ballooning on left ventriculogram with basal and apical sparing. The ECG changes resolved prior to discharge and cardiac function returned to normal on follow‑up at 3 months. The absence of obstructive coronary lesions with wall motion abnormalities on TTE and left ventriculogram, followed by the return of cardiac function to normal on follow‑up TTE highlights this case as a unique occurrence of a dobutamine‑induced, mid‑ventricular variant, atypical takotsubo cardiomyopathy. To the best of our knowledge, this is the first case of mid variant atypical takotsubo cardiomyopathy occurring secondary to dobutamine administration reported from the Indian subcontinent. The occurrence of this phenomenon points towards an extremely rare effect that may occur as a consequence of stress testing with dobutamine. JRCD 2018; 3 (6): 214–218.
{"title":"A rare case of atypical mid‑variant takotsubo cardiomyopathy during dobutamine stress echocardiography (RCD code: III‑5B)","authors":"B. Hussain, N. Basir","doi":"10.20418/jrcd.vol3no6.304","DOIUrl":"https://doi.org/10.20418/jrcd.vol3no6.304","url":null,"abstract":"Dobutamine stress echo (DSE) allows for a relatively safe and accurate assessment of ischaemia in patients, who are unable to exercise. Side effects of DSE include nausea, vomiting, and in certain cases, excessive rise and fall in blood pressures. However, in the setting of an intrinsic catecholamine surge, dobutamine stress testing can lead to transient ventricular ballooning. Here, we report a case of a 66‑year‑old female who developed chest pain with ST‑segment elevation on electrocardiogram (ECG) along with wall motion abnormalities seen on transthoracic echocardiogram (TTE) during dobutamine stress testing. Her urgent coronary angiogram showed non‑obstructive coronaries with systolic mid‑anterolateral and antero‑septal wall ballooning on left ventriculogram with basal and apical sparing. The ECG changes resolved prior to discharge and cardiac function returned to normal on follow‑up at 3 months. The absence of obstructive coronary lesions with wall motion abnormalities on TTE and left ventriculogram, followed by the return of cardiac function to normal on follow‑up TTE highlights this case as a unique occurrence of a dobutamine‑induced, mid‑ventricular variant, atypical takotsubo cardiomyopathy. To the best of our knowledge, this is the first case of mid variant atypical takotsubo cardiomyopathy occurring secondary to dobutamine administration reported from the Indian subcontinent. The occurrence of this phenomenon points towards an extremely rare effect that may occur as a consequence of stress testing with dobutamine. JRCD 2018; 3 (6): 214–218.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"40 1","pages":"213-217"},"PeriodicalIF":0.0,"publicationDate":"2018-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81598952","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 : 2018-04-13DOI: 10.20418/JRCD.VOL3NO6.313
A. Ząbek, B. Małecka, P. Matusik, Maciej Dębski, K. Boczar, J. Lelakowski
Complete atrioventricular block (AVB) is rare during pregnancy. Congenital atrioventricular block is the most common type of heart block in this group of patients. About one‑third of female patients with complete AVB remain asymptomatic until adulthood and may be first diagnosed during pregnancy. We present a case of a 31‑year‑old pregnant woman with complete AVB who was in her final stage of pregnancy. After reviewing the various advantages and disadvantages of feasible approaches with the patient, we decided to use fluoroscopy‑guided temporary backup pacemaker implantation. Estimated radiation skin dose was small and safe. The patient agreed to this treatment plan. Four days prior to scheduled cesarean delivery (39 weeks of gestation), during a one‑day stay in the hospital, the patient underwent single‑chamber temporary pacemaker implantation (using transvenous active fixation lead and external re‑sterilized pacemaker). The abdominal and pelvic regions were covered with a lead shield. The caesarean delivery was uneventful and the baby was healthy with an Apgar score of 10. JRCD 2017; 3 (6): 205–209.
{"title":"Pregnancy and congenital complete atrioventricular block : management during pregnancy and periparturient period (RCD code: VII-V)","authors":"A. Ząbek, B. Małecka, P. Matusik, Maciej Dębski, K. Boczar, J. Lelakowski","doi":"10.20418/JRCD.VOL3NO6.313","DOIUrl":"https://doi.org/10.20418/JRCD.VOL3NO6.313","url":null,"abstract":"Complete atrioventricular block (AVB) is rare during pregnancy. Congenital atrioventricular block is the most common type of heart block in this group of patients. About one‑third of female patients with complete AVB remain asymptomatic until adulthood and may be first diagnosed during pregnancy. We present a case of a 31‑year‑old pregnant woman with complete AVB who was in her final stage of pregnancy. After reviewing the various advantages and disadvantages of feasible approaches with the patient, we decided to use fluoroscopy‑guided temporary backup pacemaker implantation. Estimated radiation skin dose was small and safe. The patient agreed to this treatment plan. Four days prior to scheduled cesarean delivery (39 weeks of gestation), during a one‑day stay in the hospital, the patient underwent single‑chamber temporary pacemaker implantation (using transvenous active fixation lead and external re‑sterilized pacemaker). The abdominal and pelvic regions were covered with a lead shield. The caesarean delivery was uneventful and the baby was healthy with an Apgar score of 10. JRCD 2017; 3 (6): 205–209.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"130 1-2","pages":"204-208"},"PeriodicalIF":0.0,"publicationDate":"2018-04-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72477944","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 : 2018-04-13DOI: 10.20418/JRCD.VOL3NO6.316
A. Nowak, Karolina Bula, Karol Głowacki, Wojciech Gawin, Marcin Kalita, Konstantinos Nechoritis, M. Grabka, K. Mizia-Stec
Background: Efficacy of pulmonary arterial hypertension (PAH)-specific therapy may differ among the patients depending on the PAH aetiology. Aim: To compare the real‑life efficacy of PAH‑specific therapy between non‑congenital heart disease (non‑CHD) and CHD groups of PAH patients and to determine whether an early clinical response has an impact on prognosis. Methods: Clinical data from 41 PAH patients, 21 non‑CHD and 20 CHD patients, were included in the study. The WHO functional class (WHO‑FC), 6‑minute walk distance (6MWD) and NT‑proBNP serum level were compared at baseline and after 1 and 7 months of PAH‑specific treatment. Only patients with unmodified PAH‑specific therapy during 7‑month follow‑up were enrolled in the study. Results: Baseline characteristics revealed higher WHO‑FC and increased [loge]NT‑proBNP levels (7.74 ±1.05 vs 6.51 ±1.48; p = 0.008) in non‑CHD vs. CHD patients; baseline 6MWD was similar in both groups (283.3 ±148.5 m vs 339.2 ±114.7 m). Clinical improvement by at least one WHO‑FC after 1‑month treatment was observed more frequently in non‑CHD (55%) when compared with CHD patients (25%, p = 0.04) and was comparable (50% vs 50%) after 7‑month observation. Non‑CHD patients, who did not improve within 1 month of treatment were unlikely to achieve improvement after 7 months. The 6MWD increased during the first month of treatment in non‑CHD (p=0.009) and in CHD patients (p=0.006) when compared to baseline values and remained at this level after 7 months of treatment. [Loge]NT‑proBNP levels markedly declined only in non‑CHD patients, who had an improvement in WHO‑FC (8.0 ±1.0 vs 7.4±1.1, p=0.04) in the first month. In CHD patients, the decrease in [loge]NT‑proBNP level was seen (6.5 ± 1.5 vs 6.1 ±1.5, p=0.04) only within a 1‑month observation. Conclusion: Efficacy of 1‑month PAH‑specific therapy is aetiology‑dependent and determines clinical outcome in patients with PAH. JRCD 2018; 3 (6): 199–204
背景:肺动脉高压(PAH)特异性治疗的疗效可能因PAH病因而异。目的:比较PAH特异性治疗在非先天性心脏病(非冠心病)和冠心病组PAH患者的实际疗效，并确定早期临床反应是否对预后有影响。方法:41例PAH患者、21例非冠心病患者和20例冠心病患者的临床资料纳入研究。在基线和PAH特异性治疗1个月和7个月后比较WHO功能分级(WHO - FC)、6分钟步行距离(6MWD)和NT - proBNP血清水平。只有在7个月的随访期间接受未经改良的PAH特异性治疗的患者被纳入研究。结果:基线特征显示更高的WHO - FC和更高的[loge]NT - proBNP水平(7.74±1.05 vs 6.51±1.48;p = 0.008);两组的基线6MWD相似(283.3±148.5 m vs 339.2±114.7 m)。治疗1个月后，至少有一项WHO - FC的临床改善在非冠心病患者中更为常见(55%，p = 0.04)，而在7个月后的观察中也相当(50% vs 50%)。非冠心病患者在治疗1个月内没有改善，不太可能在7个月后达到改善。与基线值相比，非冠心病患者(p=0.009)和冠心病患者(p=0.006)在治疗的第一个月内6MWD增加，并在治疗7个月后保持在该水平。[Loge]NT - proBNP水平仅在非冠心病患者中显著下降，在第一个月who - FC有改善(8.0±1.0 vs 7.4±1.1,p=0.04)。在冠心病患者中，仅在1个月内观察到NT - proBNP水平的下降(6.5±1.5 vs 6.1±1.5,p=0.04)。结论:1个月PAH特异性治疗的疗效与病因有关，并决定PAH患者的临床预后。JRCD 2018;3 (6): 199-204
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