The Prevalence of Paroxysmal Nocturnal Hemoglobinuria Clone in Adult Patients with Idiopathic Pulmonary Hypertension

Aim: Paroxysmal nocturnal hemoglobinuria (PNH) a is a clonal disorder that may lead to several conditions such as thromboses, Budd-Chiari syndrome, renal failure, impotence, and pulmonary hypertension (pHT). Since the presentation of PNH may be occult, monitoring for clonal evolution is recommended in certain situations including aplastic anemia, Myelodysplastic syndrome, and unexplained cytopenia, and thrombosis. The prevalence of PNH clone in patients with idiopathic pHT is unknown. We designed a study to determine the prevalence of PNH clone in patients with idiopathic pHT, since it may be the first isolated presentation of the disease. Methods: A total of 45 patients with pHT were screened for PNH clone by proaerolysin conjugated with fluorescein. Results: Only two out of 45 patients had elevated lactate dehydrogenase (LDH) levels at presentation. PNH clone was detected in none of the patients. Conclusion: Screening for PNH clone in patients with pHT, who have normal LDH levels is unnecessary

İnfektif endokarditin epidemiyolojik, klinik ve prognostik sonuçları: 90 atağın retrospektif kohortu

To evaluate the epidemiological, clinical, microbiological, and echocardiographic features, as well as the prognosis and long- term outcome of patients with infective endocarditis. Methods: The clinical records and follow-up data of 90 endocarditis episodes in 86 patients diagnosed with definite and possible infec- tive endocarditis according to the modified Duke criteria in a tertiary university hospital, between 1998 and 2016, were reviewed. Results: Fifty-six patients were male (65.1%), and the mean age was 49.9 ± 14.3. Native valve endocarditis constituted 62.2% of the cases, while the remaining patients had prosthetic valve endocarditis. The aortic (34.4%) and mitral (24.4%) valves were infected more frequently. Streptococci (27.7%) and staphylococci (24.4%) were the most frequently isolated microorganisms. Embolic complications (35.5%) were the leading cause of morbidity, followed by valve insufficiency (28.8%) and heart failure (21.1%). Valve replacement surgery was performed in 28 patients (31%). The in-hospital mortality rate was 15.1% (n = 13). Chronic renal failure (P = .042) and degenerative valves (P = .036) were significantly associated with mortality. Among 43 of the 73 cases available for telephonic survey, 36 (83.7%) patients were alive and without disease, with a median follow-up of 52.9 (4-163) months. Twenty-five (69.4%) of these patients were younger than 55 years, and 24 (66.6%) had native valve endocarditis. Conclusion: Underlying cardiac conditions and chronic renal failure increase mortality in infective endocarditis, regardless of the patho- gen. Long-term survival seems promising in cases with native valve endocarditis and in younger patients with low rates of comorbidities.İnfektif endokardit tanısıyla izlenen hastaların epidemiyolojik, klinik, mikrobiyolojik, ekokardiyografik özellikleri, prognozu ve uzun dönem sonuçlarını değerlendirmek. Yöntemler: 1998 ve 2016 yılları arasında bir üniversite hastanesinde modifiye Duke kriterlerine göre kesin ve olası infektif endokardit tanısı konan 86 hastanın 90 endokardit atağının klinik kayıtları ve takip verileri retrospektif olarak incelendi. Bulgular: Çalışmamızda hastaların 56’sı (%65,1) erkek ve ortalama yaş 49,9 ± 14,3 idi. Doğal kapak endokarditi olguların %62,2’sini oluştururken, diğerlerinde protez kapak mevcuttu. En sık aort (%34,4) ve mitral (% 24,4) kapak tutulumu saptandı. Etken olarak strep- tokoklar (%27,7) ve stafilokoklar (%24,4) en sık izole edilen mikroorganizmalardı. Tüm komplikasyonlar içinde embolik komplikasyonlar (%35,5) ilk sırada yer almış, bunu kapak yetmezlikleri (%28,8) ve kalp yetmezliği (%21,1) izlemiştir. Hastaların 28’ine (%31) kapak replasman operasyonu yapılmış ve tüm hastalar içinde 13 hasta (%15,1) hastane takibinde kaybedilmiştir. Mortalite kronik böbrek yetmezliği (P = ,042) ve dejeneratif kapak hastalarında (P = ,036) istatistiksel olarak daha anlamlı bulundu. Taburculuk sonrası prognoz ve uzun dönem sonuçlar değerlendirildiğinde sağ kalan 73 hastanın 43’üne ulaşılabilmiş ve bu hastalardan 36’sının (%83,7) ortalama 52,9 ay (4-163) hayatta ve genel durumlarının iyi olduğu, 25 hastanın (%69,4) <55 yaş, 24’ünün (%66,6) doğal kapak endokarditi tanısıyla izlenmiş olduğu görülmüştür. Sonuç: İnfektif endokardit hastalarında altta yatan kalp hastalıkları ve kronik böbrek yetmezliği patojenden bağımsız olarak mortalit- eyi arttırmaktadır. Doğal kapak endokarditi ve komorbiditeleri daha az olan genç yaş hastalarda ise uzun dönem sağkalım umut verici görünmektedir

Long-term prognosis of patients with heart failure: Follow-up results of journey HF-TR study population

Background: Despite advances in therapeutic management of patients with heart failure, there is still an increasing morbidity and mortality all over the world. In this study, we aimed to present the 3-year follow-up outcomes of patients included in the Journey HF-TR study in 2016 that has evaluated the clinical characteristics and management of patients with acute heart failure admitted to the hospital and present a national registry data. Methods: The study was designed retrospectively between November 2016 and December 2019. Patient data included in the previously published Journey HF-TR study were used. Among 1606 patients, 1484 patients were included due to dropout of 122 patients due to inhospital death and due to exclusion of 173 due to incomplete data. The study included 1311 patients. Age, gender, concomitant chronic conditions, precipitating factors, New York Heart Association, and left ventricular ejection fraction factors were adjusted in the Cox regression analysis. Results: During the 3-year follow-up period, the ratio of hospitalization and mortality was 70.5% and 52.1%, respectively. Common causes of mortality were acute decompensation of heart failure and acute coronary syndrome. Angiotensin receptor blockers, beta-blockers, statin, and sacubitril/valsartan were found to reduce mortality. Hospitalization due to acute decompensated heart failure, acute coronary syndrome, lung diseases, oncological diseases, and cerebrovascular diseases was associated with the increased risk of mortality. Implantation of cardiac devices also reduced the mortality. Conclusions: Despite advances in therapeutic management of patients with heart failure, our study demonstrated that the long-term mortality still is high. Much more efforts are needed to improve the inhospital and long-term survival of patients with chronic heart failure

Serum Vitamin D Levels in Patients with Pulmonary Arterial Hypertension

Amaç: Pulmoner arteriyal hipertansiyon (PAH) hastalarında D vitamini düzeylerinin sağlıklı gönüllülerden oluşan kontrol grubuyla kıyaslanması ve hastalık şiddetiyle D vitamini düzeyi arasındaki ilişkinin araştırılması. Yöntemler: Çalışmamız tek merkezli, kesitsel bir çalışma olup, pulmoner hipertansiyon (PH) polikliniğimizde PAH tanısı ile takip edilen 36 hasta ve yaş-cinsiyet eşleştirilmiş 24 sağlıklı gönüllüden oluşmaktadır. PAH tanısı, güncel Avrupa Kardiyoloji Cemiyeti ve Avrupa Solunum Derneği 2015 PH kılavuzu tanı algoritması önerileri doğrultusunda konuldu. Kontrol grubu olarak, bilinen bir kardiyak veya sistemik-enflamatuvar hastalık öyküsü olmayan, herhangi bir ilaç kullanmayan, tüm sistem fizik muayenesi normal, elektrokardiyografik (EKG) ve ekokardiyografik (Eko) incelemede patoloji saptanmayan sağlıklı gönüllüler seçildi. Tüm hastalarda ve sağlıklı gönüllülerde sabah 8 saat açlık kan örnekleri alınarak, plazma 25-OH vitamin D düzeyleri ölçüldü (Elecsys® Vitamin D Testi). Bulgular: Çalışma popülasyonumuz 36 PAH hastası ve yaş-cinsiyet eşleştirilmiş 24 sağlıklı gönüllüden oluşmaktaydı. Hastaların 24 tanesinde (%67) doğumsal kalp hastalığıyla ilişkili PAH (PAH-DKH), 12 tanesinde (%33) idiyopatik PAH (IPAH) mevcuttu. Tüm çalışma grubunun yaş ortalaması 41.4 ± 14.0 iken PAH hastaları ve kontrol grubunda yaş ortalaması benzerdi (median 42 & 40 yaş). Her iki grupta da kadın cinsiyet baskındı. Sağlıklı gönüllüler ve PAH hastaları arasında vücut kitle indeksi, kreatinin, ürik asit, hematokrit düzeyi açısından bir fark yoktu. Vitamin D düzeyleri ise PAH hastalarında (7.7 ± 6.2 U/L) sağlıklı gönüllülere göre (21.9 ± 14.1 U/L) belirgin olarak düşük saptandı (p<0.001). Vitamin D düzeyi PAH hastalarında kontrol grubuna göre düşük saptanmakla birlikte, D vitamini düzeyi ile ne fonksiyonel kapasite (DSÖ FK III & FK II), ne de altta yatan etiyolojik neden (PAH-DKH & IPAH) arasında ilişki saptanamadı. Sonuçlar: Çalışmamızda serum D vitamini düzeyleri PAH hastalarında sağlıklı gönüllülere göre belirgin olarak daha düşüktü. Bununla birlikte PAH hastalarını altta yatan etiyolojik nedene göre incelediğimizde (IPAH & PAH-DKH), D vitamini düzeyleri arasında farklılık yoktu.Objective: To compare the vitamin D levels in patients with pulmonary arterial hypertension (PAH) with the control group of healthy volunteers and to investigate the relationship between disease severity and vitamin D level. Methods: Our study is a single center, cross-sectional study consisting of 36 patients with pulmonary artery hypertension (PAH) followed-up by pulmonary hypertension (PH) outpatient clinic and 24 age-sex matched healthy volunteers. The diagnosis of PAH was confirmed according to the diagnostic algorithm of 2015 European Society of Cardiology and European Respiratory Society PH Guidelines. As the control group, healthy volunteers who do not have a known history of cardiac or systemic-inflammatory disease, do not use any medication, has normal physical, electrocardiographic (ECG) and echocardiographic examination were selected. In all groups, 8-h fasting blood samples were obtained in the morning and plasma 25-OH vitamin D levels were measured by Elecsys® Vitamin D Test. Results: Our study population consisted of 36 PAH patients and 24 age-sex matched healthy volunteers. Twenty-four patients (67%) had PAH associated with congenital heart disease related (PAH-CHD) and other 12 patients (33%) had idiopathic PAH (IPAH). While the average age of the whole study group was 41.4 ± 14.0 years, the mean age was similar in the PAH patients and control group (median 42 & 40 years). Female sex was dominant in both groups. There was no difference between healthy volunteers and PAH patients in terms of body mass index, creatinine, uric acid and hematocrit levels. Vitamin D levels were significantly lower in PAH patients (7.7 ± 6.2 U / L) than in healthy volunteers (21.9 ± 14.1 U / L) (p <0.001). Although vitamin D level was found to be lower in PAH patients compared to the control group, no relation was found between vitamin D level and neither functional capacity (WHO FC III & FC II) nor the underlying etiological cause (PAH-CHD & IPAH). Conclusions: In our study, serum vitamin D levels were significantly lower in PAH patients than in healthy volunteers. However, when we examined PAH patients according to the underlying etiological cause (IPAH & PAH-CHD), there was no difference between vitamin D levels

Epidemiology and clinical characteristics of hospitalized elderly patients for heart failure with reduced, mid-range and preserved ejection fraction

Introduction:: Elderly patients hospitalized with heart failure (HF) have high mortality rates and require& specific evidence based theraphy, however there are few studies which have focused on patients older than 80 years hospitalized with HF. The aim of the present study is to evaluate the overall clinical characteristics, management, and in-hospital outcomes of elderly patients hospitalized with HF.Methods:: Journey-HF study was conducted in 37 different centers in Turkey and recruited 1606 patients who were hospitalized with HF between September 2015 and September 2016. In this study, clinical profile of patients >= 80 years old and 65-79 years old hospitalized with HF were described and compared based on EF-related classification: HFrEF (HF with reduced ejection fraction), HFmrEF (HF with mid-range ejection fraction) and HFpEF (HF with preserved ejection fraction).Results:: A total of 1034 elder patients (71.6% 65-79 years old and 28.4% >= 80 years old) were recruited. Of the 65-79 years old patients 67.4% had HFrEF, 16.2% had HFmrEF and 16.3% had HFpEF. Among patients >= 80 years old 61.6% had HFrEF, 15.6% had HmrEF and 22.8% had HFpEF.When compared with patients with HFrEF and HFmrEF, patients >80 years old with HFpEF were more likely to be older, have atrial fibrilation (AF), and less likely to have diabetes mellitus (DM), coronary artery disease (CAD) or to be recieving an angiotensin-converting enzyme inhibitor (ACEi) or beta blocker theraphy. When compared to patients 65-79 years old with HFpEF, patients >= 80 years with HFpEF had a higher rate of AF and less likely DM. Acute coronary syndrome was the most common precipitant factor for hospitalization in both age groups with HFrEF group. Arrhythmia was a major precipitant factor for hospitalization of patients >= 80 years old with HFpEF. Non-compliance with theraphy was a major problem of patients >= 80 years old with HFrEF.Conclusion:: Elderly patients with HFrEF, HFmrEF and HFpEF each had characterized unique patient profiles and the guideline recommended medications were less likely to be used in these patient populations. In hospital mortality rate is worrisome and reflects a need for more specific tretment strategy

Mobitz type 2 AV block dissolved with contrast injection

There are many cases in the literature concerning the occurence of atrioventricular block in acute myocardial infarction. The prevelance and management of AV block in the setting of chronic myocardial ischemia remains unclear. Our case presented with stable angina pectoris. Treadmill test revealed Mobitz Type 2 AV block which disappeared with contrast injection and re-occured after injection during PCI

A new risk score to predict in-hospital mortality in elderly patients with acute heart failure: On behalf of the journey HF-TR study investigators

We aimed to predict in-hospital mortality of elderly patients with heart failure (HF) by using a risk score model which could be easily applied in routine clinical practice without using an electronic calculator. The study population (n = 1034) recruited from the Journey HF-TR (Patient Journey in Hospital with Heart Failure in Turkish Population) study was divided into a derivation and a validation cohort. The parameters related to in-hospital mortality were first analyzed by univariate analysis, then the variables found to be significant in that analysis were entered into a stepwise multivariate logistic regression (LR) analysis. Patients were classified as low, intermediate, and high risk. A risk score obtained by taking into account the regression coefficients of the significant variables as a result of the LR analysis was tested in the validation cohort using receiver operating characteristic curve analysis. In total, 6 independent variables (age, blood urea nitrogen, previous history of hemodialysis/hemofiltration, inotropic agent use, and length of intensive care stay) associated with in-hospital mortality were included in the analysis. The risk score had a good discrimination in both the derivation and validation cohorts. A new validated risk score to determine the risk of in-hospital mortality of elderly hospitalized patients with HF was developed by including 6 independent predictors

Günlük uygulamada yeni oral antikoagülanların karşılaştırmalı takip sonuçları

Okmeydanı Training and Research Hospital, Department of CardiologyIstanbul University Institute of Cardiology, Department of CardiologyObjectives: This study aims to examine the patients using dabigatran or rivoraxaban to compare their effectiveness and adverse events. Patients and methods: Our study includes a total of 174 patients (82 male, 92 female; mean age 70.7±8.8 years), using dabigatran (n=113) and rivoraksaban (n=61), who were admitted to our outpatient clinic consecutively between August 2013 and April 2014. Physical examination was performed, patient history, electrocardiogram and biochemical results were recorded. Bleeding and ischemic stroke risk scores (HAS-BLED and CHA2DS2-VASc scores) were calculated. Patients were followed for adverse events. Results: Patients were followed for an average of 12.9±2.4 months. The CHA2DS2-VASc scores were non-significant between the two groups (3.60±1.30 for dabigatran vs 3.90±1.22 for rivoraxaban, p>0.05). The patients using rivoraxaban had significantly higher HAS-BLED scores than the patients using dabigatran (2.01±0.95 vs 1.60±0.85, p=0.004). During the follow-up period, 12 of the patients had minor bleeding, two had gastrointestinal bleeding, two had intracranial hemorrhage and seven had ischemic stroke. Adverse events were non-significant between the rivoraxaban and dabigatran using patients (p>0.05 for each). Conclusion: Bleeding and ischemic stroke are rare adverse effects among all the patients. No significant difference was observed between both of the groups in terms of preventing ischemic stroke. Rivoraxaban can be a better option in the patients with high HAS-BLED score to avoid bleeding.Amaç: Bu çalışmada dabigatran veya rivoraksaban kullanan hastalar, bu ilaçların etkinlikleri ve yan etkilerini karşılaştırmak için araştırıldı. Hastalar ve yöntemler: Ağustos 2013 - Nisan 2014 tarihleri arasında ardışık olarak polikliniğimize başvuran ve halihazırda dabigatran (n=113) ve rivoraksaban (n=61) kullanan toplam 174 hasta (82 erkek, 92 kadın; ort. yaş 70.7±8.8 yıl) çalışmamıza dahil edildi. Hastaların fizik muayeneleri yapıldı, öyküleri, biyokimyasal değerleri ve elektrokardiyogramları kaydedildi. Kanama ve iskemik inme risk değerleri (HAS-BLED ve CHA2DS2-VASc) hesaplandı. Hastalar yan etkiler açısından takip edildi. Bulgular: Hastalar ortalama 12.9±2.4 ay takip edildi. İki grup arasında CHA2DS2-VASc skoru anlamlı değildi (dabigatran için 3.60±1.30’a karşın rivoraksaban için 3.90±1.22, p>0.05). Rivoraksaban kullanan hastaların HAS-BLED skorları dabigatran kullananlarınkinden anlamlı olarak yüksekti (2.01±0.95’e karşın 1.60±0.85, p=0.004). İzlem süresince hastaların 12’sinde minör kanama, ikisinde gastrointestinal kanama, ikisinde intrakraniyal kanama ve yedisinde iskemik inme meydana geldi. Dabigatran ve rivoraksaban kullanan hastalar arasında yan etkiler açısından anlamlı fark gözlenmedi (her biri için p>0.05). Sonuç: Kanama ve iskemik inme tüm hastalar arasında nadir yan etkilerdir. İskemik inmeden koruma açısından her iki grup arasında anlamlı farklılık gözlenmedi. Kanamayı önlemek için HAS-BLED skoru yüksek olan hastalarda rivoraksaban daha iyi bir seçenek olabili

Demographics of patients with heart failure who were over 80 years old and were admitted to the cardiology clinics in Turkey

Objective: Heart failure (HF) has a high prevalence and mortality rate in elderly patients; however, there are few studies that have focused on patients older than 80 years. the aim of this study is to describe and compare the age-specific demographics and clinical features of Turkish elderly patients with HF who were admitted to cardiology clinics. Methods: the Epidemiology of Cardiovascular Disease in Elderly Turkish population (ELDER-TURK) study was conducted in 73 centers in Turkey, and it recruited a total of 5694 patients aged 65 years or older. in this study, the clinical profile of the patients who were aged 80 years or older and those between 65 and 79 years with HF were described and compared based on the ejection fraction (EF)-related classification: HFrEF and HFpEF (is considered as EF: ?50%). Results: A total of 1098 patients (male, 47.5%; mean age, 83.5±3.1 years) aged ?80 years and 4596 patients (male, 50.2 %; mean age, 71.1±4.31 years) aged 65-79 years were enrolled in this study. the prevalence of HF was 39.8% for patients who were ?80 years and 27.1% for patients 65–79 years old. For patients aged ?80 years with HF, the prevalence rate was 67% for hypertension (HT), 25.6% for diabetes mellitus (DM), 54.3% for coronary artery disease (CAD), and 42.3% for atrial fibrilation. Female proportion was lower in the HFrEF group (p=0.019). the prevalence of HT and DM was higher in the HFpEF group (p<0.01), whereas CAD had a higher prevalence in the HFrEF group (p=0.02). Among patients aged 65–79 years, 43.9% (548) had HFpEF, and 56.1% (700) had HFrEF. in this group of patients aged 65-79 years with HFrEF, the prevalence of DM was significantly higher than in patients aged ?80 years with HFrEF (p<0.01). Conclusion: HF is common in elderly Turkish population, and its frequency increases significantly with age. Females, diabetics, and hypertensives are more likely to have HFpEF, whereas CAD patients are more likely to have HFrEF. (Anatol J Cardiol 2019; 21: 196-205