Amiloidoz; organ ve yumuşak dokuların hücre dışı boşluğunda amiloid fibril birikiminin neden olduğu bir hastalıktır. Öncü proteinin tipine göre amiloidoz sınıflandırması yapılır. Kalp, böbrek, ...karaciğer, gastrointestinal sistem ve/veya otonom sinir sistemi gibi çeşitli organlarda amiloid birikimi izlenebilmekle birlikte, prognozu en kötü olan organ tutulumu türü kardiyak amiloidozdur. Birçok amiloidoz türleri arasında hemen hemen tüm klinik kardiyak amiloidoz vakalarına, transtiretin amiloidoz (ATTR) veya hafif zincir amiloidoz (AL veya primer sistemik) neden olur. Kardiyak ATTR; kalıtsal (ATTRm) veya doğal tip (ATTRwt) olabilir. Kardiyak tutulum sıklığı ve kardiyomiyopatinin prognozu amiloidoz tipleri arasında değişkenlik gösterir ve klinik belirtiler, organ tutulum paternine bağlı olarak değişir. Değişken klinik fenotip ve genellikle özgül olmayan klinik özellikler, bu hastalıkta tanıda gecikmelere neden olabilir ve tanıda multidisipliner (hematoloji, patoloji, radyoloji, nükleer tıp, nöroloji, nefroloji, kardiyoloji, romatoloji, gastroenteroloji) yaklaşım gerekir. Gelişen tanı yöntemleri klinisyene kardiyak amiloidozda erken tanı olanağını vermektedir. Kardiyak amiloidozdan şüphelenilen hastalarda; görüntüleme yöntemleri ve gerekirse doku biyopsisi ile amiloid birikimi gösterilmelidir. Amiloidoza sebep olan öncü protein saptandıktan sonra, altta yatan hastalığın tedavisi ve semptomatik hastalarda kalp yetmezliğine yönelik tedavi uygulanmaktadır. Erken tanı ve tedavi yaklaşımları ile prognozun düzeltilebildiği gösterilmiştir. Bu derlemede, kardiyak amiloidoz hastalığı hakkında farkındalığın artırılması amaçlanmış olup, günlük pratiğimizde kullanabileceğimiz güncel tanı yöntemleri ve tedavi yaklaşımları ele alınacaktır.
Amyloidosis is a disease caused by the accumulation of amyloid fibril in the extracellular space of organs and soft tissues. Amyloidosis classification is made according to the type of precursor protein. Although amyloid accumulation can be observed in various organs such as heart, kidney, liver, gastrointestinal and/or autonomic nervous system; the worst prognosis type of organ involvement is cardiac amyloidosis. Among many types of amyloidosis, almost all clinical cases of cardiac amyloidosis are caused by transtiretin amyloidosis (ATTR) or light chain amyloidosis (AL or primary systemic). Cardiac ATTR can be hereditary (ATTRm) or wild type (ATTRwt). The frequency of cardiac involvement and prognosis of cardiomyopathy varies between the types of amyloidosis and clinical symptoms vary depending on the organ involvement pattern. Variable clinical phenotype and often non-specific clinical features can cause delays in diagnosis and a multidisciplinary (hematology, pathology, radiology, nuclear medicine, neurology, nephrology, cardiology, rheumatology, gastroenterology) approach is required in diagnosis. The developing diagnostic methods give the clinician early diagnosis of cardiac amyloidosis. Amyloid accumulation should be demonstrated by imaging methods and tissue biopsy is necessary in patients with suspected cardiac amyloidosis. Once the precursor protein that causes amyloidosis has been identified, treatment of the underlying disease and heart failure is performed in symptomatic patients. It has been shown that prognosis can be corrected with early diagnosis and treatment approaches. In this review, it is aimed to raise awareness about cardiac amyloidosis disease, current diagnostic methods and treatment approaches that we can use in our daily practice will be discussed.
To investigate mean platelet volume (MPV) levels in patients with coronary slow flow (CSF).
465 stable angina pectoris cases with angiographically normal coronary arteries were recruited coronary ...slow flow group (n=76), control group (n=389) in the observational retrospective cohort study. Clinical, biochemical and demographic variables including MPV were noted and coronary blood flow was assessed with TIMI frame count (TFC).
Gender, smoking, height, serum creatinine, uric acid levels, hemoglobin, waist/hip ratio, systolic blood pressure but not MPV were significantly different among groups. Independent predictors of CSF were height (p=.029) and serum uric acid level (p=.045). Gender, height, weight, hip circumference, systolic blood pressure, fasting blood glucose, serum urea, creatinine, uric acid levels, hemoglobin and platelet count were associated with mean TFC whereas independent predictors of mean TIMI frame count were height (p=.010) and serum uric acid level (p=.041).
Height and serum uric acid level but not MPV were independent predictors of both CSF and mean TFC.
All prosthetic valves are at least mildly stenotic and have relatively high transvalvular pressure gradients that can be observed despite normal prosthesis function. Such gradients may be due to a ...mismatch between prosthesis effective orifice area (EOA) and patient's body size. Valve prosthesis-patient mismatch (VP-PM) may occur due to mismatches of both parameters, the expected hemodynamic performance of the prosthesis and the cardiac output requirements of the patient, which are largely related to the body size at rest. In other words, a prosthesis may be adequate for patients with a small body surface area (BSA) but might become obstructive for patients with a large BSA. The only parameter that has proven to be consistently and realistically useful to predict and describe VP-PM is the effective orifice area index (EOAI). The projected EOAI was identified as the best parameter to predict the VP-PM occurrence after surgery. VP-PM has been known to be independently and significantly associated with clinical outcomes. Severe VP-PM has a significant impact on early and late mortality, whereas moderate VP-PM may have a significant effect on mortality only in vulnerable subsets of patients, and particularly in those with depressed LV systolic function. The surgeon's anticipation of VP-PM prior to surgery, and successfully implented preventive strategies can reduce the incidence of VP-PM. Preventive strategies to avoid VP-PM should be individualized according to the anticipated severity of VP-PM and of the patient's baseline risk profile.
The serum paraoxonase and arylesterase activities are related to coronary artery diseases. However, there are a few data about the association of paraoxonase and arylesterase activities with in-stent ...restenosis (ISR). The aim of this study was to evaluate the relationship between paraoxonase and arylesterase activities and ISR in patients with bare metal stent (BMS).
Thirty-one patients with normal coronary artery (group 1) and 60 with BMS were enrolled in this observational study. According to the ISR, the patients were classified as group 2, without the ISR (n=29) and group 3, with the ISR (n=31). Serum paraoxonase and arylesterase activities were measured spectrophotometrically.
The paraoxonase and arylesterase activities were lower in patients with BMS than in the individuals with normal coronary artery (P < 0.001 and P = 0.001, respectively). The enzyme activities were also higher in patients without ISR than with ISR (both of P < 0.001). In bivariate correlation analyses in patients with BMS, ISR shows significant positive correlations with the presence of hypertension and hyperlipidemia, type C lesion, and stent length, but shows negative correlations with type A lesion stent diameter, high-density lipoprotein cholesterol, and paraoxonase and arylesterase activities. In regression analysis, ISR is independently associated with paraoxonase and arylesterase activities (β = -0.216, P = 0.038 and β = -0.452, P < 0.001, respectively), type A lesion (β = -0.251, P = 0.013), and stent diameter (β = -0.192, P = 0.024) in patients with BMS.
Our study shows that decreased paraoxonase and arylesterase activities play a significant role in ethiopathogenesis ISR in patients with BMS.
Endothelial dysfunction is considered the first stage in the development of atherosclerosis and assessed by flow-mediated dilatation (FMD) and aortic flow velocity propagation (AVP). Ankle-brachial ...index (ABI) is used to assess peripheral arterial disease and is associated with FMD but the relationship between ABI and AVP is unknown. In this study, we aimed to search the association between AVP, and ABI in patients with newly diagnosed hypertension.
Sixty-eight patients with newly diagnosed hypertension and 34 healthy subjects were enrolled in the cross-sectional observational study. The maximum ankle arterial pressures were divided by the maximum of the brachial arterial pressures to calculate the ABI. AVP was calculated from dividing the distance between points corresponding to the beginning and end of the propagation slope, to the duration between corresponding time points proximally descending aorta. Statistical analysis was performed using Student t-test, Chi-square test, Pearson correlation and linear regression analyses.
Age and gender of both groups were similar. Compared to control group E deceleration time of early diastolic flow velocity (184.0±32.2 vs. 217.1±38.6, p<0.001), isovolumic relaxation time (95.5±19.4 vs. 105.7±18.1, p<0.001) and body mass index (25.6±5.1 vs. 27.5±3.8, p=0.044) values were significantly higher, while ABI (1.08±0.07 vs. 1.14±0.07, p=0.001) and AVP (54.97±9.3 vs. 69.17±10.8 cm/sec, p=0.001) values were significantly lower in hypertensive patients. There was a significant correlation between AVP and ABI (r=0.279, p=0.005). Both ABI and AVP were independent predictors of hypertension (OR - 0.353, 95%CI 0.151-0.826, p=0.02 and OR - 0.133, 95%CI 0.0502-0.35, p=0.001, respectively).
Our data indicate that in patients with isolated hypertension AVP and ABI decrease. We also conclude that AVP is directly associated with ABI.
Abstract
Objectives: Thymosin beta 4 (Tβ4) plays an essential role in cardiac vessel development and is currently being developed as a therapeutic agent for the treatment of coronary artery disease ...(CAD) in some experimental studies. Thus, we aimed to investigate the association of serum Tβ4 levels and collateral formation in patients presenting with severely stenotic CAD. Methods: Thirteen patients with poor collateral development and 16 age- and sex-matched patients with good collateral development who had ≥ 95% stenosis in at least one major coronary artery on coronary angiogram (CAG) were enrolled in the study. The Gensini score was calculated for each patient by using CAG results. Collateral development was classified according to the Cohen-Rentrop method. Serum Tβ4 levels were measured with enzyme-linked immune sorbent assay. Results: There were no statistically significant differences between the two groups in regard to clinical and laboratory characteristics of the patients except for Tβ4 levels. The Tβ4 levels in the well-collateralized study group were found to be significantly higher than those of the poorly collateralized study group and serum Tβ4 levels were positively correlated with the collateral development. Conclusions: Our findings suggest that serum Tβ4 levels are significantly associated with the collateral development in severe CAD.
We have described our experience with transcatheter occlusion of congenital coronary arterial fistulas in adults.
Six symptomatic patients, mean aged 41+/-12 years (four men, two women) underwent ...transcatheter occlusion of fistulas. All had chest pain or dyspnea on exertion. Four of the patients had single fistula. Two of the patients had multiple fistulas. The fistulas originated from the left anterior descending coronary artery in four patients, and from the circumflex artery in two. They all drained into the pulmonary trunk. Graft stenting was used in two patients who had multiple fistulas, and coils in four who had a single fistula. The coils were implanted through a microcatheter, which was passed through a JL 4 8F guiding catheter.
Coils were used to completely occlude fistulas. Two 3.0x20 mm coronary graft stents were deployed in the coronary arteries to occlude the fistulas. The procedures were uncomplicated. At follow up, all patients underwent coronary angiography, and one of the patients with coil embolization and one of the patients with graft stenting had small residual flow. Patient's chest pain or dyspneas have resolved after the procedure.
Transcatheter closure in adults of congenital coronary fistulas with graft stents and coils are safe and effective, and can be regarded as an acceptable alternative to surgery.
Hemangiomas are rare benign tumors of the heart. Clinical presentation is highly variable according to the location, size, and extension of the tumor. Hemangiomas have been described in all cardiac ...chambers, but most occur on the right side of the heart and in the left atrium. Although diagnosis is typically made by echocardiography, the definite diagnosis can be made with certainty only from a very careful histopathologic examination. The authors report a case of atypically located hemangiomas originating from the left atrial appendage and right atrium in a 71-year-old woman who presented with ischemic stroke. Transthoracic and transesophageal echocardiography demonstrated an elongated left atrial mass originating in the atrial appendage and extending well into the left atrium to the mitral orifice, as well as a right atrial mass and intense biatrial spontaneous echo contrast. It was unclear whether the masses represented thrombus or an unusually located atrial tumor. Immunohistologic examinations revealed a biatrial cavernous hemangioma with no signs of malignancy.
Although the pathophysiology of paroxysmal atrial fibrillation (PAF) is not fully known, oxidative stress (OS) and atrial remodeling seem to be important triggers. Autophagy and apoptosis which are ...the types of cell death are fundamental processes in the human body. Although they investigated in many diseases, no study evaluated these parameters in PAF patients. We aimed to investigate autophagy and apoptosis which may be associated with atrial remodeling, and to show whether these factors are associated with OS in PAF patients. In this study, 44 PAF patients admitted to our clinic and 44 healthy volunteers were included. Serum total oxidative stress (TOS), total antioxidant status (TAS), oxidative stress index (OSI), and ATG5 for autophagy and serum M30 for apoptosis were studied. Serum TOS, OSI, ATG5, M30 and left atrium (LA) diameter were higher, while TAS was lower in PAF group than the control group (p < 0.001, for all). ATG5 was positively correlated with TOS, OSI and LA, whereas negatively correlated with TAS. Also, M30 was positively correlated with TOS and OSI, whereas negatively correlated with TAS. Logistic regression analysis showed that TOS (P = 0.002), ATG5 (p = 0.013) and M30 (p = 0.006) were independent predictors of the PAF. It also found that ATG5 was the only independent predictor of LA enlargement in linear regression analysis. Our study showed that ATG5 and M30 were increased, and they were correlated with OS in patients with PAF. Therefore, we suggest that autophagy and apoptosis may play an important role in the PAF process.
Optimal first-trimester anticoagulation is still challenging in pregnant women with mechanical heart valves (MHVs) requiring high-dose warfarin. This multicenter prospective study aims to determine ...the optimal anticoagulation regimens for pregnant patients with MHVs.
All women were allocated to one of three treatment options during first trimester including lone low-molecular-weight heparin (LMWH), combination of LMWH + 2.5 mg warfarin, and LMWH+4 mg warfarin. Primary maternal outcome included a combination of death, thromboembolism, severe bleeding, and need for treatment of mechanical valve thrombosis (MVT). Any fetal loss was determined as primary fetal outcome.
The study included 78 pregnancies in 65 women with MHVs. Primary maternal outcome rate was 44%, 12.5%, 3.5%, respectively. The rates of primary maternal outcome (44 vs 3.5%, P < .001), obstructive MVT (16 vs 0%, P = .04), MVT requiring treatment (28 vs 0%, P = .003), and cerebral embolism (24 vs 3.4%, P = .041) were found to be significantly higher in lone LMWH group compared to LMWH + 4 mg warfarin group. Moreover, the rates of primary maternal outcome (12.5 vs 44%, P = .015) and treatment for MHV thrombus (4.2 vs 28%, P = .049) were significantly lower in LMWH + 2.5 mg warfarin group compared to lone LMWH group. The incidences of fetal loss were 8 (32%) in the lone LMWH group, 8 (33.3%) in LMWH + 2.5 mg warfarin group, and 11 (37.9%) in LMWH + 4 mg warfarin group (P = .890 for 3-group).Warfarin related-embryopathy was not observed in any case.
The combined anticoagulation strategy of LMWH plus low-dose warfarin during the first trimester of pregnancy may result in less maternal complications with comparable fetal outcomes in patients with MHVs.
Low-molecular-weight heparin (LMWH) is thought to be safer for the fetus, however it is suspected to be less protective for the mother. To solve this dilemma, the authors suggested a novel anticoagulation strategy in pregnant women with prosthetic valves. Seventy-eight pregnancies of 65 women (median age 32 27-35 years) were included in the study. A combination of LMWH and a reduced dose warfarin were associated with low rates of thrombus-related complications in pregnant patients with mechanical heart valves.
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