Selen Bayraktaroglu

International survey of T2* cardiovascular magnetic resonance in β-thalassemia major

Accumulation of myocardial iron is the cause of heart failure and early death in most transfused thalassemia major patients. T2* cardiovascular magnetic resonance provides calibrated, reproducible measurements of myocardial iron. However, there are few data regarding myocardial iron loading and its relation to outcome across the world. A survey is reported of 3,095 patients in 27 worldwide centers using T2* cardiovascular magnetic resonance. Data on baseline T2* and numbers of patients with symptoms of heart failure at first scan (defined as symptoms and signs of heart failure with objective evidence of left ventricular dysfunction) were requested together with more detailed information about patients who subsequently developed heart failure or died. At first scan, 20.6% had severe myocardial iron (T2*≤10ms), 22.8% had moderate myocardial iron (T2* 10–20ms) and 56.6% of patients had no iron loading (T2*>20ms). There was significant geographical variation in myocardial iron loading (24.8–52.6%; P<0.001). At first scan, 85 (2.9%) of 2,915 patients were reported to have heart failure (81.2% had T2* <10ms; 98.8% had T2* <20ms). During follow up, 108 (3.8%) of 2,830 patients developed new heart failure. Of these, T2* at first scan had been less than 10ms in 96.3% and less than 20ms in 100%. There were 35 (1.1%) cardiac deaths. Of these patients, myocardial T2* at first scan had been less than 10ms in 85.7% and less than 20ms in 97.1%. Therefore, in this worldwide cohort of thalassemia major patients, over 43% had moderate/severe myocardial iron loading with significant geographical differences, and myocardial T2* values less than 10ms were strongly associated with heart failure and death.

Pulmonary and Vascular Manifestations of Behçet Disease: Imaging Findings

OBJECTIVE This article focuses on the radiologic findings related to vascular system involvement and pulmonary, pleural, and mediastinal involvement of Behçet disease. CT is an important diagnostic imaging technique in the evaluation of patients with Behçet disease. CONCLUSION CT is a valuable imaging technique in the diagnostic work-up of Behçet disease. CT effectively demonstrates vascular system involvement that is the main cause of mortality in these patients. CT is also effective in detecting mediastinal, pleural, and pulmonary parenchymal findings related to the disease.

Eponym. Scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome.

CT imaging of superior semicircular canal dehiscence: added value of reformatted images.

Abstract Conclusion: Superior semicircular canal dehiscence (SSCD) syndrome may present with various symptoms. CT scans previously interpreted as normal may show SSCD, especially if special reconstructions tailored for superior canal evaluation are added. Objectives: The purpose of this study was to investigate prevalence of SSCD, its length and its correlation with symptoms in patients who had previously undergone temporal bone CT examination that was reported normal and to demonstrate the importance of reformatted images in the diagnosis of SSCD. Methods: We retrospectively reviewed 108 patients who had undergone temporal bone CT examination for various symptoms and were reported as normal. High-resolution temporal bone CT imaging was performed with 1 mm slice thickness in the transverse plane. Each of the superior semicircular canals was evaluated in the plane of Pöschl and Stenver reformatted images together with axial images. Results: Ninety-three patients were included in the study. Nineteen patients with semicircular canal dehiscence were detected. The mean age of the study group was 45 years. Radiologic evidence of SSCD occurred in 23 of 186 temporal bones with a radiologic prevalence of 12%. The most common symptoms in dehiscent patients were vertigo, hearing loss and tinnitus. Defect lengths varied between 1 mm and 6.5 mm.

Reduction of Dialysate Calcium Level Reduces Progression of Coronary Artery Calcification and Improves Low Bone Turnover in Patients on Hemodialysis

Exposure to high Ca concentrations may influence the development of low-turnover bone disease and coronary artery calcification (CAC) in patients on hemodialysis (HD). In this randomized, controlled study, we investigated the effects of lowering dialysate Ca level on progression of CAC and histologic bone abnormalities in patients on HD. Patients on HD with intact parathyroid hormone levels ≤300 pg/ml receiving dialysate containing 1.75 or 1.50 mmol/L Ca (n=425) were randomized to the 1.25-mmol/L Ca (1.25 Ca; n=212) or the 1.75-mmol/L Ca (1.75 Ca; n=213) dialysate arm. Primary outcome was a change in CAC score measured by multislice computerized tomography; main secondary outcome was a change in bone histomorphometric parameters determined by analysis of bone biopsy specimens. CAC scores increased from 452±869 (mean±SD) in the 1.25 Ca group and 500±909 in the 1.75 Ca group (P=0.68) at baseline to 616±1086 and 803±1412, respectively, at 24 months (P=0.25). Progression rate was significantly lower in the 1.25 Ca group than in the 1.75 Ca group (P=0.03). The prevalence of histologically diagnosed low bone turnover decreased from 85.0% to 41.8% in the 1.25 Ca group (P=0.001) and did not change in the 1.75 Ca group. At 24 months, bone formation rate, trabecular thickness, and bone volume were higher in the 1.25 Ca group than in the 1.75 Ca group. Thus, lowering dialysate Ca levels slowed the progression of CAC and improved bone turnover in patients on HD with baseline intact parathyroid hormone levels ≤300 pg/ml.

Myocardial iron loading in patients with thalassemia major in Turkey and the potential role of splenectomy in myocardial siderosis.

Iron-induced cardiac disease is the leading cause of death in thalassemia major (TM). Splenectomy is performed in TM to reduce transfusion requirements and iron intake. Prevalence of myocardial siderosis and its relationship with splenectomy in 146 patients with TM were investigated. The patients with myocardial siderosis (T2*<20 ms) accounted for 42% of the cohort. Splenectomized patients had a higher incidence of myocardial siderosis (48%) compared with those having intact spleen (28%) and significantly higher myocardial iron content. Higher myocardial iron content in splenectomized patients may deserve special attention for the role of spleen in iron regulation.

Predictors of Clinical Outcome in Acute Pulmonary Embolism: Correlation of CT Pulmonary Angiography with Clinical, Echocardiography and Laboratory Findings

RATIONALE AND OBJECTIVES The aims of this study were to retrospectively evaluate whether computed tomographic (CT) parameters were predictors of in-hospital mortality within 30 days of CT imaging and to compare CT parameters with clinical, echocardiographic, and laboratory findings in patients with acute pulmonary embolism (PE). MATERIALS AND METHODS A total of 122 patients (61 women, 61 men; mean age, 64 ± 15 years) with CT scans positive for acute PE were reviewed. Two independent readers who were blinded to clinical outcomes scored pulmonary artery obstructions, evaluated cardiovascular measurements, and assessed qualitative findings. Reports of echocardiographic, clinical, and laboratory findings and clinical outcome were reviewed. Results were correlated with patient outcomes using Wilcoxons rank-sum, χ², and Students t tests. Logistic regression analyses were performed to determine predictors of patient outcomes. RESULTS Thirteen patients (11%) died related to PE within 30 days in the hospital. There were significant differences in the ratio of arterial partial pressure of oxygen to inspired fraction of oxygen and in heart rate between survivors and nonsurvivors (P < .05). No CT or echocardiographic predictor was associated with mortality. CONCLUSIONS The ratio of arterial partial pressure of oxygen to inspired fraction of oxygen and heart rate strongly predicted mortality due to PE. Neither CT pulmonary angiographic variables nor echocardiography could successfully predict in-hospital mortality in patients with acute PE.

A case of Mondini dysplasia with recurrent Streptococcus pneumoniae meningitis

Mondinis dysplasia is a developmental anomaly of the middle ear characterized by cochlear malformation with dilation of the vestibular aquaduct, vestibule, and ampullar ends of the semicircular canals. These deformities may result in a connection between subarachnoid space and the middle ear resulting in recurrent episodes of meningitis. Additionally, it is commonly associated with hearing impairment. We describe here a boy with recurrent meningitis and unilateral sensorineural hearing loss. Mondini dysplasia was demonstrated with computed tomographic scans of the temporal bones in the search for pathogenesis of recurrent meningitis.

The relationship between the myocardial T2* value and left ventricular volumetric and functional parameters in thalassemia major patients

PURPOSE Cardiac involvement in thalassemia major (TM) is mainly characterized by left ventricular dysfunction caused by iron overload. Cardiovascular magnetic resonance imaging (MRI) including myocardial T2* measurement is becoming increasingly popular for quantitatively evaluating myocardial iron overload. The aim of this study was to evaluate the relationship between the myocardial T2* value and left ventricular functional parameters and to examine the associations between the degree of cardiac iron load and various clinical parameters. MATERIALS AND METHODS A retrospective analysis of 47 patients (25 males and 22 females; mean age, 23.0±5.4 years) with TM was performed. Myocardial iron load was assessed by T2* measurements, and volumetric functions were analyzed using the steady state free precession sequence. RESULTS In patients with myocardial iron deposition (T2* < 20 ms), the mean left ventricular ejection fraction (LVEF) was 64.73±4.94%. The LVEF of patients with myocardial siderosis was significantly lower than that of patients without myocardial siderosis (r=0.35, P = 0.014). Inverse and significant correlations between both the left ventricular (LV) end-systolic volume index and the LV end-diastolic volume index and the myocardial T2* value (r=-0.32, P = 0.027 and r=-0.29, P = 0.046, respectively) were observed. There was an inverse correlation between the myocardial T2* value and the liver iron concentration (r=-0.31, P = 0.037). Cardiac T2* was not associated with serum ferritin levels, pre-transfusion hemoglobin levels or the annual red cell consumption rate. CONCLUSION Myocardial iron load assessed by cardiac MRI (T2*) is associated with deterioration in left ventricular function. Thalassemia major patients with myocardial siderosis may have LVEF values within normal limits, but this result must be interpreted cautiously.

Hydatid disease of right ventricle and pulmonary arteries: a rare cause of pulmonary embolism—computed tomography and magnetic resonance imaging findings (2009: 5b)

Hydatid disease can occur anywhere in the body and can demonstrate different imaging features that vary according to growth stage, associated complications, and affected tissue. Cardiovascular system involvement of hydatid disease is very rare. In this article, we present the cardiac magnetic resonance (MR) and thorax computed tomography (CT), MR angiography (MRA) findings of hydatid cysts located in the right ventricle and pulmonary arteries after surgical removal of hepatic hydatid cysts.