Comparison of chest computed tomography and 3-T magnetic resonance imaging results in patients with common variable immunodeficiency.

BACKGROUND: Patients with common variable immunodeficiency (CVID) have an increased incidence of pulmonary infections and require frequent follow-up computed tomography (CT) scans. PURPOSE: To evaluate the diagnostic performance of 3-T magnetic resonance imaging (MRI) in patients with CVID. MATERIAL AND METHODS: In this prospective study, 3-T MRI was performed in 20 patients with CVID. The patients were imaged with CT and MRI scans on the same day. The MRI protocol included a T2-weighted HASTE sequence (TR=1400 ms, TE=95 ms, slice thickness (ST)=3 mm), T2-weighted BLADE sequence (TR=5379 ms, TE=100 ms, ST=3 mm), and 3D VIBE sequence (TR=3.9 ms, TE=1.32 ms, ST=3 mm). Mediastinal and parenchymal changes were compared. A modified Bhalla scoring system was used in the evaluation of CT and MRI scans. RESULTS: A total of 17 (85%) patients had parenchymal abnormalities identified by CT or MRI. Similar findings were detected with CT and MRI in the assessment of the severity of bronchiectasis (P=0.083), bronchial wall thickening (P=0.157), and mucus plugging (P=0.250). Consolidations were detected with both modalities in all patients. There was excellent concordance between the two modalities in the evaluation of nodules >5 mm (nodule size 5-10 mm, P=0.317; nodule size >10 mm, P=1). However, MRI failed to detect most of the small nodules (<5 mm). CONCLUSION: 3-T MRI detected mediastinal and parenchymal alterations in patients with CVID and provided findings that correlated well with CT. Despite a few limitations, MRI is a well-suited radiation-free technique for patients requiring longitudinal imaging.

Cardiac Assessment in Children with MIS-C: Late Magnetic Resonance Imaging Features.

Multisystem Inflammatory Syndrome (MIS-C) is a new entity that emerges 2-4 weeks after the SARS-CoV-2 infection in children. MIS-C can affect all systems, the most severe of which is cardiac involvement. The duration of the cardiac symptoms is still uncertain and may be persistent or prolonged. The American College of Rheumatology Clinical Guidelines recommends cardiac magnetic resonance imaging (MRI) 2-6 months after the diagnosis of MIS-C in patients presenting with significant transient left ventricular (LV) dysfunction in the acute phase of illness (LV ejection fraction 50%) or persistent LV dysfunction. There are a few studies investigating cardiac MRI findings in MIS-C patients. In this study, we aimed to evaluate cardiac MRI findings, at the earliest 3 months after diagnosis, and compare these findings with the echocardiograms in children with MIS-C. A retrospective study including 34 MIS-C patients was conducted at a tertiary-level University Hospital between June 2020 and July 2021. Centers for Disease Control and Prevention criteria were used in the diagnosis of MIS-C. Cardiac MRI was performed at least 3 months after MIS-C diagnosis. The study included 17 (50%) boys and 17 (50%) girls with a mean age of 9.31 ± 4.72 years. Initial echocardiographic evaluation revealed cardiac abnormality in 13 (38.2) patients; 4 (11.8%) pericardial effusion, 4 (11.8%) left ventricular ejection fraction (LVEF) < 55%, and 5 (14.7%) coronary artery dilatation. Echocardiography showed normal LV systolic function in all patients during follow-up; coronary dilatation persisted in 2 of 5 (40%) patients at the 6th-month visit. Cardiac MRI was performed in 31 (91.2%) patients, and myocardial hyperemia was not detected in any patients (T1 relaxation time was < 1044 ms in all children). However, 9 (29%) patients' MRI showed isolated elevated T2 levels, and 19 (61.3%) revealed at least one of the following findings: pericardial effusion, right ventricular dysfunction, or LVEF abnormality. In patients with MIS-C, a high rate of cardiac involvement, particularly pericardial effusion was determined by cardiac MRI performed at the earliest 2-6 months after diagnosis. Even if echocardiography does not reveal any abnormality in the initial phase, cardiac MRI should be suggested in MIS-C patients in the late period. This is the first study reporting cardiac MRI findings in the late period of MIS-C patients.

Modern management of iron overload in thalassemia major patients guided by MRI techniques: real-world data from a long-term cohort study.

Monitoring liver and cardiac iron stores by magnetic resonance imaging (MRI) enables identifying patients at risk of organ-specific morbidity and better tailoring of iron chelation therapy in thalassemia. Nevertheless, serum ferritin (SF) remains the only tool for monitoring iron status in most resource-poor regions. In this study, we assessed the impact of using MRI techniques to guide iron chelation therapy on iron overload outcomes in a cohort of 99 patients with thalassemia major (TM, mean age at baselines 20.7 ± 6.9 years) followed from 2006 to 2019. We also assessed the ability of SF trends to predict changes in consecutive liver iron concentration (LIC) and cardiac T2* (cT2*) measurements. The most commonly used chelator was deferasirox at baseline (65%) and final (72%) assessments. Overall, patients with safe LIC values (< 7 mg/g dw) increased from 57 to 77%, and safe cT2* values (> 20 ms) increased from 72 to 86%. We obtained the most significant improvement in patients with severe and moderate liver (p = 0.006 and p < 0.001) and cardiac (p < 0.0013 and p < 0.0001) iron overload at baseline. SF trends were in the same direction in 64% of changes in LIC, but only 42% of changes were proportional. Most of the changes in SF (64%) and LIC (61%) could not predict changes in cT2*. Moreover, downward trends in SF and LIC were associated with worsening cardiac iron in 29% and 23.5% of consecutive cT2* measurements. Liver and cardiac MRI-driven oral iron chelation improved the iron status of subjects with TM and demonstrated the importance of using validated MRI techniques in critical clinical decisions.

The outcome of peripartum cardiomyopathy patients-single center experience.

OBJECTIVE: Peripartum cardiomyopathy (PPCM) diagnosis made by excluding identifiable causes of heart failure (HF) and occurs end of the pregnancy or during the postpartum period of five months. It presents a clinical HF spectrum with left ventricular systolic dysfunction. BACKGROUND: The purpose of this study is to retrospectively evaluate the clinical characteristics, cardiac magnetic resonance (CMR) imaging features, and end-points consisting of left ventricle recovery, left ventricular assist device implantation, heart transplantation, and all-cause mortality. METHOD: Outpatient HF records between 2008 to 2021 were screened. Thirty-seven patients were defined as PPCM. Twenty-five patients had CMR evaluation at the time of diagnosis, and six patients were re-evaluated with CMR. RESULTS: The mean age was 30.5 ± 5.6 years, and the mean LVEF was 28.2% ± 6.7%. In 13(35.7%) patients, LVEF recovered during the follow-up course. The median recovery time was 281(IQR [78-358]) days. LVEF on CMR was 35.3 ± 10.5, and three patients exhibited late gadolinium enhancement(LGE) patterns. Sub-endocardial and mid-wall uptake pattern types were detected. 18(75%) patients met the Petersen left ventricle non-compaction cardiomyopathy(LVNC) criteria. Patients with NC/C ratio lower than 2.3 had lower LVEDVi and LVESVi (124.9 ± 35.4, 86.4 ± 7.5, p = .003; 86.8 ± 34.6, 52.6 ± 7.6, p = .006), respectively. The median follow-up time was 2129 (IQR [911-2634]) days. The primary endpoint-free 1-year survival was 88.9% (event rate 11.1%), and 5-year survival was 75.7% (event rate 24.3%). CONCLUSION: In a retrospective cohort of PPCM patients, 35.7% of patients' LVEF recovered, and the primary end-point of free-5-year survival was 75%. Twenty-five patients were assessed with CMR; three of four met the Petersen CMR-derived LVNC at initial evaluation.

Serial CT analysis in idiopathic pulmonary fibrosis: comparison of visual features that determine patient outcome.

AIMS: Patients with idiopathic pulmonary fibrosis (IPF) receiving antifibrotic medication and patients with non-IPF fibrosing lung disease often demonstrate rates of annualised forced vital capacity (FVC) decline within the range of measurement variation (5.0%-9.9%). We examined whether change in visual CT variables could help confirm whether marginal FVC declines represented genuine clinical deterioration rather than measurement noise. METHODS: In two IPF cohorts (cohort 1: n=103, cohort 2: n=108), separate pairs of radiologists scored paired volumetric CTs (acquired between 6 and 24 months from baseline). Change in interstitial lung disease, honeycombing, reticulation, ground-glass opacity extents and traction bronchiectasis severity was evaluated using a 5-point scale, with mortality prediction analysed using univariable and multivariable Cox regression analyses. Both IPF populations were then combined to determine whether change in CT variables could predict mortality in patients with marginal FVC declines. RESULTS: On univariate analysis, change in all CT variables except ground-glass opacity predicted mortality in both cohorts. On multivariate analysis adjusted for patient age, gender, antifibrotic use and baseline disease severity (diffusing capacity for carbon monoxide), change in traction bronchiectasis severity predicted mortality independent of FVC decline. Change in traction bronchiectasis severity demonstrated good interobserver agreement among both scorer pairs. Across all study patients with marginal FVC declines, change in traction bronchiectasis severity independently predicted mortality and identified more patients with deterioration than change in honeycombing extent. CONCLUSIONS: Change in traction bronchiectasis severity is a measure of disease progression that could be used to help resolve the clinical importance of marginal FVC declines.

Insulin secretion-sensitivity index-2 could be a novel marker in the identification of the role of pancreatic iron deposition on beta-cell function in thalassemia major.

The purpose of this study is to evaluate the impact of insulin secretion-sensitivity index-2 (ISSI-2) in the identification of the role of pancreatic iron deposition on beta-cell function in thalassemia major. Tissue iron stores were measured with magnetic resonance imaging (MRI) in the liver (R2), pancreas (R2*), and heart (T2*). ISSI-2 was assessed as a novel oral glucose tolerance test-based measure of beta-cell function. Also, the Stumvoll index showing the insulin sensitivity and Stumvoll index estimating first and second phase insulin secretion were calculated. Fourteen of the 51 Thalassemia Major patients, aged 8-34 (mean 21.1 ± 7.2) years-old, had either an impaired glucose tolerance test (n = 9, 17.6%) or diabetes mellitus (n = 5, 9.8%)-referred to as the glucose dysregulation (GD) group. The median serum ferritin and the mean liver R2 and cardiac T2* values were not significantly different between the GD and normal glucose tolerance (NGT, n = 37) groups whereas pancreas R2* was significantly higher in the GD group compared to the NGT group (p = 0.004). Patients with GD showed significantly lower ISSI-2 index (p < 0.001) as well as the Stumvoll index and Stumvoll first and second phase indices compared to those with NGT (p < 0.001). All patients with GD displayed a pancreas R2* >50 Hz and ISSI-2 <2. In conclusion, Pancreas R2* MRI combined with ISSI-2 index may be valuable parameters to identify patients at the highest risk for developing glucose dysregulation.

MRI assessment of pituitary iron accumulation by using pituitary-R2 in ß-thalassemia patients.

Background Patients with thalassemia major (TM) require repeated blood transfusions, which leads to accumulation of iron in a wide variety of tissues. Accumulation of iron in the pituitary gland can lead to irreversible hypogonadotropic hypogonadism (HH) in this group of patients. Purpose To investigate the reliability of pituitary-R2 as a marker to estimate the extent of pituitary iron load by comparing the pituitary magnetic resonance imaging (MRI) findings with hepatic iron load and serum ferritin levels. Material and Methods A total of 38 ß-TM patients were classified into HH (group A, n = 18) and non-HH (group B, n = 17) groups. A third group, group C, consisted of 17 healthy participants. Each participant underwent 1.5-T MRI examinations. Pituitary gland heights (PGH), pituitary-R2 values, and liver-R2 values were measured by using multi-echo spin-echo sequences. Results Pituitary-R2 values were significantly higher in group A compared with group B ( P < 0.05). A positive correlation was detected between the pituitary-R2 values and serum ferritin levels in TM patients ( P < 0.01). A threshold value of 14.1 Hz for pituitary-R2 was found to give a high specificity and sensitivity in distinguishing the TM patients with HH from those with normal pituitary functions. PGH measurements were significantly lower in group A compared with group B ( P < 0.05). Conclusion MRI-assessed pituitary-R2 seems to be a reliable marker for differentiating the TM patients with normal pituitary function from those with secondary hypogonadism due to iron toxicity.

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.

Multidetector computed tomographic assessment of the normal diameters for the thoracic aorta and pulmonary arteries in infants and children.

BACKGROUND: Knowledge of the normal size of the thoracic aorta and pulmonary arteries is important regarding the detection of the abnormal and valuable in the treatment of patients with congenital and acquired cardiovascular diseases. PURPOSE: To determine the normal diameters of the thoracic vascular structures of pediatric participants on contrast-enhanced multidetector computer tomography (MDCT) scans. MATERIAL AND METHODS: Between July 2010 and July 2014, the MDCT examinations obtained from 520 participants (age range, 0-18 years; mean age, 8.49 years ± 5.54 [standard deviation]; male:female ratio, 1.6:1) with normal cardiovascular examinations were retrospectively evaluated. Patients were divided into six groups according to their age. Diameters of the ascending aorta (AA), descending aorta (DA), main pulmonary artery (MPA), right pulmonary artery (RPA), and left pulmonary artery (LPA) were measured. RESULTS: The diameters of the thoracic vascular structures increased with age and the statistical difference among the age groups and genders were significant. The vascular structures in male patients tended to have higher dimensions than female patients. The LPA demonstrated higher mean values than the RPA in each age group. The mean ratio PA (MPA/AA) is 0.93 ± 0.08 for pediatric participants and it is slightly higher in girls compared to boys (0.93 ± 0.07 and 0.92 ± 0.07, respectively). The level of pulmonary artery bifurcation moves caudally with increasing age with thoracal 6 vertebra being the most common for all age groups (53.3%). CONCLUSION: We believe that the results of our study can serve as a potential reference in differentiating the normal from the abnormal size of the aorta and pulmonary arteries on chest MDCT studies in the pediatric population.

The effect of HFE polymorphisms on cardiac iron overload in patients with beta-thalassemia major.

OBJECTIVE: We aimed to investigate the effect of human hemochromatosis protein (HFE) polymorphisms on cardiac iron overload in patients with beta-thalassemia major. METHODS: Our study included 33 patients diagnosed with beta-thalassemia major who were treated with regular transfusions and chelation therapy. M-mode, tissue Doppler, and pulsed wave Doppler echocardiography were performed on all patients. T2* magnetic resonance imaging (MRI) scans were also performed. The HFE polymorphisms (H63D, C282Y, S65C, Q283P, E168Q, E168X, W169X, P160delC, Q127H, H63H, V59M, and V53M) were studied using polymerase chain reaction. RESULTS: The H63D polymorphism was detected in six patients with beta-thalassemia major. Five patients were heterozygous for the H63D polymorphism, while one was homozygous. There were no other polymorphisms. There was no relationship between the HFE polymorphisms and either the serum ferritin levels or the T2-weighted MRI values (P > .05). Moreover, conventional echo and tissue Doppler echo findings were not correlated with the HFE polymorphisms. Pulmonary vein atrial reversal flow velocity, which is a manifestation of diastolic dysfunction measured with pulse wave echo, was higher in the patients with HFE polymorphisms (P = .036). CONCLUSIONS: The HFE polymorphisms had no effect on cardiac iron overload. However, pulmonary vein atrial reversal flow velocity measurements can provide important information for detecting diastolic dysfunction during cardiac follow-up of patients with HFE polymorphisms. Studies with more patients are needed to provide more information regarding this matter.

[Impact of the practice of "Extended Focused Assessment with Sonography for Trauma" (e-FAST) on clinical decision in the emergency department]. / Acil serviste "Genisletilmis Acil Travma Ultrasonografisi" uygulamalarinin klinik karar üzerine etkisi.

BACKGROUND: We aimed to show the sensitivity of Extended Focused Assessment with Sonography for Trauma (e-FAST) for detection of pneumothorax, hemothorax and intraabdominal injury. We also investigated the relationship between e-FAST and need for invasive treatment. METHODS: This study included patients who experienced multiple trauma. The emergency physician, who had no clinical information about the patient, performed e-FAST. Findings on a supine chest X-ray and invasive interventions were recorded. The results of abdomen and thorax computed tomography (CT) were reviewed (the size of the pneumothorax was scored). RESULTS: Compared with CT, the sensitivities of e-FAST for intraabdominal injury and hemothorax were 54.5% and 71%, respectively. The patients with hemothorax and intraabdominal injuries were not identified with e-FAST, didn't need for invasive intervention. Pneumothorax diagnosis was established in 27 patients with e-FAST (sensitivity 81.8%) from among 33 (30.8%) pneumothorax patients. According to the grading on CT, pneumothoraces less than 1 cm in width and not exceeding the midcoronal line in length were not identified. e-FAST was positive for all patients performed with tube thoracostomy. CONCLUSION: e-FAST can be used with high sensitivity for determination of pneumothorax requiring invasive procedure. It has low sensitivity in the diagnosis of intraabdominal injury and hemothorax; however, e-FAST can predict the need for invasive procedures.

Drug-induced lung disease: a brief update for radiologists.

Pulmonary adverse events and drug-induced lung disease (DILD) can occur when treating many conditions. The incidence of DILDs in clinical practice and the variety of radiological findings have increased, mainly due to the increased use of novel therapeutic agents. It is crucial to determine whether the newly emerging clinical and imaging findings in these patients are due to the progression of the underlying disease, infection, pulmonary edema, or drug use, as this will change the patient management. Although the diagnosis of DILD is usually obtained by excluding other possible causes, radiologists should be aware of the imaging findings of DILD. This article reviews the essential radiological results of DILD and summarizes the critical clinical and imaging findings with an emphasis on novel therapeutic agents.

Coronary artery bypass grafting in a patient with situs inversus totalis.

Situs inversus totalis (SIT) describes a complete mirror image of the visceral organs in the thoracic and abdominal cavities. Dextrocardia, in combination with SIT, is a rare congenital anomaly with a frequency of 1:10 000, and coronary heart disease may occur with a similar frequency and manifestation as in the general population. Coronary computed tomography (CT) angiography is useful for accurately assessing the coronary artery origin and position for preprocedural planning of difficult coronary artery catheterisation in SIT. In this case, invasive coronary angiography (ICA) was performed from the same angle but on the opposite side compared to standard angiography. With the use of volume-rendered three-dimensional and curved reformatted images reconstructed from coronary CT angiography, the advancement of guidewires and catheters during ICA as well as the planning of surgical procedures can be performed more safely.

Traumatic left main coronary artery dissection in a young adult following blunt chest trauma - A case report.

Blunt chest trauma may cause variable degrees of thoracic injuries. Most of the patients may remain asymptomatic after sustaining blunt chest trauma. But in rare instances, life-threatening conditions such as coronary artery dissection may occur. The authors present a 29-year-old male adult with persistent chest pain following blunt trauma with a rise in cardiac troponins and elevated ST segment in ECG. Coronary CT and conventional angiography demonstrated dissection of the left main coronary artery. It is deemed necessary to suspect cardiac injury in patients with a history of blunt chest trauma in appropriate clinical settings. Early recognition of coronary artery dissection is vital to reduce morbidity and mortality. ECG combined with cardiac enzymes can be essential tools helping the physicians raise the suspicion towards a cardiac injury followed by cross-sectional and conventional angiographies for confirmation.

Primary Carnitine Deficiency as a Treatable Cause of Heart Failure in Young Patients.

Non-ischemic dilated cardiomyopathy is the most common subgroup of heart failure in young adults. Several metabolic defects could be the underlying etiology in these young heart failure patients. However, most cases are considered idiopathic. Primary carnitine deficiency is an overlooked inherited metabolic disease causing cardiomyopathy in these patients. Oral carnitine replacement therapy could prevent primary carnitine deficiency patients from progressing to advanced heart failure and life-threatening arrhythmias. In this case report, we present an index primary carnitine deficiency case and his brother's diagnosis and successful treatment period to draw attention to primary carnitine deficiency as a treatable cause of heart failure in young adults.

Case report: Therapy adherence, MTTP variants, and course of atheroma in two patients with HoFH on low-dose, long-term lomitapide therapy.

Background: Homozygous familial hypercholesterolemia (HoFH) is a rare and devastating genetic condition characterized by extremely elevated levels of low-density lipoprotein cholesterol (LDL-C) leading to an increased risk of premature atherosclerosis. Patients with Homozygous familial hypercholesterolemia mostly present with mutations in LDLR; however, herein, we present two cases with concomitant microsomal triglyceride transfer protein mutations, who showed different clinical courses and treatment adherence on long-term therapy with the new MTTP inhibitor lomitapide. Objectives: We aimed to present the possibility of preventing the progression of atherosclerotic burden with effective and safe LDL-C reduction in patients with Homozygous familial hypercholesterolemia on low-dose lomitapide therapy and emphasize the role of treatment adherence in therapy success. Methods: We present two patients with phenotypically Homozygous familial hypercholesterolemia, a compound heterozygous woman and a simple homozygous man, both with LDLR and additional MTTP mutations, who were treated with the MTTP-inhibiting agent lomitapide, with different treatment compliances. The role of impulsivity was investigated through Barratt Impulsivity Scale 11, and the extent of the atherosclerotic burden was followed up using coronary artery calcium scoring, echocardiographic and sonographic findings, and, eventually, through a strict follow-up of laboratory parameters. The patients were on lomitapide for 8 and 5 years, respectively, with no adverse effects. Conclusion: When accompanied by good adherence to therapy, low-dose lomitapide on top of standard lipid-lowering therapy with decreased frequency of lipid apheresis prevented the progression of atherosclerotic burden. Non-compliance might occur due to patient impulsivity and non-adherence to a low-fat diet.

Characteristics and long-term survival of patients with left ventricular non-compaction cardiomyopathy.

AIMS: Left ventricular non-compaction cardiomyopathy (LVNC) is a poorly understood entity resulting in heart failure. Whether it is a distinct form of cardiomyopathy or an anatomical phenotype is a subject of discussion. The current diagnosis is based on morphologic findings by comparing the compacted to non-compacted myocardium. The study aimed to compare demographic and prognostic variables of patients with dilated cardiomyopathy (DCM) and LVNC. Emphasis was given to cardiac magnetic resonance (CMR) imaging analysis. Data on survival were also assessed. METHODS AND RESULTS: We retrospectively evaluated the characteristics and outcomes of 262 non-ischaemic cardiomyopathy patients with LVNC and DCM phenotypes. Petersen's CMR criteria of non-compacted to the compacted myocardial ratio 2.3 were used to diagnose LVNC. The primary endpoint was a composite endpoint of major adverse cardiovascular events comprising cardiovascular-related death, left ventricular assisted device implantation, or heart transplantation. A total of 262 patients with CMR data were included in the study. One hundred fifty-five patients who fulfilled CMR criteria were diagnosed as LVNC. CMR findings revealed that LVNC patients had higher left ventricular end-diastolic (137.2 ± 51.6, 116.8 ± 44.6, P = 0.002) and systolic volume index (98.4 ± 49.5, 85.9 ± 42.7, P = 0.049). Cardiac haemodynamics, cardiac output (5.61 ± 2.03, 4.96 ± 1.83; P = 0.010), stroke volume (73.9 ± 28.8, 65.1 ± 25.1; P = 0.013), and cardiac index (2.85 ± 1.0, 2.37 ± 0.72; P < 0.0001), were higher in LVNC patients. Of all the 249 patients, 102 (40.9%) patients demonstrated late gadolinium enhancement (LGE). According to Petersen's criteria, the Kaplan-Meier survival outcome did not reveal significant differences (hazard ratio [HR]: 1.53, 95% confidence interval [CI]: [0.89-2.63], P = 0.11). The presence or pattern of LGE did not show significant importance for endpoint-free survival. Most of the sub-epicardial LGE pattern was found in LVNC patients (94.4%). When receiver operator characteristics analysis was applied to NC/C ratio to discriminate the primary endpoint, a higher NC/C ratio of 2.57 was associated with adverse events (HR: 1.90, 95% CI: [1.12-3.24], P = 0.016). CONCLUSIONS: Our study questions the criteria being used for the diagnosis of LVNC. Further evaluation of CMR variables and association of these findings with demographic variables and survival is mandatory.

The effect of reducing repetition time TR on the measurement of liver R2 for the purpose of measuring liver iron concentration.

The effects of reducing the pulse repetition time from 2500 ms to 1000 ms when using spin-density-projection-assisted R2-magnetic resonance imaging for the purpose of measuring liver iron concentration were evaluated. Repeated liver R2 measurements were made using both protocols on 60 subjects with liver iron concentrations ranging from 0.5 to 48.6 mg Fe (g dry tissue)(-1). The mean total scan time at repetition time 1000 ms was 42% of that at repetition time 2500 ms. The repeatability coefficients for the two protocols were not significantly different from each other. A systematic difference in the measured R2 using each protocol was found indicating that an adjustment factor is required when one protocol is used to replace the other. The 95% limits of agreement between the two protocols were not significantly different from their repeatability coefficients indicating that the protocols can be interchanged without any significant change in accuracy or precision of liver iron concentration measurement.

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.