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.

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.

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.

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.

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.

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.

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.

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.

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.

Prevalence of Incidental Gynecomastia by Chest Computed Tomography in Patients with a Prediagnosis of COVID-19 Pneumonia.

OBJECTIVE: In this study, we aimed to determine the prevalence of gynecomastia by evaluating computed tomography (CT) images of male patients who were admitted to our hospital during the coronavirus disease-2019 (COVID-19) pandemic. MATERIALS AND METHODS: This study included a total of 1,877 patients who underwent chest CT for prediagnosis of COVID-19 pneumonia between March 15th and May 15th, 2020. All images were evaluated for the presence of gynecomastia. Gynecomastia patterns were evaluated according to morphological features, and diagnoses were made by measuring the largest glandular tissue diameter. Statistical analysis was performed with IBM SPSS software version 25.0. RESULTS: The prevalence of gynecomastia was 32.3%. In terms of pattern, 22% were nodular, 57% were dendritic, and 21% were diffuse glandular gynecomastia. A significant correlation was found between age and gynecomastia pattern (p<0.001). The incidence of nodular, dendritic, and diffuse glandular gynecomastia increased with advancing age. A significant difference was found in the analysis of the correlation between age groups and glandular tissue diameters (p<0.001). With an increase in glandular tissue diameter, the gynecomastia pattern changed from a nodular to a diffuse glandular pattern. CONCLUSION: In our study, gynecomastia diagnosis was made through axial CT images. Although CT should not replace mammography and ultrasonography for clinical diagnosis of gynecomastia, chest CT scans can be used to evaluate patients with suspected gynecomastia.

Atypical chest CT findings of COVID-19 pneumonia: a pictorial review.

Coronavirus disease 2019 (COVID-19) first emerged in China and rapidly spread in the world causing a pandemic. Chest computed tomography (CT) continues to play an important role in the diagnosis and follow-up of the disease due to shortcomings of the real-time reverse transcription-polymerase chain reaction test, which is the gold standard in the diagnosis of this disease. Typical chest CT findings of COVID-19 pneumonia have been widely reported in the literature. However, atypical findings such as central involvement, peribronchovascular involvement, isolated upper lobe involvement, nodular involvement, lobar consolidation, solitary involvement, pleural and pericardial fluid, and subpleural sparing can also be seen. Knowing these atypical findings is important to avoid misdiagnosis. This review summarizes the atypical findings that can be seen in the course of the disease and may be confused with other diseases.

The novel coronavirus pneumonia (COVID-19): a pictorial review of chest CT features.

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), was first reported in Wuhan, China. The infection rapidly spread to more than 200 countries around the world. The clinical presentation of the disease may vary from mild illness to severe pneumonia such as acute respiratory distress syndrome (ARDS). The chest computed tomography (CT) has an important complementary role in diagnosis of the disease. The predominant CT findings of the disease are ground glass opacities and consolidations located in subpleural areas of lower lobes. Widespread ground-glass opacities, consolidation, air bronchograms, central involvement of lung parenchyma, mediastinal lymphadenopathy are more common in patients with the severe form of the disease. CT imaging also guides in differentiation of alternative diagnosis or in assessment of associated pulmonary embolism during the course of the disease. In this pictorial review we aim to review the CT features of COVID-19 pneumonia and mention the changes throughout the disease process.

CT imaging features of COVID-19 pneumonia: initial experience from Turkey.

PURPOSE: We aimed to demonstrate the computed tomography (CT) findings observed at the initial presentation of coronavirus disease 2019 (COVID-19) pneumonia and reveal the most frequent infiltration and distribution patterns of the disease. METHODS: One hundred and eighty-five patients (87 men, 98 women; mean age, 48.7 years), who underwent RT-PCR sampling and high-resolution CT examination in our hospital between March 15, 2020, and April 15, 2020, and got a definitive diagnosis of COVID-19 disease via initial or follow-up RT-PCR test, were included in the study. We comprehensively analyzed the most common and relatively rare CT imaging features (e.g., distribution pattern, density of the lesions, additional CT signs) in patients diagnosed with COVID-19 pneumonia. RESULTS: Thirty-eight patients (20.6%) had no evidence of pneumonia on their initial high-resolution CT images. Among 147 patients (79.4%) who had parenchymal infiltration consistent with pneumonia, 10 (6.8%) had a negative baseline RT-PCR test, and positivity was detected as a result of repeated tests. Most of the patients had multifocal (89.1%) and bilateral (86.4%) lesions. The most common location, right lower lobe, was affected in 87.8% of the patients. Lesions were distributed predominantly at peripheral (87.1%) and posterior (46.3%) areas of lung parenchyma. Most of the patients had pure ground glass opacity (GGO) (82.3%) followed by GGO with consolidation (32.7%) and crazy paving pattern (21.8%). Pure consolidation, solid nodules, halo sign, reverse halo sign, vascular enlargement, subpleural line, air-bronchogram, and bronchiectasis were the other findings observed in at least 15% of the cases. Halo sign, acinar nodules, air-bubble sign, pleural thickening and effusion, mediastinal and/or hilar lymphadenopathy were seen rarely (2%-12.9%). Pericardial effusion, pneumothorax, cavitation, and tree-in-bud pattern were not detected in our study group. CONCLUSION: Multifocal and bilateral GGO infiltration predominantly distributed in peripheral, posterior, and lower lung areas was the most common infiltration pattern.

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.

Evaluation of Liver Iron Content by Magnetic Resonance Imaging in Children with Acute Lymphoblastic Leukemia after Cessation of Treatment

Objective: There are a limited number of studies evaluating iron overload in childhood leukemia by magnetic resonance imaging (MRI). The aim of this study was to determine liver iron content (LIC) by MRI in children with acute lymphoblastic leukemia (ALL) who had completed treatment and to compare those values with serum iron parameters. Materials and Methods: A total of 30 patients between the ages of 7 and 18 who had completed ALL treatment were included in the study. Serum iron parameters (serum iron, serum ferritin [SF], and total iron-binding capacity) and liver function tests were studied. R2 MRI was performed for determining LIC. Results: Normal LIC was detected in 22 (63.4%) of the cases. Seven (23.3%) had mild and 1 (3.3%) had moderate liver iron deposition. In contrast, severe iron overload was not detected in any of the cases. LIC levels were correlated with the numbers of packed red blood cell (pRBC) transfusions (r=0.637, p<0.001), pRBC transfusion volume (r=0.449, p<0.013), SF levels (r=0.561, p=0.001), and transferrin saturation (r=0.353, p=0.044). In addition, a positive correlation was found between the number of pRBC transfusions and SF levels (r=0.595, p<0.001). Conclusion: We showed that the frequency of liver iron deposition was low and clinically less significant after the end of treatment in childhood ALL patients. LIC was demonstrated to be related to SF and transfusion history. These findings support that SF and transfusion history may be used as references for monitoring iron accumulation or identifying cases for further examinations such as MRI.

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.