Right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery.

PURPOSE: Here, we report a case of the right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery. METHODS: A 42-year-old male patient underwent a whole-body computed tomography angiography (CTA) examination upon the clinical suspicion of vasculitis. RESULTS: CTA revealed a right-sided aortic arch with the isolation of the left innominate artery and hypoplasia of the left internal carotid artery. CONCLUSION: The right-sided aortic arch, with the isolation of the left innominate artery, is a scarce vascular variation that may occur with other cardiovascular anomalies such as ventricular septal defect. It can be asymptomatic or can present with symptoms of subclavian steal syndrome. Although its association with the agenesis of the left internal carotid artery has been reported, its association with the hypoplasia of the left internal carotid artery has not been reported previously to the best of our knowledge.

Dual origin of the left vertebral artery: a rare anatomic variation.

Duplication of the vertebral artery is a very rare vascular variant. We describe a case of a duplicated left vertebral artery. Computed tomography (CT) angiography examination of the head and neck vessels of a 63-year-old man with a history of imbalance was undertaken. A duplicated left vertebral artery was incidentally noted. The medial limb directly originated from the arcus aorta between the left common carotid artery and the left subclavian artery. The lateral limb originated from the proximal left subclavian artery as the first branch and entered the transverse foramen of the C6 vertebra as usual. Prior to neck procedures requiring the anterior cervical approach and carotid endarterectomy, the awareness and diagnosis of the duplicated vertebral artery are important due to the potential risk of inadvertent vertebral artery injury. Therefore, this variation should be taken into account while evaluating cross-sectional imaging studies.

A blind-ending branch of celiac artery: a persistent embryologic vascular remnant?

The celiac artery classically divides into three major branches, but it may have variations in branching pattern. Here we report a case of celiac artery having a blind-ending branch in a 54-year-old man who underwent computed tomography of the abdomen before the kidney transplantation surgery. Preprocedural assessment of celiac artery variations is critical for surgical and angiographic procedures involving the upper abdomen.

Type 4 retro-aortic left renal vein with an aneurysm.

Type 4 retro-aortic left renal vein (RLRV) and renal vein aneurysms are rare entities which may have clinical and surgical implications. Here we present computed tomography (CT) angiography images of a case of retro-aortic left renal vein draining into the left common iliac vein with a venous aneurysm.

Multiparametric assessment of right ventricular function in heart transplant recipients by echocardiography and relations with pulmonary hemodynamics.

OBJECTIVE: Right ventricular (RV) dilatation and dysfunction are usually present in heart transplant (HTx) patients and worsened with residual pulmonary hypertension (PH). We aimed to determine the ability of different echocardiographic modalities to evaluate RV function in comparison with cardiac magnetic resonance (CMR) and their relations with pulmonary hemodynamics in HTx patients. METHODS: A total of 62 data sets [echocardiographic, hemodynamic, and CMR] were acquired from 35 HTx patients. Comprehensive echocardiography, including two-dimensional (2D) transthoracic echocardiography, speckle tracking echocardiography, and three-dimensional (3D) echocardiography, was performed. Mean pulmonary artery pressure (mPAP) was obtained invasively from right heart catheterization. The correlations between all echocardiographic parameters and CMR imaging data and the differences between patients with and without residual PH were evaluated. RESULTS: Diastolic and systolic RV volumes and RV ejection fraction (RVEF) by 3D echocardiography correlated strongly with CMR-derived volumes and RVEF (r = .91, r = .79, r = .64; p < .0001 for each, respectively). Among other parameters, RV fractional area change (r = .439; p < .001) and RV free wall longitudinal strain (RVFW-LS) (r = -.34; p < .05) correlated moderately with CMR-RVEF, whereas tricuspid annulus S' velocity (r = .29; p < .05) and tricuspid annular systolic plane excursion (r = .27; p < .05) correlated weakly with CMR-RVEF. Additionally, 3D-RVEF and RVFW-LS were significantly decreased in studies with mPAP ≥ 20 mm Hg in comparison to those with mPAP < 20 mm Hg (47.7 ± 3.7 vs. 50.9 ± 5.3, p = .04 and -15.5 ± 3.1 vs. -17.5 ± 3, p = .03, respectively). CONCLUSION: The best method for the evaluation of RV function in HTx recipients is 3D echocardiography. Besides, the subclinical impact of residual PH on RV function can be best determined by RVFW-LS and 3D-RVEF in these patients.

The combined intrathyroidal course of the brachiocephalic artery, right common carotid artery and right subclavian artery.

PURPOSE: We present an extremely rare vascular variant in which the brachiocephalic artery, right common carotid artery, and right subclavian artery course through the right lobe of the thyroid gland. METHODS: A 54-year-old woman underwent a coronary computed tomography (CT) angiography examination with the suspicion of infective endocarditis. RESULTS: Unexpectedly, the distal brachiocephalic artery, the proximal right common carotid artery, and right subclavian artery had a course through the right lobe of the thyroid gland. Otherwise, the arcus aorta branching pattern was normal. CONCLUSION: The supraaortic major branches seldom have intrathyroidal course. The intrathyroidal course of the right common carotid artery was described previously only in one case. But, to our best knowledge, the combined intrathyroidal course of these three major vessels has not been previously reported. Although asymptomatic, such variations may complicate lower neck procedures involving thyroidectomies and thyroid biopsies if undetected and unreported. So, the awareness of this atypical course while reporting CT examinations is crucial prior to neck interventions.

Cross-sectional imaging findings of cardiac outpouchings.

A cardiac outpouching (CO) is a protrusion in a heart chamber's internal anatomical lining. Most COs are clinically insignificant, but some are of vital importance, requiring immediate surgery. Cross-sectional imaging findings of COs, such as location, morphology, size, and accompanying wall motion abnormalities, play an essential role in determining the correct diagnosis and appropriate clinical management. Therefore, radiologists should be familiar with them. This article reviews the key cross-sectional imaging findings and differential diagnoses of COs.

Approach to optimal assessment of right ventricular remodelling in heart transplant recipients: insights from myocardial work index, T1 mapping, and endomyocardial biopsy.

AIMS: Right ventricular (RV) dysfunction is an important cause of graft failure after heart transplantation (HTx). We sought to investigate relative merits of echocardiographic tools and cardiac magnetic resonance (CMR) with T1 mapping for the assessment of functional adaptation and remodelling of the RV in HTx recipients. METHODS AND RESULTS: Sixty-one complete data set of echocardiography, CMR, right heart catheterization, and biopsy were obtained. Myocardial work index (MWI) was quantified by integrating longitudinal strain (LS) with invasively measured pulmonary artery pressure. CMR derived RV volumes, T1 time, and extracellular volume (ECV) were quantified. Endomyocardial biopsy findings were used as the reference standard for myocardial microstructural changes. In HTx recipients who never had a previous allograft rejection, longitudinal function parameters were lower than healthy organ donors, while ejection fraction (EF) (52.0 ± 8.7%) and MWI (403.2 ± 77.2 mmHg%) were preserved. Rejection was characterized by significantly reduced LS, MWI, longer T1 time, and increased ECV that improved after recovery, whereas RV volumes and EF did not change MWI was the strongest determinant of rejection related myocardial damage (area under curve: 0.812, P < 0.0001, 95% CI: 0.69-0.94) with good specificity (77%), albeit modest sensitivity. In contrast, T1 time and ECV were sensitive (84%, both) but not specific to detect subclinical RV damage. CONCLUSION: Subclinical adaptive RV remodelling is characterized by preserved RV EF despite longitudinal function abnormalities, except for MWI. While ultrastructural damage is reflected by MWI, ECV, and T1 time, only MWI has the capability to discriminate functional adaptation from transition to subclinical structural damage.

Breast artery calcification as an opportunistic predictor of cardiovascular disease.

BACKGROUND: Atherosclerotic cardiovascular disease is still the leading cause of mortality for women. Breast cancer screening with mammography is recommended in all women aged over 40 years. AIMS: Whether breast artery calcification (BAC) is associated with cardiovascular disease is not clear. We aimed to evaluate the association between BAC and the presence of coronary atherosclerosis determined by CT. METHODS: All patients who underwent both mammography and coronary CT angiography between January 2010 and December 2016 were screened, and patients with a duration of less than 12 months between CT and mammography were included. RESULTS: A total of 320 women were included and BAC was detected in 47 (14.6%) patients. BAC was correlated with age and CT coronary calcium score. Both the frequency of critical coronary artery stenosis (34% vs 10.6%; p = 0.001) and CT coronary calcium score (5.5 vs 0; p = 0.001) was significantly higher in patients with BAC. The absence of BAC was a strong predictor of the absence of significant coronary artery disease (p = 0.001). BAC was independently associated with all-cause mortality after excluding patients with breast cancer (HR: 5.32; p = 0.013). CONCLUSION: Breast artery calcification is associated with coronary calcium score and significant coronary stenosis. A high BAC score is related to increased mortality.

Comprehensive assessment and outcomes of patients with chronic periaortitis.

OBJECTIVES: Chronic periaortitis (CP) is a less known but more frequently diagnosed fibro-inflammatory disorder, but we know little about it and data regarding follow-up and outcome are still very limited. This study aims to identify the clinicopathologic, laboratory, and radiologic features, as well as outcomes of CP patients. METHODS: Patients with CP from HUVAC database were included in the study. CP was diagnosed based on compatible imaging findings and histopathological evaluation (if available), in addition to clinical findings. Demographics, laboratory, clinical, and imaging data were retrospectively reviewed from medical records. RESULTS: A total of 51 (male/female:37/14) patients were included in the study. Median (IQR) age was 63 (53-69) years and follow-up duration was 40 (4-60) months. 32 of the patients were IgG4-related CP. The most common form of CP in our cohort was idiopathic retroperitoneal fibrosis (82%), followed by inflammatory abdominal aortic aneurysms (12%) and peri-aneurysmal retroperitoneal fibrosis (8%). 8 (15.6%) patients had thoracic periaortitis and 16 (31.6%) venous involvement. Cyclophosphamide (CYC) combined with steroids was the most preferred treatment modality (43%), followed by rituximab (RTX) (31.3%). Follow-up imaging was done after a median (IQR) of 7(3-11) months, 30% of the patients were stable and 64.1% showed regression. A total of 18 (35.2%) had been taken off therapy at the last visit. CONCLUSIONS: Idiopathic retroperitoneal fibrosis was the most frequent presentation, whereas 15.6% of patients had thoracic involvement. Venous involvement was also not uncommon. Optimal time for follow-up imaging was determined as 6-9 months. Steroids along with CYC/RTX had a favourable outcome in the treatment of these patients.

Treatment of childhood-onset Takayasu arteritis: switching between anti-TNF and anti-IL-6 agents.

OBJECTIVES: Biologics are new treatment alternatives in Takayasu arteritis (TA), although data in childhood are limited. The aim of this study was to share our experience in seven childhood-onset TA patients who received a TNF-α inhibitor (adalimumab) or an IL-6 receptor inhibitor (tocilizumab) and the effect of switching therapy. METHODS: We retrospectively evaluated the medical treatment records of seven patients with TA, followed between August 2005 and January 2021 at the Pediatric Rheumatology Department of Hacettepe University Faculty of Medicine. RESULTS: The median age of patients was 14 (IQR 4) years, and six were female. All of the patients had severe disease and high acute-phase reactants. The patients initially received only steroids or steroids+CYC. Prednisone was decreased, and biologic agents were started once the acute phase reactants decreased, and the Indian Takayasu Activity Score (ITAS) returned to normal. Initially, four patients received tocilizumab (TCZ) [median 25.5 (IQR 41) months] and three patients received adalimumab (ADA) [median 13 (IQR 31) months]. However, due to the progression of MR angiography findings or persistent elevation in acute-phase reactants, the biologic agents were switched from TCZ to ADA in four patients and from ADA to TCZ in three patients. The patients' median follow-up time after changing was 50 (IQR 77) months, and median ITAS was evaluated as '0' after 2 (IQR 4) months. CONCLUSIONS: In conclusion, both TNF-α and IL-6 inhibitors are effective alternatives in treating patients with childhood-onset TA. However, prospective randomized controlled trials are needed for the comparison of their effectiveness.

Evaluation of atrial fibrosis in atrial fibrillation patients with three different methods.

BACKGROUND: The presence of atrial fibrosis has already been known as a risk factor for atrial fibrillation (AF) development. We aimed to evaluate atrial fibrosis with previously defined three different methods, which were cardiac magnetic resonance imaging (C-MRI), echocardiographic strain imaging, and biomarkers and show the relationship between these methods in patients with AF scheduled for cryoballoon ablation. METHODS: A total of 30 patients were enrolled. Atrial T1 relaxation durations were measured using C-MRI before the procedure of atrial fibrillation catheter ablation. Fibroblast growth factor-21 (FGF-21) and fibroblast growth factor-23 (FGF-23) levels were measured at serum derived from the femoral artery (Peripheral FGF 21 and 23) and left atrium blood samples (Central FGF 21 and 23) before catheter ablation. Preprocedural transthoracic echocardiography was performed. The median follow-up duration for atrial tachyarrhythmia (ATa) recurrence was 13 (12-18 months) months. RESULTS: The mean ages of the study group were 55.23 ± 12.37 years, and there were 17 (56.7%) female patients in study population. There were negative correlations between post contrast T1 relaxation durations of both posterior and posterosuperior atrium, and central FGF-23 (r: - 0.561; p = 0.003; r:-0.624; p = 0.001; Posterior T1 vs. central FGF-23 levels and Posterosuperior T1 vs central FGF-23 levels, respectively). The positive correlations were observed between postcontrast posterior T1 relaxation durations and left ventricle ejection fraction (r:0.671; p = 0.001); left atrial emptying fraction (r:0.482; p = 0.013); peak atrial longitudinal strain (r:0.605; p = 0.001), and peak atrial contraction strain (r:0.604; p = 0.001). Also negative correlation was observed between postcontrast posterior T1 relaxation durations, and left atrial volume index (r: - 0.467; p = 0.016).

Specific FSTL1 polymorphism may determine the risk of cardiomyopathy in patients with acromegaly.

BACKGROUND: We have investigated the role of a cardiomyokine, follistatin-like 1 (FSTL1), and its single nucleotide polymorphism on acromegalic cardiomyopathy. METHODS: The study was performed as a cross-sectional case research in a Tertiary Referral Centre. Forty-six patients with acromegaly (29 F-17 M, mean age: 50.3 ± 12.1 years) were included. FSTL1 levels were measured and the rs1259293 region of the FSTL1 gene was subjected to polymorphism analysis. 1.5 Tesla MRI was used to obtain cardiac images. RESULTS: There were 15 active (6 F-9M) and 31 (22 F-9M) controlled patients. Active patients had a higher left ventricular mass (LVM) and left ventricular mass index (LVMi). GH levels were positively correlated with left ventricular end-diastolic volume index (LVEDVi), stroke volume index (SVi), cardiac index (Ci), LVM and LVMi; r = 0.35, 0.38, 0.34, 0.39 and 0.39, respectively. IGF-1 index was positively correlated with LVEDVi, left ventricular end-systolic volume index (LVESVi), SVi, Ci, LVM and LVMi; r = 0.36, 0.34, 0.32, 0.31, 0.42 and 0.42, respectively. Twenty out of 46 patients with acromegaly (43.5%) had myocardial fibrosis. FSTL1 levels were neither correlated with disease activity nor with any functional and structural cardiac parameter. Multivariate linear regression analysis revealed no association between FSTL1 and any study parameters. The rs1259293 variant genotype CC was significantly associated with low left ventricular mass. CONCLUSIONS: Serum FSTL1 levels are not associated with functional and structural measures of myocardium in patients with acromegaly. However, the risk of left ventricular hypertrophy is reduced in CC genotyped individuals of FSTL1.

Coronary (peri)-arteritis in patients with IgG4-related disease: A case series from the Central Anatolia Region of Turkey.

OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory disease which affects many systems, as well as the cardiovascular system. Identifying the coronary involvement like periaortitis, coronary periarteritis and pericarditis is important, as they often cause unfavorable outcomes. METHODS: Eighty-one patients with IgG4-RD were retrospectively evaluated for symptomatic coronary artery involvement from Hacettepe University Vasculitis Research Center (HUVAC) database. The demographic, laboratory, radiologic and clinical characteristics of the patients were assessed. RESULTS: Among 81 patients with IgG4-RD, 6 patients (M/F:5/1) had coronary artery involvement. The patients' median age was 57 and serum IgG4 levels were above normal except for one case. All patients with coronary arteritis revealed an increased coronary vessel wall thickening and stenotic lesions. The coronary aneurysm and pericarditis were observed in half of the patients. Immunosuppressive treatments were given to all the patients and most of them followed in stable condition. CONCLUSION: Coronary arteritis is a rare but notable manifestation of IgG4-RD. Although coronary periarteritis can cause significant morbidity and mortality, it seems better results can be achieved with early diagnosis and treatment.

Common ostium of inferior pulmonary veins: An extremely rare variant described by preprocedural computerized tomography angiography.

The common ostium of left and right inferior PVs is an extremely rare variant which was only reported in 16 cases undergoing catheter ablation. Thus, electrophysiologists should be careful about such an exremely rare PV variants for the safety and efficacy of ablation. Pre-procedural CTA is a valuable tool to decide on the ablation strategy in patients with such a very rare PV anomaly.

Cholesterol efflux promoting function of high-density lipoproteins in calcific aortic valve stenosis.

Background and aims: Cholesterol efflux capacity is a functional property of high-density lipoproteins (HDL) reflecting the efficiency of the atheroprotective reverse cholesterol transport process in humans. Its relationship with calcific aortic valve stenosis (CAVS) has not been fully assessed yet. Methods: We evaluated HDL-CEC in a patient population with varying degrees of aortic valvular calcific disease, assessed using echocardiography and cardiac computed tomography. Measurement of biomarkers that reflect osteogenic and tissue remodeling, along with dietary and gut microbiota-derived metabolites were performed. Results: Patients with moderate-severe CAVS had significantly lower HDL-CEC compared to both control and aortic sclerosis subjects (mean: 6.09%, 7.32% and 7.26%, respectively). HDL-CEC displayed negative correlations with peak aortic jet velocity and aortic valve calcium score, indexes of CAVS severity (ρ = -0.298, p = 0.002 and ρ = -0.358, p = 0.005, respectively). In multivariable regression model, HDL-CEC had independent association with aortic valve calcium score (B: -0.053, SE: 0.014, p < 0.001), GFR (B: -0.034, SE: 0.012, p = 0.007), as well as with levels of total cholesterol (B: 0.018, SE: 0.005, p = 0.002). Conclusion: These results indicate an impairment of HDL-CEC in moderate-severe CAVS and may contribute to identify potential novel targets for CAVS management.

Donor Nephrectomy May Compromise the Cardiovascular System: A Retrospective, Single-Center Study.

OBJECTIVES: Our goal was to determine the short-term effects of donor nephrectomy on the cardiovascular system and to gain a better understanding of the recently recognized long-term increased risk of end-stage renal disease and cardiovascular mortality. MATERIALS AND METHODS: Living kidney donors who underwent donor nephrectomy between January 2010 and January 2015 at the Hacettepe University Transplantation Unit were retrospectively screened. Echocardiographic parameters, kidney volumes, and renal functions before nephrectomy were compared with measurements after nephrectomy. Flow-mediated dilatation values of living kidney donors were compared with healthy individuals. RESULTS: The study included 73 female and 31 male living kidney donors with a mean age of 46.1 ± 10.8 years. In the comparative analysis of donors versus 35 healthy individuals, the changes in flow-mediated dilatation were 12.3 ± 5.7% and 15.4 ± 6.3%, respectively (P = .016). In the comparative analysis of preoperative versus the last visit transthoracic echocardiographic results, left ventricular end-systolic and end-diastolic diameters decreased and left ventricular posterior wall thickness and septum thickness increased (P = .025, P = .002, P = .026, and P = .019, respectively). CONCLUSIONS: Nephrectomy may cause several hemodynamic changes in living kidney donors, which may exacerbate cardiovascular risks in this population.