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Introduction: Microvasculopathy and endothelial dysfunction play important roles in the development of post-transplant complications, including graft-versus-host disease (GVHD). We assessed structural microvasculopathy by employing nailfold video capillaroscopy (NFVC) and endothelial dysfunction via flow-mediated dilatation (FMD) of the brachial artery in recipients of hematopoietic stem cell transplantation. Patients and methods: Recipients of stem cell transplantation were included in this study post day+100 and divided into two cohorts. The first cohort consisted of 35 recipients of allogeneic hematopoietic stem cell transplantation (HCT) and the second cohort was comprised of 31 recipients of autologous HCT. A third cohort included 35 healthy individuals. NFVC was conducted on the second to fifth fingers of both hands using an Optilia video capillaroscope at 200× magnification, and the images were analyzed according to the European Alliance of Associations for Rheumatology (EULAR) criteria. The following parameters were used to measure vasculopathy: (a) median capillary density, derived from the capillary density of eight fingers, (b) median capillary diameter, derived from maximum capillary apical diameters of eight fingers, and (c) significant neoangiogenesis (neoangiogenesis present in ≥2 fingers). FMD of the right brachial artery was observed by high-resolution ultrasonography using the principle of post-occlusive reactive hyperemia, and video images were analyzed using edge-detecting software. Results: The median capillary diameter was significantly higher in the allo-HCT cohort (20.56±5.17 micrometer) compared to the auto-HCT cohort (16.19±3.31 micrometer; p<0.001) and healthy controls (14.66±2.61 micrometer; p<0.001). The median capillary density was significantly lower in the allo-HCT cohort (median: 6 capillaries/mm, range: 5-9 capillaries/mm) compared to the auto-HCT cohort (median: 8.5 capillaries, range: 5-12 capillaries/mm; p<0.001) and healthy controls (median: 8 capillaries/mm, range: 7-10.5 capillaries/mm; p<0.001). The allo-HCT cohort had a higher proportion of patients with significant neoangiogenesis (86%) than the auto-HCT cohort (10%) and healthy controls (9%). The presence of significant neoangiogenesis was more frequent in the subgroup of patients with a history of GVHD (93%) compared to the subgroup of patients without any history of GVHD (57%; p=0.044). No significant differences in NFVC parameters or FMD were observed between recipients of myeloablative and reduced-intensity conditioning regimens. There was no significant difference in NFVC parameters between the auto-HCT cohort and healthy controls. There was no significant difference in FMD among the three cohorts; however, a higher proportion of patients in the allo-HCT cohort (28%) had lower FMD than those in the auto-HCT cohort (3%) and healthy controls (6%), suggesting endothelial dysfunction. Conclusions: Our findings demonstrate the presence of structural microvasculopathy in allo-HCT recipients and suggest a possible role of alloreactivity in the pathogenesis of post-HCT microvasculopathy.

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Background: Transthoracic echocardiography (TTE) has traditionally been the primary method for coronary imaging in children with Kawasaki disease (KD). We aimed to evaluate coronary artery lesions (CALs) of the left circumflex artery (LCx) in KD on computed tomography coronary angiography (CTCA). Methods: Over a 9-year period (November 2013-December 2022), 225 children with KD underwent radiation-optimized CTCA on a 128-slice dual-source platform. TTE was performed on the same day, or a day prior or after CTCA. Findings: On CTCA, LCx CALs were seen in 41/225 (18.2%) patients. However, TTE detected CALs in only one third of these patients [15/41 (36.6%)]. CTCA showed 47 LCx CALs in 41 patients-aneurysms in 39 patients (40 fusiform, 2 saccular; 7 giant aneurysms), stenoses in 3, and thrombosis in 2. Thromboses and stenoses were both missed on TTE. Proximal LCx aneurysms were seen in 39 patients-of these, 12 had distal extension. Six patients had distal LCx aneurysms without proximal involvement and 2 non-contiguous multiple aneurysms. Four (9.75%) patients had isolated LCx involvement. Based on CTCA findings, treatment protocols had to be modified in 3/41 (7.3%) patients. Interpretation: This study highlights anatomical findings of LCx involvement in KD. Isolated LCx CALs were noted in 4/41 (9.75%) patients. TTE alone proved inadequate for LCx assessment in children with KD. With abnormalities detected in 18.2% of cases, including those missed by TTE, CTCA emerges as an essential imaging modality. The findings have implications for treatment planning and follow-up strategies in children with KD. Funding: None.

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There is evidence to suggest that M-type phospholipase A2 (PLA2R) antibodies activate the mannose-binding lectin (MBL) cascade, resulting in glomerular damage and proteinuria in patients with primary membranous nephropathy (PMN). Furthermore, there are few reports indicating that aberrant MBL activation is associated with endothelial dysfunction and accelerated atherosclerosis. While PMN is a common cause of adult nephrotic syndrome, and patients are at increased risk of cardiovascular disease (CVD), there is a lack of research that explores the factors that contribute to this condition. This study aims to determine the MBL levels in PMN and their relation to the clinical activity and endothelial dysfunction in PMN. The MBL levels of 22 biopsy-confirmed PMN patients were assessed at baseline and after 6 months of immunosuppressive therapy. In order to evaluate endothelial dysfunction in PMN patients, flow-mediated vasodilation (FMD) was measured at baseline and after treatment. A total of 22 healthy controls were included in this study to measure MBL levels and FMD. A significant difference was observed between MBL levels in PMN patients and healthy controls (p < .01). MBL levels decreased significantly after immunosuppressive therapy (p = .04). The baseline MBL levels and FMD levels exhibited a strong correlation (Spearman correlation coefficient [ρ] = 0.51: p = .01). In conclusion, the study signals the activation of the MBL cascade and its association with endothelial dysfunction in PMN patients.

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Detecting cardiac sarcoidosis; a potentially life-threatening condition is challenging and requires a multimodality imaging approach using echocardiography, PET/CT and CMR. Although 18F-FDG is the recommended PET tracer for evaluating cardiac sarcoidosis, it is limited by physiological cardiac FDG uptake and requires stringent patient preparation/ dietary modifications before imaging. We hereby present a case of cardiac sarcoidosis demonstrating myocardial FAPI uptake on cardiac PET, highlighting the potential role of 68Ga-FAPI PET in the evaluation of cardiac sarcoidosis.

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BACKGROUND: Pulmonary artery to left atrium fistula is an unusual structural cause of silent cyanosis. Only less than 100 cases have been reported so far. A high index of clinical suspicion and proper evaluation with bubble contrast echocardiography and cardiac computed tomography (CT) will help to detect this treatable anomaly. The advent of safer percutaneous closure methods has replaced the need for more invasive surgical closure. CASE PRESENTATION: We report an adolescent boy, who presented with long-standing cyanosis and progressive dyspnea with normal clinical cardiovascular examination. On evaluation, echocardiography and bubble contrast study revealed a large right pulmonary artery (RPA) to left atrium (LA) fistula. Cardiac CT confirmed the same with normal pulmonary venous drainage s/o a large 20 mm Type I RPA LA Fistula. He underwent successful percutaneous closure of the fistula tract with a 22 × 24 mm Cera™ duct occluder via transseptal approach uneventfully. CONCLUSION: Our case enlightens the methodological approach to diagnosing this rare anomaly as well as the feasibility of percutaneous intervention in such cases as it is one of the largest fistula tracts closed percutaneously to date.

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Autoimmune gastrointestinal (GI) disorders comprise a heterogeneous group of diseases with non-specific clinical manifestations. These are divided into primary and secondary. A high index of clinical suspicion complemented with endoscopic and radiological imaging may allow early diagnosis. Due to the relatively low incidence of autoimmune disorder, the imaging literature is sparse. In this review, we outline the pathogenesis, classification, and imaging appearances of autoimmune GI disorders.

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Partial anomalous pulmonary venous drainage is a congenital cardiac disorder characterized by abnormal drainage of one or more pulmonary veins into the systemic circulation. It can be isolated or associated with other congenital cardiac anomalies, most commonly atrial septal defect and patent ductus arteriosus. The clinical presentation is variable and depends on the degree of shunting and associated cardiac anomalies. Many patients usually remain asymptomatic until late in life. In this article, we presented a complex case of obstructive partial anomalous pulmonary venous drainage with dual drainage of bilateral accessory pulmonary veins with intact interatrial septum in conjunction with a patent ductus arteriosus and a ventricular septal defect. This pattern is incredibly rare and to the best of our knowledge has not been previously reported. Computed tomography played a pivotal role in precisely elucidating the intricate anatomy in this case with a complex pattern of anomalous pulmonary venous drainage.

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Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation. This article describes extremely rare complete duplication of inferior vena cava involving both suprarenal and infrarenal segments. Moreover, the complete duplication of inferior vena cava is seen in association with concomitant double superior vena cava, in a patient with visceroatrial situs solitus and associated congenital heart disease, which to the best of our knowledge, has not been reported so far in literature. This study also highlights the utility of multidetector computed tomography in accurate identification of such anomalies.

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Iatrogenic injuries are unavoidable complications of surgeries and minimally invasive procedures. They are generally classified into vascular and nonvascular injuries and based on the time of injury into early and late injuries. Iatrogenic injuries, particularly vascular injuries, increase the mortality and morbidity, with prolongation of hospital-stay. Multidetector computed tomography (MDCT) is a highly sensitive, and often the first imaging modality in suspected iatrogenic injuries. This pictorial review elucidates the imaging considerations and appearances of iatrogenic injuries of the abdominopelvic organs on MDCT.