Thromboembolic and bleeding risk in cardiac amyloidosis.

Cardiac amyloidosis represents a spectrum of conditions characterized by the accumulation of insoluble fibrils, resulting in progressive deposition and myocardial dysfunction. The exact mechanisms contributing to the heightened risk of thromboembolic events and bleeding tendencies in cardiac amyloidosis remain unclear. Proteins such as transthyretin in transthyretin amyloidosis and light chains in light-chain amyloidosis, along with acute phase proteins in amyloid A (AA) amyloidosis, play complex roles in the coagulation cascade, affecting both coagulation initiation and fibrinolysis regulation. The increased occurrence of atrial fibrillation, systolic and diastolic left ventricular dysfunction, and atrial myopathy in patients with cardiac amyloidosis may predispose them to thrombus formation. This predisposition can occur regardless of sinus rhythm status or even with proper anticoagulant management. Bleeding events are often linked to amyloid deposits around blood vessels, which may increase capillary fragility and cause coagulation disturbances, leading to unstable international normalized ratio levels during anticoagulant therapy. Thus, comprehensive risk assessment for both thrombotic and hemorrhagic complications, especially before commencing anticoagulant therapy, is imperative. This review will explore the essential pathophysiological, epidemiologic, and clinical aspects of thromboembolic and bleeding risk in cardiac amyloidosis, evaluating the existing evidence and uncertainties regarding thrombotic and bleeding risk assessment and antithrombotic treatment.

Air embolism as a rare complication of lung biopsy: A case report.

Lung biopsy is an important interventional radiology procedure allowing the characterization of lesions with suspected malignancy. The most frequent complications are pneumothorax and hemorrhage. Air embolism is a rare but potentially fatal occurrence. In this case report, we present an air embolism after core needle CT-guided biopsy showing CT and MRI features that radiologists should expect in the everyday clinical practice.

Common Clinical and Molecular Pathways between Migraine and Sarcoidosis.

Migraine and sarcoidosis are two distinct medical conditions that may have some common biological and clinical pathways. Sarcoidosis is a chronic granulomatous disease characterized by the formation of granulomas in various organs, including the lungs, skin, cardiovascular system, lymph nodes, and brain. Migraine is a common comorbidity in sarcoidosis patients and a common neurological disorder characterized by recurrent headaches that can be accompanied by other symptoms, such as nausea, vomiting, and sensitivity to light and sound. There have been several reports of individuals with neurosarcoidosis experiencing migraines, though the exact relationship between the two disorders is not well understood. Both conditions have been associated with inflammation and the activation of the immune system. In sarcoidosis, the formation of granulomas is thought to be an immune response to the presence of an unknown antigen. Similarly, the pain and other symptoms associated with migraines are thought to be caused by inflammation in the brain and the surrounding blood vessels. There is also evidence to suggest an interplay of environmental and genetic factors playing a role in both conditions, but evidence is inconsistent with the hypothesis of shared genetic susceptibility. This review aims to illustrate common clinical and biological pathways between migraine and sarcoidosis, including inflammation and dysregulation of the immune system, with a focus on the cumulative burden of concurrent disorders and therapeutic implications.

Imaging findings of right cardiac amyloidosis: impact on prognosis and clinical course.

Cardiac involvement from amyloidosis is of growing interest in the overall literature. Despite cardiac amyloidosis (CA) has been considered for a long time a rare disease, the diagnostic awareness is increasing mainly thanks to the improvement of diagnostic softwares and of imaging techniques such as cardiac magnetic resonance  (CMR). Some authors have observed an increase of prevalence rate of CA; moreover it's often underestimated because clinical manifestations are aspecific. The interstitial infiltration of the left ventricle has been extensively studied, while the involvement of the right ventricle (RV) has been less investigated. Involvement of the RV, even in the absence of pulmonary hypertension or clearly left ventricle infiltration, plays an important role as prognostic factor and is useful to achieve an early diagnosis. Therefore, the use of fast and low-cost diagnostic methods such as ultrasound strain of the right ventricle could be used to recognize cardiac amyloidosis early. Herein the importance of evaluating the right ventricular involvement, which can predict the most severe course of the disease also without overt clinical manifestations. The role of imaging, in particular of echocardiography, CMR, and scintigraphy is here reported.

Sarcoidosis and COVID-19: At the Cross-Road between Immunopathology and Clinical Manifestation.

Coronavirus disease 2019 (COVID-19) has been associated with dysregulation of the immune system featuring inappropriate immune responses, exacerbation of inflammatory responses, and multiple organ dysfunction syndrome in patients with severe disease. Sarcoidosis, also known as Besnier-Boeck-Schaumann disease, is an idiopathic granulomatous multisystem disease characterized by dense epithelioid non-necrotizing lesions with varying degrees of lymphocytic inflammation. These two diseases have similar clinical manifestations and may influence each other at multiple levels, eventually affecting their clinical courses and prognosis. Notably, sarcoidosis patients are at high risk of severe COVID-19 pneumonia because of the underlying lung disease and chronic immunosuppressive treatment. In this narrative review, we will discuss interactions between sarcoidosis and COVID-19 in terms of clinical manifestations, treatment, and pathogenesis, including the role of the dysregulated renin-angiotensin system, altered immune responses involving increased cytokine levels and immune system hyperactivation, and cellular death pathways.

Clinical Features and Diagnosis of Cardiac Sarcoidosis.

Cardiac sarcoidosis (CS) is an unusual, but potentially harmful, manifestation of systemic sarcoidosis (SA), a chronic disease characterized by organ involvement from noncaseating and nonnecrotizing granulomas. Lungs and intrathoracic lymph nodes are usually the sites that are most frequently affected, but no organ is spared and CS can affect a variable portion of SA patients, up to 25% from post-mortem studies. The cardiovascular involvement is usually associated with a bad prognosis and is responsible for the major cause of death and complications, particularly in African American patients. Furthermore, the diagnosis is often complicated by the occurrence of non-specific clinical manifestations, which can mimic the effect of more common heart disorders, and imaging and biopsies are the most valid approach to avoid misdiagnosis. This narrative review summarizes the main clinical features of CS and imaging findings, particularly of CMR and 18-Fluorodeoxyglucose Positron Emission Tomography (18F-FDG PET) that can give the best cost/benefit ratio in terms of the diagnostic approach. Imaging can be very useful in replacing the endomyocardial biopsy in selected cases, to avoid unnecessary, and potentially dangerous, invasive maneuvers.

Appropriate use criteria for cardiovascular magnetic resonance imaging (CMR): SIC-SIRM position paper part 1 (ischemic and congenital heart diseases, cardio-oncology, cardiac masses and heart transplant).

Cardiac magnetic resonance (CMR) has emerged as new mainstream technique for the evaluation of patients with cardiac diseases, providing unique information to support clinical decision-making. This document has been developed by a joined group of experts of the Italian Society of Cardiology and Italian society of Radiology and aims to produce an updated consensus statement about the current state of technology and clinical applications of CMR. The writing committee consisted of members and experts of both societies who worked jointly to develop a more integrated approach in the field of cardiac radiology. Part 1 of the document will cover ischemic heart disease, congenital heart disease, cardio-oncology, cardiac masses and heart transplant.

Clinical Features, Histopathology and Differential Diagnosis of Sarcoidosis.

Sarcoidosis is a chameleon disease of unknown etiology, characterized by the growth of non-necrotizing and non-caseating granulomas and manifesting with clinical pictures that vary on the basis of the organs that are mainly affected. Lungs and intrathoracic lymph nodes are the sites that are most often involved, but virtually no organ is spared from this disease. Histopathology is distinctive but not pathognomonic, since the findings can be found also in other granulomatous disorders. The knowledge of these findings is important because it could be helpful to differentiate sarcoidosis from the other granulomatous-related diseases. This review aims at illustrating the main clinical and histopathological findings that could help clinicians in their routine clinical practice.

The Multi-modality Cardiac Imaging Approach to Cardiac Sarcoidosis.

BACKGROUND: Sarcoidosis is a multisystem granulomatous disease with a neglected but high prevalence of life-threatening cardiac involvement. DISCUSSION: The clinical presentation of Cardiac Sarcoidosis (CS) depends upon the location and extent of the granulomatous inflammation, with left ventricular free wall the most common location followed by interventricular septum. The lack of a diagnostic gold standard and the unpredictable risk of sudden cardiac death pose serious challenges for the validation of accurate and effective screening test and the management of the disease. In the last few years advanced cardiac imaging modalities such as Cardiac Magnetic Resonance (CMR) and Positron Emission Tomography (PET) have significantly improved our knowledge and understanding of CS, and have also contributed in risk stratification, assessment of inflammatory activity and therapeutic monitoring of the disease. CONCLUSION: In this review, we will discuss the state of the art in the diagnosis of CS focusing on the role and importance of multi-modality cardiac imaging.

Prevalence and Clinical Relevance of Extracardiac Findings in Cardiovascular Magnetic Resonance Imaging.

PURPOSE: To assess the prevalence of extracardiac findings (ECF) during cardiovascular magnetic resonance (CMR) examinations and their downstream effect on clinical management. MATERIALS AND METHODS: We retrospectively identified 500 consecutive patients. Trans-axial balanced steady-state free precession nongated images acquired from the upper thorax to the upper abdomen were evaluated independently by 2 radiologists. ECF were classified as nonsignificant (benign, with no need for further investigation), significant (mandatory to be reported/monitored), and major (clinically remarkable pathology, mandatory to be reported/investigated/treated). Fifteen-month clinical follow-up information was collected through hospital records. RESULTS: Of 500 patients, 108 (21.6%) showed a total of 153 ECF: 59 (11.8% of the entire study population; 38.5% of all ECF) nonsignificant, 76 (15.2%; 49.7%) significant, and 18 (3.6%; 11.8%) major ECF. The most frequent ECF were pleural effusion, hepatic cyst, renal cyst, and ascending aorta dilatation. Of 94 significant and major ECF, 46 were previously unknown and more common in older patients. Newly diagnosed major ECF (n=11, 2.2% of the entire study population, and 7.2% of all ECF)-including 5 tumors (1% of study population)-were confirmed by downstream evaluations and required specific treatment. Patients with major ECF were significantly older than patients without with major ECF. Newly diagnosed clinically significant and major ECF prompted downstream diagnostic tests in 44% and 100% of cases, respectively. CONCLUSIONS: The detection of significant and major ECF is common during CMR reporting. The knowledge and the correct identification of most frequent ECF enable earlier diagnoses and faster treatment initiation of unknown extracardiac pathologies in patients referred to CMR imaging.

Aortic valve bypass surgery in severe aortic valve stenosis: Insights from cardiac and brain magnetic resonance imaging.

OBJECTIVE: To investigate and describe the distribution of aortic and cerebral blood flow (CBF) in patients with severe valvular aortic stenosis (AS) before and after aortic valve bypass (AVB) surgery. METHODS: We enrolled 10 consecutive patients who underwent AVB surgery for severe AS. Cardiovascular magnetic resonance imaging (CMR) and brain magnetic resonance imaging were performed as baseline before surgery and twice after surgery. Quantitative flow measurements were obtained using 1.5-T magnetic resonance imaging (MRI) scanner phase-contrast images of the ascending aorta, descending thoracic aorta (3 cm proximally and distally from the conduit-to-aorta anastomosis), and ventricular outflow portion of the conduit. The evaluation of CBF was performed using 3.0-T MRI scanner arterial spin labeling (ASL) through sequences acquired at the gray matter, dorsal default-mode network, and sensorimotor levels. RESULTS: Conduit flow, expressed as the percentage of total antegrade flow through the conduit, was 63.5 ± 8% and 67.8 ± 7% on early and mid-term postoperative CMR, respectively (P < .05). Retrograde perfusion from the level of the conduit insertion in the descending thoracic aorta toward the aortic arch accounted for 6.9% of total cardiac output and 11% of total conduit flow. We did not observe any significant reduction in left ventricular stroke volume at postoperative evaluation compared with preoperative evaluation (P = .435). No differences were observed between preoperative and postoperative CBF at the gray matter, dorsal default-mode network, and sensorimotor levels (P = .394). CONCLUSIONS: After AVB surgery in patients with severe AS, cardiac output is split between the native left ventricular outflow tract and the apico-aortic bypass, with two-thirds of the total antegrade flow passing through the latter and one-third passing through the former. In our experience, CBF assessment confirms that the flow redistribution does not jeopardize cerebral blood supply.

Endovascular Treatment of Visceral Artery Pseudoaneurysms in Patients with Chronic Pancreatitis: Our Single-Center Experience.

BACKGROUND: To analyze our experience in endovascular treatment as first-line approach for severe and acute hemorrhage in patients with chronic pancreatitis. METHODS: From 2010 to 2016 at our institution, 12 males (mean age 66 years) with bleeding pseudoaneurysms (PSAs) underwent urgent visceral angiography and endovascular treatment. All patients had chronic pancreatitis. True visceral artery aneurysms and PSAs arising after surgery were excluded from the study. RESULTS: Pancreatitis was caused by alcohol abuse in 9 (9/12, 75%) patients and biliary lithiasis in 3 (3/12, 25%). Involved arteries were gastroduodenal (5/12, 50%), splenic (2/12, 16%), common hepatic (2/12, 16%), middle colic (1/12, 9%), and celiac trunk (1/12, 9%). All patients underwent computed tomography angiography (CTA) scan and visceral angiography followed by endovascular treatment using different devices. Technical success rate was 100%. Bleeding was stopped in all patients, and no one required reembolization. No major complications occurred. There were 2 complications associated with the endovascular procedure: in one case, a coil migration and in another case, in-stent restenosis at 6 months. Follow-up included CTA performed during hospitalization and at 6 months after the procedure. CONCLUSIONS: Our experience confirms the role of CTA and visceral angiography as diagnostic and therapeutic tool, respectively.

The lung in systemic vasculitis: radiological patterns and differential diagnosis.

The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis. Pulmonary involvement is less frequent in immune-complex-mediated small-vessel vasculitides, such as Behçet's disease and Goodpasture's syndrome. Pulmonary involvement associated to large-vessel (gigantocellular arteritis and Takayasu's disease) or medium-vessel (nodose polyarteritis and Kawasaki's disease) vasculitides is extremely rare. The present review describes the main clinical and radiological features of pulmonary vasculitides with major purpose to correlate HRCT findings (solitary or multiple nodules, cavitary lesions, micronodules with centrilobular or peribronchial distribution, airspace consolidations, "crazy paving", tracheobronchial involvement, interstitial disease) with pathological results paying particular attention to the description of acute life-threatening manifestations. A thorough medical history, careful clinical examination and the knowledge of radiological patterns are mandatory for a correct and early diagnosis.

Coronary artery calcium score on low-dose computed tomography for lung cancer screening.

AIM: To evaluate the feasibility of coronary artery calcium score (CACS) on low-dose non-gated chest CT (ngCCT). METHODS: Sixty consecutive individuals (30 males; 73 ± 7 years) scheduled for risk stratification by means of unenhanced ECG-triggered cardiac computed tomography (gCCT) underwent additional unenhanced ngCCT. All CT scans were performed on a 64-slice CT scanner (Somatom Sensation 64 Cardiac, Siemens, Germany). CACS was calculated using conventional methods/scores (Volume, Mass, Agatston) as previously described in literature. The CACS value obtained were compared. The Mayo Clinic classification was used to stratify cardiovascular risk based on Agatston CACS. Differences and correlations between the two methods were compared. A P-value < 0.05 was considered significant. RESULTS: Mean CACS values were significantly higher for gCCT as compared to ngCCT (Volume: 418 ± 747 vs 332 ± 597; Mass: 89 ± 151 vs 78 ± 141; Agatston: 481 ± 854 vs 428 ± 776; P < 0.05). The correlation between the two values was always very high (Volume: r = 0.95; Mass: r = 0.97; Agatston: r = 0.98). Of the 6 patients with 0 Agatston score on gCCT, 2 (33%) showed an Agatston score > 0 in the ngCCT. Of the 3 patients with 1-10 Agatston score on gCCT, 1 (33%) showed an Agatston score of 0 in the ngCCT. Overall, 23 (38%) patients were reclassified in a different cardiovascular risk category, mostly (18/23; 78%) shifting to a lower risk in the ngCCT. The estimated radiation dose was significantly higher for gCCT (DLP 115.8 ± 50.7 vs 83.8 ± 16.3; Effective dose 1.6 ± 0.7 mSv vs 1.2 ± 0.2 mSv; P < 0.01). CONCLUSION: CACS assessment is feasible on ngCCT; the variability of CACS values and the associated re-stratification of patients in cardiovascular risk groups should be taken into account.

Extrapleural and cardiophrenic lymph nodes: prevalence, clinical significance and diagnostic value.

PURPOSE: The aim of this study was to evaluate the prevalence, clinical meaning and diagnostic value of extrapleural and cardiophrenic nodes occasionally observed on computed tomography (CT) scans of the chest. MATERIALS AND METHODS: We included 750 consecutive patients who underwent CT of the chest for different clinical purposes (340 nonneoplastic patients, 270 with extrathoracic neoplasms, 120 with intrathoracic neoplasms, 20 with pleural metastasis) and 91 patients with histologically proven malignant pleural mesothelioma (MPM). For each group of patients, we analysed the presence of extrapleural and cardiophrenic nodes, their number (single or multiple) and their size. RESULTS: The prevalence of cardiophrenic nodes between 6 and 10 mm and >10 mm was significantly higher in patients with MPM (28.6 and 26.4 %, respectively) than in all other categories of patients, except for patients with pleural metastasis (30 and 25 %, respectively). The prevalence of extrapleural nodes, independently from their size, was significantly higher in patients with MPM (68 %) compared with all other groups, including patients with pleural metastasis (5 %; p < 0.0001). CONCLUSIONS: Cardiophrenic nodes >5 mm and extrapleural nodes of any size have a significant diagnostic value in malignant pleural disease, either primary or secondary, whereas they are extremely rare in other neoplastic or nonneoplastic diseases.