Behçet\'s syndrome (BS) is a rare chronic multisystemic inflammatory disorder of unknown etiopathogenesis. BS is classified as a vasculitis of variable vessel size, which can manifest in both arterial and venous blood vessels. BS commonly presents with mucocutaneous and ocular manifestations. Superficial and deep vein thrombosis is present in 50% of patients, with atypical venous thrombosis affecting the inferior vena cava, superior vena cava, hepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses, and right atrium and ventricle. Arterial manifestations include in situ thrombosis, pulmonary artery aneurysms, aneurysms of the abdominal aorta, and aneurysms of visceral and peripheral arteries. This article reports a new case of BS in a 28-year-old female patient who presented with severe dyspnea and hemoptysis. Echocardiography and cardiovascular magnetic resonance imaging led to the diagnosis of endomyocardial fibrosis and a large right ventricular thrombus with pulmonary embolism. Computed tomography angiography revealed multiple pulmonary aneurysms and emboli. Rare findings such as endomyocardial fibrosis and Budd-Chiari syndrome were noted. This case highlights the role of medical imaging modalities in diagnosing rare syndromes such as BS, as demonstrated in the current case.

OBJECTIVE: Pulmonary artery involvement is a severe complication of Behcet\'s disease (BD). Although venous thrombosis is common in BD, pulmonary embolism is considered to be rare because the inflammatory nature makes the thrombi strongly adherent to the venous walls. This study aimed to define the radiological characteristics of pulmonary artery thrombosis (PAT) on computed tomography (CT) imaging in BD patients.
METHODS: We retrospectively evaluated 165 BD patients with vascular involvement. Among the patients with venous involvement (n = 146), we identified 65 patients who had undergone thorax CT imaging previously. Fourteen patients who were diagnosed with PAT were included in the study. Expert radiologists re-evaluated the patients\' initial and control thorax CT scans, classified the PAT as acute or chronic based on their radiological features.
RESULTS: The patients\' median age was 35 (min-max: 15-60) years at the time of the initial CT scan, and nine were male. Twelve (85.7%) patients were symptomatic at the time of CT evaluation. Upon re-evaluating the thorax CTs, acute PAT was diagnosed in six (42.8%); chronic PAT was detected in eight (57.1%) patients. Two patients with chronic PAT also had acute PAT. Pulmonary artery aneurysms were present in three (21.4%) patients, and intracardiac thrombus was found in three (21.4%) patients.
CONCLUSIONS: A significant number of BD patients with venous involvement had radiological findings consistent with acute PAT potentially due to pulmonary emboli in this study. The clinical importance of these lesions has to be defined with future studies.

UNASSIGNED: Venous thromboembolism (VTE) with the prevalence of pulmonary microcirculatory thrombosis is considered a common complication of novel coronavirus disease (COVID-19) that develops despite anticoagulation.
UNASSIGNED: The clinical course of the disease and the autopsy findings of seven deceased patients with verified COVID-19 were analyzed. The chest computed tomography (CT) scan was routinely performed while CT pulmonary angiography and a duplex ultrasound scan (DUS) of the lower limbs were used in cases of suspected VTE. The VTE prophylaxis was administered to all patients with intermediate or therapeutic doses of low-molecular-weight heparin. The histological examination of the lung tissue and other organs was performed with particular attention paid to the pulmonary vasculature.
UNASSIGNED: Venous thromboembolism, including deep vein thrombosis in one patient and pulmonary artery thrombosis in two patients, was confirmed by imaging tests despite anticoagulation. Systemic thrombolysis was performed in two patients with putative and confirmed pulmonary embolism. An autopsy revealed the signs of acute respiratory distress syndrome in all seven patients. Abnormalities of lung vessels were found in all cases and were represented by dystrophy and necrosis in the endothelium and muscle fibers, and by infiltration by plasmatic cells, neutrophils, and lymphocytes. Multiple clots of variable maturity were observed. All those changes developed despite anticoagulation and were preserved after systemic thrombolysis.
UNASSIGNED: Inflammatory and prothrombotic changes in the arterial wall in parallel with the lack of lung perfusion may cause diffuse arterial thrombosis in the lungs. This background may be responsible for the low response to systemic anticoagulation and thrombolysis in severe forms of COVID-19.

We present a case of a nine-year-old female patient who presented with recurrent syncope and was ultimately diagnosed with pulmonary hypertension (PH) secondary to pulmonary artery thrombi in the context of anti-phospholipid syndrome (APS). Extensive investigations including imaging studies revealed PH. Thromboembolic workup confirmed multiple pulmonary artery thrombi, and anti-phospholipid antibody testing confirmed APS. The patient received anticoagulation therapy tailored to APS management. Follow-up assessments demonstrated significant improvement in PH leading to cessation of syncope episodes. In this case, we underscore the importance of considering rare causes of syncope in the pediatric age group, particularly autoimmune disorders. Timely recognition and appropriate management are crucial for favorable outcomes in such cases. This report contributes to understanding the diverse clinical presentations of APS and emphasizes the need for a comprehensive diagnostic approach in patients with unexplained syncope.

BACKGROUND: Pulmonary artery thrombosis presents a rare and formidable challenge, especially in individuals with untreated complex congenital heart diseases and concurrent valvular abnormalities.
METHODS: This case details a distinctive instance of pulmonary artery thrombosis in a patient with untreated congenital atrial septal defect, severe pulmonary hypertension, pulmonary aneurysm, valvular heart disease, and triple-positive antiphospholipid antibodies. Collaborative decision-making by a heart team guided the diagnostic and therapeutic processes, resulting in surgical interventions encompassing pulmonary thrombectomy, mitral and tricuspid regurgitation repair, and atrial septal defect closure.
CONCLUSIONS: The case underscores the nuanced management of pulmonary artery thrombosis within the realm of congenital heart disease, highlighting the imperative role of a multidisciplinary approach in achieving accurate diagnoses and timely interventions. The multifaceted treatment strategy implemented further accentuates the need for continual monitoring and personalized interventions in intricate clinical scenarios.
CONCLUSIONS: In conclusion, this case emphasizes the complexity of addressing pulmonary artery thrombosis in the context of congenital heart disease. The collaborative efforts of a multidisciplinary team proved pivotal in navigating diagnostic uncertainties and formulating a tailored treatment plan. Ongoing monitoring and personalized strategies remain crucial for optimizing outcomes in such intricate cases.

COVID-19-related thrombosis affects the venous and arterial systems. Data from 156 autopsies of COVID-19 patients were retrospectively analyzed to investigate the pattern of thrombotic complications and factors associated with pulmonary artery thrombosis and thromboembolism. Thrombotic complications were observed in a significant proportion (n = 68, 44%), with pulmonary artery thrombosis the most frequently identified thrombotic event (42, 27%). Multivariate analysis revealed that the length of hospital stay (OR 1.1, p = 0.004), neutrophil infiltration in the alveolar spaces (OR 3.6, p = 0.002), and the absence of hyaline membranes (OR 0.1, p = 0.01) were associated with thrombotic complications. Neutrophil infiltration in the alveolar spaces (OR 8, p < 0.001) and the absence of hyaline membranes (OR 0.1, p = 0.003) were also independent predictors of pulmonary artery thrombosis. The association of pulmonary artery thrombosis with an absence of hyaline membranes suggests it occurs later in the course of COVID-19 infection. As neutrophil infiltration in the alveolar spaces may indicate bacterial infection, our studies suggest the consideration of bacterial infections in these critically ill patients.

Behcet\'s disease (BD) is a chronic systemic inflammatory vasculitis with a relapsing and remitting course. The disease predominantly affects males between the ages of 20 and 40 years. The disease is more prevalent in Middle Eastern and Asian countries but is less common in North American countries. BD typically presents as recurrent oro-genital ulcers and ocular inflammation. Pulmonary vasculitis with pulmonary arterial involvement is a unique manifestation, with most pulmonary manifestations occurring later in the disease course. Here, we report a case with pulmonary arterial aneurysms and variable arterial-venous thrombosis in a young African American Male diagnosed with BD after he presented with pulmonary manifestations. This report emphasizes that a high index of suspicion is needed to diagnose a rare condition with such variable manifestations as Behcet\'s disease and that early detection and immunosuppression therapy can confer improved prognosis.

Spontaneous pulmonary artery thrombosis in neonates is rare and can be life-threatening. Clinical presentation may mimic pulmonary hypertension or CHD. Further, not all children present with identifiable risk factors. We report the case of two infants with pulmonary artery thromboses who underwent rapid diagnosis and therapy, one with percutaneous intervention and the other with anticoagulation. We also conducted a literature review to highlight the importance of early identification and referral to a centre capable of performing appropriate medical and interventional therapies.

Hughes-Stovin syndrome (HSS) is a systemic inflammatory condition of unknown origin that is considered to be part of the Behçet\'s syndrome (BS) spectrum. Recurrent venous thrombosis and superficial thrombophlebitis in combination with bilateral pulmonary artery aneurysms (PAA) represent the hallmark of HSS. The diagnostic evaluation includes computed tomography pulmonary angiography to detect signs of pulmonary vasculitis. The management of HSS is based on the European Alliance of Associations for Rheumatology (EULAR) recommendations for BS and mainly comprises immunosuppressive therapy with glucocorticoids and cyclophosphamide. In addition to drug therapy, PAA should be evaluated for interventional treatment. Spontaneous PAA rupture due to fragile vessel architecture can occur even in cases of remission and/or PAA regression.
UNASSIGNED: Das Hughes-Stovin-Syndrom (HSS) ist eine entzündliche Systemerkrankung unklarer Genese, die inzwischen dem Spektrum des Behçet-Syndroms (BS) zugeordnet wird. Wegweisende Befunde sind rezidivierende Thrombosen des venösen Systems und oberflächliche Thrombophlebitiden in Kombination mit beidseitigen Pulmonalarterienaneurysmen (PAA). Die Pulmonalisangiographie mittels Computertomographie ist von entscheidender diagnostischer Bedeutung, um die (entzündliche) Beteiligung der Pulmonalarterien darzustellen. Die Therapie des HSS orientiert sich an den Empfehlungen der European Alliance of Associations for Rheumatology (EULAR) für das BS und sieht primär eine Immunsuppression mit Cyclophosphamid und Glukokortikoiden vor. Neben einer medikamentösen Therapie sollte eine interventionelle Versorgung der PAA evaluiert werden. Eine spontane PAA-Ruptur muss auch bei Remission der Erkrankung und/oder deutlicher Regredienz des PAA-Durchmessers aufgrund einer fragilen Gefäßarchitektur bedacht werden.

COVID-19 infection caused by the new coronavirus called severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is an infection with symptoms and results ranging from mild flu-like symptoms to severe respiratory failure leading to death. The risk of thrombosis increases due to hypercoagulation in COVID-19 infection. All causes (endothelial injury, stasis, and hypercoagulopathy) known as Virchow\'s triad contribute to thrombosis in COVID-19 infection. However, the pathogenesis of hypercoagulability in COVID-19 is still unknown. In this article, we discuss the unique multiple thrombosis events following recovery from COVID-19 infection and our treatment strategy for pulmonary thrombosis. The patient had symptoms of dry cough, fever, and myalgia two months ago. His polymerase chain reaction (PCR) test for COVID-19 was positive, but there was no need for hospitalization. His symptoms resolved within seven days. But, thrombosis of the superior mesenteric artery (SMA) occurred one month after the COVID-19 infection, and bowel resection was performed. He was admitted to our hospital with dyspnea, chest pain, palpitations, and hoarseness. Further evaluation showed tachycardia, hypotension, tachypnea, and anxiety. Peripheral oxygen saturation (SpO2) was 86% at room air. He had hemodynamic instability, right ventricular (RV) dysfunction, and D-Dimer elevation. Pulmonary Embolism Severity Index (PESI) was calculated as 149. The patient was in the high-risk group. Our Pulmonary Embolism Response Team (PERT) decided to apply catheter-directed thrombolysis (CDT) for the treatment of pulmonary thrombosis. The EkoSonic™ Endovascular System (EKOS) (Boston Scientific Corporation, Marlborough, USA) was used for the treatment.