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INTRODUCTION & IMPORTANCE: Pulmonary Artery Aneurysm is defined as the localized dilation of the pulmonary artery >1.5 times the upper normal limit or pulmonary artery measuring 4 cm. Pulmonary artery aneurysm is considered as a rare disorder having an incidence of 1 in 14,000 post-mortem examinations. CASE PRESENTATION: Presented below is a case of a 28 year old gentleman, who presented with exertional dyspnea and orthopnea and was diagnosed with having a pulmonary artery aneurysm of 76 cm × 56 cm × 53 cm arising from the main pulmonary artery upon Computed Tomography Scan which is a rare finding according to the available literature. CLINICAL DISCUSSION: Clinical manifestations of pulmonary artery aneurysm are varying and rarely occur. However the use of radiological imaging has aided in the diagnosis. No specific treatment guidelines have been mentioned yet in the literature however, medical management, surgical resection and endovascular therapy are one of the multiple options available. CONCLUSION: Pulmonary Artery Aneurysm presents with non-specific symptoms which makes the diagnosis very challenging for the physicians, in process, delaying the accurate management of the disease. However, pulmonary artery aneurysm must be considered as a differential diagnosis and appropriate management options, whether medical or surgical should be opted for keeping in mind the size and the complications of the disease.

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Background: Hughes-Stovin syndrome (HSS) is a rare vasculitis characterized by the association of thrombophlebitis with pulmonary artery aneurysms (PAAs). Because it is rarely reported, there are currently no established diagnostic criteria or standardized treatment guidelines for HSS. While conventional immunosuppressants are generally effective as first-line treatment, relapsing and refractory cases urge the need to investigate alternative therapies, such as TNF-alpha inhibitors. However, with only five cases published in the literature, knowledge of their efficacy in HSS is very limited. Case summary: A 28-year-old man, with no past medical history, presented with haemoptysis, chest pain, and dyspnoea on exertion. Physical examination found bilateral leg swelling, with no associated lesions. CT angiography showed multiple bilateral PAA, proximal pulmonary artery thrombosis (PAT), and deep venous thrombosis (DVT) in the superior mesenteric vein and spleno-mesaraic confluence. Echocardiography was performed, identifying right intracardiac thrombosis (ICT). Initial management included high-dose corticosteroids and monthly cyclophosphamide cycles, followed by maintenance treatment with oral azathioprine. Eighteen months later, the patient presented with haemoptysis revealing a relapse of ICT and two new PAA. Infliximab was initiated, allowing complete and sustained remission after one year of follow-up. Discussion: We report the challenging case of an HSS patient presenting with multiple PAA, proximal PAT, right ICT, and extended abdominal DVT. The positive response of our patient to infliximab, following a relapse under conventional immunosuppressants, supports the efficacy of TNF-alpha inhibitors as second-line treatment in relapsing/refractory HSS.

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Pulmonary artery aneurysm (PAA) is a rare pulmonary vascular disease with nonspecific symptoms and various etiologies. As the disease progresses, in addition to the dilation of the pulmonary arteries, it may be accompanied by remodeling of the cardiac structure and changes in the morphology of the aorta. Recognizing the cause of PAA is therefore a clinically challenging task. In this review article, we provide an overview of various causes of PAA with the support of corresponding imaging findings on computed tomography pulmonary angiography (CTPA) examination. Firstly, from the perspective of hemodynamics, a logical diagnosis is provided according to whether the main pulmonary artery (MPA) is dilated, and whether the PA is dilated locally or diffusely. Secondly, for the imaging examination of vascular wall lesions, due to the limitations of ultrasound examination and interventional procedures, the irreplaceability of dual-phase CTPA examination in disease assessment is especially emphasized. Finally, for highly suspected disorders, it is necessary to comprehensively check with the patient whether there is a family history or past medical history. For patients with PAA, especially those with Marfan syndrome (MFS) or arteritis, adequate preoperative imaging evaluation, regular postoperative radiographic follow-up, and concurrent treatment of the underlying disease (if necessary) are crucial, which are related to the prognosis and long-term quality of life of such patients. Despite the nonspecific features of PAA presentation, a thorough examination of the patient's clinical history and imaging characteristics will play an important role in diagnosing PAA and planning patient management strategies.

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Cardiac tamponade from ruptured intrathoracic organs can lead to sudden cardiac death. In rare circumstances, the pulmonary artery can be the source of hemopericardium. We describe a case of a 62-year-old woman with no significant past medical history, who presented with sudden unexpected death. A forensic autopsy revealed 500 ml of hemopericardium. Further dissection demonstrated a saccular aneurysm in the pulmonary artery trunk, along with the evidence of prior dissection, i.e., neointimal layer. Persistent ductus arteriosus (PDA) was also present. Pulmonary artery aneurysms (PAA) are rare and often associated with congenital heart disease (CHD). PDA is the most common CHD related to PAA. Secondary pulmonary hypertension makes the pulmonary artery vulnerable to medial degeneration and increases the risk of dissection and rupture. Careful inspection of the great vessels and congenital anomalies are essential in the forensic autopsies for sudden death investigation.

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Idiopathic pulmonary arterial hypertension is a serious condition that carries a poor prognosis. While exertional dyspnea is the most common symptom, angina like chest pain, most often due to right ventricle ischemia, may occur at advanced stages. We present a patient with pulmonary hypertension symptomatic for dyspnea and angina in whom computed coronary tomography angiography showed compression of the left main coronary artery by a large pulmonary artery aneurysm. Percutaneous coronary intervention and stenting was performed resulting in significant clinical improvement. This case emphasizes the role of different cardiovascular imaging modalities for the diagnosis of rare conditions.

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Behçet's disease is a chronic systemic inflammatory vasculitis of unknown etiology. It is characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. This disease affects many organs and systems, showing a wide range of clinical features. Although pulmonary artery involvement is not common in Behçet's disease, its presence carries a substantial risk of mortality. This report provides a detailed history of a 25-year-old male who was admitted with productive cough, hemoptysis, dyspnea on minimal exertion, fever, and chest pain. He had recurrent orchitis and epididymitis for 7 years, as well as oral and genital ulcers and severe headache. Clinical examination revealed decreased breath sounds at the right middle lung. Thoracic computed tomography angiography confirmed multiple pulmonary artery aneurysms bilaterally. The patient was diagnosed with Behçet's disease, and immunosuppression therapy was initiated. During follow-up, the patient did not report any complications. This case report underscores the significance for clinicians to consider Behçet's disease as a differential diagnosis in patients presenting with hemoptysis and a history of orchitis and epididymitis, given that Behçet's disease rarely causes pulmonary artery aneurysms.

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Giant septic pulmonary artery aneurysms (PAAs) are rare but important entities, with few cases having been reported worldwide. Early diagnosis and prompt treatment are crucial in the management of such cases. We report a 56-year-old female patient presenting with fatigue, nausea and vomiting who was first diagnosed with diabetic ketoacidosis (DKA) and developed life-threatening giant infectious PAA secondary to SARS-CoV-2 infection and Actinomyces odontolyticus sepsis. The patient did not develop any specific symptoms, and enhanced computed tomography (CT) revealed a massive PAA of 5.6 × 4.9 cm in size at the left pulmonary hilar with normal pulmonary artery (PA) pressures. After multidisciplinary discussion and after considering the critical condition accompanied by sepsis increased the risk of surgery, endovascular treatment was the first therapy of choice for the patient; nevertheless, the patient ultimately opted for hospice care. This case report aims to raise awareness of PAAs, which are rare but potentially fatal complications of infectious diseases such as COVID-19 pneumonia and Actinomyces odontolyticus sepsis.

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Pulmonary artery pseudoaneurysms (PAPs) are uncommon, yet they frequently result in hemoptysis and are associated with a poor prognosis. We report a case of an 87-year-old male patient. Initially, he was admitted to a previous hospital, and diagnosed with a lung abscess in the left lower lobe. On the second hospital day, he developed hemoptysis. A contrast-enhanced chest computed tomography (CT) identified an infectious pulmonary artery pseudoaneurysm. On the ninth hospital day, pulmonary artery coil embolization was successfully performed, significantly improving the patient's condition.

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Pulmonary Artery Aneur ysm (PAA), whether congenital or acquired, is a rare diagnostic find ing com pare d to aor tic aneur ysms. There have been fe w cases where PA As were documented as a complication of untreated Patent Ductus Ar teriosus (PDA) due to long-standing Pulmonary Arterial H ypertension (PAH). However, it is quite rare for a case of PAA to be reported with co-existing PDA without PAH. This report highlights a case of a five -year-old girl who was presented with palpitations, easy fatigability, fever, c yanos is, and vomiting. A Chest X-ray s howed mo derate cardiomega ly. A PDA of 6 mm was diagnosed on Transthoracic E chocardiog rap hy ( TTE ) and a large cavity con necte d with LPA raised suspicion of a possible LPA aneur ysm. A Chest CT scan confirm ed the diagnosis of a saccular aneurysm, originating from the distal part of the main Left Pulmonary Artery (LPA) just proximal to the point of bifurcation into lobar branches, measuring 7.5x6.5 cm. During surgery, the aneurysm was opened, emptied with suction and closed without resecting the aneur ysmal walls. The patient had an uneventful post-op course and is doing well during regular interval follow up visits.

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Introduction: Pulmonary artery aneurysm (PAA) is a rare abnormality of pulmonary vasculature. It can be idiopathic or secondary to various pathologies, frequently with multiple factors leading to its formation. We report the case of a man with concomitant sarcoidosis and PAA. Case description: A 75-year-old male with a diagnosis of pulmonary sarcoidosis was referred to the Cardiology department due to heart failure with reduced left ventricular ejection fraction (LVEF). The transthoracic echocardiogram revealed mildly reduced LVEF, aortic root and pulmonary artery (PA) dilatation, and no signs of pulmonary hypertension (PH). Cardiac magnetic resonance imaging was performed, revealing mild left ventricular dilation, LVEF of 40%, main PA dilation (43 mm) and a pattern of late gadolinium enhancement suggestive of cardiac sarcoidosis. At follow-up, a thoracic computed tomography (CT) angiography scan revealed ascending aorta ectasia and giant main PA aneurysm (60 mm). A right heart catheterisation was performed, and a mean PA pressure of 34 mmHg was obtained. Given the clinical context, the patient was considered to have PH due to lung disease and left heart disease, and PAA was possible due to vascular granulomatous involvement by sarcoidosis. Conclusion: PAA is a rare finding and mostly occurs in the setting of PH. Sarcoidosis is a granulomatous disease that mostly affects the lungs, but the sarcoid involvement of great vessels has been described. In this clinical case, the probable cause for the PA fragility leading to aneurysm formation remains sarcoid vascular infiltration, regarding the discrepancy between the PA dimensions and mildly elevated PA pressure. LEARNING POINTS: Pulmonary artery aneurysm is a rare abnormality of pulmonary vasculature that can be idiopathic or a consequence of pulmonary hypertension, congenital heart disease, infection, vasculitis or collagenopathies.Due to the low incidence of this disease, there are no guidelines for its diagnosis, management or follow-up, and treatment is based on the underlying aetiology, aneurysm dimensions and occurrence of symptoms.Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas that mostly involve the lungs, but can also affect skin, eyes, and lymph nodes. Sarcoid involvement of great vessels has been rarely described.

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A 30-year-old woman with ankylosing spondylitis was referred to our clinic with abnormal fetal echocardiography findings, including ascending aortic dilatation, giant main pulmonary artery aneurysm, and aortic and pulmonary valve stenosis at 22 weeks of gestation. The full-term male neonate was born by cesarean section and was transferred to the cardiac intensive care unit soon after delivery for respiratory distress with low percutaneous oxygen saturation. Based on cardiovascular and genetic analysis findings, the patient was diagnosed with Marfan syndrome. Surgery was performed; however, the patient died due to cardiac arrest. In conclusion, main pulmonary artery dilatation and aneurysms are uncommon in Marfan syndrome; therefore, presentation with these findings during the fetal life, as in the present case, is likely a sign of severe Marfan syndrome-related cardiac involvement.

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Nontuberculous mycobacterial (NTM) infection sometimes leads to the development of pulmonary artery aneurysm (PAA), a rare but life-threatening complication. We herein report a 64-year-old woman with a history of NTM infection who presented with severe hemoptysis. Computed tomography revealed a ruptured PAA, which was treated successfully with pulmonary artery embolization. Subsequent right total pneumonectomy was performed to control infection. This case emphasizes the need to consider PAA in patients with NTM infection who present with hemoptysis. Early detection and appropriate management are critical for preventing this fatal complication.

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INTRODUCTION: Behcet's disease (BD) is a chronic inflammatory disorder with arterial vasculitis. Although, pulmonary artery aneurysm (PAA) is accepted as the prototypic arterial disorder, an increasing presence of pulmonary artery thrombosis (PAT) with or without aneurysms was also reported in recent studies. In this study, we aimed to describe computed tomography pulmonary angiography (CTPA) findings of pulmonary involvement and its correlation with symptoms and acute phase response in BD. METHOD: In this retrospective study, 153 CTPA of BD patients were assessed by two radiologists. Clinical and laboratory data were collected from the patient files. Pulmonary artery involvement (PAI) was defined as thrombus or aneurysm in CT angiography. RESULTS: Most of (85.6 %) our patients were male and median age was 33.7 ± 10 years during angiographic assessments. Sixty-two (40.5 %) angiographies presented a thrombus: 14 subsegmental, 29 segmental, 13 lobar and 6 main branches. Among these, 82.3 % (n = 51) had bilateral involvement. Isolated PAT was present in 58 (93.5 %) angiographies with only 4 (2.6 %) angiographies displaying an aneurysm together with a thrombus. Pulmonary infarction was detected in 9 angiographies. Forty-four (29.3 %) patients, almost all of them under immunosuppressive treatments for other indications, were screened for asymptomatic pulmonary involvement (without any symptoms or increased acute-phase response (APR)), and one fourth of these were diagnosed as having a segmental or subsegmental PAT. CONCLUSION: Our results show that isolated pulmonary thrombosis is the main form of PAI, and isolated pulmonary aneurysm formation is rare in our BD cases. In the presence of pulmonary symptoms with or without increased APRs, involvement of segmental or more proximal parts of pulmonary arteries is most commonly detected. We also observed that PAI may be seen in about one fourth of especially male BD patients without symptoms or increased APR. Our results suggest that BD patients with pulmonary symptoms should be screened by CTPA for PAI, however, further research is needed to clarify the role of routine CTPA screening in asymptomatic BD patients.

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INTRODUCTION: Pulmonary artery thrombosis presents a rare and formidable challenge, especially in individuals with untreated complex congenital heart diseases and concurrent valvular abnormalities. PRESENTATION OF CASE: 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. DISCUSSION: 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. CONCLUSION: 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.

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Behçet's disease is a chronic inflammatory condition that predominantly affects the body's blood vessels, exhibiting various clinical manifestations and complications. The exact cause remains unclear, but genetic predisposition, immune responses, and vascular activation are believed to contribute to its development. This disease is more prevalent in certain geographic regions and primarily affects young adults, particularly males. Pulmonary aneurysm, a complication of Behçet's disease, is the leading cause of mortality in Behcet disease. In this review, we summarize the complications of Behcet disease with a focus on pulmonary artery aneurysms. We discussed the medical, endovascular, and surgical management of pulmonary aneurysms in Behcet disease and the indications and outcomes of the different treatment options. Corticosteroids and cyclophosphamide remain the preferred first-line therapy. However, clinical improvement with infliximab or adalimumab, tumor necrosis factor-alpha (TNFα) blocking agents, have been reported after treatment failure with recommended first-line agents. In patients who fail medical therapy or those with life-threatening hemoptysis, endovascular or surgical intervention is the next option. Endovascular interventions include pulmonary artery embolization with coils or acrylic glue and using plugs, occluders, or stents. Endovascular interventions usually have fewer adverse effects than surgery. Although the risk of surgical procedures is high in pulmonary artery aneurysms, it could be a life-saving procedure in patients with life-threatening hemoptysis. Surgical options, including pulmonary artery ligation, aneurysmorrhaphy, segmentectomy, lobectomy, or pneumonectomy are available. However, the results of surgical therapy for Behçet aneurysms are often disappointing.

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Aneurysms are characterized by focal dilation of the blood vessel wall due to weakening. The involvement of two layers of the vessel wall is classified as a pseudoaneurysm while the involvement of all three layers is called a true aneurysm. Involvement of neoplastic lesions is rare, but the few reported cases have been associated with pulmonary artery pseudoaneurysms as opposed to true pulmonary artery aneurysms (PAAs). Our case of a true left PAA of a patient with metastatic sarcoma of the lung shows an association that has previously not been reported to the best of our knowledge.

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Key Clinical Message: In suspected cases of systemic vasculitis, imaging studies should include the pulmonary artery. This is a rare case of Takayasu arteritis with a large pulmonary aneurysm. Medical management is the first line and vascular intervention if fails prior. Abstract: Takayasu arteritis (TA) should be suspected in young women presented with hypertension, carotidynia, and claudications. Pulmonary artery involvement is frequent, occurring in 20%-50% of patients with TA. However, this case highlights the rare presentation of TA with a large pulmonary aneurysm and minimal aortic involvement. Medical management including immunosuppressive agents and biological therapies remains an important role, with vascular intervention remains as an option if medical therapy failed.

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BACKGROUND: Pulmonary artery (PA) aneurysms are usually diagnosed radiographically and present as small or large lesions resembling inflammation or a neoplasm on chest radiography. It has rarely been reported as an endobronchial mass. CASE SUMMARY: We report the case of a 64-year-old man who presented with recurrent hemoptysis. Bronchoscopy revealed a tumorous protrusion blocking the right middle lobe bronchus, which was confirmed to be a PA aneurysm using endobronchial ultrasound bronchoscopy and computed tomography angiography. CONCLUSION: Although endobronchial PA aneurysms are rare, bronchoscopists need to add this lesion to the list of endobronchial masses for which a biopsy is to be assiduously avoided.

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A peripheral pulmonary artery aneurysm (PAA) is a dilatation involving all 3-vessel wall layers (the intima, media, and adventitia) of a distal pulmonary artery. It represents a rare but potentially life-threatening condition. There are only some reviews of transcatheter embolization of unruptured idiopathic peripheral PAAs. Association with cardiac diseases, infections, vascular anomalies, pulmonary hypertension, and vasculitis has been noted. We report a case of a 38-year-old woman, with a history of third-degree atrioventricular (AV) block, treated with pacemaker placement, who presented a PAA in the left pulmonary lobe. Transcatheter coil embolization was performed, using a triple coaxial catheter system (a 6F outer, a 5F intermediate, and a 2.4F inner catheter) to prevent rupture and the aneurysm was successfully embolized. Although there is no consensus on the treatment for unruptured idiopathic peripheral PAAs, transcatheter embolization may be a promising treatment option.

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First-line treatment of pulmonary artery aneurysms/pseudoaneurysms (PAA/PAPA) is percutaneous or endovascular embolization. The present case of a Rasmussen aneurysm, a PAPA caused by Tuberculosis (TB), was successfully treated with ethylene-vinyl alcohol (EVOH), a radiopaque liquid embolic agent with favorable characteristics. A 35-year-old man presented as a new patient with hemoptysis, and CT imaging revealed multiple cavitary lesions and a 2.1 cm aneurysm in the upper right lobe. Endovascular treatment was delivered and a complete lack of filling of the lesion was noted on post-treatment angiography. The patient's history includes risk factors and past TB infection. Despite the suspicious imaging, diagnostic tests were negative for active TB in this patient. He was then found to have MRSA bacteremia and a mediastinal lymph node positive for M. avium. The etiology of this aneurysm is suspicious for the superinfection of a chronic tuberculous cavity with M. avium, MRSA, or both.

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