Pulmonary Aneurysm Research Articles

A Rare Case of Pulmonary Artery Trunk Aneurysm.

Pulmonary artery trunk aneurysm (PATA) is a rare and complex vascular anomaly characterized by the abnormal dilation of the initial portion of the pulmonary artery, posing significant diagnostic and therapeutic challenges. This clinical case report aims to describe the follow-up of a patient with PATA, emphasizing the role of imaging in diagnosis and monitoring, as well as discussing potential associations with other conditions. The study is designed as a clinical case report, detailing the longitudinal follow-up of a single patient with PATA. The subject of this study is a 48-year-old female patient with a history of idiopathic hypertension who developed a PATA. Data Collection and/or Analysis: Since 2010, the patient underwent various imaging exams, including echocardiography, computed tomography, and catheter angiography, to detect and evaluate the aneurysm at different stages. The imaging results indicated a progression of the aneurysm over time, underscoring the importance of imaging in the early identification and monitoring of PATA. The report also explores the possible association of PATA with conditions such as pulmonary hypertension, Behçet's disease, and Hughes-Stovin syndrome, highlighting the diagnostic complexity. Imaging diagnosis is crucial for the detection, characterization, and monitoring of PATA, providing essential information for selecting appropriate treatment options and achieving a satisfactory prognosis. An individualized treatment approach, considering both medical and surgical options, is necessary based on the clinical characteristics of each patient.

Multiple pulmonary aneurysms and infective endocarditis as initial presentation of persistent ductus arteriosus

Multiple pulmonary aneurysms and infective endocarditis as initial presentation of persistent ductus arteriosus

Interventional occlusion treatment of pulmonary artery-aortic aneurysm residual cavity fistula after aortic repair.

Interventional occlusion treatment of pulmonary artery-aortic aneurysm residual cavity fistula after aortic repair.

Massive pericardial hemorrhage due to incidentally rupture of a saccular pulmonary aneurysm

Massive pericardial hemorrhage due to incidentally rupture of a saccular pulmonary aneurysm

Pulmonary aneurysms associated with thrombosis in Behçet’s disease: a case report

Pulmonary aneurysms associated with thrombosis in Behçet’s disease: a case report

Multiple pulmonary aneurysms and infective endocarditis as initial presentation of persistent ductus arteriosus.

Multiple pulmonary aneurysms and infective endocarditis as initial presentation of persistent ductus arteriosus.

Ruptured popliteal artery aneurysm: An initial presentation of Behçet’s disease

Behçet’s Disease (BD) is an uncommon systemic vasculitic disorder, that can affect multiple organ systems. Arterial and venous aneurysmal and occlusive manifestations can occur. We report a case of a 25-year-old male presenting with a 4-week history of left leg pain and swelling, with acute deterioration due to ruptured popliteal aneurysm, confirmed on imaging. He underwent emergency popliteal bypass with ipsilateral reversed great saphenous vein. Subsequent workup revealed pulmonary aneurysms and features suggestive of BD. He received aggressive immunosuppressive therapy to good effect. The management of the patient’s popliteal aneurysm, diagnostic procedure and subsequent management are discussed.

A Rare Case of Angio-Behcet Revealed by Hemoptysis of Great Abundance

Behçet's disease (BD) is a chronic, relapsing vasculitis of unknown etiology that is characterized by mucocutaneous, ocular, articular, vascular, gastrointestinal, and central nervous system.It mainly affects young subjects, generally 20 to 30 years old with a predominance of men. Thoracic involvement of Behcet's disease is unusual but serious. It is related to the well known vascular tropism of the disease. It may involve the superior vena cava, pulmonary arteries, aorta and subclavian vessels. These vascular attacks can be arterial or venous thrombosis, aneurysms or pseudo-aneurysms .In Behçet's disease, pulmonary artery aneurysms are considered exceptional. these aneurysms manifest themselves by recurrent hemoptysis as is the case with our patient who consulted in pneumolgy department 20 august 1953 at the university hospital Ibn Rochd Casablanca, for a moderate to great abundance hemoptysis revealing an angio-behcet. This is a negative element in patients with BD. it is a diagnostic and therapeutic emergency.The treatment of BD is not well codified but it depends on the severity of the systemic manifestations. Regarding vascular involvement, surgical treatment of an aneurysm with all its risks in patients with Behçet's disease frequently results in aneurysm recurrence. The arterial aneurysms have a poor prognosis, especially pulmonary aneurysms.

Angiographic findings of pulmonary arterial involvement in Behcet's Disease: Do they correlate with symptoms and acute phase response?

IntroductionBehcet'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. MethodIn 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. ResultsMost 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. ConclusionOur 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.

Pulmonary Artery Aneurysm in Behcet Disease: Medical, Endovascular or Surgical Intervention.

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.

A Rare but Fatal Behçet Variant: The Hughes-Stovin Syndrome-Successful Case Report and New Evidence from Literature Review.

Hughes-Stovin syndrome (HSS) is a rare potentially fatal vasculitis supposedly belonging to the spectrum of Behçet disease without ocular involvement. HSS tends to play by a temporal pattern, starting with thrombosis and followed by formation of pulmonary aneurysms. Since its mortality can reach 25% of cases, early recognition and appropriate therapy represent the major clinical challenges. We describe a rare case of HSS successfully treated via multidisciplinary management by an endovascular approach and immunosuppressive therapy.

Pulmonary Artery Aneurysm Dilemma in pregnancy: a Case Report

Pulmonary Artery Aneurysm (PAA), defined as greater than 40 mm dilation of the main pulmonary artery wall, in female and above 43 mm in males is a rare and fatal defect. Since there is a high risk for rupture, especially in cases of symptomatic or severe dilatation, surgical intervention is suggested. There is no recommendation about therapeutic methods based on the diameter of the pulmonary aneurysm in pregnancy in the guidelines. In this rare and unique report, we described a 26-year-old pregnant woman with previous history of biologic pulmonary valve replacement referred to the joint clinic of heart disease and pregnancy at 15 weeks of pregnancy because transthoracic echocardiography showed an aneurysm of the main pulmonary artery (55mm). Due to pulmonary artery diameter and risk of dissection, we informed her about the risks and recommended therapeutic abortion, but she refused and, fortunately no complication occurred during close observation in pregnancy and few months later. There is no specific recommendation about therapeutic methods based on the diameter of the pulmonary aneurysm in pregnancy in the guidelines, but referring to the aortic aneurysm guidelines recommendation, pregnancy termination when PA diameter > 5.5 cm because of the higher risk of dissection. Other factors should be considered to determine the risk of dissection in pregnancy include; categorized PA aneurysms to high or low intravascular PA pressure, PA diameter growth rate, and causative mechanisms. Thus, if pregnancy occurs, decisions about each patient will vary depending on risk factors

Targeting Vascular Smooth Muscle Cell Senescence: A Novel Strategy for Vascular Diseases.

Vascular diseases are a major threat to human health, characterized by high rates of morbidity, mortality, and disability. VSMC senescence contributes to dramatic changes in vascular morphology, structure, and function. A growing number of studies suggest that VSMC senescence is an important pathophysiological mechanism for the development of vascular diseases, including pulmonary hypertension, atherosclerosis, aneurysm, and hypertension. This review summarizes the important role of VSMC senescence and senescence-associated secretory phenotype (SASP) secreted by senescent VSMCs in the pathophysiological process of vascular diseases. Meanwhile, it concludes the progress of antisenescence therapy targeting VSMC senescence or SASP, which provides new strategies for the prevention and treatment of vascular diseases.

Pulmonary artery aneurysm-related fatal hemoptysis in Behcet's disease: A case report with literature review

Introduction: Behcet's disease is a multisystemic disorder of unknown etiology. Vascular involvement is identified by the development of aneurysms and occlusion of the vessels. the current report aimed to discuss a case of Behcet disease presented with a bilateral pulmonary aneurysm.
 Case presentation: A 41-year-old woman presented with recurrent attacks of hemoptysis for two years. a chest computed tomography scan bilateral pulmonary artery aneurysm. Repeated bronchoscopy showed bleeding from the right lower lobe bronchus. She underwent a right-side thoracotomy with a right lower lobectomy. She was scheduled for left-side intervention as well. Before discharge, she was seen by a rheumatologist and was diagnosed with a case of Behcet disease. She developed attacks of massive hemoptysis (huge and bright blood) after two weeks which led to her passing away.
 Conclusion: Although it is rare, Behcet disease could involve major arteries and lead to fatal complications. Surgical intervention remained a viable option for the management.

Behçet’s Disease: Pulmonary Aneurysms Resolution with Immunosuppressive Therapy

Behçet’s Disease: Pulmonary Aneurysms Resolution with Immunosuppressive Therapy

Frail Silk: Is the Hughes-Stovin Syndrome a Behçet Syndrome Subtype with Aneurysm-Involved Gene Variants?

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The etiology and pathogenesis of HSS are incompletely known. The current consensus is that vasculitis underlies the pathogenic process, and pulmonary thrombosis follows arterial wall inflammation. As such, Hughes-Stovin syndrome may belong to the vascular cluster with lung involvement of Behçet syndrome, although oral aphtae, arthritis, and uveitis are rarely found. Behçet syndrome is a multifactorial polygenic disease with genetic, epigenetic, environmental, and mostly immunological contributors. The different Behçet syndrome phenotypes are presumably based upon different genetic determinants involving more than one pathogenic pathway. Hughes-Stovin syndrome may have common pathways with fibromuscular dysplasias and other diseases evolving with vascular aneurysms. We describe a Hughes-Stovin syndrome case fulfilling the Behçet syndrome criteria. A MYLK variant of unknown significance was detected, along with other heterozygous mutations in genes that may impact angiogenesis pathways. We discuss the possible involvement of these genetic findings, as well as other potential common determinants of Behçet/Hughes-Stovin syndrome and aneurysms in vascular Behçet syndrome. Recent advances in diagnostic techniques, including genetic testing, could help diagnose a specific Behçet syndrome subtype and other associated conditions to personalize the disease management.

Incidental case of an idiopathic pulmonary arterial aneurysm masquerading as a stable pulmonary nodule for 17 years

A true pulmonary arterial aneurysm is the dilation of all three vascular walls in a pulmonary artery. True pulmonary arterial aneurysms are very rare, and the relatively minor cases on this topic present with symptoms pertaining to the causes of trauma, Behçet’s disease, pulmonary vasculitis, granulomatous lung infections, and cartilage instability. A rupture of the pulmonary arterial aneurysm can be life-threatening and often requires surgical repair. Our case presents a 70-year-old male who was referred to pulmonary clinic for evaluation of an asymptomatic, incidental finding in his right lower lobe that was considered to represent a pulmonary nodule based on its presentation on computed tomography imaging. The referring provider had requested a possible biopsy. Before proceeding with a biopsy, the presence of the pulmonary aneurysm was confirmed only after the fluorodeoxyglucose-positron emission tomography scan and a computed tomography angiography were performed. Our case illustrates the importance of including pulmonary aneurysm in the differential during work-up of a pulmonary nodule, as diagnostic sampling could have been detrimental.

Hemothorax Due to Aneurysm of the Left Pulmonary Vein in a Pregnant Patient. Case Report

Pulmonary venous aneurysm often presents as a mediastinal mass, in most cases patients are asymptomatic and surgical treatment is not required unless there are complications. We present the case of a 34-year-old woman, with a normal evolutionary pregnancy of 36 weeks of gestation, with dyspnea on small to medium efforts, to which was added pleuritic pain in the left hemithorax, evidence of left pleural effusion greater than 80%, with drainage of 700 ml of hematic fluid. The gynecology and obstetrics service identified anhydramnios, deciding to terminate the pregnancy through the abdomen. A simple and contrast- enhanced chest tomography was performed, as well as papanicolaou staining of pleural fluid with a negative report for malignancy. The angiotomography reported an aneurysm of the left lobar vein with a fistulous tract, so cardiothoracic surgery performed resection of the saccular aneurysm by thoracotomy without complications. Pulmonary vein aneurysms are such rare lesions that there are no data in the literature to predict their natural history or pathogenesis. During pregnancy, cardiovascular causes of mortality predominate; on this spectrum, hemothorax could be a true surgical emergency.

The Eye as a Window to the Brain: Prominent Retinal Vasculopathy Points to Neuro-Behcet Diagnosis for an Undifferentiated Solitary Brain Lesion

Objective To report a perplexing case of Behcet disease (BD) presenting as a focal parenchymal lesion that reached a diagnosis after noting a prominent retinal vasculopathy, highlighting the importance of ophthalmologic evaluation in undifferentiated CNS disease. Background BD can have variable systemic manifestations driven by a vasculitis, including oral or genital ulcers, pulmonary aneurysms, and uveitis. Neurologic involvement is present in less than 10% of patients, most commonly as a meningoencephalitis. Design/Methods We present the case of a woman who developed asymmetric sub-acute sensorineural hearing loss at age 31 followed by transient right facial weakness at age 40, and most recently presented with right facial numbness and arm weakness at age 47. Brain MRI revealed a left frontal enhancing lesion with associated T2/FLAIR hyperintensity extending from the periventricular to the juxtacortical area with a thin rim of a reduced diffusion. CSF and serum studies were negative for inflammation, infection and malignancy except for elevated ESR and CRP. Brain biopsy revealed non-specific gliosis. Persistent enhancement on MRI was noted over 3 months, with spontaneous clinical improvement. Patient endorsed insidious vision changes over recent years, and visual testing was performed. Results Dilated ophthalmic examination demonstrated striking peripheral attenuation and sclerosis of retinal vasculature, with evidence of nonperfusion and skip lesions on retinal fluorescein angiography (FA). Findings of occlusive retinal peripheral vasculopathy suggested an underlying vasculitis as the etiology of the brain lesion and prior deficits, raising the likelihood of BD. Patient was homozygous for HLA-B*51, further supporting this diagnosis even with lack of mucosal ulcers and negative history of pathergy. Conclusions Neurologic manifestations of BD can be diverse including retinal occlusive vasculopathy; ulcers are not universally present. Ophthalmologic examination, even when minimally symptomatic, can inform the diagnosis of CNS lesions. Patient was started on Prednisone, Infliximab and Methotrexate, achieving disease remission.

P170 Life threatening hemoptysis in COVID-19 associated Pulmonary Aspergillosis (CAPA)

Poster session 2, September 22, 2022, 12:30 PM - 1:30 PMObjectiveWe report a case of large pulmonary mycotic aneurysms with massive hemorrhage as an unusual complication of CAPA which required vascular radiological interventions along with appropriate antifungal treatment.Method and ResultsA 71-year-old female patient had COVID-19 and was admitted elsewhere and she received steroids, aspirin, antibiotics, and fluconazole. Cough, dyspnea, and hemoptysis started 3 weeks later which markedly worsened over 4 days, requiring emergency hospitalization due to shock, and she received a large number of packed RBCs. The chest CT scan revealed two large nodular opacities with central breakdown and CT pulmonary angiography revealed large mycotic aneurysms within the nodular opacities (Fig. 1).The important task at the moment of presentation was pulmonary artery embolization to arrest hemoptysis; which took precedence over the diagnosis and treatment of the likely infectious process. Both aneurysms were embolized using coils and vascular plugs resulting in their obliteration.Common causes of such nodules with central necrotic cavitation and formation of pseudoaneurysms are angioinvasive molds like Aspergillus and Mucorales. In the post-COVID-19 and post steroid setting in India, invasive Mucormycosis (IM) is thought to be more common than invasive Aspergillosis (IA). A mixed infection with these molds appears to be present in about 15% of the cases. Absence of DM or hyperglycemia was somewhat against a diagnosis of Mucormycosis. TB was considered less likely due to the imaging features and Nocardia was considered less likely due to the tempo of the illness and the severity of hemoptysis.Since the clinical distinction between Aspergillosis and Mucormycosis was not possible and posaconazole can be potentially useful for both, it was chosen for treatment in preference to voriconazole. Both amphotericin B and isavuconazole were in short supply at that time. Posaconazole was used for the patient intravenously followed by gastroresistant tablets. Expectorated sputum revealed narrow, septate, acute angle branching fungal hyphae. Therapeutic drug monitoring was used later to assure an adequate drug exposure. Serum galactomannan and BDG reports were received soon after and were 2.49 ODI (Optical density Index) and >523pg/ml respectively. The mold grew in culture as shown in and was identified by morphological features and by MALDI TOF MS as Aspergillus fumigatus (Fig. 2). Sensititre MIC to posaconazole was found to be 0.12 which is considered in the susceptible range.Despite these findings indicating IA, posaconazole was not replaced by voriconazole as IM could not be ruled out as part of a mixed infection. The patient showed clinical improvement, had no further hemoptysis, serum galactomannan turned negative and CT showed obliteration of the aneurysms.ConclusionPosaconazole and isavuconazole may indeed emerge as good contemporary choices over voriconazole for IA and over amphotericin B for IM.This case adds to the clinical experience of using medical treatment with posaconazole as the sole drug and not undertaking surgery in cases such as these, due to certain extenuating circumstances.