Pulmonary Artery Aneurysm Research Articles

POS1345 BEYOND PULMONARY ARTERY ANEURYSM; PULMONARY INVOLVEMENT IN BEHCET’S SYNDROME

BackgroundPulmonary involvement (PI) other than pulmonary artery aneurysm (PAA) in Behcet’s syndrome (BS) is still an area of investigation.ObjectivesHerein, we aimed to retrospectively evaluate all types of pulmonary involvement associated with Behcet’s syndrome (BS).MethodsAmong 800 BS patients according to International Study Group for Behcet’s Disease (ISG) criterias, 28 patients were selected based on their radiologic examination consistent with BS-related PI. Demographic features, other clinical manifestations of BS, treatment modalities and types of PI were analyzed.ResultsThe overall prevalence of PI was estimated 3,5% among all BS patients. PI was more common in males (82.1% vs 17.9%). Mean age for BS diagnosis and onset of PI were as following; 32 ± 10,9 and 37 ± 11,4 years. Deep vein thrombosis (DVT) was the most common accompanying vascular involvement (53,6%). PAA, pulmonary vasculitis (PV), and pulmonary thromboembolism (PTE) were seen in 7 (25%), 13 (46,3%), and 18 (64,4%) of patients, respectively. In 5 patients, intracardiac thrombosis was present simultaneously in the right ventricle. Cyclophosphamide (CYC) was the most common preferred agent (78%) followed by azathioprine (AZA) as first line. Warfarin used in 18 patients (Table 1) Mortality was seen in 3 patients during follow up (1 due to PAA bleeding, others with unknown causes).Table 1.Characteristics of pulmonary involvement in BS patientsMale/Female, (n), (%)23/5 (82,1%/17,9%)Age of diagnosis, years (Mean± SD)32 ± 10,9Age of pulmonary involvement, years (Mean ± SD)37 ± 11,4Oral ulcer, (n), (%)28 (100%)Genital ulcer, (n), (%)20 (71,4%)Osteofollicular lesion,(n), (%)20 (71,4%)Erythema nodosum, (n), (%)13 (46,4%)Uveitis, (n), (%)8 (28,6%)DVT, (n), (%)15 (53,6%)Cardiac involvement, (n), (%)5 (17,8%)Pulmonary involvement, (n)-PAA, (n), (%)287 (25%)-PV, (n), (%)13 (46,4%)-PTE, (n), (%)18 (64,3%)Immunosuppression (first line agents)26/28-Steroid, (n), (%)26/28-CYC, (n), (%)22/26 (84,6%)-AZA, (n), (%)4/26 (15%)Anticoagulation (warfarin), (n), (%)18PAA; pulmonary artery aneurysm, PTE; pulmonary thromboembolism, PV; pulmonary vasculitis, CYC; cyclophosphamide, AZA; azathioprine, DVT; deep vein thrombosisConclusionDespite the importance of PAA in BS patients, capillaritis and thromboembolism other than PAA may occur commonly in BS as well. Onset at young age, male gender and previous DVT seem to be significant risk factors for the development of PI.

A Case Report and Review of the Literature: Infectious Aneurysm Formation in the Pulmonary Arteries-A Rare but Perilous Sequela of Persisting Infection With Klebsiella pneumoniae.

Septic aneurysms of the pulmonary artery are rare conditions, with few cases having been reported worldwide. They are assumed to result from septic emboli that cause a local inflammatory reaction of the arterial wall, ultimately leading to degenerative changes. We report the case of a 63-year-old female patient presenting with Klebsiella pneumoniae urosepsis and first diagnosis of diabetes mellitus, who developed a life-threatening infectious pulmonary artery aneurysm secondary to bacteremia with Klebsiella pneumoniae. The patient required a lobectomy due to pulmonary hemorrhage. We review the clinical hallmarks of Klebsiella pneumoniae related septic pulmonary embolic disease and summarize currently known risk factors for the development of infectious aneurysmatic disease including diabetes mellitus and other states of immunosuppression. The featured case aims to increase the awareness for this seldom but life-threatening complication of infectious diseases such as Klebsiella pneumoniae urosepsis.

Saccular Pulmonary Aneurysm that Mimicking a Pulmonary Mass : a Case Report

Introduction: Pulmonary artery aneurysm (PAA) is a rare disorder. The pathophysiology of PAA was varies. Radiological modalities was very important due to an atypical clinical manifestations. Diagnosis approach of PAA is very important to avoid miss diagnosis and miss treatment. Case: A 51-year-old female patient came with chief complaints of shortness of breath since 5 years ago that was worsen 2 days before being admitted to emergency room. The complaints was felt throughout the day, that usually only appears if she did heavy activity due to history of chronic heart failure (CHF). Patient had a history of uncontrolled hypertension for years that leads to renal failure and CHF. A post pleural effusion evacuation Chest X-Ray evaluation revealed a well-defined but partially lobulated consolidation that is suspected as a lung mass. A CT scan with contrast showed a pulmonary aneurysm. A Doppler ultrasound revealed a pulmonary artery aneurysm showed by the turbulence of vascular flow. The patient got conservative oral therapy and monitoring for signs of aneurysm complications while waiting for the patient's agreement to be referred to tertiary hospital for CTPA (Computed Tomography Pulmonary Angiography) which is the gold standard on diagnosed PAA. Conclusion: PAA is a rare cardiovascular disorder with atypical clinical manifestations. Radiological examinations such as Doppler ultrasound, Trans Thoracic Echocardiography and Computed Tomography could be done for diagnostic approach of PAA in the hospital with limitation of radiologic modality. Diagnosis of PAA should be considered in the differential diagnosis of a pulmonary nodule or mass.

Emerging Roles of Extracellular Non-Coding RNAs in Vascular Diseases.

Extracellular vesicles (EVs) are secreted by cells and carry diverse components, including proteins, lipids, nucleic acids, and metabolites. EVs could be found in blood and other biofluids. They vary greatly in size, function, cargo, and cellular origin. Accumulating evidence shows that extracellular non-coding RNAs, the dominant extracellular RNAs encapsulated into EVs, function as critical mediators of cell-cell communication and play critical roles in human health and disease. Blood vessels form a dense network that nourishes all of the body's tissues. These vascular networks' dysregulated functions contribute to vascular diseases, such as pulmonary arterial hypertension (PAH), hypertension, atherosclerosis, and aneurysm. With the increase in unhealthy lifestyle-associated obesity and metabolic disorders, vascular diseases are becoming serious medical and public health issues with a profound global economic burden. The present review summarizes the latest advances on extracellular non-coding RNAs in pathological vascular remodeling-associated diseases, briefly describing vessel-associated extracellular non-coding RNAs and their mechanisms of action.

Pulmonary valve perforation with multiple cardiac anomalies: a case report

BackgroundLarge pulmonary valve perforation, which is rarely seen with infective endocarditis, general atrophy, or congenital fenestration, often leads to potentially fatal outcomes, including heart failure.Case presentationTransthoracic and transesophageal echocardiographic evaluation of a 69-year-old woman revealed a severely eccentric pulmonary regurgitation with concomitant pulmonary valve stenosis, patent ductus arteriosus, patent foramen ovale, and pulmonary artery aneurysm. In the operation, a large perforation was found in the pulmonary valve leaflet. She underwent complicated surgery that involved closure of the congenital heart defects and replacement of a pulmonary valve with successful results. But the cause of her pulmonary valve perforation remained undetermined.ConclusionThis case highlights two important points: the need for timely management of congenital heart disease and being aware of the possibility of pulmonary valve perforation, which in this case was indicated by an eccentric pulmonary regurgitant jet seen on echocardiography.

Ortner's Syndrome Due to Giant Thoracic Aortic Aneurysm.

Ortner's syndrome is hoarseness of the voice caused by enlarged cardiac structures compressing the left recurrent laryngeal nerve (LRLN). The LRLN is vulnerable for impingement by cardiac structures due to its anatomical course. LRLN is longer and it hooks below the arch of the aorta, posterior to the ligamentum arteriosum before ascending toward the tracheo- esophageal groove. Ortner's syndrome has been commonly described with left atrial enlargement due to mitral stenosis; however, other causes like aortic aneurysm, pulmonary artery aneurysm, and aortic dissection have been described. Although rare, hoarseness of the voice could be the only presenting complaint in aortic aneurysms as in the case described herein.

Syndromic pulmonary artery aneurysm

Syndromic pulmonary artery aneurysm

Surgical correction of massive main pulmonary artery aneurysm due to giant cell arteritis causing left main coronary artery compression.

Surgical correction of massive main pulmonary artery aneurysm due to giant cell arteritis causing left main coronary artery compression.

Bilateral lung transplant for pulmonary hypertension with pulmonary artery aneurysm.

Bilateral lung transplant for pulmonary hypertension with pulmonary artery aneurysm.

Surgical management of giant pulmonary artery aneurysms in patients with severe pulmonary arterial hypertension.

Giant aneurysm of the pulmonary artery (PAA) is an extremely rare condition that may develop in patients with pulmonary arterial hypertension (PAH) which may be complicated by rupture, dissection or intravascular thrombus formation. The aim of this study was to examine available literature with regard to surgical strategies in patients undergoing transplantation for PAH with PAA. These patients were traditionally considered for heart-lung transplantation but more recently, there have been reports of successful lung transplantation with reconstruction of the pulmonary artery. Unless there is a mandatory indication for heart-lung transplantation, patients with PAH and PAA can undergo lung transplantation and reconstruction of the pulmonary artery without compromising the outcome.

Pulmonary Artery Aneurysm with Tetralogy of Fallot: A Rare Entity

Pulmonary artery aneurysms is a rare entity with varied causes, its association with Tetralogy of Fallot (TOF) having low pressure pulmonary system is further more uncommon. Here, the authors present a case of 15-year-old male patient presenting with symptoms of cyanosis and dyspynoea for which echocardiography, cardiac catheterisation and Computed Tomography (CT) angiography was done and diagnosis of tetralogy of fallot with left pulmonary artery aneurysm was made for which patient was admitted and underwent surgical correction. Pulmonary artery aneurysm with tetralogy of fallot is an extremely rare anatomical combination requiring treatment strategy modification, hence merit proper evaluation

Comment on: Behçet's disease in Emergency Department: a rare case presenting with haemoptysis and massive pulmonary arterial aneurysms. On behalf of the Hughes-Stovin syndrome (HSS) international study group.

Comment on: Behçet's disease in Emergency Department: a rare case presenting with haemoptysis and massive pulmonary arterial aneurysms. On behalf of the Hughes-Stovin syndrome (HSS) international study group.

Thrombosis of aneurysmal pulmonary arteries in patent ductus arteriosus with Eisenmenger syndrome.

ABSTRACTA 45-year-old lady with patent ductus arteriosus with Eisenmenger's syndrome had presented with hemoptysis. Computed tomography revealed aneurysmally dilated pulmonary arteries with a large calcified organized thrombus.

Computed tomography findings of isolated peripheral pulmonary artery aneurysms

Background: In this study, we aimed to evaluate computed tomography(CT) findings of peripheral pulmonary artery aneurysms(PPAA) associated with Behcet Disease(BD), Hughes Stovin Syndrome(HSS), and idiopathic origin. Methods: Contrast-enhanced CT scans of the patients were retrospectively reviewed regarding PPAA. The patients with PPA were classified into BD, HSS, and idiopathic groups according to the etiology. The groups were compared for demographical features including age and gender, multiplicity, distribution thrombosis and accompanying pulmonary artery embolism(PAE), and deep venous thrombosis(DVT) history. Results: A total of 30 PPAA (25.4±13.4 (11-62) mm) were detected in 10(2.3%) (mean age 39.8±22-1.0[8-73] years, female/male:3/7) among 4391 patients reviewed. In 7 patients multiple aneurysms were detected while in 3 a solitary lesion was seen. Most commonly lower lobes (right 8-left 8, 53.2%) involvement was observed. A thrombosis was detected within 19(63.4%) aneurysms. Among 10 patients with PPAA 4(40%) ones have BD, 2(20%) HS and 4(40%) idiopathic origin. In 5(50.%) patients there was accompanying PAE and 3 (30%) DVT history. Patients with BD nad HSS tended to have multiple lesions than with idiopathic origin. Accompanying PAE was observed in 2(100%) of HSS, 2(50%) BD, and 1(25%) patient in the idiopathic group. A DVT history was recorded in 2(100%) of HSS, 1(25%) BD. None of in the idiopathic group had a DVT history. The only rupture was observed in the HSS group. Conclusion: Vasculitic diseases lead to PPAA, including BD and HSS are more likely to be associated with complications and additional morbid conditions than idiopathic processes.

Pneumonectomy in Extensive Pulmonary Aspergillosis

A 38-year-old man, without relevant medical history, was referred to our intensive care unit for abundant hemoptysis 3 months after being diagnosed with multivisceral aspergillosis. After mechanical ventilation initiation a chest computed tomography revealed evolved multifocal pulmonary aspergillosis (Figure 1) complicated with a left pulmonary artery aneurysm located after the culmen branch and right before the origin of the lingular and inferior lobe branches (Figure 2, arrow). Active bleeding had stopped.

Triple oral combination therapy in patients with idiopathic pulmonary arterial hypertension and recurrent vessel dissection of inoperable pulmonary artery aneurysm

Kontext: Aneurysma na plicnici je vzĂĄcnĂŠ postiĹženĂ­ v dĹŻsledku zĂĄtěže cĂŠvnĂ­ stěny při postupnĂŠ dilataci. JednoznačnĂĄ definice a Ăşdaje o prevalenci tohoto postiĹženĂ­ nejsou k dispozici. PlicnĂ­ hypertenze je navĂ­c Ĺživot ohroĹžujĂ­cĂ­ a progredujĂ­cĂ­ postiĹženĂ­ vyĹžadujĂ­cĂ­ specifickou léčbu. Kazuistika: Popisujeme případ 62letĂŠ běloĹĄky s idiopatickou plicnĂ­ hypertenzĂ­ a inoperabilnĂ­m obrovskĂ˝m aneurysmatem na opakovaně disekovanĂŠ plicnici. DiagnĂłza byla stanovena snadno echokardiograficky a vĂ˝početnĂ­ tomografiĂ­. ObtĂ­ĹžnĂŠ bylo naopak rozhodovĂĄnĂ­ o léčbě, protoĹže operatéři odmĂ­tli provĂŠst chirurgickĂŠ odstraněnĂ­ aneurysmatu. Pacientku jsme léčili farmakologicky medikacĂ­ specificky určenou pro plicnĂ­ hypertenzi s cĂ­lem snĂ­Ĺžit tlak na plicnici. Diskuse: Popisujeme velmi vzĂĄcnĂŠ a nesmĂ­rně zĂĄvaĹžnĂŠ postiĹženĂ­ charakterizovanĂŠ přítomnostĂ­ plicnĂ­ hypertenze účinně léčenĂŠ vhodnou perorĂĄlnĂ­ trojkombinacĂ­ spolu s inoperabilnĂ­m aneurysmatem na opakovaně disekovanĂŠ plicnici. © 2021, ČKS.

A gray zone in the literature: indications, options, and outcomes for surgical repair of pulmonary artery aneurysms.

Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary.This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05).Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.

Misdiagnosis of pulmonary artery aneurysm with eroding thrombus into the airways. A fatal case of suffocation

Abstract We present a fatal case of hemoptysis following a thrombus-eroding pulmonary artery aneurysm into the left upper bronchus of a 79-year-old male with a history of multiple hospital contacts and examinations due to cough, hemoptysis, and reflux symptoms. A postmortem computed tomography (CT) scan revealed a hyperdense, condensed area in the left lung in relation to the lung hilus. At autopsy, the forensic specialist discovered a large, organized thrombus in a pulmonary artery aneurysm. The thrombus was adherent to the pulmonary artery aneurysm wall with an underlying defect directly communicating to the left upper bronchus. The cause of death was asphyxia due to blood in the airways (i.e., suffocation). The combination of pulmonary artery hypertension, previous pulmonary embolism, and hemoptysis should lead to a particularly thorough inspection of the lungs with a focus on the pulmonary circulation. This case report emphasizes the importance of early detection of patients at risk of pulmonary artery rupture and attentiveness when performing biopsies during bronchoscopy to prevent communication between the artery and the airway. The risk of rupturing an aneurysm should be taken into account when performing biopsies on excrescence intruding into the bronchus in patients with medical histories of pulmonary hypertension, cough, and sporadic hemoptysis.

Pulmonary artery diameter: means and normal limits-assessment by computed tomography angiography.

OBJECTIVESNormal pulmonary artery (PA) diameter remains blurred and the definitions of PA aneurysm are heterogenous. We aimed to assess PA diameters, identify a threshold for normal diameters, define PA aneurysms, possible predictors of PA size and evaluate the correlation with mid-ascending aortic diameters.METHODSBetween April 2018 and August 2019, 497 consecutive patients who underwent whole-body computed tomographic angiography were reviewed. Clinical and imaging data were collected from our institutional database. Precise three-dimensional centreline measurements were taken. Linear regression analysis was performed to detect parameters associated with PA diameter. A two-stage model was created to identify potential predictors and the resulting statistically significant interactions were tested. Data were grouped and PA, standard deviation, and upper normal limits were calculated.RESULTSAmong 497 patients with an average age of 51.4 (20.2) (74.6% males), the mean PA diameter measured 32.0 (4.6) mm [female: 31.2 (4.7) mm vs male: 32.2 (4.5) mm; P = 0.032]. The mean PA length, left PA and right PA diameters were similar between male and female patients. We found a significant correlation (r = 0.352; P < 0.001) between the PAs and mid-ascending aortic diameters. Body surface area (P = 0.032, β = 4.52 [0.40; 8.64] 95% CI) was the only significant influencing variable for PA diameter.CONCLUSIONSThe normal mean PA diameter in a reference cohort is 32.0 (4.6) mm. Body surface area is the only influencing variable of PA diameter. The normal diameters measured and corresponding upper limits of normal revealed that a PA aneurysm should not be considered below a threshold of 45 mm.

Abstract 12813: Left Main Disease—Not as Straightforward as It Seems

A 51 year-old female with a past medical history of hypertension presents with chest pain that began 12 hours earlier. EKG showed biphasic T waves in the anterior leads and ST depressions in the lateral leads with a dynamic trend in troponins. LHC showed severe LM stenosis extending into the proximal LAD and LCx. An IABP was placed due to persistent angina despite being on a nitroglycerin drip. Given the minimal involvement the other coronary arteries, a coronary CT angiogram was pursued to evaluate for other non-atherosclerotic etiologies. The CT showed a periaortic soft tissue mass that encased the left main and the right pulmonary artery. The differential for conditions causing compression of the coronaries includes aortic or pulmonary artery aneurysms, abscesses, thymoma, lymphoma, teratoma and bronchogenic cysts. A PET scan showed metabolic activity of a supraclavicular lymph node, the mediastinal mass and a pericardial effusion. Her lymph node biopsy and mediastinal mass biopsy were consistent with a thymic carcinoma. Although thymomas are the most common anterior mediastinal tumor in adults, thymic carcinoma are rarer, more aggressive and thus spread outside the thymus. For thymic carcinomas, the ideal treatment is complete surgical resection while non-surgical candidates should undergo chemotherapy and radiation. Due to the proximity of the carcinoma the aorta and pulmonary artery, our patient was deemed not a surgical candidate and underwent radiation. This unique case highlights that non-athlerosclerotic etiologies should be entertained in cases of isolated left main disease and the importance of utilizing multimodality imaging to identify such causes.