Pulmonary Artery Aneurysm Research Articles

Comparative Data are Needed for Pulmonary Artery Aneurysm Management Guidelines

Silent giant: 80 mm pulmonary artery aneurysm.

Behçet’s disease (BD) is a chronic, multisystem, autoinflammatory disorder classically characterized by recurrent oral ulcers, genital ulcers, and uveitis. However, atypical presentations including vascular thrombosis, pulmonary artery aneurysms, gastrointestinal lesions, and neurological involvement can precede the classic triad, making early recognition challenging. We report three unusual cases: (1) a 38-year-old male with multisite vascular thrombosis, (2) a 39-year-old female with pulmonary artery aneurysms, and (3) a 30-year-old male with parenchymal neuro-Behçet’s disease. All cases required multidisciplinary management and early immunosuppressive therapy to prevent life-threatening complications. These cases underscore the importance of high clinical suspicion and thorough investigation in patients presenting with atypical symptoms suggestive of BD.

The patient is a 73-year-old woman who was referred to our department for diagnostic surgery. A part-solid ground-glass nodule, suspected to be lung cancer, was identified in the right S6 on a preoperative chest contrast computed tomography (CT). Additionally, a continuous bronchial artery was observed at the root of A6. Based on the CT findings, we diagnosed a bronchial-pulmonary artery fistula (BPAF) and an associated pulmonary artery aneurysm. The patient exhibited no subjective symptoms, such as blood-streaked sputum or hemoptysis. She subsequently underwent a right S6 segmentectomy. Intraoperative examination of the pulmonary artery revealed a continuous bronchial artery dorsal to A6, as anticipated. Intravenous indocyanine green (ICG) administration after root ligation of A6 showed that ICG flowed into A6 and peripherally to S6 beyond the ligated point via the bronchial artery, confirming the diagnosis of BPAF.

ABSTRACTPulmonary artery aneurysm is a serious but under‐recognized complication of Behçet's disease that may present with hemoptysis. Early diagnosis coupled with timely initiation of immunosuppressive therapy is crucial in preventing aneurysmal progression and associated life‐threatening complications.

This case highlights a giant pulmonary artery aneurysm with a diameter of 107.7 mm, which is so far one of the most massive pulmonary aneurysms detected in a young person. Management may be extremely challenging in a resource-poor setting where surgical interventions are not readily available.

Pulmonary involvement of Behçet disease (BD) typically manifests as vascular involvement in the form of pulmonary artery thrombosis and/or aneurysm, although various parenchymal lung findings may also be observed. We aimed to analyze the indications for imaging and thoracic computed tomography (TCT) findings in BD patients. In this medical records review, single-center, comparative cohort study, 196 BD patients who underwent TCT for any reason between July 2020 and July 2024 were included. The patients' demographic data, disease-related clinical features, indications for TCT, and TCT findings were recorded. The mean age of the patients was 40.0 ± 12.0 years, with a female-to-male ratio of 56/140 and disease duration of 8.9 ± 9.1 years. The most common indications for TCT imaging were suspected pulmonary vascular involvement (PVI) (139/196), unexplained acute phase elevation (46/196), and infection (36/196). PVI was present in 23 patients. Patients with PVI also exhibited additional parenchymal findings. Ground-glass opacities and atelectasis were significantly more common in patients with PVI compared with those without. TCT imaging is essential for identifying both vascular and parenchymal pulmonary complications in BD, especially in patients with atypical symptoms or elevated inflammatory markers.

One-stage surgical repair via clamshell incision for a giant pulmonary artery aneurysm and bilateral huge lung bullae associated with pulmonary hypertension

Pulmonary artery aneurysms are extremely rare and can be fatal if ruptured. We report the case of a 72-year-old man who underwent coil embolization of bilateral peripheral pulmonary artery aneurysms. Ten years ago, a left upper lobe nodule was incidentally noted on a chest computed tomography (CT). The nodule was suspected as a benign tumor and followed up. As it gradually enlarged, the patient referred to our hospital. Chest CT showed a 2.0-cm nodule in S1+2 of the left lung and a 0.6-cm nodule in S10 of the right lung. Contrast-enhanced CT showed that each nodule had the same contrast enhancement as the pulmonary artery. The left upper lobe nodule and the right lower lobe nodule were connected by a shunt with A1+2b and A10. They were diagnosed as pulmonary artery aneurysms, and coil embolization was performed. Eighteen months later, no enlargement of the pulmonary artery aneurysms or new lesions were observed.

Behçet's disease (BD), also called Behçet's syndrome, is a rare multisystemic vasculitis that causes blood vessel inflammation throughout the body. It may affect multiple organs and less frequently involves the central nervous system, potentially resulting in thrombotic events and aneurysm formation, the most notably pulmonary artery aneurysms (PAA). We present a case of a 44-years-old female who sought medical attention due to prolonged hemoptysis combined with chest pain. Ascribed to these symptoms, initial computer tomography (CT) (native and arterial phases) revealed an oval mass in the left hilum. Subsequent CT angiography demonstrated an aneurysm of the left interlobar artery compressing the segmental bronchus with associated thrombus formation. The patient refused surgical intervention. One year later, a follow-up CT angiography showed the resolution of the initial aneurysm but revealed a new aneurysm in the right main pulmonary artery. This case highlights the rare migratory nature of pulmonary aneurysms in Behçet vasculitis, underscoring the need for early diagnosis, imaging follow-up and interdisciplinary management

Left Pulmonary Artery Aneurysm in an Adult Patient With Uncorrected Tetralogy of Fallot and Patent Ductus Arteriosus: A Case Report

CT imaging of pulmonary artery aneurysms in the emergency department - What you need to know.

Pulmonary artery aneurysm and dissection associated with ventricular septal defect

Background Behçet's Disease (BD) is characterized by oral and genital ulcers, arthritis, skin manifestations, uveitis, gastrointestinal tract, and central nervous system involvement. Although it is known to be more severe in men, there are studies in the literature with conflicting results regarding gender and the distribution of clinical findings. This study aimed to examine the relationship between clinical findings and gender in BD patients and to compare our results with the literature. Methods 506 patients diagnosed with Behçet's disease were included in the study. Demographic data, laboratory, and clinical findings of the patients were obtained retrospectively from hospital records. The distribution of clinical findings according to gender was evaluated. Results A total of 280 males (55.3%) and 226 females (44.7%) were included in the study. There was no significant difference between male and female patients regarding age at diagnosis (p=0.662). Genital ulcer (47.6% vs 52.4%, p=0.011), superficial thrombophlebitis (20.9% vs 79.1%, p=0.002), uveitis (33.7% vs 66.3%, p=0.02), deep vein thrombosis (22.5% vs 77.5%, p=0.00) and pulmonary artery aneurysm (11.1% vs 88.9%, p=0.046) were more common in males. There was no significant difference between the sexes in other clinical findings, HLA B5, and pathergy positivity. Conclusion Gender impacts the clinical manifestations of BD and should be considered in patient follow-up. However, it is a heterogeneous disease, other factors may certainly affect the emergence of clinical findings.

Pulmonary arterial aneurysm in a pulmonary hypertension patient.

Surgical Correction of a Large Pulmonary Artery Aneurysm and Dysplastic Pulmonary Valve in a 6-Year-Old Patient: A Case Report and Literature Review

Behçet's disease is a complex multisystem vasculitis of unknown aetiology, characterised by different clinical manifestations and the occurrence of successive attacks interspersed with remissions. Mediastino-pulmonary involvement is rare but severe, resulting mainly in thrombosis of the superior vena cava and pulmonary artery aneurysm. The association between Behçet's disease and chylothorax and/or chylopericardium is rare, with a few cases reported in the literature. We present a case of chylothorax and chylopericardium with thrombosis of the superior vena cava as clinical manifestations in a patient with Behçet's disease. Management was multidisciplinary, based on pleuropericardial drainage, immunosuppressants, anticoagulants and nutritional management.

Surgical Therapy for Pulmonary Artery Aneurysm

ObjectivePulmonary flow restrictors (PFRs) are interesting devices, but their surgical removal outcomes are poorly understood. MethodsRetrospective review of clinical data from children with bilateral PFRs who underwent device removal during follow-up surgery. ResultsThirty-four PFRs were explanted from 17 patients (41.2% males) at a median of 2 months (IQR, 1.2-5.2) post-implantation, with a median patient age of 2.5 months (IQR, 1.6-5.8). One patient experienced life-threatening bilateral pulmonary artery (PA) aneurysms two months after PFR implantation, necessitating urgent surgery. Two PFRs were found migrated across left PA's upper lobe branch origin. Twenty-six were removed intact, 1 in two fragments, and 7 piecemeal. No thrombus was noted. Neoendothelium was observed on 11 PFRs. Seven PFRs caused endothelial damage, requiring sharp and blunt dissection for removal. Six right and four left PA arteriotomies were patched. Hegar dilators, with median sizes of 7 mm (IQR, 6.8-8.3) for right PA and 7 mm (IQR, 7-8) for left PA, confirmed branch patency. At a median follow-up of 14.8 months (IQR, 10.2–18.3), echocardiographic maximum velocities in 13 biventricular patients and 2 awaiting future biventricular repair were 1.5m/s (IQR, 1.4–1.7) for the left PA and 1.6m/s (IQR, 1.4–1.7) for the right PA. One patient with deferred Norwood had normal PAs and well-positioned PFRs on pre-stage-II catheterization. A patient who underwent stage-II Norwood 3.2 months post-PFR implantation died from sepsis 1 month later, but post-Glenn angiogram revealed no stenosis. ConclusionsPFR removal is safe and effective. Complications are manageable, with no PA stenosis observed.

Hughes-Stovin Syndrome (HSS) is a rare and severe disorder characterized by the combination of pulmonary artery aneurysms and deep vein thrombosis. We report the case of a 42-year-old male presenting with recurrent hemoptysis revealing HSS. Management consisted of high-dose corticosteroids and cyclophosphamide. The patient showed marked clinical and radiological improvement. HSS, as a rare form of Behçet’s disease, is associated with high morbidity and mortality due to thrombosis and potential rupture of aneurysms. Immunosuppression remains the cornerstone of management. Anticoagulation is controversial and requires careful consideration. Early diagnosis and prompt treatment are critical in managing HSS.