We report a case of a 10-year-old male patient with pulmonary artery aneurysm (PAA) caused by infective endarteritis of the pulmonary artery attributed to patent ductus arteriosus. He was found to have patent ductus arteriosus at the age of 2, but he was not treated because of the absence of symptoms and normal physical development. He sought medical attention for fever and cough in August 2022. Echocardiography showed pulmonary artery aneurysm, intrapulmonary artery bulge, patent ductus arteriosus, and pericardial effusion. Contrast-enhanced CT showed pulmonary artery aneurysm, patent ductus arteriosus, and a slight compression of the left main bronchus. Surgery was performed to reconstruct the main pulmonary trunk and repair the ductus arteriosus in November 2022. The surgical outcomes were satisfactory.

Pulmonary artery aneurysm (PAA) is a rare disorder that may be classified as congenital, acquired, or idiopathic, in the case of unclear etiology. When associated with severe idiopathic pulmonary arterial hypertension, such a case of PAA may present to the operating room as an indication for lung transplantation. In this article, we present such a case of a patient with a giant main and right PAA that underwent a double lung transplant. We describe the pathophysiology and natural course of this PAA and discuss the role of intraoperative transesophageal echocardiography in the management of patients with this rare diagnosis.

Aneurysms are uncommon, but potentially life-threatening abnormalities of the pulmonary arteries. Aneurysm of the main pulmonary artery (MPA) defined as MPA diameter over 40 mm was reported in 1 : 14 000 autopsies. The most frequent location is the main pulmonary artery (89% of cases), whereas the maximum described diameter is 106-170 mm. Clinical manifestations are usually nonspecific or asymptomatic. Right heart failure symptoms, pulmonary regurgitation, trachea or bronchi compression or pulmonary emboli caused by enlarged MPA are the most commonly described clinical manifestations. Pulmonary artery aneurysm dissection is an uncommon complication but associated with a high mortality rate. Unfortunately, guidelines regulating the optimal time for the surgical intervention still have not been developed. We present the history of 76-year-old patient suffering from an aneurysm of the pulmonary artery (74 × 61 mm), as well as mitral and aortic valve disease, who was successfully operated on in our hospital.
Tętniaki są rzadkimi, ale potencjalnie zagrażającymi życiu patologiami tętnicy płucnej. Częstość ich występowania szacuje się na 1 : 14 000 autopsji. Zgodnie z definicją tętniaka pnia tętnicy płucnej rozpoznaje się, gdy średnica naczynia przekracza 40 mm. Najczęstszą jego lokalizacją jest pień płucny (89% przypadków), podczas gdy największy opisany dotąd tętniak miał średnicę 106–170 mm. Objawy są najczęściej niespecyficzne lub choroba przebiega bezobjawowo. Prawokomorowa niewydolność serca, niedomykalność zastawki płucnej, ucisk na tchawicę, oskrzela lub zator płucny spowodowany przez ucisk powiększającego się tętniaka są najczęstszymi objawami zgłaszanymi przez chorego. Rozwarstwienie tętniaka tętnicy płucnej jest bardzo rzadkim powikłaniem, ale obarczonym wysoką śmiertelnością. Niestety dotychczas nie zostały opracowane żadne wytyczne dotyczące postępowania u pacjentów z tą chorobą. Przedstawiamy przypadek 76-letniego pacjenta z tętniakiem tętnicy płucnej (74 × 61 mm), wadą zastawki aortalnej i mitralnej, który został zoperowany w naszym ośrodku.

Pulmonary artery aneurysms (PAAs) are very rare condition. In this paper, we report a case of a twenty-year-old female patient with the aneurysm of the pulmonary trunk secondary to the patent arterial duct (PDA) and infection. Diagnosis of PAA was confirmed by echocardiography. The pulmonary artery computed tomographic angiography also revealed the aneurysm and drawn a similar conclusion as echo did. The patient underwent surgery of ligation of PDA and PAA aneurysmectomy repairing with pericardium graft.

Hemoptysis is a life-threatening complication of Behcet\'s disease that is likely related to pulmonary artery aneurysm (PAA). Vascular interventional radiology may offer effective emergency therapeutic option, but has not been thoroughly investigated in this setting. A case series of a French referral center for hemoptysis combined with a literature review of case reports was conducted. Between 1995 and 2016, 12 patients were referred to our center for hemoptysis revealing or complicating the course of Behcet\'s disease. Pulmonary artery aneurysm (PAA) was the mechanism of hemoptysis in ten patients, nine of whom were treated by a transcatheter embolotherapy. Combining an additional 8 case reports from the literature, 17 patients treated by transcatheter embolotherapy for PAA were analyzed. The duration of the course of Behcet\'s disease was 22 months [IQR 3-45] at the time of PAA diagnosis. Transcatheter embolotherapy of PAA was successful for immediately controlling hemoptysis in all patients, without major complication except for one. Hemoptysis recurred in seven patients (41%) within 5 months [IQR 1-12]. The use of coils for transcatheter embolotherapy was associated with hemoptysis recurrence. A bronchosystemic hypervascularization related to the previously occluded PAA was the main mechanism of bleeding recurrence, leading to bronchosystemic artery embolization in four patients and surgery in two patients. Behcet\'s disease-related hemoptysis is mainly due to PAA. Transcatheter embolotherapy should be considered as the first-line emergency treatment for PAA-related hemoptysis, in association with the immunosuppressive regimen. Hemoptysis may recur in half of the cases, involving preferentially a bronchosystemic arterial mechanism.

Pulmonary artery (PA) vasculitis occurs in association with primary vasculitides-in particular, giant cell arteritis, Takayasu\'s arteritis, or Behçet\'s disease-or secondary vasculitis as a result of infections or malignancy. However, PA vasculitis in isolation and with concomitant aneurysmal dilation is an unusual finding. We present a rare case of PA aneurysm secondary to isolated PA vasculitis in an asymptomatic patient with no features of systemic vasculitis. This case highlights one of the first cases of PA vasculitis managed with surgical resection alone.

The occurrence of pulmonary artery aneurysm (PAA) is extremely rare in the clinical setting. Careful treatment should be considered because of the possibility of fatal complications including rupture, dissection, pulmonary embolism and heart failure. Our goal is to contribute a better understanding of this disease and its treatment.
Information on patients diagnosed with PAA was retrieved from the institute\'s database. The detailed data including aetiology, clinical presentation, diagnostic methods, operation methods and long-term outcomes of the surgical cases were outlined and analysed.
In total, 21 patients were diagnosed with PAA in Peking Union Medical College Hospital from 1980 to 2015, among whom 5 patients received surgical treatment, including 2 patients with giant PAAs. The complete remission rate of surgical cases was 80%, and the average postoperative hospital stay was 8.5 ± 1.29 days. One postoperative death occurred due to distributive shock.
PAAs must be seriously classified by aetiology to be treated appropriately. Patients with giant-size PAAs, and those with pulmonary hypertension, anatomical anomalies, and rapid growth and compression of neighbouring critical structures, are proper candidates for surgery. Surgical options include aneurysm repair and replacement with allogeneic/synthetic grafts, depending on the situation. Additionally, the correction of associated abnormalities should be performed simultaneously during surgery. Surgical outcomes are effective, and long-term prognoses are satisfactory.

Behçet\'s disease (BD) is a systemic inflammatory disorder of unknown aetiology. Pulmonary haemorrhage from ruptured pulmonary artery aneurysms (PAA) in this condition carries a high mortality but treatment has largely been empiric with use of glucocorticoids and cyclophosphamide. Tumour necrosis factor α (TNF-α) was recently recognised as a mediator in the pathogenesis of BD inflammatory lesions. TNFα inhibitors have been shown in various case reports/series to have beneficial effects in uveoretinitis, entero-Behçet\'s, neuro-Behçet\'s and BD arthritis. We describe the efficacy and tolerability of infliximab in 2 patients with Behçet\'s disease complicated by pulmonary vasculitis admitted to our unit during the years 2004-2015, and discuss the previously published data in this area.

BACKGROUND: Pulmonary artery (PA) aneurysms are rare and their related complications like dissection or rupture have been so far reported in a few reports, and a systematic description of the disease is lacking. To identify patients with PA aneurysm, at high-risk for complications, is critical. We performed a systematic review of the literature to determine characteristics that could identify high-risk patients.
METHODS: A systematic search strategy was established and executed in Pubmed, Embase, Cochrane Central Register of Controlled Trials and Google scholar. Case reports were included if a minimal set of data were described.
RESULTS: After executing the search strategy and exclusion of non-relevant or duplicate articles, 38 original articles, reviews and 169 case reports could be included. Articles were classified in high and low-pressure PA aneurysms and subdivided in six groups on basis of the causative mechanisms. PA dilatation was most common in association with pulmonary hypertension, but only one dissection was reported in 6 original articles containing 153 patients. Analysis of the case reports suggests that predictors of high-risk patients are: pulmonary hypertension in congenital heart disease, fast PA diameter growth (>2 mm/year), tissue weakness due to infection and possibly pregnancy especially in combination. Except for 2 cases, PA dissection did not occur, when the PA diameter was <75 mm and the PA pressure <50 mmHg.
CONCLUSIONS: High-risk PA aneurysms maybe identified by evaluating: the causative mechanism(s) for PA dilatation, absolute PA diameter and growth rate and by evaluating the PA systolic pressure.