Pulmonary Arterial Aneurysm with Thrombus Formation and Atrial Septal Defect with Pulmonary Hypertension

Introduction@#Pulmonary arterial aneurysm (PAA) is a rare condition with varying symptomatology. Patients are often referred due to incidental finding of intrathoracic mass on chest radiograph. Common causes include structural cardiac and vascular abnormality, pulmonary hypertension, infection, vasculitis, connective tissue disease, and trauma.@*Case presentation@#A 37-year-old female came in due to shortness of breath associated with easy fatigability, exertional dyspnea, orthopnea, and occasional palpitations. Workup revealed 2D echocardiography findings of atrial septal defect (ASD) with left to right shunt; dilated right atrium and right ventricle; severe pulmonary hypertension; dilated main pulmonary artery. Contrast-enhanced computed tomography (CT) angiography showed PAA in the right main trunk, left and right pulmonary artery with thrombus formation. Cardiac catheterization and hemodynamic study were intended prior to definitive surgical treatment however patient did not opt to proceed. She was managed with warfarin, bisoprolol, digoxin, and trimetazidine. In spite of symptom relief, the patient still needs regular follow-up for reassessment to monitor disease progression.@*Discussion@#Pulmonary arterial aneurysm (PAA) is a rare complication of ASD, a common congenital heart disease. In this case, it presented as a mediastinal mass with nonspecific symptoms diagnosed after routine chest x-ray, verified through echocardiogram. Contrast-enhanced CT angiography ruled out aortic aneurysm and provided additional information of the extent of thrombus formation. However, pulmonary angiography still remains the diagnostic gold standard. Due to the rarity of the disease, optimal treatment of PAA is still uncertain. It is reasonable, however, to treat conservatively for asymptomatic patient with no significant pulmonary hypertension. A more aggressive surgical approach has been advocated for patients with complications.@*Conclusion@#It is important to emphasize that PAA can present with non-specific manifestations thus high index of suspicion is critical for the diagnosis. It is also vital for early detection of the aneurysm and correction of the etiology to prevent disease morbidity and mortality.

A Surgical Case for a Huge Pulmonary Artery Aneurysm 40 Years after Waterston Operation / 日本心臓血管外科学会雑誌

Surgical cases for pulmonary artery aneurysm after palliative operation for congenital heart disease are rare. A man in his 40s underwent Waterston operation (side-side anastomosis of the ascending aorta and right pulmonary artery) for pulmonary atresia and ventricular septal defect at the age of one. Medical follow-up was continued at a local clinic without definitive repair. He had complained of exertional dyspnea and cough for one month. CT scan on admission showed a large right pulmonary artery aneurysm and atelectasis of the right lung. An urgent operation was planned for the huge pulmonary artery aneurysm. Under cardiac arrest, the proximal end-to-side anastomosis to the aorta was conducted using a Y-shaped vascular prosthesis at the previous Waterston anastomosis. The peripheral pulmonary arteries were reconstructed by inclusion technique. Percutaneous cardiopulmonary support was necessary for one day after operation due to unstable hemodynamics. He was extubated on postoperative day (POD) 4 and transferred to our general ward on POD 5. He was discharged home on POD 38. He is now free from heart failure symptoms and recurrence of aneurysm one year after surgery. Careful follow-up is necessary for the dilatation of the pulmonary artery and aortic root. A definitive operation—Rastelli and closure of the ventricular septal defect—may be considered in the future if the pulmonary artery resistance is suitable for the repair.

Death after Bronchoscopic Biopsy of a Pulmonary Artery Aneurysm Mimicking Bronchial Polyp / 대한법의학회지

Pulmonary artery aneurysms (PAAs) are rare, and massive hemoptysis can lead to death if appropriate diagnosis and treatment is not provided. PAAs can be of congenital, acquired, or idiopathic origin, and the clinical symptoms are various. Among all reported cases, one-third of the patients died due to rupture. Optimal treatment or guidelines for PAAs remain uncertain. Herein, we report autopsy findings from a woman with PAA. The patient was taking medication for tuberculosis. On bronchoscopy, a polypoid lesion was found, suspected to be an inflammatory polyp. Biopsy was performed and massive bleeding into the airway occurred. The bleeding could not be controlled by bronchoscopic suction, and cardiac arrest occurred 30 minutes after biopsy; the patient subsequently died. Autopsy revealed a round, calcified PAA in the bronchus of the right middle lobe; the end of the PAA was torn. Hypovolemic signs, including weak postmortem lividity and pallor of the skin and conjunctivae, were observed. Visual inspection and histopathological examination of the right lung revealed tuberculosis and congestion. Cases related PAA are not uncommon, but autopsy cases of death occurring after biopsy of PAA mimicking bronchial polyps are rarely reported.

Idiopathic pulmonary artery aneursym.

Idopathic pulmonary artery aneurysm (PAA) is a rare lesion. Clinical experience with this condition is limited and current knowledge is mainly derived from autopsy findings. We report a patient who came to us with complaints of chest pain, breathlessness on exertion and pedal oedema and was diagnosed to have PAA.

Aneurisma de la arteria pulmonar asociado a estenosis valvular pulmonar: reporte de un caso de cardiopatía infrecuente en el adulto / Pulmonary Artery Aneurysms Associated to Pulmonary Valve: report of a rare case of heart disease in the adult

Resumen: Arch Med Interna 2013 - 35(1):19-22 La estenosis de la válvula pulmonar es una patología infrecuente constituyendo entre 8 y 10% de las cardiopatías congénitas. Se clasifica según sus características anatómicas en: típica, con displasia leve/moderada y con displasia severa. Su severidad se mide según el gradiente de presión siendo: leve < 30 mmHg, moderada entre 30 y 80 mmHg y severa > 80 mmHg. La valvuloplastia pulmonar percutánea es el procedimiento de elección en todas las edades y en todos las variantes anatómicas. Está indicada en casos de estenosis moderada a severa o en presencia de sintomatología, siendo menos efectiva en casos con válvulas displásicas. Es una técnica segura y efectiva con baja incidencia de mortalidad y complicaciones. En caso de ser ineficaz está indicada la cirugía. Los aneurismas de la arteria pulmonar presentan una baja prevalencia, estando descritos únicamente casos clínicos aislados y pequeñas series de casos lo que no permite estimar su prevalencia. Generalmente se presentan en pacientes asintomáticos o con síntomas inespecíficos y el diagnóstico se realiza como hallazgo en estudios de imagen. Pueden ser congénitos o adquiridos, asociando los congénitos otras malformaciones cardiovasculares. Los adquiridos son secundarios a: hipertensión pulmonar ya sea primaria o secundaria; estenosis valvular pulmonar; vasculitis; infecciones; neoplasias o iatrogénicas. La complicación más temida es la rotura del aneurisma con una elevada mortalidad. El tratamiento médico es limitado, atendiendo al control de la hipertensión pulmonar y a la etiología causal en el caso de los secundarios. El tratamiento quirúrgico queda reservado para los aneurismas inestables, con hemoptisis, o aneurismas mayores a 60 mm.

Abstract: Arch Med Interna 2013 - 35(1):19-22 The Pulmonary Artery Aneurysm is a rare condition that accounts for 8 to 10% of all congenital heart diseases. It is classified according to its anatomy as typical, with mild/moderate dysplasia and with severe dysplasia. Severity is defined based on the pressure gradient, being: mild < 30 mmHg, moderate between 30 and 80 mmHg and severe > 80 mmHg. The percutaneous pulmonary valve repair is a safe and effective technique that entails a low incidence of complications and a low mortality rate. It is the procedure of choice at all ages, regardless of the anatomical type. It is indicated in cases of moderate to severe stenosis or in the presence of symptoms, being less effective in cases that occur with dysplastic valves. Surgery is indicated when the percutaneous route has failed. Pulmonary artery aneurysms have a low prevalence; being there only isolated reports and small case series, their prevalence is difficult to estimate. They usually occur in patients that are either asymptomatic or presenting with non specific symptoms; diagnosis typically results from incidental imaging findings. These aneurysms may be congenital or acquired, being the former associated to additional cardiovascular malformations. Acquired PA aneurysms may be secondary to pulmonary hypertension, which may in turn be primary or secondary to episodes of pulmonary thromboembolism, pulmonary valve stenosis, vasculitis, infections, malignancies or resulting from iatrogenia. The most feared complication of the aneurysm is its rupture, a highly lethal event. Medical therapy is limited, and requires keeping pulmonary hypertension under control and acting upon the root cause in the secondary cases. Surgical therapy is reserved only to unstable aneurysms, patients with haemoptysis, or aneurysms over 60.

A Case of Behcet's Disease Complicated with a Pulmonary Artery Aneurysm and Deep Vein Thrombosis, Separately

Behcet'sdisease is a chronic inflammatory disease characterized by oral ulcers, genital ulcers, uveitis, and skin lesions. Furthermore, Behcet's disease can manifest as vascular lesions, such as, those of vasculitis, venous thrombosis, or thrombophlebitis or as an arterial aneurysm. Here, the authors report the case of a pulmonary artery aneurysm and deep vein thrombosis in a 41-year-old woman with a previous diagnosis of Behcet's disease. The patient presented with hemoptysis and a cough, and was found to have a bleeding pulmonary artery aneurysm at the right lower lung. Pulmonary arteriography was performed and the aneurysm was embolized with coils. As a result, hemoptysis did not subsequently recur. However, five years later, deep vein thrombosis occurred in the left leg. Left leg pain improved after the regional infusion of thrombolytics.

A Case of Behcet's Disease Complicated with a Pulmonary Artery Aneurysm and Deep Vein Thrombosis, Separately

Behcet'sdisease is a chronic inflammatory disease characterized by oral ulcers, genital ulcers, uveitis, and skin lesions. Furthermore, Behcet's disease can manifest as vascular lesions, such as, those of vasculitis, venous thrombosis, or thrombophlebitis or as an arterial aneurysm. Here, the authors report the case of a pulmonary artery aneurysm and deep vein thrombosis in a 41-year-old woman with a previous diagnosis of Behcet's disease. The patient presented with hemoptysis and a cough, and was found to have a bleeding pulmonary artery aneurysm at the right lower lung. Pulmonary arteriography was performed and the aneurysm was embolized with coils. As a result, hemoptysis did not subsequently recur. However, five years later, deep vein thrombosis occurred in the left leg. Left leg pain improved after the regional infusion of thrombolytics.

Endovascular Treatment of a Ruptured Pulmonary Artery Aneurysm in a Patient with Behcet's Disease Using the Amplatzer Vascular Plug 4

A pulmonary artery aneurysm is a common manifestation and the leading cause of mortality in Behcet's disease. We describe a case of spontaneous rupture of a pulmonary artery aneurysm that, due to the inadequacy of medical therapy and the disadvantages of surgery, became the ideal candidate for endovascular management and was successfully performed by using the Amplatzer Vascular Plug 4.

Peripheral Pulmonary Artery Aneurysm Secondary to Tricuspid Valve Infective Endocarditis in an Intravenous Drug User / 日本心臓血管外科学会雑誌

We report a case of tricuspid infective endocarditis with peripheral pulmonary artery aneurysm. A 31-year-old man with a history of intravenous drug abuse was admitted to our institution. Echocardiography showed severe tricuspid valve insufficiency and large vegetation (10 mm) attached to the tricuspid valve. Computed tomography (CT) revealed a right peripheral pulmonary artery aneurysm. We operated because of the large amount of vegetation. Before the operation, we performed coil embolization for peripheral pulmonary aneurysm. During the operation, we removed the posterior leaflet with vegetation, and performed tricuspid valve repair. The postoperative course was uneventful. Postoperative echocardiography did not show any tricuspid valve insufficiency or vegetation.

Bronchial Artery Embolization for Massive Hemoptysis in a Patient with Behcet's Disease / 대한류마티스학회지

Behcet's disease is an important cause of fatal vasculitis in young adult males and vascular involvement has been reported in more than 25% of patients. Pulmonary vascular involvement has been found in 1~7.7% of patients and is known to be related to poor prognosis. A incidence of fatal hemoptysis is high in patients whose Behcet`s disease is complicated by pulmonary artery aneurysm (PAA). The recurrence of hemoptysis in PAA of Behcet's disease may result from inadequate medical therapy and complications of surgical treatment. We report a case with Behcet's disease whose fatal hemoptysis has been successfully treated with bronchial artery embolization.

A Case of Coronary Arteriovenous Fistula Associated with Pulmonary Artery Aneurysm Confirmed by Multi Detector-Row Helical CT

A congenital coronary arteriovenous fistula is a rare condition, which is an abnormal communication of the coronary artery with the right or left ventricle, the right or left atrium or the pulmonary artery. A coronary arteriovenous fistula, complicated with a pulmonary aneurysm, is quite uncommon. A case of 68-year-old woman, complaining of resting chest pain for 1 week, is reported. The coronary arteriovenous fistula associated, with a pulmonary aneurysm, was confirmed by coronary angiography and multidetector-row helical CT (MDCT), and was surgically ligated.

A Case of Suspected Behcet's Desease Diagnosed by Manifestation of Pulmonary Artery Aneurysm / 결핵

Behcet's disease is a chronic systemic inflammatory disorder with an unknown etiology. It affects many organs and is characterized by recurrent attacks. Pulmonary artery aneurysms occur more frequent in males, and it is o ne of the rare pulmonary complications of Behcet's disease. It has a poor and prognosis, and is also one of the leading causes of death from Behcet's disease. Here we report a case of suspected Behcet's disease diagnosed by a manifestation of a pulmonary artery aneurysm in a 37-year-old woman.

Involvement of large pulmonary vessels in Behcet's disease:the clinical imaging features of 13 cases / 中国实用内科杂志

Objective To analyze the clinical characteristics of large pulmonary vessel involvement in Behcet's diseaseMethods The clinical data of Behcet's disease patients with large pulmonary vessel involvement,who were treated in Peking Union Medical College Hospital from 1983 to 2008,were retrospectively analyzed.Results Thirteen patients had large pulmonary vessel involvement among a total of 418 patients with Behcet's disease.The 13 patients had a mean age of (37.5?12)years old,with 10(69.2%)having hemoptysis,8(61.5%)having dyspnea.Helical CT/CTPA showed that pulmonary vascular involvement included pulmonary arterial aneurysms(53.8%),pulmonary artery occlusion(46.2%),and pulmonary thrombi(46.2%).All the 13 patients were treated with steroid and immunosuppressant;five received steroid pulse therapy;and four received vascular intervention therapy.The mean follow-up was (3?1.6)years,with a follow-up rate of 92.5%.The 3-year survival rate was 83.9% for the 13 patients.Conclusion The manifestation of pulmonary vascular involvement in Behcet's disease is various,and the diagnosis mainly depends on the manifestations of CT pulmonary angiography.

A Case of chronic necrotizing pulmonary aspergillosis with pulmonary artery aneurysm / 결핵

Pulmonary aspergillosis is classified as a saprophytic, allergic, and invasive disease. Chronic necrotizing pulmonary aspergillosis is categorized as an invasive pulmonary aspergillosis. Most invasive pulmonary aspergillosis have acute and toxic clinical features but chronic necrotizing pulmonary aspergillosis is characterized by a sub-acute infection, most commonly seen in patients with altered local defense system from preexisting pulmonary disease of in mild immunocompromised patients. Pulmonary artery aneurysm due to this infection is termed as a mycotic aneurysm, etiology of which are tuberculosis, syphilis, bacteria and fungus. We report a case chronic necrotizing pulmonary aspergillosis complicating pulmonary aneurysm is a 62 year-old man who was presented with cough, sputum, and fever. Chest radiographs showed a rapid, progressive cavitary lesion and pulmonary artery aneurysm. Angioinvastion of aspergillus was revealed by pathology after operative removal of left upper lobe containing the pulmonary artery aneurysm. He was treated with itraconazole.

A Case of Giant Pulmonary Artery Aneurysm with Severe Pulmonary Hypertension

Aneurysms of the pulmonary artery are uncommon in general but have a potentially fatal prognosis. This is derived from the potential for rupture of the aneurysm from the fact that there is commonly underlying severe pulmonary hypertension. Most cases are documented in the large postmortem series. Aneurysms of the pulmonary artery can be classified as congenital or acquired. Possible symptoms include dyspnea on exertion, cough, hemoptysis, and thoracic pain. When a large aneurysm formation of the pulmonary artery is diagnosed, surgical intervention is indicated because conservative treatment of the aneurysm will undoubtedly result in rupture with fatal outcome. We report a case of 46-year old patient with a giant left pulmonary artery aneurysm associated with severe pulmonary hypertension. A Doppler echocardiogram and a computed tomographic scan showed a giant saccular aneurysm of the left pulmonary artery (12 cm in diameter) and estimated systolic right ventricular pressure of 80 mmHg.

Congenital Giant Aneurysm of Pulmonary Artery-Associated with Ventricular Septal Defect and Pulmonary Stenosis : A Case Report

Aneurysm of the pulmonary artery is a rate entity. A neonate was seen with cyanosis and tachypnea. There was a grade 4/6 systolic murmur along the left sternal border. The chest X-ray showed a round mass shadow in the left parahilar region. Echocardiogram showed large Ventricular Septal Defect and mild Pulmonary Stenosis. The cardiac angiogram showed giant aneurysm of pulmonary artery. Surgical intervention was advised. However, the patient was discharged against operative plan. And the patient died two weeks later.

A Case of Pulmonary Artery Aneurysm with Pulmonary Hypertension

Clinical experiences of pulmonary artery aneurysm are distinctly rare and current knowledge is mainly derived from autopsy findings. Pulmonary artery aneurysms are associated with congenital heart disease, most frequently patient ductus arteriosus, followed in decreasing order by ventricular septal defect, atrial septal defect. In the remaining cases, such aneurysms are associated mainly with secondary acquired lesions, such as Behcet's disease, bacterial endocarditis, syphilis, tuberculosis, atherosclerosis, vasculitis, hypertension, trauma. Angiography was the gold standard for diagnosing pulmonary artery aneurysms, but MRI imaging has been potentially useful diagnostic modality in demonstrating aneurysm and other fine pathologic processes with high resolution. Surgery was main, but there are no firm guidelines for optimal treatment of pulmonary artery aneurysm. We experienced a case of pulmonary artery aneurysm with pulmonary hypertension in a 58 year-old female and observed for 2 years. The aneurysmal size was 5.19 cm initially and inreased to 5.5cm for two years, accompanying by pulmonary hypertension. We report this case of pulmonary artery aneurysm with a brief review of literature.

Clinical Manifestations of the Lung Involvement in Behet's Syndrome / 결핵

Background: Behet's syndrome is a chronic multisystemic disease affecting many organs such as skin, mucosa, eye, joint, central nervous system and blood vessels. Lung involvement occurs in 5% of Behet's syndrome and is thought to be due to the pulmonary vasculitis leading to thromboembolism, aneurysm and arteriobronchial fistula. Pulmonary vasculitis in Behet's syndrome is a unique clinical feature, differing from other vascuitis affecting the lung and is one of the major causes of death. Therefore, we examined the incidence, the clinical features, the radioloic findings and the clinical courses of the lung involvement in Behet's syndrome. Methods: We retrospectively reviewed the medical records and radiologic studies of 10 cases of the lung involvement in Behet's syndrome diagnosed at Yongdong Severance Hospital and Severance Hospital from 1986 to 1995. We analysed the clinical features, the radiological findings, the treatment modalities and the clinical courses. Results: 1) The incidence of the lung involvement in Behet's syndrome was 2%(10/487). The male to female ratio was 8: 2 and the mean age was 34 years. The presenting symptom was hemoptysis in 5 of 10 cases, and massive hemoptysis was noted in 2 cases. Other pulmonary symptoms were cough(6/10), dyspnea(4/10), and chest pain(2/10). Other manifestations were oral ulcers(10/10), genital ulcers(9/10), skin lesions(7/10), and eye lesions(6/10). 2) The laboratory findings were nonspecific. The posteroanterior views of chest radiographies showed multiple infiltrates(6/10), nodular or mass-like opacities(4/10), or normal findings(2/10). The chest CT scans showed multifocal consolidations(6/8), and aneurysms of the pulmonary arteries(4/8). The pulmonary angiographies were performed in 3 cases, and showed pulmonary artery aneurysms in 2 cases. The ventilation-perfusion scans in 2 cases of normal chest x-ray showed multiple mismatched findings. 3) The patients were treated with combination therapy consisting of corticosteroids, cyclophosphamide, and colchicine or anticoagulant agents. Surgical resection was performed in one case with a huge aneurysm. 4) We have followed up nine of ten cases. Three cases are well-being with medical therapy, two cases are severely disabled now and four cases died due to massive hemoptysis, massive pulmonary embolism, or sepsis. Conclusion: Pulmonary vasculitis is a main feature of the lung involvement of Behet's syndrome, causing hemorrhage, aneurysmal formation, and/or thromboemboism. The lung involvement of Behet's syndrome is uncommon but is one of the most serious prognostic factors of the disease. Therefore, an aggressive diagnostic work-up for early detection and proper treatment are recommended to improve the clinical course and the survival.

Clinical Manifestations of the Lung Involvement in Behet's Syndrome / 결핵

Background: Behet's syndrome is a chronic multisystemic disease affecting many organs such as skin, mucosa, eye, joint, central nervous system and blood vessels. Lung involvement occurs in 5% of Behet's syndrome and is thought to be due to the pulmonary vasculitis leading to thromboembolism, aneurysm and arteriobronchial fistula. Pulmonary vasculitis in Behet's syndrome is a unique clinical feature, differing from other vascuitis affecting the lung and is one of the major causes of death. Therefore, we examined the incidence, the clinical features, the radioloic findings and the clinical courses of the lung involvement in Behet's syndrome. Methods: We retrospectively reviewed the medical records and radiologic studies of 10 cases of the lung involvement in Behet's syndrome diagnosed at Yongdong Severance Hospital and Severance Hospital from 1986 to 1995. We analysed the clinical features, the radiological findings, the treatment modalities and the clinical courses. Results: 1) The incidence of the lung involvement in Behet's syndrome was 2%(10/487). The male to female ratio was 8: 2 and the mean age was 34 years. The presenting symptom was hemoptysis in 5 of 10 cases, and massive hemoptysis was noted in 2 cases. Other pulmonary symptoms were cough(6/10), dyspnea(4/10), and chest pain(2/10). Other manifestations were oral ulcers(10/10), genital ulcers(9/10), skin lesions(7/10), and eye lesions(6/10). 2) The laboratory findings were nonspecific. The posteroanterior views of chest radiographies showed multiple infiltrates(6/10), nodular or mass-like opacities(4/10), or normal findings(2/10). The chest CT scans showed multifocal consolidations(6/8), and aneurysms of the pulmonary arteries(4/8). The pulmonary angiographies were performed in 3 cases, and showed pulmonary artery aneurysms in 2 cases. The ventilation-perfusion scans in 2 cases of normal chest x-ray showed multiple mismatched findings. 3) The patients were treated with combination therapy consisting of corticosteroids, cyclophosphamide, and colchicine or anticoagulant agents. Surgical resection was performed in one case with a huge aneurysm. 4) We have followed up nine of ten cases. Three cases are well-being with medical therapy, two cases are severely disabled now and four cases died due to massive hemoptysis, massive pulmonary embolism, or sepsis. Conclusion: Pulmonary vasculitis is a main feature of the lung involvement of Behet's syndrome, causing hemorrhage, aneurysmal formation, and/or thromboemboism. The lung involvement of Behet's syndrome is uncommon but is one of the most serious prognostic factors of the disease. Therefore, an aggressive diagnostic work-up for early detection and proper treatment are recommended to improve the clinical course and the survival.

A Case of Hughes - Stovin Syndrome / 결핵

Hughes-Stovin Syndrome is an exceedingly rare combination of distal pulmonary arterial aneurysm and deep vein thrombosis, mostly found in young patients. There are striking similarities between the vascular manifestation of Behcet's disease and Hughes-Stovin Syndrome. It has been suggested that they may have a similar pathogenesis. Most patients died of massive hemoptysis due to rupture of aneurysm. Recently we have experienced the first case of Hughes-Stovin Syndrome in Korea. A 37 year old male patient was admitted because of recurrent hemoptysis and intermittent fever. He had a history of recurrent aphthous ulcers and erythema nodosum-like skin rash, But no other findings of Behcet's disease was found. Angiography showed multiple pulmonary arterial aneurysm and deep vein thrombosis in Right lower extremity. Histologic examination of specimens of open lung biopsy revealed leukocyto- clastic angiitis. Pulmonary arterial aneurysms were successfully treated by coil embolization and he is in good condition with corticosteroid and cyclophosphamide therapy.