Intracardiac thrombus and pulmonary aneurysms in an adolescent with Beh?et disease

Behçet disease (BD) is an inflammatory disorder of unknown origin. We present here an unusual case of juvenile Behçet with hemoptysis due to large pulmonary artery aneurysms (PAA), large intra-cardiac thrombus and prolonged fever, which posed several therapeutic challenges. In this case, a 14-year-old boy was admitted with a 3-month history of fever, painful oral ulcers, skin rash and intermittent hemoptysis. A high resolution helical computed tomography angiogram demonstrated thrombi in the right ventricle, two large aneurysms located in the right lung and two smaller ones in the left. The patient was successfully treated with colchicine, prednisone, cyclophosphamide and enoxaparine. A discussion about PAA and intracardiac thrombi and their role in BD is provided in this case.     

Left recurrent laryngeal nerve palsy associated with primary pulmonary hypertension and patent ductus arteriosus

Two patients with left recurrent laryngeal nerve paralysis in association with pulmonary artery hypertension are described. One had primary pulmonary hypertension and the other had patent ductus arteriosus. The greatly dilated pulmonary artery in these patients resulted in compression of the left recurrent laryngeal nerve and produced a cardiovocal (Ortner's) syndrome. The pathogenesis of the vocal cord palsy was documented by cross-sectional computed tomography. In conclusion, computed tomography is of great help in differentiating this syndrome from other diseases such as mediastinal mass or lymphadenopathy whenever hoarseness is complicated by pulmonary hypertension.     

Changes in plasminogen activator inhibitor 1 and tissue-type plasminogen activator during exercise in patients with coronary artery disease

As depressed fibrinolysis is implicated in the pathogenesis of coronary artery disease, we have studied the activation of fibrinolysis during maximal, symptom-limited exercise in a group of 68 men. After exercise they were divided, according to their coronary angiography and exercise 201Tl emission computed tomography results, into three groups. Group 1: persons with normal exercise 201Tl emission computed tomography results and no underlying diseases who served as controls; group 2: patients with coronary artery disease without exercise-induced myocardial ischemia, and group 3: patients with coronary artery disease with transient, exercise-induced myocardial ischemia. Before and at peak exercise we measured the plasminogen activator activity (PAA) in the euglobulin fraction of plasma by an amidolytic method and the concentrations of tissue plasminogen activator (t-PA), activator-inhibitor complex - plasminogen activator inhibitor 1 (PAI-1) complexed with t-PA - and total PAI-1 by enzyme immunoassay. The concentration of free PAI-1 in plasma was calculated by subtraction of the concentration of activator-inhibitor complex from that of total PAI-1. Under basal conditions, group 3 had significantly higher free and total PAI-1 levels than group 1. There were no statistically significant differences between the three groups in PAA, t-PA, and activator-inhibitor complex levels. At peak exercise, group 1 showed the highest release of t-PA accompanied with highest increases in PAA as well as in activator-inhibitor complex, the proportion of released t-PA antigen not bound to PAI-1 being highest in group 1. Free PAI-1 decreased significantly, but there were no differences between individual groups.(ABSTRACT TRUNCATED AT 250 WORDS)     

The Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma: Two Case Reports

Pulmonary artery sarcoma (PAS) is considered a very rare tumor with a poor prognosis. We herein report two cases of PAS that were diagnosed by positron emission tomography (PET)/computed tomography (CT). In both cases, PET was an effective option for diagnosing tumors, and surgical resection was a valid treatment for these diseases. If a pulmonary artery tumor is suspected, PET/CT is useful for diagnosing PAS and very helpful for choosing the surgical treatment strategy.     

肺动脉栓塞在多层螺旋CT强化及后处理中的影像表现及诊断评价

目的观察肺动脉栓塞（PAE）在多层螺旋CT（MSCT）强化下扫描及后处理图象中的特征,评价其对肺动脉栓塞的诊断价值。方法35例可疑PAE病人均行MSCT强化扫描,并进行多平面重建（MPR）、最大密度投影（MIP）、容积再现（VR）及仿真内窥镜（VE）后处理。结果35例受检者主干肺动脉至段肺动脉的显示率为100％;对亚段及5级肺动脉的显示率为80．23％。在35例中32例可见到不同部位、不同类型和形态的充盈缺损,并有不同种类的间接征象。3例未见肺动脉内充盈缺损,其中2例各有1种间接征象。结论多层螺旋CT扫描及重建后处理是肺动脉栓塞的快速、有效、安全、简便可行的诊断方法。     

An unexpected pulmonary arterial aneurysm in a COPD patient

We present a case of an idiopathic pulmonary artery aneurysm in an asymptomatic patient who was treated for an irrelevant medical condition. Pulmonary artery aneurysms (PAA) are quite rare and can either be congenital or acquired. Congenital aneurysms are usually associated with cardiac malformations leading to pulmonary hypertension. Acquired aneurysms can be idiopathic or associated with infections (tuberculosis, syphilis), trauma, pulmonary valvular stenosis, or collagen diseases. Pulmonary artery aneurysms are not common and an idiopathic pulmonary artery aneurysm is a rare finding that could be diagnosed incidentally.     

Transesophageal echocardiographic detection of a pulmonary artery aneurysm complicated by thrombus

The incidence of aneurysms of the pulmonary artery is known to be very low. Although diagnosis and evaluation of pulmonary artery aneurysms may be difficult without angiography, computed tomography and MRI's have emerged as useful noninvasive techniques. However, a transthoracic echocardiogram may reveal a pulmonary artery aneurysm. To our knowledge, transesophageal echocardiographic findings of pulmonary artery aneurysm with thrombus have not been reported in detail. Here, a case of thrombosed aneurysm of the main pulmonary artery diagnosed by transesophageal echocardiography and confirmed by computed tomography and MRI is reported.     

Anomalous origin of the left anterior descending artery from the main pulmonary artery with multi-detector row computed tomography coronary angiography

Anomalous origin of the left anterior descending artery from the main pulmonary artery is a rare congenital anomaly of the coronary artery. We present a case report of a 31-year-old male patient with anomalous origin of the left anterior descending artery from the main pulmonary artery that was diagnosed by multi-detector row computed tomography coronary angiography and correlated with conventional angiography. The results indicate that multi-detector row computed tomography plays an important role in the diagnosis of some rare coronary anomalies that conventional angiography cannot clarify.     

Absent pulmonary valve syndrome with ascending aortic aneurysm

Absent pulmonary valve syndrome is rare. We report the case of a child with absent pulmonary valve syndrome noted after birth. Progressive dilatation of the main pulmonary artery and ascending aorta were noted by echocardiography and confirmed by heart computed tomography. He underwent ascending aorta graft replacement and pulmonary artery reduction angioplasty at the age of 6. Good outcome was achieved at 18-month follow-up where subsequent computed tomography revealed a normal size ascending aorta and main pulmonary artery.     

Surgical correction of isolated unilateral absence of right pulmonary artery

A 2-year-old girl with isolated unilateral absence of right pulmonary artery is described. Catheterization at 5 months demonstrated hypoplastic right pulmonary artery by pulmonary venous wedge angiography, and the patient underwent right Blalock-Taussig shunt and angioplasty of right pulmonary artery with autologous pericardial roll as an initial step. At 2 years, she underwent anastomosis of right pulmonary artery to main pulmonary artery with an autologous pericardial tube. Postoperative computed tomography showed a patent reconstructed right pulmonary artery.     

Proximal pulmonary artery aneurysms in patients with pulmonary artery hypertension: complicated cases

Cases with proximal pulmonary artery aneurysm (PAA) sometimes have severe complications. We report 4 cases of proximal PAA complicated by pulmonary hypertension. Three cases had proximal PAA and one had both proximal and peripheral PAA. Complications associated with proximal PAA are compression of the bronchus, dissection and/or rupture of the pulmonary artery, and thrombus of the pulmonary artery. The available medical treatments have limitations. Two of our patients with proximal PAA are awaiting lung transplantation.     

MDCT and 3D evaluation of type 2 hypoplastic pulmonary artery sling associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis

The early diagnosis and complete anatomic evaluation of pulmonary artery sling, a congenital vascular anomaly in which left pulmonary artery arises from the right pulmonary artery, is paramount for proper patient management, because patients with this disorder frequently have other congenital anomalies resulting in high morbidity and mortality. Until recently, pulmonary artery sling in the neonate has been established with standard radiologic imaging studies such as plain radiographs, barium swallow studies, fluoroscopy-guided airway studies, and echocardiograms. However, with the development and widespread availability of multidetector computed tomography, pulmonary artery sling is increasingly evaluated with this newer technology. This case report presents a rare incidence of type 2 hypoplastic pulmonary artery sling in a neonate associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis. Multidetector computed tomography combined with 3-dimensional evaluation was particularly helpful in making a correct diagnosis of the complicated anatomic anomalies found in this case.     

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.     

Bilateral proximal pulmonary artery aneurysms simulating hilar adenopathy.

Proximal pulmonary artery aneurysms (PAAs) are rare. Most are associated with secondary pulmonary hypertension or a variety of rare systemic disorders. An asymptomatic adult patient presented with bilateral hilar enlargement on a routine chest roentgenogram. Computed tomography of the chest revealed 5 cm bilateral proximal PAAs with a normal pulmonary trunk. The clinician should consider proximal PAA in the differential diagnosis of hilar enlargement.     

Evaluation of diagnostic accuracy of computed tomography to assess the angioarchitecture of pulmonary sequestration

Bronchopulmonary sequestration is an unusual congenital malformation consisting of abnormal lung tissue that lacks normal communication with the tracheobronchial tree. The diagnosis of pulmonary sequestration is based on identifying this systemic arterial supply. We aimed to evaluate the sensitivity of multidetector computed tomography in demonstrating the feeding artery and draining veins. Between 2003 and 2008, 8 patients (6 males, 2 females) ranging in age from 5 to 49 years with a diagnosis of pulmonary sequestration were identified. All patients underwent evaluation with chest tomography (spiral or multi detector tomography) and digital subtraction angiography. Aberrant systemic arterial supply was demonstrated in all cases: from the descending thoracic aorta (n= 6); arcus aorta (n= 1), internal mammarial artery (n= 1), intercostal arteries (n= 2) and celiac axis (n= 1). Four patients underwent surgery which confirmed the angioarchitecture depicted on angiography. One patient underwent angiography with embolization using. Computed tomography especially multidetector computed tomography is a powerful noninvasive technique for the detection of pulmonary sequestration.     

Pseudopulmonary artery sling in a 22q11 deletion newborn with tetralogy of Fallot and isolated left subclavian artery

We report an interest and mystifying cardiovascular imaging in a 22q11 deletion neonate with rare congenital heart defects, including anomalous origin of the left pulmonary artery arising from the right pulmonary artery and in front of the trachea, called pseudo-pulmonary artery sling, combined with isolated left subclavian artery confirmed by multidetector row computed tomography.     

Fever of unknown origin: Importance of 18F-FDG PET/CT in the diagnosis of a late infectious complication after aneurysm bypass

Persistent blood flow in aneurysmal sac after bypass-exclusion is well documented in the literature. Aneurysm enlargement, local compressive symptoms and even sac rupture are commonly described complications. Late secondary infection of popliteal artery aneurysm (PAA) following ligation and venous bypass is exceptional. We report the case of late PAA infection six years after bypass-exclusion in a 75 year-old man which was diagnosed by 18F-FDG PET/CT. The patient was successfully treated by aneurysm resection and antibiotics. The diagnosis of popliteal aneurysm infection is often clinical, echographic and sonographic, but computed tomography scan can be false negative in chronic low-grade infection. 18F-FDG PET/CT is able to accurately diagnose and localize infection with high sensibility and specificity.     

Large pulmonary artery aneurysm associated with Marfan syndrome

Pulmonary artery aneurysms are rare, and published data on the subject are limited. The first case of Marfan syndrome associated with a large pulmonary artery aneurysm complicated by pulmonary artery thrombi and pulmonary hypertension is described. Serial echocardiograms showed progressive dilation of the aneurysm, which reached a massive size of 90 mm. Cardiac catheterization, pulmonary angiography and computed tomography angiography were used in the preoperative evaluation. The patient died suddenly while awaiting a decision for surgical intervention, thereby underscoring the need for aggressive management.     

Pulmonary artery aneurysms in Behçet's disease: contribution of imaging in 5 cases

Pulmonary involvement in Beh?et's disease is an uncommon condition (12%). Thromboembolism of the superior vena cava and/or other mediastinal veins, aneurysms of the aorta and pulmonary arteries are the main vascular manifestations in addition to pulmonary infarct and intrathoracic hemorrhage. Despite their scarcity, respiratory symptoms may be life-threatening. The aim of this study was to assess the contribution of thoracic imaging for one of the most serious aspects of the disease: pulmonary artery aneurysm. We report five patients with pulmonary artery aneurysms (mean age: 39.5 years). Hemoptysia revealed Beh?et's disease in three. Initially explored by conventional radiography, computed tomography and angiography, pulmonary artery aneurysms are currently investigated well with helicoidal computed tomography, digital angiography, magnetic resonance imaging (MRI) and angio-MRI. These imaging techniques provide helpful information for the diagnosis of Beh?et's disease.     

A self-resolving,post-traumatic aortopulmonary shunt

A 34-year-old Hispanic man sustained a stab wound to his chest complicated with hemopericardium and pericardial tamponade. He underwent emergent clamshell thoracotomy as well as repair to the pulmonary artery. A transthoracic echocardiogram showed no evidence of intracardiac shunt. Two months later, a new murmur was noted, with a transthoracic echocardiogram revealing high-velocity flow between the left coronary sinus and the main pulmonary artery, with which a coronary computed tomography angiogram concurred. A transesophageal echocardiogram was performed which revealed an aortopulmonic fistula from the left coronary sinus of Valsalva, approximately 1cm anterior to the ostium of the left main coronary artery, to the main pulmonary artery just distal to the pulmonic valve. Pulmonary insufficiency was minimal. The main pulmonary artery was dilated, measuring 3.2 cm by coronary computed tomography angiogram. Right ventricular systolic function was normal. Right and left heart catheterizations were performed to further assess hemodynamics and coronary anatomy; pulmonary artery pressures were 16/8 mm Hg. Aortopulmonary fistula was seen on aortogram. Surgery was deferred in view of lack of symptoms and uncertainty in its natural history in the setting of traumatic etiology. A repeat transthoracic echocardiogram at six-month follow-up showed spontaneous closure of the fistula.