Aneurysms and Pseudoaneurysms of the Pulmonary Vasculature
Section snippets
Pulmonary Hypertension
Numerous conditions have been reported associated with PAA, but, probably, the most common underlying factor to many of them is the presence of pulmonary arterial hypertension (PAH) of any cause. Congenital deficiencies of the vessel wall, valvular and supravalvular stenosis, and increased flow secondary to left-to-right shunt are common factors. Hypoplastic pulmonic valve and bicuspid pulmonic valve have also been implicated. Left-to-right shunt (eg, atrial septal defect, ventricular septal
Vasculitis
Two types of vasculitis have commonly been associated with PAA: Behçet disease (BD) and Hughes–Stovin syndrome (HSS). Takayasu arteritis can also involve the pulmonary arteries; however, more commonly, it produces pulmonary artery narrowing and occlusion rather than dilation.21, 22 In patients with Takayasu arteritis with extensive damage to the pulmonary vascular bed who develop PAH, pulmonary artery dilation and aneurysm may occur.23, 24
BD is a multisystem inflammatory disorder characterized
Connective Tissue Abnormalities
Aneurysmal dilation of the pulmonary arteries associated with soft tissue disorders is much less common than aneurysm of the aorta and systemic arteries. Pulmonary artery dilation can be seen in as much as 75% of patients affected by Marfan syndrome, both at the level of the aortic root as well as at the level of the pulmonary artery bifurcation (Fig. 12).37 Even though frank PAA in Marfan have been reported, including multiple pulmonary aneurysm in the same patient, this is an uncommon finding
Infection
Both true and false aneurysms can develop as infectious or mycotic aneurysms of the lung. Aggressive and virulent organisms (ie, staphylococcus) produce tissue necrosis and destruction involving all layers of the arterial wall, resulting in the formation of pseudoaneurysms, whereas indolent organisms more often cause true aneurysms, in which the arterial wall is less severely damaged (ie, syphilis). The 3 main pathogenic mechanisms responsible for the formation of mycotic aneurysms are
Malignancy
Pulmonary pseudoaneurysms associated with lung cancer are rare, and the majority of pulmonary pseudoaneurysms are secondary to squamous cell carcinoma, in patients who typically present with massive hemoptysis. Direct tumoral invasion and erosion of the arterial wall results in arterial rupture, that when contained will manifest as a pseudoaneurysm. Noncontained rupture with massive bleeding if left untreated is associated with high mortality.57, 58, 59, 60, 61, 62 On contrast-enhanced CT, the
Trauma
Penetrating and nonpenetrating trauma may result in pulmonary artery or vein pseudoaneurysm when there is tear or disruption of all layers of the vessel wall with the extravasated blood contained by clot and extravascular tissue.66 Despite the large number of patients with chest trauma, posttraumatic pulmonary pseudoaneurysms are uncommon even after penetrating lung injury. In a series of 22 patients with penetrating lung injury, 15 of them with gunshot wounds and 8 with stab wounds who
Iatrogenic
Iatrogenic perforation of a pulmonary artery may also result from the use of flow-directed pulmonary artery catheters (also called Swan–Ganz catheters), widely used in the critically ill patients. Complications are rare but may result in pulmonary hemorrhage, thrombosis, pseudoaneurysm, pulmonary infarction, and death. Pulmonary artery rupture occurs in 0.03%-0.5% of patients, with a mortality rate as high as 70%.75 Contained rupture results in a pseudoaneurysm, which occurs in 0.16% of the
Bronchial Artery Aneurysms
BAAs may be mediastinal or intrapulmonary in location. Different congenital and acquired medical conditions are associated with BAA. Pulmonary TB and atypical mycobacterial infection are some of the most common causes of BAA, particularly in developing countries. They are reported in about 1% of all selective bronchial arteriograms and in 7% of patients with TB presenting with hemoptysis.52, 81 Other causes of BAA include bronchiectasis, pulmonary sequestration, pulmonary agenesis, sarcoidosis,
Pulmonary Venous Aneurysms
Pulmonary vein aneurysms can present as an isolated finding or in association with other cardiopulmonary diseases. A localized aneurysmal dilatation of a pulmonary vein, also referred to as a pulmonary varix in the literature, can be an incidental finding in healthy individuals or be associated with conditions like pulmonary venous hypertension, mitral regurgitation, acquired and congenital heart disease, and anomalous pulmonary venous return. They have been reported also in association with
Conclusions
Although uncommon, pulmonary aneurysms and pseudoaneurysms are important vascular abnormalities with significant morbidity and mortality and often reflect the presence of a serious underlying medical condition. Catheter angiography has been largely replaced by noninvasive cross-sectional imaging in the examination of pulmonary vascular diseases. MDCT in particular plays an important role in detecting and characterizing the different types of aneurysms of the bronchopulmonary vasculature. MDCT
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2022, Respiratory Medicine Case ReportsCitation Excerpt :Other causes of PAPAs have been described in several case reports. These include trauma, iatrogenic due to catheters, lung cancers, pulmonary embolism, vasculitis, traction bronchiectasis and pulmonary fibrosis among others [2,6]. COVID-19 has been linked to a vasculitis-like process due to upregulation of proinflammatory mediators and endothelial damage and dysfunction.