UNASSIGNED: A 75-year-old male with a diagnosis of pulmonary sarcoidosis was referred to the Cardiology department due to heart failure with reduced left ventricular ejection fraction (LVEF). The transthoracic echocardiogram revealed mildly reduced LVEF, aortic root and pulmonary artery (PA) dilatation, and no signs of pulmonary hypertension (PH). Cardiac magnetic resonance imaging was performed, revealing mild left ventricular dilation, LVEF of 40%, main PA dilation (43 mm) and a pattern of late gadolinium enhancement suggestive of cardiac sarcoidosis. At follow-up, a thoracic computed tomography (CT) angiography scan revealed ascending aorta ectasia and giant main PA aneurysm (60 mm). A right heart catheterisation was performed, and a mean PA pressure of 34 mmHg was obtained. Given the clinical context, the patient was considered to have PH due to lung disease and left heart disease, and PAA was possible due to vascular granulomatous involvement by sarcoidosis.
UNASSIGNED: PAA is a rare finding and mostly occurs in the setting of PH. Sarcoidosis is a granulomatous disease that mostly affects the lungs, but the sarcoid involvement of great vessels has been described. In this clinical case, the probable cause for the PA fragility leading to aneurysm formation remains sarcoid vascular infiltration, regarding the discrepancy between the PA dimensions and mildly elevated PA pressure.
CONCLUSIONS: Pulmonary artery aneurysm is a rare abnormality of pulmonary vasculature that can be idiopathic or a consequence of pulmonary hypertension, congenital heart disease, infection, vasculitis or collagenopathies.Due to the low incidence of this disease, there are no guidelines for its diagnosis, management or follow-up, and treatment is based on the underlying aetiology, aneurysm dimensions and occurrence of symptoms.Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas that mostly involve the lungs, but can also affect skin, eyes, and lymph nodes. Sarcoid involvement of great vessels has been rarely described.
■一名诊断为肺结节病的75岁男性因左心室射血分数(LVEF)降低的心力衰竭被转诊到心脏科。经胸超声心动图显示LVEF轻度降低,主动脉根部和肺动脉(PA)扩张,没有肺动脉高压(PH)的迹象。进行了心脏磁共振成像,显示轻度左心室扩张,LVEF为40%,主要PA扩张(43毫米)和钆晚期增强模式提示心脏结节病。在后续行动中,胸部计算机断层扫描(CT)血管造影扫描显示升主动脉扩张和巨大的主PA动脉瘤(60mm)。进行了右心导管插入术,并且获得34mmHg的平均PA压力。鉴于临床背景,患者被认为是由于肺部疾病和左心疾病导致的PH,结节病可能是血管肉芽肿累及PAA。
■PAA是一种罕见的发现,主要发生在PH的设置中。结节病是一种肉芽肿性疾病,主要影响肺部,但是已经描述了大血管的结节病。在这个临床病例中,PA脆性导致动脉瘤形成的可能原因仍然是结瘤样血管浸润,关于PA尺寸和轻度升高的PA压力之间的差异。
结论:肺动脉瘤是一种罕见的肺血管异常,可以是特发性或肺动脉高压的结果,先天性心脏病,感染,血管炎或胶原病。由于这种疾病的发病率低,它的诊断没有指导方针,管理或后续行动,治疗是基于潜在的病因,动脉瘤的尺寸和症状的发生。结节病是一种病因不明的多系统疾病,其特征是主要累及肺部的非干酪样肉芽肿。但也会影响皮肤,眼睛,和淋巴结。很少描述大血管的结节病参与。