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先天性心脏病成年患者的肺动脉瘤样扩张

Pulmonary artery aneurysmal dilatation in adult patients with congenital heart disease.

作者信息

Martínez-Quintana Efrén, Rodríguez-González Fayna, Nieto-Lago Vicente

机构信息

Cardiology Service, Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Spain.

出版信息

World J Pediatr Congenit Heart Surg. 2011 Jul 1;2(3):375-9. doi: 10.1177/2150135111405075.

DOI:10.1177/2150135111405075
PMID:23803989
Abstract

BACKGROUND

Aneurysmal dilatation of the main pulmonary artery and its peripheral branches are rare lesions that account for less than 1% of all thoracic aneurysms and have a number of possible pathogenetic causes such as congenital heart disease (CHD), pulmonary artery hypertension, vasculitis, mycotic aneurysm, neoplasm, iatrogenic causes, trauma-related events, or connective tissue abnormalities.

METHODS

We conducted a retrospective review of the demographic data and the results of clinical examinations, laboratory tests, echocardiography, and angiography of patients managed at the Adult Congenital Heart Disease Unit of the Complejo Hospitalario Universitario Insular-Materno Infantil, between January 2004 and May 2010.

RESULTS

A total of 352 adult patients with CHD were studied. Of these, 8 (2.3%) patients had pulmonary artery aneurysmal dilatation (PAAD): 4 with low pressure of the pulmonary artery and 4 with pulmonary hypertension. Only 1 patient showed PAAD-related symptoms. Patients with CHD having PAAD had significantly higher prevalence of pulmonary artery hypertension and higher levels of C-reactive protein (CRP) than those without PAAD (incidence: 4 patients [50%] vs 18 patients [5.2%], P < .000; CRP in mg/dL: 0.52 [0.4; 1.2] vs 0.2 [0.0; 1.6], P = .016). No significant differences were found in cholesterol levels (total cholesterol, low-density lipoprotein [LDL], high-density lipoprotein [HDL], or triglycerides) between CHD patients with or without PAAD.

CONCLUSION

In patients with CHD, PAAD is a rare finding. The PAAD size, etiology, symptoms, and association with pulmonary hypertension should guide decisions on whether conservative or surgical treatment should be applied.

摘要

背景

主肺动脉及其外周分支的动脉瘤样扩张是罕见病变,占所有胸主动脉瘤的比例不到1%,其发病原因有多种,如先天性心脏病(CHD)、肺动脉高压、血管炎、霉菌性动脉瘤、肿瘤、医源性原因、创伤相关事件或结缔组织异常。

方法

我们对2004年1月至2010年5月期间在Insular-Materno Infantil大学综合医院成人先天性心脏病科接受治疗的患者的人口统计学数据以及临床检查、实验室检查、超声心动图和血管造影结果进行了回顾性分析。

结果

共研究了352例成年CHD患者。其中,8例(2.3%)患者存在肺动脉瘤样扩张(PAAD):4例肺动脉压力低,4例有肺动脉高压。只有1例患者出现与PAAD相关的症状。患有PAAD的CHD患者肺动脉高压的患病率和C反应蛋白(CRP)水平显著高于无PAAD的患者(发生率:4例[50%]对18例[5.2%],P <.000;CRP以mg/dL计:0.52[0.4;1.2]对0.2[0.0;1.6],P =.016)。有或无PAAD的CHD患者在胆固醇水平(总胆固醇、低密度脂蛋白[LDL]、高密度脂蛋白[HDL]或甘油三酯)方面未发现显著差异。

结论

在CHD患者中,PAAD是一种罕见的发现。PAAD的大小、病因、症状以及与肺动脉高压的关联应指导关于采用保守治疗还是手术治疗的决策。

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