Etiological classification and manifestation of pulmonary artery dissection: A literature review and case analysis

Qi-Xian Zeng; Qun-Ying Xi; Qin Luo; Zhi-Hong Liu; Chang-Ming Xiong

doi:10.1016/j.heliyon.2023.e22570

Etiological classification and manifestation of pulmonary artery dissection: A literature review and case analysis

Heliyon. 2023 Nov 24;9(12):e22570. doi: 10.1016/j.heliyon.2023.e22570. eCollection 2023 Dec.

Authors

Qi-Xian Zeng¹, Qun-Ying Xi², Qin Luo¹, Zhi-Hong Liu¹, Chang-Ming Xiong¹

Affiliations

¹ State Key Laboratory of Cardiovascular Disease, Center of Pulmonary Vascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, North Lishi Road, Xicheng District, No. 167, Beijing, 100037, China.
² Center for Pulmonary Vascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences, Shenzhen, No. 12, Langshan Road, Nanshan District, Shenzhen, 518057, China.

Abstract

Background: Pulmonary artery dissection (PAD) is an uncommon condition associated with high mortality rates. To date, a comprehensive examination of various underlying causes of PAD remains unexplored.

Methods: We used the search words "pulmonary artery dissection," "pulmonary artery dilatation," and "pulmonary artery rupture" in the public database, and examined the medical records of PAD patients treated in our hospital. Data on demographics, aetiology, PAD locations, histopathology, treatments, and outcomes, were collected.

Results: A total of 145 patients with PAD (135 cases from the literature and 10 cases from our hospital) were analysed. PAD aetiology was categorized into four groups: congenital heart diseases (CHD) associated with pulmonary arterial hypertension (PAH), non-CHD associated with pulmonary hypertension (PH), aortic dissection-related, and miscellaneous causes. The most frequent cause, accounting for 32.4 % of PAD cases, was congenital heart disease, followed by idiopathic PAH (13 %) and chronic obstructive pulmonary disease (6 %). Patients with CHD were typically younger at the time of PAD diagnosis (median age: 35 years old) when compared with those suffering from aortic dissection, PH-associated conditions, or other causes (median age: 45, 55, and 56 years old, respectively). Imaging of the pulmonary artery proved effective in diagnosing PAD. The outcomes were generally poor. 44.7 % (21/47) of patients with CHD associated with PAH and 47.7 % (21/44) of non-CHD PH-associated diseases died during follow-up. Multidisciplinary team consultations are crucial when making decisions on management of PAD. Characteristic PAD histopathology features included thickened intima and hypertrophied media with atheromatous degeneration, disrupted elastic fibres, and lymphocytic infiltration.

Conclusions: PAD aetiology can be divided into four main categories, with CHD associated with PAH being the leading cause. Despite the similar histopathology features, clinical manifestations and outcomes vary according to the aetiology.

Keywords: Aetiology; Pathology; Pulmonary artery dissection; Pulmonary hypertension; outcomes.

Publication types

Review