Coronary artery dissection in alpha‐1‐antitrypsin deficiency

Pregnancy-related spontaneous coronary artery dissection: Two case reports and a comprehensive review of literature

“…Other factors such as use of oral contraceptives, immunosuppressant drugs, drug reactions, blunt trauma to chest,[27] type IV Ehlers Danlos syndrome[28] Marfan's syndrome,[29] and α1-antitrypsin deficiency[30] have also been described as possible mechanisms leading to arterial dissection. [31–34] Crystal medial necrosis has also been implicated in SCAD.…”

Echocardiographic evaluation in subjects with α1‐Antitrypsin deficiency

“…The most recognized pathological feature of AATD consists of emphysema-related COPD, but the possible involvement of the cardiovascular system is the hypothesis generating this study. Some reports of ruptured mesenteric aneurysm and cerebral, carotid or coronary arteries dissection in patients with AATD suggest a possible link between these lesions and damage of elastic fibres of arterial walls due to an abnormal equilibrium between elastase and AAT [2][3][4][5][6][7][8][17][18][19][20][21]. Although the pathogenesis of these associate vascular disorders is not clearly established, the absence or reduced activity of AAT, which usually acts as a serum protease inhibitor, seems to compromise the vascular wall integrity.…”

“…Although the pathogenesis of these associate vascular disorders is not clearly established, the absence or reduced activity of AAT, which usually acts as a serum protease inhibitor, seems to compromise the vascular wall integrity. Thus, an underlying arteriopathy in AATD patients has been claimed as a potential determinant of such complications . Therefore, in the vascular wall, AATD appears to promote connective tissue degeneration which was erroneously believed to be limited to lung parenchyma.…”

“…Involvement of the vascular system has been also suggested with derangement of blood vessels wall. Actually, abdominal aortic aneurysm , dissection of the descending aorta , left ventricular (LV) pseudoaneurysm , dissection of coronary and cervical arteries and intracerebral aneurysms have been described in AATD patients. Recently, we performed an echocardiographic study in a relatively large cohort of ZZ genotype AATD subjects to investigate the properties of ascending aorta, and incidentally, we observed an increase in the LV mass as compared to normal controls .…”

Aortic degeneration in α1‐antitrypsin deficiency

“… 2 Clinical observations include the report of a ruptured mesenteric aneurysm with α1‐antitrypsin deficiency emphysema, 3 and carotid, cerebral, or coronary artery dissections. 4–6 An experimental model 7 perfusing the aortas of rats with pancreatic elastase to produce medial elastin degeneration is also known to result in experimental emphysema, suggesting that elastolytic activity causes elastin degeneration in both vessels and lung parenchyma. Further investigation is merited, and we suggest that strict control of hypertension in patients with α1‐antitrypsin deficiency may decrease the risk of aortic artery dissection.…”