Published 2023-11-15
The following article contains content based on the documents:
First International Consensus on the diagnosis and management of fibromuscular dysplasia (link) and Diagnostics and management of spontaneous coronary artery dissection. Opinion of experts from the Association of Cardiovascular Interventions PTK (link).
Any updates to the article below can be found at the links provided. The article presented below contains numerous quotations and abbreviations of the above documents in order to provide information about the disease fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection (SCAD). Our intention is to provide the readers with detailed and verified information, based on reliable scientific sources, about both fibromuscular dysplasia and spontaneous coronary artery dissection. All presented graphics also come from the documents indicated in the links above and from the database of the Culture Fly! Foundation.
Author of the article: Beata Budzkowska, Culture Fly! Foundation.
Medical consultation: dr hab. n.med. Jacek Kądziela, National Institute of Cardiology in Warsaw.
What is FMD?
FMD (Fibromuscular Dysplasia), Fibromuscular dysplasia is an arterial disease without inflammation or atherosclerosis, manifested by an abnormal structure of the artery wall. FMD manifests primarily as bead-shaped (multifocal) or focal lesions in medium-sized or small arteries. FMD most commonly affects the renal arteries and the extracranial carotid and vertebral arteries, but almost all arterial beds can be involved, and involvement of multiple vessels is quite common.
Approximately 80–90% of FMD patients are women.
Although less common, FMD also develops in men and may have a more aggressive course with a higher incidence of aneurysms and dissections.
The causes of FMD are currently unknown, it is assumed that the development of dysplasia is probably related to a combination of genetic and environmental factors..
What is SCAD?
SCAD (spontaneous coronary artery dissection) is a rare cause of acute myocardial infarction. It mostly affects young and middle-aged women, with several percent of cases occurring during or shortly after pregnancy. SCAD is responsible for 10–25% of cases of acute myocardial infarction in women under 50 years of age, and for half of cases of infarcts occurring in the postpartum period. Unlike an atherosclerotic infarction, in which the main pathophysiological event is the formation of a thrombus in the lumen of the coronary artery at the site of rupture or erosion of the atherosclerotic plaque, flow disturbances in the coronary artery in SCAD result from external compression of the true blood flow channel by a hematoma growing in the artery wall (forming a “channel” false” and, as it were, “dissecting” the artery). This hematoma occurs either as a result of a rupture (dissection) of the inner layer of the artery and blood penetrating its wall (A) or as a result of spontaneous bleeding of small blood vessels located in the arterial wall (B).
The causes of SCAD are unknown.
Familial cases have been described and rare cases associated with inherited connective tissue disorders have been reported, but most cases of SCAD are sporadic. SCAD has been shown to be associated with increased coronary artery tortuosity. SCAD can usually be diagnosed angiographically by coronary angiography, although intracoronary imaging (e.g., optical coherence tomography) may be helpful in equivocal cases.
In most cases, arterial dissection heals spontaneously within a few weeks.
Although an association between FMD and SCAD has been established (i.e., the relatively common co-occurrence of FMD in SCAD survivors), it is more controversial, first, whether SCAD is a distinct disease entity or simply a clinical complication of FMD, and, second, whether there are specific coronary manifestations of FMD other than coronary artery dissection.
Doctors are still looking for answers to these questions.