pontaneous coronary artery dissection (SCAD) is a nonatherosclerotic disease of the coronary arteries and usually presents as acute coronary syndrome. 1,2 The pathogenesis of SCAD involves development of a dissection plane within the coronary artery wall with concurrent intramural hematoma and/or intimal flap, ultimately obstructing flow and causing myocardial ischemia and infarction. Spontaneous coronary artery dissection was previously considered rare, with only individual cases and small case series published. [3][4][5] However, the advent of dedicated online SCAD patient communities and social media communication has led to increased awareness of the condition and robust patient-initiated research activity. More recent studies 6,7 with advanced intracoronary imaging suggest that 1% to 4% of patients with acute coronary syndrome may have SCAD. More importantly, SCAD may be responsible for one-third of the acute coronary syndromes in females younger than 50 years and is the most common cause of peripartum myocardial infarction. [6][7][8][9] The emerging recognition that both the treatment and outcomes of SCAD differ from those of acute coronary syndrome caused by atherosclerosis 1,10,11 highlights the need to increase awareness and diagnostic acumen among health care professionals.Spontaneous coronary artery dissection occurs most commonly in young women with no or minimal cardiovascular risk factors. The exact pathophysiologic mechanisms associated with SCAD are unclear, but associations with the peripartum period, extreme exercise or emotion, connective tissue disorders, and fibromuscular dysplasia have been reported. 1,2,12 Because the estimated 10-year incidence of major adverse cardiovascular events, including death, recurrent SCAD, heart failure, and myocardial infarction after initial SCAD, is high (47%), 1 identification of novel risk factors is critical. To our knowledge, the significance of family history in SCAD has not been previously reported.
MethodsThis study was approved by the Mayo Clinic institutional review board. Written informed consent was obtained for participation in the "Virtual" Multicenter Mayo Clinic SCAD Registry, 1,13 which included obtaining medical records from other institutions. We reviewed the medical records of 412 participants with SCAD in the Registry for a history of SCAD or IMPORTANCE Spontaneous coronary artery dissection (SCAD) is a major cause of acute coronary syndrome in young women, especially among those without traditional cardiovascular risk factors. Prior efforts to study SCAD have been hampered by underrecognition and lack of registry-based studies. Risk factors and pathogenesis remain largely undefined, and inheritability has not been reported.OBSERVATIONS Using novel research methods, patient champions, and social media, the Mayo Clinic SCAD Registry has been able to better characterize this condition, which was previously considered rare. Of 412 patient enrollees, we identified 5 familial cases of SCAD comprising affected mother-daughter, identical twin sis...