Two separate episodes of spontaneous coronary artery dissection in a patient with familial spontaneous coronary artery dissection background: a case report.

Background: Spontaneous coronary artery dissection (SCAD) is a potentially life-threatening condition in patients with acute coronary syndrome (ACS). Individuals with a family history of SCAD are rare, and genetic testing in monozygotic twin sisters has not been previously reported. Case summary: This case report describes a 51-year-old woman presenting with signs of ACS. Three years prior to the present admission, she was diagnosed with SCAD in the right coronary artery. The recent episode, precipitated by intense emotional stress, resulted in a dissection of the left circumflex artery, complicated by in-hospital cardiac arrest. The patient underwent conservative management, and a secondary prophylactic, implantable cardioverter-defibrillator was implanted before discharge. At follow-up, computed tomography angiography showed features indicative of fibromuscular dysplasia, but genetic testing for possible hereditary connective tissue or syndromic conditions showed no abnormalities. The patient's fraternal aunt has a history of SCAD. Furthermore, the patient has a monozygotic twin sister who prior to her death was diagnosed with SCAD on three separate occasions. Previous genetic investigations were normal. The sister died recently due to her third SCAD event. Discussion: While a thorough clinical assessment for rare connective tissue disorders is required for all SCAD patients, the present case indicates that evaluation with broader genetic panels is needed in patients with recurrent or familial SCAD.