A 48-year-old woman was admitted to the emergency department with sudden onset severe chest tightness whilst doing yoga. This was associated with pins and needles in both arms, nausea, and abdominal discomfort. It lasted for 1 hour before it self-resolved and was not related to exertion. She reported being under increased emotional stress in the preceding month prior to presentation. She had no significant past medical history and was not on any regular medications.
She had a mother who died suddenly from an ascending aortic dissection in her 50s. Her mother was not hypertensive and suffered no symptoms or comorbidity suggestive of systemic illness.
The patient was hypotensive with a blood pressure of 90/60 mmHg and a heart rate of 80 bpm with no respiratory compromise or fever. Her troponin I (high-sensitivity assay) was 54 ng/dl, 79 ng/dl, and 27 ng/dl, respectively. Her electrocardiogram (ECG) showed sinus bradycardia with no ischaemic changes. A CT pulmonary angiogram was performed and excluded pulmonary embolus and showed no other cause for chest pain. An echocardiogram showed preserved biventricular function with no significant valvulopathy and normal ascending aorta dimensions. A coronary angiogram performed on day 2 of admission showed type 1 distal left anterior descending (LAD) coronary artery dissection with thrombolysis in antiplatelets and myocardial infarction (TIMI) 3 flow (Figure
(a–c) Type 1 spontaneous coronary artery dissection (SCAD) (contrast dye staining of the arterial wall with multiple arterial lumens) in the distal LAD. Increased arterial tortuosity
Spontaneous coronary artery dissection (SCAD) is a cause of acute coronary syndrome (ACS) varying in severity from unstable angina to sudden cardiac death. This is the only case report to our knowledge of a patient with SCAD having a first-degree relative with aortic dissection. Twenty percent of patients with aortic dissection will have an underlying connective tissue disease [
The incidence of patients with SCAD having fibromuscular dysplasia (FMD) has been reported as 74% [
Marfan syndrome (MFS) has an incidence of 1/10000 per year [
Vascular Ehlers Danlos (vEDS) syndrome causing SCAD is extremely rare; we have identified seven case reports in the literature, including a patient in pregnancy with SCAD being the first presentation of vEDS [
Some inflammatory conditions such as systemic lupus erythematosus (SLE) have been associated with SCAD in case reports, but this is highly uncommon; it is thought that 5% of SCAD have underlying inflammatory connective tissue disease [
Loeys-Dietz syndrome is a rare connective tissue disorder that is caused by autosomal dominant inheritance of TGFBR-1 mutation that causes defects in elastogenesis leading to arterial tortuosity, aneurysm, and rupture. This affects any segment of the aorta, but has rarely been described in case reports to affect the coronary artery. Other associated features include cleft palate/uvula, hypertelorism, craniosynostosis, and other congenital heart defects such as ASD or bicuspid aortic valve [
Our case shows a presentation of SCAD with an unusual family history. The main learning points from this case are that a high index of suspicion of SCAD is needed in a young patient presenting with ACS and that connective tissue disease and extracoronary arterial screening needs to be considered in these situations. There is no obvious disease that links both presentations although we propose FMD as the most likely culprit. It is clear from literature review that if the predisposition to dissection was inherited from mother to daughter, one of them suffered a rare manifestation of their underlying disease. SCAD is a life-changing event for the patient due to the complications of myocardial infarction and the threat of lifelong recurrence.
Consent has been obtained from the patient prior to submission for publication.
Neither author has any conflict of interest to declare and have not received any funding.