Case Presentation: A 32 year old South Asian man with a history of hyperlipidemia presented to the emergency department (ED) for severe, crushing, mid-sternal chest pain associated with nausea and diaphoresis that awoke him from sleep. In the ED, he was found to have a troponin elevation to 1.159 ng/ml (normal < 0.055 ng/ml) and initial electrocardiogram (ECG) with sinus rhythm and Q waves in II, III, and AvF suggestive of prior completed inferior infarct. Given high concern for acute coronary syndrome, he was started on aspirin, ticagrelor, and heparin infusion. Shortly after admission, his heart rate dropped to 59 and ECG showed junctional escape rhythm and new inferior infarct. He went for urgent cardiac catheterization which revealed aneurysmal changes of all coronaries and an acute 100% thrombotic occlusion of the proximal right coronary artery (RCA). RCA aspiration thrombectomy was performed with placement of drug-eluting stent achieving TIMI Grade 3 flow. Upon further history, the patient recalled a hospitalization for febrile illness at age 5, making his presentation most consistent with cardiac sequelae of Kawasaki Disease. A full rheumatological and infectious workup including human immunodeficiency virus, viral hepatitis, tuberculosis, lupus, antiphospholipid antibody, antineutrophil cytoplasmic antibodies, and coagulation studies was unrevealing. He was discharged with apixaban, ticagrelor, and rosuvastatin and was able to attend his wedding two days after discharge.
Discussion: Acute coronary syndrome (ACS) in young adults is a rare but life-threatening cause of chest pain, and its differential diagnosis should be familiar to hospital clinicians. Common causes of ACS in young adults includes Kawasaki Disease (KD), cocaine use, spontaneous coronary artery dissection (SCAD), connective tissue disorders (CTD), pro-coagulant states due to medications or Factor V Leiden, and early-onset atherosclerosis due to severe dyslipidemia syndromes. KD is a childhood vasculitis, typically presenting with fever that does not abate with antibiotics, cervical lymphadenopathy, conjunctivitis, and a maculo-papular erythematous rash over the lips, palms, and soles. Intravenous immunoglobulin given at the time of presentation reduces duration and serious sequelae of disease, such as cardiac involvement. Coronary artery aneurysm occurs in 20-25% of patients and predisposes to coronary artery disease, calcification, and thrombus formation leading to STEMI. In a long-term study of patients with coronary aneurysms, 35% regress without complications, while 65% require cardiac intervention such as percutaneous intervention or bypass. Patients benefit from aggressive risk factor reduction such as lipid and glycemic control, exercise, weight management, and abstaining from tobacco. Coronary artery bypass is the definitive treatment of KD-related coronary aneurysmal disease.
Conclusions: It is essential for hospitalists to recognize ACS as a life threatening cause of chest pain in young adults, especially since many patients will require life-long management of their underlying disease. The differential diagnosis of ACS in young adults differs from that of older adults and includes drug use, SCAD, CTD, coagulopathies, and sequelae of Kawasaki Disease, as in this case.
