Abstract
Mitral valve prolapse is not commonly on the list of differential diagnosis when a patient presents in the emergency department (ED) in severe distress, presenting with non-specific features such as abdominal pain, tachycardia and dyspnea. A healthy 55-year-old man without significant past medical history arrived in the ED with a unique presentation of a primary mitral valve prolapse with an atrial septal defect uncommon in cardiology literature. Early recognition of mitral valve prolapse in high-risk patients for severe mitral regurgitation or patients with underlying cardiovascular abnormalities such as an atrial septal defect is crucial to prevent morbid outcomes such as sudden cardiac death.