Nosology spectrum of the bicuspid aortic valve condition: Complex-presentation valvulo-aortopathy

Patient presentation: A 24-year-old man, who smoked 1 pack a day of cigarettes, presented to the emergency department with sudden onset of 7/10 chest pain radiating to the back. His medical history was significant for anxiety and depression and a bicuspid aortic valve (BAV) diagnosed at age 5 years because of an auscultatory click. Outside transthoracic echocardiogram 3 years previously had shown normal valve function and aortic sinuses (root) of 42 mm. His family history was notable for a mother and uncle with BAV and aortic dilatation, but there was no early sudden cardiac death or aortic dissection in the family. The patient denied illicit drug use. On examination, he was in mild distress from chest pain. His heart rate was 75 beats/minute, his blood pressure was 124/72 mm Hg and equal in both arms, and room air oxygen saturation was 96%. Physical examination was unremarkable with normal cardiac auscultation. The ECG showed an early repolarization pattern and troponins were undetectable. A triplerule-out computed tomography angiogram (CTA) showed no evidence of pulmonary embolus, coronary disease, or aortic dissection. The aortic root measured 48 mm with asymmetric noncoronary sinus dilatation (Figure 1) and no aortic coarctation. The remainder of the thoracic aorta was normal. After resolution of chest pain had occurred with analgesics administration, the patient was discharged and scheduled for outpatient cardiology follow-up in 2 weeks. The cause of his chest pain remained unknown.