
Patient Background:
A 39-year-old male presented with exertional dyspnea, orthopnea, and shortness of breath. He was an active smoker with a history of pulmonary edema 10 months prior. On admission, his blood pressure was 120/70 mmHg, and he was receiving bisoprolol, ramipril, and furosemide. Though family history was unspecified, aortic dilation prompted consideration of first-degree relative screening, as recommended in bicuspid valve disease.
Assessment & Diagnosis:
Transthoracic echocardiography revealed severe aortic regurgitation (grade 4+), a left ventricular end-diastolic diameter of 65 mm, and preserved ejection fraction of 56%. The ascending aorta measured 54 mm. Preoperative imaging suggested a bicuspid aortic valve; however, intraoperative inspection revealed a rare pentacuspid aortic valve with five variably sized cusps, some exhibiting partial fusion. The final diagnosis was severe aortic regurgitation and ascending aortic aneurysm due to a pentacuspid valve anomaly.
- Why is pentacuspid aortic valve important in the workup of severe AR?
- How does early surgical intervention influence long-term outcomes in such patients?

