(240) Congenital Aortic Stenosis Unleashing as Flash Pulmonary Edema in a 22-Year-Old Female

Introduction: Aortic stenosis secondary to unicuspid aortic valve, affects ~0.02% of adults, (M/F: 4:1 ratio), and usually presents around 40-50 years of age with syncope and exercise intolerance. Post-surgery survival rates are ~97% at 5 and 10 years. It has been reported to cause flash pulmonary edema and mimic type 2 MI in elderly individuals; life-threatening presentations in young adults are rare

Description: A 22-year-old female with no significant past medical history presented with shortness of breath and throat pain lasting one day. On arrival at the ER, she was hypotensive and in severe respiratory distress, with an SpO2 of 70% on room air, and was intubated and sedated. Chest X-ray suggested flash pulmonary edema, and EKG revealed atrial flutter with RVR. Laboratory results showed severe respiratory acidosis (pH 7.16, PO2 51 mmHg, PCO2 55 mmHg), an initial troponin level of 300 ng/L, and a BNP of 708 pg/mL. CT chest indicated multilobar diffuse infiltrates, raising concerns for pulmonary edema or pneumonia. Bedside echocardiography showed normal left ventricular ejection fraction and no pericardial tamponade.

Emergent bronchoscopy revealed pink frothy fluid, consistent with acute pulmonary edema. The patient was admitted to the ICU, requiring vasopressors and sedation with propofol and fentanyl. Serial troponin levels rose to 6000 ng/L, and repeat EKGs showed significant ST changes in V1-V2 with reciprocal changes in V4-V6. Formal echocardiogram identified severe aortic stenosis with a unicuspid aortic valve, an aortic valve area of less than 0.25 cm², and a transvalvular gradient of 84 mmHg with a peak gradient of 145 mmHg. Cardiothoracic surgery was consulted, and she underwent valve repair with mechanical valve placement

Discussion: While severe congenital AS is diagnosed in infancy, subclinical AS presenting with pulmonary edema in young adults is less known. In such cases, diuresis can be harmful due to preload dependence from both aortic stenosis and demand ischemia. Atrial flutter, which deprives the left ventricle of the crucial "atrial kick," can worsen the situation. The thickened aortic valve can narrow the coronary ostium, exacerbating myocardial ischemia. This case was managed with a short course of pressors, one day of diuresis, and emergent surgical valve replacement.