LATE PRESENTATION OF A RARE CONGENITAL CARDIOPULMONARY DEFECT: SCIMITAR SYNDROME

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Scimitar syndrome (SS) is a rare congenital disease. It characterized by abnormal drainage of right pulmonary vein into inferior vena cava or atrium instead left atrium. commonly associated with hypoplastic lung and artery as well dextroposition the heart. The name derived from radiographic manifestation anomalous resembling Turkish sword. CASE PRESENTATION: A 45 years old woman history obstructive sleep apnea, hypertension, morbid obesity, recurrent mild respiratory infections since childhood. She complained cough dyspnea on exertion for four months. Over that period, she was repeatedly treated several courses antibiotics steroids. During this also gained 40 pounds. With progression shortness breath need supplemental oxygen, referred to pulmonology clinic. Chest CT contrast reported prominent draining junction atrium, compatible Syndrome. On further workup, PFT showed moderate restrictive pattern, echocardiogram evidence hypertension. then tertiary center eventually underwent surgical anastomosis via present atrial septal defect patch enlargement ostium scimitar at its entrance, tricuspid valve repair commissuroplasty. After surgery, her improved significantly having rest only activity. oxygen weaned continuous 2 liters nasal canula. continues improve cardiopulmonary rehabilitation has recently returned work. DISCUSSION: SS accounts 0.5-1 percent all heart disease cases. prevalence estimated be 1-3 every 100,00 livebirths. Two types have been described, infantile adult forms. Infantile form usually other thoracic vascular abnormalities. Adult smaller shunt more isolated. Atrial most common cardiac anomaly in both Echocardiogram color doppler studies computed tomography are mainstay diagnostic tools. Most patients asymptomatic birth. Clinical symptoms similar ASD. Exercise intolerance complaint. Depending volume, may range completely infections. CONCLUSIONS: rarely seen adulthood can progressive Many proposed. repairs designed divert connection between veins systemic drain flow REFERENCE #1: Vida, Vladimiro L., Alvise Guariento. "A sword threating heart: syndrome." JTCVS Techniques (2020). #2: Gupta, Manohar Lal, Rajeev Bagarhatta, Jyotsna Sinha. "Scimitar syndrome: unusual presentation." Lung India: official organ Indian Society 26.1 (2009): 26. DISCLOSURES: No relevant relationships Muneer Al Zoby, source=Web Response Thomas Marnejon, Abolfazl Sodagar,