SESSION TITLE: Medical Student/Resident Genetic and Developmental Disorders Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Cor triatriatum sinister (CTS) is a rare congenital cardiac condition characterized by the division of the left atrium by an abnormal fibromuscular membrane potentially causing obstruction of blood flow within the left atrium. The condition is found in less than 0.1% of patients diagnosed with cardiomyopathies and typically diagnosed in childhood. We describe a case of an incidentally diagnosed CTS in an adult. CASE PRESENTATION: A 45-year old male with a past medical history of supraventricular tachycardia and coronary artery disease status post percutaneous stenting was admitted to the hospital for evaluation of shortness of breath, productive cough, fever and chills. On chest x-ray, there was evidence of a left lower lobe pulmonary infiltrate and he was treated for community acquired pneumonia with ceftriaxone and azithromycin. His hospital course was complicated by hypoxemic respiratory failure. Echocardiography was performed, due to persistent tachycardia and shortness of breath and he was found to have CTS (Figure 1). On further questioning he declined a history of dyspnea on exertion, orthopnea or other respiratory complaints since childhood. He was discharged home after resolution of acute community-acquired pneumonia. DISCUSSION: There are three theories on the embryology of CTS; malincorporation, malseptation and entrapment. Among these, malincorporation of the common pulmonary vein into the left atrium creating two chambers separated by a narrowing chamber, is the most accepted theory. Up to 80% of cases are associated with other congenital abnormalities and presentation varies from asymptomatic patients to septic shock with pulmonary edema and respiratory failure. In adults, asymptomatic patients likely have a large foramen (fenestrations) with no intra-atrial pressure gradient unlike symptomatic patients who have no means for compensation and develop elevated left atrial pressure and subsequent pulmonary congestion. On physical exam, no pathognomonic findings are seen, and the absence of an opening snap or loud S1 differentiates the murmur of CTS from mitral stenosis. Echocardiography is a simple, non-invasive tool that can delineate the morphology and diagnose CTS as well as assess severity, describing severe obstruction when the doppler velocity gradient is greater than 2 m/s. Further studies are not needed, but cardiac catheterization, and cardiac magnetic resonance imaging can be useful for diagnosis and management. Management is based on symptoms and ranges from no treatment, to preload reducing agents, to surgical intervention. CONCLUSIONS: Establishing the diagnosis is critical as untreated, hemodynamically-significant CTS can lead to arrhythmias, pulmonary hypertension and right-sided heart failure that can be prevented with favorable outcomes after surgical correction. We present this case due to its rarity and asymptomatic nature in an adult patient. Reference #1: Nassar PN, Hamdan RH. Cor triatriatum sinistrum: classification and imaging modalities. The European journal of cardiovascular medicine. 2011;1(3):84. Reference #2: Ather B, Siddiqui WJ. Cor Triatriatum. In: StatPearls [Internet]. StatPearls Publishing; 2019. Reference #3: Chen Q, Guhathakurta S, Vadalapali G, Nalladaru Z, Easthope RN, Sharma AK. Cor triatriatum in adults: three new cases and a brief review. Tex Heart Inst J. 1999;26(3):206-210. DISCLOSURES: No relevant relationships by Israel Acosta Sanchez, source=Web Response No relevant relationships by Floyd Burke, source=Web Response No relevant relationships by Somshukla Ghosh, source=Web Response No relevant relationships by Hiren Patel, source=Web Response No relevant relationships by Irene Riestra Guiance, source=Web Response
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