Erlenmeyer-shaped Heart in a Patient with Giant Left Atrium due to Mixed Mitral Valve Pathology: A Neglected Case in a Rural Area in Indonesia

Abstract

Giant left atrium (GLA) is a rare condition typically found in patients with rheumatic mitral valve disease. On chest radiographs, this condition often mimics another cause of cardiomegaly. A 52-year-old man was admitted to the hospital with complaints of shortness of breath and orthopnea. Mid-diastolic and grade IV pansystolic murmurs could be heard at the apex radiating to the axilla on cardiac auscultation. The electrocardiogram showed a junctional tachycardia rhythm, while the chest radiograph displayed extreme cardiomegaly with Erlenmeyer-shaped heart. Massive pericardial effusion was suspected. Transthoracic echocardiography (TTE) was then performed, revealing GLA with a left atrial volume index (LAVi) of 1101 ml/m2. Meanwhile, transoesophageal echocardiography (TEE) confirmed calcification of P2 and P3 extending to the border of the mitral leaflet up to one third of the chordae tendineae, with moderate mitral stenosis and eccentric mitral regurgitation jet due to prolapse of A2. The patient was treated with optimal medical therapy while awaiting cardiac surgery. GLA is a rare complication of mitral valve disease and an almost forgotten cause of cardiomegaly. Diagnostic imaging procedures with echocardiography are a less invasive and readily available modality to establish the diagnosis of cardiomegaly.