Azaje Andinet, Abebe Alula, Fentahun Shibabaw, Molalegn Befekadu, Tadesse Abilo
Department of Internal Medicine, School of Medicine, College of Medicine and Health Sciences, University of Gondar, Gondar, Ethiopia.
SAGE Open Med Case Rep. 2023 Jul 29;11:2050313X231189772. doi: 10.1177/2050313X231189772. eCollection 2023.
Single atrium is a rare congenital heart disease characterized by complete absence of the interatrial septum. It occurs as an isolated cardiac defect, or as a part of skeletal, muscular, ophthalmologic, and vascular malformations to signify congenital syndromes. A 78-year-old male patient presented with generalized body swelling of 2 weeks duration. He had associated shortness of breath, orthopnea, productive cough, and palpitation. Upon physical examination, blood pressure = 150/75 mmHg, pulse rate = 50 bpm, respiratory rate = 24 bpm, and ° = 36.7 °C. He had signs of pleural effusion on left lung field. Cardiovascular examination revealed mean heart rate of 50 beats/min with irregularly irregular pulse rhythm, raised jugular venous pressure, and pansystolic murmur at left lower sternal border. He had tender hepatomegaly, ascites, and pitting leg edema. Chest X-ray showed cardiomegaly, prominent pulmonary trunks, and left-sided pleural effusion. Electrocardiography revealed atrial fibrillation, bifascicular block (right bundle branch block and left anterior fascicular block) with mean heart rate of 50 beats/min. Two-dimensional transthoracic echocardiography showed complete absence of interatrial septum (single atrium) without atrioventricular defect and interventricular communication. Diagnosis of heart failure secondary to congenital heart disease (single atrium) with atrial fibrillation and bifascicular block was made based on clinical and imaging evaluation. Well-tolerated symptoms of a single atrium until late adulthood could be explained by the presence of streaming or incomplete mixing of blood within the atrium, in which the more oxygenated blood is directed to the systemic circuit.
单心房是一种罕见的先天性心脏病，其特征为房间隔完全缺失。它可作为孤立的心脏缺陷出现，也可作为骨骼、肌肉、眼科和血管畸形的一部分，提示先天性综合征。一名78岁男性患者出现全身肿胀2周。他伴有呼吸急促、端坐呼吸、咳痰和心悸。体格检查时，血压 = 150/75 mmHg，脉搏率 = 50次/分，呼吸率 = 24次/分，体温 = 36.7℃。左肺野有胸腔积液体征。心血管检查显示平均心率为50次/分，脉律绝对不齐，颈静脉压升高，左胸骨下缘全收缩期杂音。他有压痛性肝肿大、腹水和凹陷性腿部水肿。胸部X线显示心脏增大、肺动脉主干突出和左侧胸腔积液。心电图显示心房颤动、双分支阻滞（右束支阻滞和左前分支阻滞），平均心率为50次/分。二维经胸超声心动图显示房间隔完全缺失（单心房），无房室缺损和室间隔交通。根据临床和影像学评估，诊断为先天性心脏病（单心房）继发心力衰竭，伴有心房颤动和双分支阻滞。单心房在成年后期症状耐受性良好，这可能是由于心房内存在血流或血液不完全混合，其中氧合较好的血液流向体循环。
