Cardiology Unit, Department of Medicine, Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife, Osun State, Nigeria.
Pan Afr Med J. 2022 Apr 28;41:342. doi: 10.11604/pamj.2022.41.342.29903. eCollection 2022.
Lutembacher syndrome (LS) is a rare syndrome comprising a combination of atrial septal defect (ASD) and mitral stenosis. We present the case of a 28-year-old man, who presented with progressively worsening dyspnea of 2 months associated with orthopnea, paroxysmal nocturnal dyspnea, bilateral leg swelling and productive cough. Chest X-ray revealed plethoric lung fields with prominent pulmonary conus and cardiomegaly. Transthoracic echocardiography revealed a large ostium secundum ASD with left to right shunt, mild mitral stenosis, severe mitral and tricuspid regurgitations and pulmonary hypertension. A diagnosis of Lutembacher syndrome in heart failure with pulmonary hypertension was made. The patient was managed conservatively, but declined surgery primarily because of financial reasons. This rare case of LS presenting with heart failure and complicated by pulmonary hypertension is the first reported case in our centre and our region. The patient's inability to afford the cost of definitive care posed a significant problem in his management.
卢特巴赫综合征(LS)是一种罕见的综合征,包括房间隔缺损(ASD)和二尖瓣狭窄。我们报告了一例 28 岁男性病例,该患者出现进行性恶化的呼吸困难,病程为 2 个月,伴有端坐呼吸、阵发性夜间呼吸困难、双侧腿部肿胀和咳嗽有痰。胸部 X 线显示肺部过度充气,肺动脉圆锥突出,心脏增大。经胸超声心动图显示继发孔 ASD 大,左向右分流,二尖瓣轻度狭窄,二尖瓣和三尖瓣重度反流和肺动脉高压。诊断为心力衰竭合并肺动脉高压的卢特巴赫综合征。患者接受了保守治疗,但主要由于经济原因拒绝手术。这例罕见的 LS 合并心力衰竭和肺动脉高压的病例是我们中心和我们地区首例报告。患者无力承担确定性治疗的费用,这在他的管理中构成了一个重大问题。