Meriç Murat, Yüksel Serkan
Department of Cardiology, Medicine Faculty of Ondokuz Mayıs University, Samsun, Turkey.
Turk Gogus Kalp Damar Cerrahisi Derg. 2018 Sep 16;26(4):649-652. doi: 10.5606/tgkdc.dergisi.2018.16106. eCollection 2018 Oct.
Total anomalous pulmonary venous return is observed in 1.5 to 3% of congenital heart diseases. Urgent surgical intervention is required following the diagnosis, since severe heart failure is accompanied by cyanosis from the first days of life in most of the patients. This rare congenital heart disease is often managed in adulthood with surgery. Patients who survive until adulthood without a surgical intervention have a large atrial septal defect. A 36-year-old female patient was admitted with complaints of fatigability, shortness of breath, and cyanosis of the lips. Total anomalous pulmonary venous return, secundum-type atrial septal defect, ventricular septal defect, and pulmonary hypertension were diagnosed by transthoracic echocardiography and catheter angiography. After medical treatment, surgery was planned for the patient who responded to the pulmonary vasoreactivity test with iloprost.
完全性肺静脉异位引流在1.5%至3%的先天性心脏病中可见。诊断后需紧急进行手术干预,因为大多数患者从出生第一天起就伴有严重心力衰竭和青紫。这种罕见的先天性心脏病在成年期通常通过手术治疗。未经手术干预而存活至成年的患者有较大的房间隔缺损。一名36岁女性患者因疲劳、呼吸急促和嘴唇青紫而入院。经胸超声心动图和导管血管造影诊断为完全性肺静脉异位引流、继发孔型房间隔缺损、室间隔缺损和肺动脉高压。经过药物治疗后,计划对用伊洛前列素进行肺血管反应性试验有反应的患者进行手术。