Mishra Vijay Kumar, Chaudhuri Souvik, Singh Rajesh Kumar, Singh Ajay Kumar
Department of Critical Care, Bhagwan Mahavir Medica Superspecialty Hospital, Ranchi, Jharkhand, India.
Anesth Essays Res. 2018 Jul-Sep;12(3):765-767. doi: 10.4103/aer.AER_105_18.
Unilateral pulmonary artery agenesis (UPAA) is a rare condition where the patient may remain asymptomatic for a long period till adulthood. Presentation may be in the form of dyspnea, cough, hemoptysis, decreased exercise tolerance, and recurrent respiratory tract infections. Ventilation-perfusion mismatch and excessive blood flow to the normal lung may eventually lead to unilateral acute respiratory distress syndrome (ARDS). We present a case of a 40-year-old gentleman with nonresolving pneumonia who was diagnosed to have unilateral ARDS due to UPAA. Even though ARDS involves bilateral lung, unilateral ARDS which does not show resolution with lung protective strategies should always raise the suspicion of a rare disease like UPAA among clinicians. To date, there is no specific prescribed treatment for UPAA.
单侧肺动脉发育不全（UPAA）是一种罕见病症，患者在成年前很长一段时间可能都没有症状。症状表现可能为呼吸困难、咳嗽、咯血、运动耐量下降以及反复呼吸道感染。通气-灌注不匹配和过多血流流向正常肺最终可能导致单侧急性呼吸窘迫综合征（ARDS）。我们报告一例40岁男性，患有不愈的肺炎，诊断为因UPAA导致的单侧ARDS。尽管ARDS累及双侧肺，但对于采用肺保护性策略后仍未好转的单侧ARDS，临床医生应始终怀疑像UPAA这样的罕见疾病。迄今为止，尚无针对UPAA的特定规定治疗方法。
