Department of Emergency Medicine, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India.
Department of Medicine, All India Institute of Medical Sciences, New Delhi, India.
Am J Emerg Med. 2021 Nov;49:185-188. doi: 10.1016/j.ajem.2021.06.015. Epub 2021 Jun 7.
Management of acute pulmonary hypertension in the Emergency Department(ED) can be challenging. The treatment is specialised, requires rapid identification and correction of the precipitating cause; failing which the patient enters a vortex of deterioration. We describe a lesser-known cause for the same, Thiamine responsive acute pulmonary hypertension (TRAPH) syndrome where timely appropriate treatment can result in dramatic improvement.
Medical records with ICD code E51.12 (Wet Beriberi) from Mar 2018 to Mar 2020 were screened. The data regarding presenting symptoms, initial vitals, lab and radiological investigations, and treatment received were retrieved from patient files and the Hospital Informatics System, entered into an MS Excel sheet and compared.
The study includes eight cases, which we believe to be TRAPH syndrome. Majority were young adult males, ethanol users. All patients presented with acute shortness of breath with tachypnea and shock index more than 0.9. Gross right atrioventricular dilatation, tricuspid regurgitation and mild to moderate pulmonary arterial hypertension was identified in echocardiography. The initial blood gas revealed median pH 6.98 (IQR 6.81-7.09), Bicarbonate 3.4 meq/L (IQR 2.5-5) and lactate 172 mg/dL (IQR 132-200) which improved within 12-16 h of admission. Patients received median 400 mg IV Thiamine. The mean duration of ICU stay was 2.5 days and total hospital stay was 7 days.
Thiamine Responsive Acute Pulmonary Hypertension (TRAPH) Syndrome is an under-recognised entity which should be included in differentials for acute right ventricular dysfunction in the ED. Early diagnosis and rapid protocolised management of the same can cause quick recovery of patients.
急诊科（ED）急性肺动脉高压的管理具有挑战性。治疗需要专业知识，需要快速识别和纠正诱发原因；否则患者会陷入恶化的漩涡。我们描述了一种不太为人知的原因，即硫胺素反应性急性肺动脉高压（TRAPH）综合征，及时给予适当的治疗可以显著改善病情。
筛查 2018 年 3 月至 2020 年 3 月 ICD 编码为 E51.12（湿性脚气病）的病历。从患者档案和医院信息系统中检索有关临床表现、初始生命体征、实验室和影像学检查以及所接受的治疗的数据，并将其输入 MS Excel 工作表进行比较。
本研究包括 8 例患者，我们认为他们患有 TRAPH 综合征。大多数为年轻成年男性，为乙醇使用者。所有患者均表现为急性呼吸急促伴呼吸急促和休克指数大于 0.9。超声心动图显示右心房和右心室明显扩大，三尖瓣反流和轻度至中度肺动脉高压。初始血气分析显示 pH 值中位数为 6.98（IQR 6.81-7.09），碳酸氢盐 3.4 meq/L（IQR 2.5-5），乳酸 172 mg/dL（IQR 132-200），这些指标在入院后 12-16 小时内得到改善。患者接受了中位数为 400 mg 的静脉注射硫胺素。ICU 住院时间的平均值为 2.5 天，总住院时间为 7 天。
硫胺素反应性急性肺动脉高压（TRAPH）综合征是一种认识不足的实体，应将其纳入 ED 急性右心室功能障碍的鉴别诊断中。早期诊断和快速的规范化管理可以使患者迅速康复。
