The conundrum of Thiamine Responsive Acute Pulmonary Hypertension (TRAPH) syndrome in the Emergency Department.

Abstract

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.