Aims & Objectives:
Pulmonary Hypertension remains a leading cause of mortality despite many advances in its management. Thiamine deficiency is identified as a reversible cause of pulmonary hypertension in exclusively breastfed infants. Shoshin beriberi a fulminant form of cardiovascular beriberi characterized by tachycardia, hypotension, severe pulmonary hypertension and lactic acidosisand has been designated as ‘a rapidly curable hemodynamic disaster’.The aim of this report is to call attention to a fatal, poorly recognized, easily reversible cause of pulmonary hypertension, thiamine deficiency.
We present retrospective analysis of three cases of pulmonary hypertension which were likely due to thiamine deficiency admitted to a tertiary PICU in Bengaluru between August 2015 to August 2017
A total of nine children were admitted with pulmonary hypertension over a period of two years out of which three children (33%)responded to IV Thiamine.Mean age of presentation was 67 days.
These three infants were exclusively breast-fed presented well - nourished with sudden onset of excessive irritability, rapidly deteriorating respiratory distress with unexplained bi-ventricular failure and severe pulmonary hypertension. Acute metabolic acidosis was a universal feature with mean pH of 6.92 ± 0.1 and average lactate of 7 ± 2.5 mmol/L.They responded to IV Thiamine dramatically with immediate improvement in pulmonary pressures, metabolic acidosis and reversal of shock in 6–12 hours.
Shoshin beriberi is a rapidly progressive fatal disease without proper treatment.Clinician awareness of its possibility, even in well-nourished infants with unexplained metabolic acidosis and high pulmonary pressures, is important for early recognition and prevention of deaths with a trial of thiamine infusion.