Non-Cardiac Pulmonary Arterial Hypertension in Infants Presenting with Respiratory Distress: Assessing the Efficacy of Thiamine Supplementation in a Clinical Setting

Our project aims to investigate Pulmonary Arterial Hypertension (PAH) in hospitalized infants aged 1 to 12 months, without heart problems, and determine if treating them with Vitamin B1 (Thiamine) improves their condition. Using Echocardiography (ECHO), we’ll identify infants with PAH and test their blood for Thiamine levels.

 Currently, the Standard Care for treating PAH is giving Oxygen and a medicine called Sildenafil. Interestingly, some case series have reported that giving thiamine is beneficial in treating PAH in infants who do not have any obvious causes like heart or lung issues. However, Thiamine isn’t yet a standard treatment for PAH. There are also a few reports stating that thiamine deficiency is still quite common in Northeast India. Therefore we want to examine the effect of thiamine on PAH and explore if thiamine will be an effective adjunct therapy for PAH.

 For this, we’ll split our selected patients into two equal groups: One group will receive Standard Care alone and the other group Standard Care with the addition of thiamine. The test results of thiamine levels is expected to take approximately 4-5 days to come. In the meantime, we propose to randomly give thiamine to one group alongwith the Standard Care. We will repeat the ECHO at 12, 24, 48 and 72 hours. After 72 hours, if some of the children who did not receive thiamine earlier still have PAH, then we will try thiamine in them too. Our goal is to see whether the group who received thiamine on top of Standard Care had a better response to PAH or not. Now, if the thiamine blood test results come back before 72 hours then the patient will immediately receive thiamine if not given earlier and response monitored as mentioned earlier.

 If we can demonstrate a positive response of PAH to thiamine, then it could become a part of the standard treatment for infant PAH.