Severe PAH in Healthy Infants

A recent study examined the clinical presentation and management of acute pulmonary arterial hypertension (PAH) in healthy infants below 6 months old. 

The study entailed reviewing records of 56 infants diagnosed with sudden-onset PAH via 2D echocardiography. All infants received supportive care and pulmonary vasodilator therapy, while 28 of those selected received thiamine as per the institute's protocol. 

It was observed that 80% of the cases had complete recovery. Infants who failed to survive had lower pH and serum bicarbonate, higher arterial lactate, and increased incidences of ventricular dysfunction and shock. Thiamine supplementation did not show additional benefits, with comparable outcomes in terms of recovery and mortality between thiamine-receiving and non-receiving groups. 

It was inferred that a significant number of infants with PAH improve with supportive and vasodilator therapy alone, and thiamine supplementation may not offer additional benefits. Further large-scale prospective studies are needed to explore the etiology, role of thiamine, risk factors, and optimal management of acute PAH in this population.

Source: Aroor S, Handattu K, Mundkur SC, Verma A, Samuel PC, Teli AS, Banga G. Indian pediatrics. 2023:S097475591600551.