Pulmonary arterial hypertension in infancy: A case series
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Updated: May 13, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
N V Shashikala1, K M Arun2, R C Ashwini3
1Resident, Department of Paediatrics, Bapuji Child Health Institute and Research Centre, Davanagere, Karnataka, India.
Insights
Thiamine deficiency causes severe pulmonary arterial hypertension (PAH) in infants. Prompt thiamine therapy rapidly reverses this condition, offering excellent outcomes for affected infants.
Area of Science:
- Pediatrics
- Cardiology
- Nutritional Science
Background:
- Pulmonary arterial hypertension (PAH) in infants can have various causes.
- Thiamine deficiency is a known but often overlooked cause of reversible PAH.
Purpose of the Study:
- To investigate the link between thiamine deficiency and severe PAH in exclusively breastfed infants.
- To evaluate the efficacy of thiamine therapy in treating infant PAH.
Main Methods:
- Retrospective case series of infants aged 12 months with echocardiographically confirmed severe PAH.
- Analysis of dietary habits of mothers, focusing on polished rice consumption.
- Assessment of clinical response to thiamine therapy.
Main Results:
- Eight exclusively breastfed infants (median age 2.5 months) presented with severe PAH.
- All mothers consumed polished rice-based diets, suggesting thiamine deficiency.
- Thiamine therapy led to rapid clinical improvement within 48 hours and complete resolution within two weeks.
- No deaths were reported in the study group.
Conclusions:
- Thiamine deficiency is a critical, reversible cause of severe PAH in early infancy.
- Early diagnosis and prompt thiamine treatment are crucial for excellent outcomes in affected infants.
- Pediatricians should consider thiamine deficiency in infants presenting with acute severe PAH, especially those exclusively breastfed by mothers on polished rice diets.
Abstract:
Thiamine deficiency is a reversible cause of severe pulmonary arterial hypertension (PAH) in early infancy, particularly among exclusively breastfed infants of mothers consuming polished rice-based diets. This retrospective case series (July 2023-July 2025) included infants aged-12 months with echocardiographically confirmed severe PAH and no structural heart disease. Eight exclusively breastfed infants (median age 2.5 months) presented with respiratory distress. All mothers consumed polished rice-based diets. Echocardiography revealed severe PAH with right-sided cardiac dilation. Thiamine therapy resulted in rapid clinical improvement within 48 h and complete resolution within two weeks, with no deaths. Thiamine deficiency should be suspected in infants with acute severe PAH, as prompt treatment leads to excellent outcomes.
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