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Thiamine-Responsive Acute Pulmonary Hypertension of Early Infancy (TRAPHEI)-A Case Series and Clinical Review

Nalinikanta Panigrahy1, Dinesh Kumar Chirla1, Rakshay Shetty2

  • 1Rainbow Children's Hospitals, Hyderabad 500034, India.

Children (Basel, Switzerland)
|October 31, 2020
PubMed

Insights

Infants in India can develop severe pulmonary hypertension due to thiamine deficiency, often linked to a polished rice diet. Early diagnosis and thiamine treatment significantly improve outcomes for this condition, known as thiamine-responsive acute pulmonary hypertension of early infancy.

Area of Science:

  • Pediatrics
  • Cardiology
  • Nutritional Science

Background:

  • Persistent pulmonary hypertension of the newborn (PPHN) is a global condition characterized by elevated pulmonary vascular resistance (PVR) in neonates.
  • Recent Indian case reports highlight severe pulmonary hypertension in infants aged 1-6 months, distinct from typical PPHN, often associated with maternal polished rice diets.
  • These infants present with respiratory distress, potentially triggered by infections like laryngotracheobronchitis, leading to cardiac and hepatic complications.

Purpose of the Study:

  • To review four cases of thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) in India.
  • To discuss the pathophysiology, clinical presentation, diagnostic criteria, and therapeutic strategies for TRAPHEI.
  • To emphasize the role of thiamine deficiency as a cause of severe pulmonary hypertension in this age group.

Main Methods:

  • Retrospective review of four infant cases presenting with acute pulmonary hypertension.
  • Analysis of clinical data, dietary history (maternal polished rice consumption), and response to thiamine therapy.
  • Discussion of diagnostic findings including echocardiographic evidence of right heart strain and pulmonary edema.

Main Results:

  • All four infants showed significant improvement with respiratory support, vasodilators, inotropes, diuretics, and crucially, thiamine infusion.
  • The clinical presentation mimicked PPHN but was responsive to thiamine, suggesting a deficiency-related etiology.
  • Thiamine deficiency was identified as the likely cause of acute pulmonary hypertension in these infants.

Conclusions:

  • Thiamine deficiency is a critical, treatable cause of severe pulmonary hypertension in infants (TRAPHEI), particularly in regions with polished rice-based diets.
  • Prompt diagnosis and administration of thiamine, alongside supportive care, can dramatically improve outcomes and reduce mortality.
  • Awareness of TRAPHEI is essential for pediatricians and cardiologists managing infants with unexplained pulmonary hypertension.

Keywords:
Indiaberiberinitric oxidepulmonary hypertensionsildenafilthiamine

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