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Thiamine-responsive acute severe pulmonary hypertension in exclusively breastfeeding infants: a prospective observational study
  1. Usha M K Sastry1,
  2. Jayranganath M1,
  3. Raman Krishna Kumar2,
  4. Santu Ghosh3,
  5. Bharath A P1,
  6. Anand Subramanian1,
  7. Ameetkumar Managuli1,
  8. Madhu Gangadhara1,
  9. Cholenahally Nanjappa Manjunath4
  1. 1Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India
  2. 2Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Centre, Cochin, Kerala, India
  3. 3Department of Biostatistics, St John’s National Academy of Health Sciences, Bangalore, Karnataka, India
  4. 4Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India
  1. Correspondence to Dr Usha M K Sastry, Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore 560069, India; docmkusha{at}


Objectives Severe pulmonary hypertension (PH) causing right heart failure can occur due to thiamine deficiency in exclusively breastfeeding infants. This study describes the clinical profile and management of thiamine-responsive acute pulmonary hypertension.

Methods A prospective observational study of infants presenting with severe PH without any other significant heart or lung disease. History of symptoms, clinical examination, echocardiography and basic investigations were performed. Dietary patterns of mothers were recorded. Thiamine was administered and serial echocardiography was performed.

Results A total of 250 infants had severe PH and 231 infants responded to thiamine. The mean age was 3.2±1.2 months. Fast breathing, poor feeding, vomiting and aphonia were the main symptoms. Tachypnoea, tachycardia and hepatomegaly were found on examination. Echocardiogram revealed grossly dilated right heart with severe PH. Intravenous thiamine was administered to all the babies based on clinical suspicion. Clinical improvement with complete resolution of PH was noticed within 24–48 hours. Babies were followed up to a maximum of 60 months with no recurrence of PH. All the mothers consumed polished rice and followed postpartum food restriction.

Conclusion Thiamine deficiency is still prevalent in selected parts of India. It can cause life-threatening PH in exclusively breastfeeding infants of mothers who are on a restricted diet predominantly consisting of polished rice. It can contribute to infant mortality. Thiamine administration based on clinical suspicion leads to remarkable recovery. High degree of awareness and thiamine supplementation in relevant geographical areas is required to tackle this fatal disease.

  • nutrition
  • infant feeding
  • cardiology
  • general paediatrics
  • intensive care
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  • Contributors UMKS conceptualised the study, drafted the protocol and was primarily responsible for data acquisition and drafting the manuscript, analysis and interpretation of data. JM, BAP, AS, AM: conception of the study design, drafting the protocol, data compilation, suggestions, reviewed and revised the manuscript. RKK: scientific review, critical analysis and manuscript editing. SG: statistical analysis and interpretation of data, critical review of the manuscript for intellectual content. MG: designing the protocol, assistance in drafting the manuscript, compiling references, editing the manuscript and critical suggestions. CNM: conception of study design and protocol, supervision of the study, final approval of the submission version. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work. Dr Nisha C Babu and Dr Ravishankar: assistance in data acquisition.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Ethics approval Institutional ethics committee approval was obtained. Parents gave informed consent for the study. Ethics Committee clearance obtained: Sri Jayadeva Ethics Committee: 25 Jan 2013.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available on reasonable request. All data relevant to the study are included in the article or uploaded as online supplementary information. Individual participant data that underlie the results will be available after deidentification 1 year after the publication of the article to those researchers who submit a methodologically sound proposal for the purpose of meta-analysis. Data will be available on requesting the corresponding author (

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