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Thiamine-responsive acute severe pulmonary hypertension in exclusively breastfeeding infants: a prospective
Usha M K Sastry1, Jayranganath M2, Raman Krishna Kumar3
1Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India docmkusha@gmail.com.
Insights
Thiamine deficiency in infants can cause severe pulmonary hypertension (PH). Prompt thiamine administration led to rapid recovery in most cases, highlighting the need for awareness and supplementation.
Area of Science:
- Pediatrics
- Cardiology
- Nutritional Science
Background:
- Severe pulmonary hypertension (PH) in infants can lead to right heart failure.
- Thiamine deficiency is a potential, yet often overlooked, cause of PH in exclusively breastfed infants.
Purpose of the Study:
- To describe the clinical presentation and management of infants with acute pulmonary hypertension caused by thiamine deficiency.
- To evaluate the efficacy of thiamine treatment in resolving PH and improving outcomes in affected infants.
Main Methods:
- A prospective observational study involving 250 infants presenting with severe PH.
- Clinical evaluation, echocardiography, and maternal dietary history were recorded.
- Intravenous thiamine was administered based on clinical suspicion, with serial echocardiography to monitor PH resolution.
Main Results:
- Out of 250 infants with severe PH, 231 (92.4%) responded positively to thiamine treatment.
- Clinical improvement and complete resolution of PH occurred within 24-48 hours of thiamine administration.
- Mothers of affected infants commonly consumed polished rice and adhered to postpartum dietary restrictions.
Conclusions:
- Thiamine deficiency remains a significant cause of life-threatening pulmonary hypertension in exclusively breastfed infants in certain regions.
- Early clinical suspicion and prompt thiamine supplementation are crucial for remarkable recovery and preventing infant mortality.
- Increased awareness and targeted thiamine supplementation programs are necessary in high-risk geographical areas.
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.
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