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

Abstract

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of high pulmonary vascular resistance (PVR) commonly seen all over the world in the immediate newborn period. Several case reports from India have recently described severe pulmonary hypertension among infants in the postneonatal period. These cases typically present with respiratory distress in 1–6-month-old infants, breastfed by mothers on a polished rice-based diet. Predisposing factors include respiratory tract infection such as acute laryngotracheobronchitis with change in voice, leading to pulmonary hypertension, right atrial and ventricular dilation, pulmonary edema and hepatomegaly. Mortality is high without specific therapy. Respiratory support, pulmonary vasodilator therapy, inotropes, diuretics and thiamine infusion have improved the outcome of these infants. This review outlines four typical patients with thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) due to thiamine deficiency and discusses pathophysiology, clinical features, diagnostic criteria and therapeutic options.

Thiamine responsive pulmonary hypertension: case series

“…The review of literature including our patients have shown infantile PH due to thiamine deficiency, which is mostly seen in developing countries like South Asian, African and other sub continents and very few cases have been noticed in developed countries like UK and USA. 3 Main source of thiamine in developing countries includes rice, whole grains, poultry, nuts, soyabeans. 5 The recommended daily intake (RDI) levels in infancy are 0.2 mg/day and its RDI levels slightly increase with age.…”

“…Wet beri beri can cause congestive heart failure most commonly right heart failure, which can lead to decrease in circulatory volume, lactic acidosis, but lately PH in thiamine deficiency cases were reported in India. 3 Lack of awareness and late recognition of thiamine deficiency may result in high mortality. A high index of suspicion is required for early diagnosis and management to decrease the severity and morbidity and thereby preventing long term implications on neurological development.…”

“…A high index of suspicion is required for early diagnosis and management to decrease the severity and morbidity and thereby preventing long term implications on neurological development. 3 Venkat CG et al Int J Contemp Pediatr. 2021 Jun;8(6)…”

Neonatal and Postneonatal Pulmonary Hypertension

“…More recently, there have been several reports of acute PH in early infancy. [20] These infants demonstrate rapid deterioration with high mortality without specific therapy. Treatment with thiamine has led to a dramatic improvement in PH.…”

“…After the neonatal period, in the absence of BPD or other lung conditions, PH is uncommon. More recently, there have been several reports of acute PH in early infancy [ 20 ]. These infants demonstrate rapid deterioration with high mortality without specific therapy.…”