Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India
Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India.
Arch Dis Child. 2021 Mar;106(3):241-246. doi: 10.1136/archdischild-2019-318777. Epub 2020 Sep 3.
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.
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.
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.
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.
由于单纯母乳喂养的婴儿缺乏硫胺素，可能会导致严重的肺动脉高压（PH）引起右心衰竭。本研究描述了硫胺素反应性急性肺动脉高压的临床特征和治疗方法。
对出现严重 PH 且无其他明显心脏或肺部疾病的婴儿进行前瞻性观察研究。记录了母亲的饮食模式。对婴儿进行了症状史、临床检查、超声心动图和基本检查。给予硫胺素，并进行了连续的超声心动图检查。
共有 250 名婴儿患有严重 PH，其中 231 名对硫胺素有反应。平均年龄为 3.2±1.2 个月。主要症状为呼吸急促、喂养不良、呕吐和声音嘶哑。检查时发现呼吸急促、心动过速和肝肿大。超声心动图显示右心明显扩张，且存在严重 PH。所有婴儿均根据临床怀疑给予静脉注射硫胺素。在 24-48 小时内，临床症状明显改善，PH 完全缓解。对婴儿进行了最长 60 个月的随访，未再出现 PH 复发。所有母亲都食用精米，并遵循产后饮食限制。
在印度的某些地区，硫胺素缺乏仍然很普遍。它可导致单纯母乳喂养的婴儿出现危及生命的 PH，而这些婴儿的母亲主要食用精制米，并遵循产后饮食限制。它可能导致婴儿死亡。根据临床怀疑给予硫胺素治疗可显著恢复。需要在相关地区提高对该病的认识，并进行硫胺素补充。
