ObjectivesSevere 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.MethodsA 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.ResultsA 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.ConclusionThiamine 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.

Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.

Dilated cardiomyopathy (DCM) is an uncommon complication of Takayasu arteritis (TA) with a prevalence of about 6%. We report a case of 14-year-old girl who presented with dyspnea, bipedal edema, loss of weight, and easy fatigability for three months. She was being treated for DCM for the same duration. Clinical examination revealed absence of both upper limb pulses. Echocardiography revealed features of DCM with severe biventricular dysfunction (ejection fraction 30%). Computed tomography angiogram confirmed the diagnosis of TA and revealed the presence of bilateral renal artery stenosis. Bilateral renal angioplasty was done, and immunosuppressant therapy with oral prednisolone and weekly oral methotrexate was started.

A 6-year-old girl born of a consanguinous marriage, with an uneventful birth history presented with cyanosis, clubbing, and effort intolerance. Her physical examination revealed cyanosis (oxygen saturation 78%), clubbing, single second heart sound, and a grade II/VI ejection systolic murmur over the left parasternal region. Echocardiography (Philips HD7 XE System, Eindhoven, The Netherlands) revealed the presence of a large subaortic ventricular septal defect, overriding of aorta, aneurysm of the left sinus of Valsalva at the level of the left coronary artery and pulmonary atresia (Fig. 1). Parasternal shortaxis view at the aortic valve level demonstrated confluent pulmonary arteries and left coronary to pulmonary artery fistula with a giant aneurysm at Figure 1. Parasternal long-and short-axis views show a large subaortic ventricular septal defect (VSD) with pulmonary atresia (P At) and an aneurysm (An) at the left sinus at the origin of the left coronary artery (marked with red arrow).Figure 2.Parasternal short-axis view shows the aneurysm at the origin of left coronary artery with left coronary to pulmonary artery fistula connecting to main pulmonary artery (PA) (marked with red arrow).

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