Journal of Pediatric Cardiology and Cardiac Surgery

Cardiovascular beriberi may occur because of an unbalanced diet or eating disorder, and can lead to circulatory insufficiency. Here, we report a 3-year-old-boy with avoidant/restrictive food intake disorder who developed severe pulmonary artery hypertension (PAH) due to cardiovascular beriberi and was treated with a phosphodiesterase type 5 inhibitor in addition to thiamine. The patient had limited oral intake for one month due to a mouth ulcer. On admission, he was sluggishness, hypotensive, tachycardic and tachypneic. Severe PAH was suspected based on echocardiography showing enlargement of the right ventricle and the right atrium and deformity of the interventricular septum. A sildenafil administration test was performed under oxygen administration by high flow nasal cannula; PAH improved one hour later. Based on the dietary history, thiamine was started for possible cardiovascular beriberi due to vitamin B1 deficiency which was eventually confirmed by the blood sample on admission. Since idiopathic PAH could not be ruled out initially, tadalafil was started on the day of admission. With these treatments, oral intake increased. Tadalafil was gradually reduced and eventually discontinued 7 months later. Cardiac catheterization 8 months after the onset of the PAH episode did not show severe PAH. This case indicates that a potential of PAH due to cardiovascular beriberi should be kept in mind if the physical condition deteriorates in a child with an eating disorder or unbalanced diet. Important issues are timely assessment using echocardiography, treatments according to appropriate understanding of pathophysiology, and supplementation of vitamin B1.