Absent pulmonary valve syndrome is a congenital heart defect usually associated with tetralogy of Fallot. Rarely, absent pulmonary valve syndrome can occur independently of tetralogy of Fallot and presents with an intact ventricular septum and a patent ductus arteriosus. This case report describes the prenatal diagnosis of absent pulmonary valve syndrome with intact ventricular septum via echocardiogram and the postnatal management of the resulting physiologic effects secondary to this rare congenital heart disease.
A 5-year-old healthy boy had a witnessed ingestion of hematite (iron oxide rock) at school. He had self-limited gagging and blood-tinged secretions. An X-ray showed a radiopaque foreign body measuring 3.5 cm by 2.2 cm in the right upper quadrant. Due to concern for iron toxicity, (1) an iron level was obtained about 3 hours after the ingestion and was 56 mg/dL, increasing to 97 mg/dL after 3 hours. The child remained asymptomatic but because of increasing iron levels with presumed risk for toxicity, the decision was made to proceed with endoscopy for removal. It was uncertain how rapidly iron could reach toxic levels as no similar cases were found in the literature. The hematite rock was successfully removed endoscopically with a Roth Net from the gastric fundus. No injuries to the stomach or esophagus were identified. Iron poisoning is a risk after iron ingestion. (1-3) However, it is unknown how rapidly the absorption occurs when an iron-containing foreign body is retained in the gastrointestinal tract. Our case evidences a rapid increase in iron levels by 1.7-fold in 3 hours, suggesting that the potential for iron poisoning does exist with the ingestion of hematite rocks (Figs. 1 and 2).
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