Celiac Disease in Association with a Congenital Heart Disease

Celiac disease is a form of enteropathy affecting the (small) intestine in genetically predisposed children and adults, precipitated by the ingestion of gluten-containing foods. It is also referred to as celiac sprue, gluten-sensitive enteropathy, and nontropical sprue. Though its presentation is commonly associated with the gastrointestinal system other systemic associations like hepatobiliary system, nervous system, hematologic system, cardiovascular system are not uncommon. We hereby present a case of a 14 year old girl with a history of global growth retardation. Systemic examination suggested severe anemia, massive pleural and pericardial effusions subsequently confirmed by chest x ray and echocardiography which also revealed an underlying atrial septal defect. Raised levels of serum anti-transglutaminase antibody (IgA) arose suspicion of Celiac disease which was confirmed after the histopathological study of the duodenal biopsy. We are reporting this case to highlight a rare association of celiac disease with a congenital heart disease.