Idiopathic pulmonary hemosiderosis in a patient with tetralogy of Fallot and pulmonary hemorrhage

Abstract

Pulmonary hemorrhage and hemoptysis are well-known complications in patients with congenital heart disease and are commonly associated with aorto-pulmonary collateral arteries (APCA). We report a rare case of idiopathic pulmonary hemosiderosis in a patient with pulmonary hemorrhage who underwent pediatric cardiac surgery. A child with tetralogy of Fallot presented with recurrent pulmonary hemorrhage before and after intracardiac repair. Coil embolization for APCA was not effective against the pulmonary hemorrhage. Although her symptoms were improved by administration of recombinant activated factor VII and blood transfusion, the cause of the pulmonary hemorrhage was still obscure. She was admitted to our hospital urgently at 4 years of age with severe anemia and hypoxemia secondary to diffuse alveolar hemorrhage. Histopathological examination of bronchoalveolar lavage fluid revealed hemosiderin-laden macrophages. We diagnosed her with idiopathic pulmonary hemosiderosis after excluding all other sources of hemorrhage. She was treated with oral corticosteroids and immunosuppressants and showed a good clinical response. This case highlights that clinicians must consider rare conditions such as idiopathic pulmonary hemosiderosis as a cause of pulmonary hemorrhage even without evidence of APCA in patients with congenital heart disease showing recurrent symptoms.