Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease

Abstract The bronchial anatomy of a series of patients with congenital heart disease plus either or both abnormal visceral situs and abnormal spleen (absent, multiple, bifid or with unusually large accessory spleen or spleens) was studied by dissection and bronchographic methods. The results suggest that 5, and perhaps 6, syndromes can be delineated: (1) asplenia (Ivemark syndrome), characterized by bilateral eparterial bronchi (bilateral right or 3-lobed lung) and abnormal visceral situs; (2) polysplenia, characterized by bilateral hyparterial bronchi (bilateral left or 2-lobed lung) and abnormal visceral situs; (3) M-anisosplenia (third syndrome), characterized by bilateral eparterial bronchi and a relatively normal visceral situs; (4) F-anisosplenia (fourth syndrome), characterized by bilateral hyparterial bronchi with a visceral situs that is less abnormal than that observed in asplenia or polysplenia; (5) situs inversus, characterized by reversed bronchial pattern (eparterial bronchus on left and hyparterial on right) and reversed visceral situs; and (6) a group characterized by total anomalous pulmonary venous return, normal visceral situs, normal bronchial pattern, and 1 or more accessory spleens. Typical cardiac malformations were demonstrated in each of these syndromes. The results suggest that air or contrast bronchography can be of value in assessing patients with congenital heart disease plus abnormal visceral situs.