Kartagener's syndrome with corrected transposition. Conducting system studies and coronary arterial occlusion complicating valvular replacement.
An 18-year-old man whose sister has classic Kartagener's syndrome was found to have sinusitis, bronchiectasis, and corrected transposition with normal visceral situs. Congenital complete heart block was secondary to absence of conducting-system pathways between a small posterior atrioventricular node and the transposed His bundle and bundle branches. No anterior atrioventricular node was present. Prosthetic valvular replacement of the left-sided (morphologic right) atrioventricular valve was complicated by coronary arterial occlusion by suture, with subsequent myocardial infarction. The case appears to represent an unusual variant of Kartagener's syndrome with the abnormality of laterality being expressed as corrected transposition.
D000293:Adolescent; D004194:Disease / Q000209:etiology*; D006329:Heart Conduction System / Q000473:pathology*; D006350:Heart Valve Prosthesis / Q000009:adverse effects*; D006801:Humans; D007619:Kartagener Syndrome / Q000150:complications*; D008297:Male; D009206:Myocardium / Q000473:pathology; D014188:Transposition of Great Vessels / Q000150:complications*