Polydactyly in a patient with a cardiovascular anomaly.

A 34-year-old Montenegrin maintenance man, of South Slavic ancestry, presented for evaluation of nonexertional polyarthralgia, mainly involving the shoulders, knees, and ankles. The patient denied functional limitations in his daily activities. His medical history was remarkable for a congenital heart defect, manifested as a partial atrioventricular septal defect with a single common atrium, persistent left superior vena cava, and cleft mitral valve, surgically corrected at age 17 years. Examination revealed an otherwise well-developed man with symmetric hexadactyly of all extremities, with well-developed and functional extra digits present on the ulnar and fibular sides (i.e., type A postaxial polydactyly). Radiographs confirmed postaxial polydactyly. The patient reported that his brother had a similar anomaly. Both declined genetic testing. As there was no evidence of inflammatory or metabolic arthropathy, symptomatic treatment was recommended. Polydactyly is the most common congenital limb deformity but is rarely encountered in adult practice, being usually corrected in early childhood. Postaxial polydactyly affects the lateral aspect of the limb and, as an isolated finding, confers a favorable prognosis. Postaxial hexadactyly associated with a partial or complete atrioventricular septal defect with common atrioventricular junction has been reported in only 10 cases, including 1 lethal outcome in early infancy. Levin and Vanderdonck reported 6 cases and suggested that this constellation constitutes a true syndrome, although the karyotype in 4 cases was normal. Our case contributes to this rare clinical constellation. In the world of piano music, a 5-finger exercise is used to train all one’s fingers to develop fundamental playing skills. Despite his potential advantages, our patient reported that he played no musical instrument.