Holt-Oram: when the key to a broken heart is in the hand.

To cite: Naderi N, McCurdy MT, Reed RM. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014203851 DESCRIPTION A man in his 50s had a history of a congenital atrial and ventricular septal defect requiring repair decades prior. He also had a history of heart block requiring pacemaker placement. During an evaluation for pacemaker battery replacement, he was noted to have nodules at the metacarpophalangeal joint of his fifth digit, bilaterally (figure 1). The nubbins were consistent with ulnar (also known as postaxial) polydactyly and, given the cardiac history, a diagnosis of Holt-Oram syndrome was considered highly likely. The patient tolerated his battery replacement without complication, and years later passed away from causes unrelated to his medical condition.