We report a case of 2 year old female child who was referred to cardiology out-patient department with congenital left upper limb deformity. On examination she had a small forearm with radial deviation of hand and hypoplasia of four fingers. On retrospective evaluation she was found to have repeated respiratory tract infection. Chest roentgenogram revealed cardiomegaly and echocardiogram revealed a 10-11 mm Atrial septal defect (ASD) with left to right shunt. In view of limb deformity, she underwent soft tissue release followed by JESS fixator application and distraction. We emphasise the importance of syndromic diagnosis in all paediatric cases presenting with limb abnormalities.
Key words: Holt Oram syndrome, Atrio-digital dysplasia, Heart-hand syndrome, Atrial Septal defect.