Published on:January 2016
    Journal of Cardiovascular Disease Research, 2016; 7(1):53-55
    Case Report | doi:10.5530/jcdr.2016.1.11

    The Classical Holt Oram Syndrome

    Authors:

    Nitin Naik,1 M Sudhakar Rao,2 Sneha S,1 Sravan Reddy2

    1Department of Medicine, KMC Manipal, Manipal University, Madhav Nagar, Near Tiger circle, Manipal, Karnataka, INDIA.

    2Department of Cardiology, KMC Manipal, Manipal University, Madhav Nagar, Near Tiger circle, Manipal, Karnataka, INDIA.

    Abstract:

    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.

    Article Download