A RARE CASE OF PREGNANCY IN WOMAN WITH SINGLE ATRIUM

Ellis-van Creveld syndrome is a rare genetic disorder. About 300 cases have been reported worldwide. Amongst the affected population more than half do not survive beyond infancy or childhood. Thus we are presenting this case of a 28 year old short primigravida with 8 months gestation with positive family history with complaints of breathlessness and chest pain and an echocardiography revealing common atrium with severe pulmonary hypertension. Associated clinical features of short limbs, postaxial polydactyly and narrow chest led to the diagnosis of Ellis-van Creveld syndrome