Summary
Persistent patency of the arterial duct is a congenital heart abnormality defined as continued patency in term infants older than 3 months. Most patients are asymptomatic. Isolated patent arterial duct is found in around 1 in 2000 full term infants, but the prevalence of this condition is higher in preterm infants. The familial occurrence of patent arterial duct is uncommon and the usual mechanism of inheritance is considered to be polygenic with a recurrence risk of 3%. Rare families have been described in which the mode of inheritance appears to follow a dominant or recessive pattern of inheritance in the absence of any associated abnormalities. Familial incidence of patent arterial duct has also been linked to certain syndromes and associations, including Char syndrome, familial thoracic aortic aneurysm/dissection associated with patent arterial duct, and familial patent arterial duct and bicuspid aortic valve associated with hand abnormalities. Patency of the arterial duct is recognised on detection of the characteristic heart murmur. There may be history of prematurity, asphyxia, or maternal rubella. Large shunts may lead to failure to thrive, recurrent infection of the upper respiratory tract and congestive heart failure. Antenatal diagnosis of patent arterial duct is not possible as it is a normal structure during antenatal life. In preterm infants with symptomatic heart failure secondary to persistent patency of the arterial duct, treatment may be achieved by surgical ligation or with a prostaglandin synthetase inhibitor, such as ibuprofen. In 2004, this molecule has obtained EU marketing authorisation as an orphan drug for the treatment of preterm newborn infants less than 34 weeks of gestational age. Transcatheter closure of the duct is usually offered to older children. *Authors: Drs O.A. Elmasry and R.P. Martin (August 2007)*.
