Cardiac problems in Down syndrome

Children with Down syndrome (DS) have long been known to have an increased incidence of pulmonary arterial hypertension (PAH), but this has not been clearly understood until recently. DS patients have an increased incidence of congenital heart disease (CHD) and upper airway obstruction (UAO) amongst other co-morbidities. Even though CHD is usually quickly recognized in early infancy with current echocardiographic assessment and corrected with early surgery, many children with Down syndrome will still have ongoing PAH which may be unrecognized. A better understanding of the interplay between these conditions, managed by a multi-disciplinary team, is required to maximize their long term quality of life.