Key Points : Congenital Heart Disease in Down Syndrome 40 to 60 percent of babies born with Down syndrome will have a congenital heart anomaly Diagnosis may be made antenatally by specialised fetal echocardiography Clinical examination alone is insufficient to detect all of even the most serious heart disease in newborns and young babies A normal echocardiogram does not rule out heart disease, but should exclude the major conditions Less serious conditions such as small holes, persistent arterial ducts and atrial septal defects may not be detected by examination or echocardiogram, so continue vigilance is required. The risk of pulmonary hypertension is greater in those with Down syndrome than in the general population and may be due to congenital heart disease, with or without concurrent airways disease. Without surgical correction of cardiac defects irreversible pulmonary vascular disease can occur causing significant morbidity and premature death. Screening for congenital heart disease in the newborn period is essential with prompt cardiological opinion where necessary to allow for early surgical correction