The role of cardiac catheterization in Trisomy 21 and pulmonary hypertension

Children with Trisomy 21 are at increased risk for pulmonary hypertension. The reasons for this are multi-factorial but include an abnormal pulmonary vascular bed with increased propensity for congenital heart disease and upper airway obstruction. And although the association of pulmonary hypertension with Trisomy 21 is well established, this case report highlights the complexity of pulmonary hypertension in this vulnerable population, the limitations of echocardiography and critical contribution of cardiac catheterization in informing clinical management.