In the past decade, there have been more patients with congenital heart disease (CHD) surviving to adulthood; whether due to late repair, or complex underlying CHD, many of these patients will be faced with pulmonary arterial hypertension (PAH) associated with CHD (APAH-CHD). In this review, the authors discuss the most commonly encountered forms of APAH-CHD, how to interpret the hemodynamic data, and how to classify the patients into meaningful subgroups that have similar management strategies. The current state of targeted medical treatments available to patients with APAH-CHD is also discussed.