Approximately 5-10% of adolescent and adult patients with congenital heart disease (CHD) will develop pulmonary arterial hypertension (PAH). Patients with PAH associated with CHD (PAH-CHD) exhibit several similarities to those with idiopathic and other associated forms of PAH, especially with regards to their non-specific, cardinal symptoms. The development of PAH-CHD can lead to lifelong impairment although, paradoxically, survival may be better versus idiopathic PAH. Patients with PAH-CHD may experience social limitations, and emotional and psychological issues, arising from their disease burden. Nurses and other allied healthcare professionals are well placed to deliver the individually-tailored care that patients with PAH-CHD require. Activities known to be of particular benefit include patient engagement and education, patient empowerment, colleague training, and ensuring effective communication across the multidisciplinary team. Recent developments in the management of PAH-CHD have led to changes in the medical needs and optimal care of this patient population. This review aims to provide an overview of the natural course, diagnosis, symptoms and impact of PAH-CHD. We also aim to communicate the current standards in management of patients with PAH-CHD, and how their outlook can be improved in the future.