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The presence of pulmonary arterial hypertension (PAH) increases morbidity and mortality in patients with congenital heart disease (CHD), but treatment patterns in these patients are not well studied. We aimed to evaluate PAH-CHD treatment patterns and understand adherence to guideline recommended care. Data were drawn from the 2022 Adelphi Real World PAH-CHD Disease Specific Programme, a point in time survey which collected physician-provided information on US PAH-CHD patients. Cardiologists and pulmonologists completed record forms for PAH-CHD patients including their PAH treatment and clinical characteristics. Results were stratified by NYHA Functional Classification (FC I-IV) and physician affiliation with PAH expert centres. Data on 191 patients were analysed. Mean patient age was 38.8 years (SD 15.9); 62% were male; 49% had PH associated with single ventricle Fontan palliation and 51% with other forms of CHD. Overall, 34% of patients were classified as FC I, 58% as FC II and 7% as FC III. Most patients (76%) received treatment for their PAH with PAH- and non-PAH-specific pharmacotherapies; few patients (7%) had a treat-and-repair strategy. Overall, 37% of patients were receiving monotherapy and 48% were prescribed combination therapy for their PAH-CHD, most frequently with PDE5i or PDE5i + ERA combination, respectively. Patients receiving PDE5 inhibitors were typically treated with sildenafil (50%) and tadalafil (48%). Levels of combination therapy usage were similar across FC I & II at 40% and 48%, respectively, but were higher at 75% in FC III. Prostacyclin pathway agents were more frequently prescribed in FC III patients (60%). Combination therapy use was also similar for those treated by physicians affiliated with PAH expert centres, or those who were not (45% and 46%, respectively) and was similar for PAH-CHD patients with Fontan palliation at 44%, to other forms of CHD at 51%. A low proportion of physicians reported using guidelines (10%) or risk assessment (10%) to guide treatment. PDE5 inhibitor treatment was the most prescribed medication in our sample; however, combination therapy should be considered for patients with higher FC as PAH is a progressive disease. Continued risk assessment could be a tool to assess need for treatment escalation in this high-risk population. [ABSTRACT FROM AUTHOR]