Introduction: Noonan syndrome (NS) is an autosomal dominant genetic disorder associated with a high incidence of cardiovascular disease. We sought to investigate pulmonary hypertension (PH) in patients with NS, as well as characterize the hemodynamics, cardiovascular interventions, genetics, and transplant-free survival. Methods: A retrospective, descriptive cohort study was conducted among patients with NS who underwent cardiac catheterization at Boston Children’s Hospital between 2000 and 2020. Patient demographic, clinical, and hemodynamic data were collected for cohorts of patients with and without PH. Results: Eight-seven patients with Noonan syndrome underwent cardiac catheterization in the study period. Fifty-nine (68%) patients had a NS-associated pathogenic variant, while 28 (32%) had a clinical diagnosis of NS. Eleven (13%) patients had PH by 6 th World Symposium on Pulmonary Hypertension criteria: two patients with Group 1 Pulmonary Arterial Hypertension (PAH), seven Group 2 PH, and two Group 4 PH. There were significant differences in NS-associated causative gene in patients with and without PH, with RAF1 (p=0.013) and KRAS1 (p=0.015) more common among PH patients. A clinical diagnosis of hypertrophic cardiomyopathy (p=0.009) was more common in PH patients, while valvar, subvalvar, or supravalvar pulmonary stenosis (p=0.011) was less common. PH patients were significantly more likely to have aortic-valve interventions (p=0.025) and less likely to have valvar right-sided heart interventions (p=0.037). Transplant-free survival at five years was lower in the PH group (73%) compared to the non-PH group (99%). Conclusion: In a cohort of patients with Noonan syndrome undergoing cardiac catheterization, we noted differences among those with and without PH for NS-associated causative gene, associated hypertrophic cardiomyopathy or structural heart disease, interventions, and transplant-free survival. The prevalence of PH among this population demonstrates the need for future studies on the association between NS and pulmonary vascular disease.