Risk factors for PH and management approach in patients with NTDT. Patients with NTDT should be screened annually with a transthoracic echocardiogram with assessment of the tricuspid regurgitant velocity (TRV). For patients who are symptomatic or have a TRV >3.2 m/s, right heart catheterization is appropriate to confirm the diagnosis of PH. Other secondary causes should be excluded. A ventilation/perfusion (V/Q) scan should be obtained to evaluate for chronic thromboembolic disease. If asymptomatic and 2.5 < TRV ≤ 3.2 m/s, close follow-up is warranted, and management of reversible risk factors should be considered. RBC, red blood cell. Figure created with BioRender.