Intravenous Treprostinil in Precapillary and Postcapillary Pulmonary Hypertension Due to Cardiac Amyloidosis and Aortic Stenosis.

backgroundPulmonary hypertension (PH) associated with cardiac amyloidosis is challenging to manage. Limited data on intravenous treprostinil exist in this setting. CASE SUMMARY: An 82-year-old female with chronic thromboembolic pulmonary hypertension (CTEPH) and newly diagnosed cardiac amyloidosis presented with symptomatic tricuspid regurgitation. She was on intravenous treprostinil (25 ng/kg/min) for CTEPH. Hemodynamic evaluation confirmed postcapillary PH. Tricuspid edge-to-edge repair was performed successfully. Nine months later, progression of aortic stenosis necessitated transcatheter aortic valve implantation. Post-transcatheter aortic valve implantation follow-up showed clinical improvement with stable right and left ventricular function. DISCUSSION: This case demonstrates the safe use of individualized treprostinil in a patient with precapillary and postcapillary PH and CTEPH. The case also underscores the importance of screening for cardiac amyloidosis in patients with unexplained PH. TAKE-HOME MESSAGE: The use of high-dose intravenous treprostinil was uneventful in this case of postcapillary PH secondary to cardiac amyloidosis and aortic stenosis.