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

BACKGROUND: Pulmonary 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.