# A Case Report of Pulmonary Arterial Hypertension Associated With Hereditary Hemorrhagic Telangiectasia Successfully Treated With Riociguat

## TL;DR

A 44-year-old woman with HHT and pre-capillary PH was successfully treated with riociguat, showing improvement without worsening bleeding symptoms.

## Contribution

This case report presents a successful treatment of pre-capillary PH in HHT using riociguat, a novel therapeutic approach.

## Key findings

- Right heart catheterization confirmed severe pulmonary arterial hypertension without portal hypertension.
- Treatment with riociguat improved pulmonary vascular resistance without exacerbating epistaxis or causing adverse effects.

## Abstract

Hereditary hemorrhagic telangiectasia (HHT) is a rare vascular disorder that is occasionally complicated by pulmonary hypertension (PH). In most cases where PH is associated with HHT, it is typically of a post-capillary type, while pre-capillary PH is relatively rare. Herein, we report a rare case of pre-capillary PH in a 44-year-old woman. She presented with recurrent epistaxis, mucocutaneous telangiectasias, a family history of hemorrhagic events, and hepatic arteriovenous malformations. She met the four Curaçao criteria and was diagnosed with HHT. Right heart catheterization demonstrated severe pulmonary arterial hypertension in the absence of portal hypertension. Given the risks of bleeding, management of both HHT and PH presents notable challenges. In this case, treatment with riociguat improved pulmonary vascular resistance (PVR) without exacerbating epistaxis or causing other adverse effects. These findings suggest that riociguat may represent a promising therapeutic option for HHT-associated pre-capillary PH.