March 2023 PVRI Community Call with Sudarshan Rajagopal & Gayathri Viswanathan

In this PH Community Call we discussed: 

• Risk Assessment Models and Event-Free Survival in Pulmonary Arterial Hypertension
• PINK1/Parkin Deficiency Enhances Vascular Remodeling and Aggravates Hypoxia-induced Pulmonary Hypertension

Pulmonary tumor thrombotic microangiopathy (PTTM) is a severe and fatal disease that rapidly causes pulmonary hypertension (PH). While imatinib shows potential for treating PH related to PTTM, it remains unclear if imatinib should continue or be discontinued after improvement in PH. Here, we present a case with PTTM in which PH was controlled for 9 months on imatinib, but recurred immediately after discontinuing of imatinib.

Pulmonary arterial hypertension (PAH) is a disorder characterized by progressive remodeling of small pulmonary arteries, leading to increased pulmonary vascular resistance, right ventricular failure and premature death (1-2). Over the past 30 years, significant advancements have been made in the treatment of PAH, including the recent approval of sotatercept, a first-in-class fusion protein that acts as a ligand trap for activins and growth differentiation factors, which are key players in the transforming growth factor β (TGF-β) superfamily (3-4).