My Introduction to Pulmonary Hypertension

In 1976, my wife and I moved to Salt Lake City to start my fellowship in pulmonary and critical care medicine. The University of Utah program had applied principles of physiology to the care of critically ill patients; and that interested me. As a critical care fellow, I learned to perform pulmonary artery catheterization and to interpret hemodynamic measurements in patients with adult respiratory distress syndrome (ARDS).

My journey into studying nitric oxide (NO) bioavailability developed as I explored the role of nitrite ions. The traditional view that nitrite was merely an inactive byproduct of NO oxidation was a perspective I sought to challenge. As I continued my research at Papworth and Addenbrooke's Hospitals under Professor Higenbottam, I hypothesized that nitrite ions could be a source of NO bioactivity.

The article, “Survival Outcomes and Impact of Targeted PAH Therapy in Portopulmonary Hypertension in the PVRI GoDeep Meta-Registry” by Jose et al. addresses the controversial question on the survival benefit of PAH drugs in portopulmonary hypertension (PoPH) [1]. Treatment strategies in PoPH are often extrapolated from other PAH subtypes as PoPH have largely been excluded from large PAH drug randomized trials. In this context, the present study represents an important contribution to the management of PoPH.

Pfizer initiated their Phase III trial of sildenafil for the treatment of PAH (SUPER study), which later led to the publication of the results [4] and the approval of sildenafil (20 mg, three times a day) in 2006 for the management of PAH. This period marked a pivotal moment in the medical history of treating PAH. I witnessed firsthand how this development transformed the landscape of treatment options for patients suffering from this severe condition.