Learning and research

This serendipitous encounter, initially sparked by a single patient, began my profound and enduring interest in pulmonary vascular disease, particularly pulmonary hypertension. Learning from this, my career has been shaped, to a large extent, by clinical observation, translational research, and collaboration with peers, all driven by a curiosity to understand the complex mechanisms of pulmonary vascular disease. This journey underscores the value of curiosity-driven research, demonstrating how unexpected clinical encounters can profoundly influence a medical career, hopefully leading to advancements in understanding and treating pulmonary hypertension. You could easily say that pentazocine hooked me.

Acute lung injury (ALI) involves inflammatory cytokines and chemokines, resulting in lung and multiple organ injuries. This study explored the mechanism of mitophagy and cGAS/STING pathway in oleic acid (OA)-induced ALI. Mice and pulmonary microvascular endothelial cells were divided into four groups: control group (Con), ALI group, FUNDC1−/− control group (F-Con), and FUNDC1−/− ALI group (F-ALI). After 24 h of modeling, proceed with tissue collection. Lung tissues were stained using hematoxylin eosin.

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.

In 1979, during my cardiology fellowship, a 16-year-old with primary pulmonary hypertension (PPH), a rare and severe condition at that time, spurred my research. There was limited literature then. My pediatric cardiology colleagues, who were experienced in dealing with pulmonary hypertension linked to congenital heart diseases, introduced me to vasoreactivity testing, a method involving the administration of oxygen and tolazoline to assess operability. Tolazoline administration resulted in a significant decrease in pulmonary artery (PA) pressure, suggesting potential therapeutic responsiveness, though diuretics were the only treatment for pulmonary hypertension and right heart failure at that time. This prompted my search for a viable solution.

Pulmonary arterial hypertension (PAH) in intermediate-risk patients poses a challenge for clinicians, particularly in determining the optimal timing for escalating pharmacological treatment. We conducted a multicenter cross-sectional study that analyzed data from the Colombian Pulmonary Hypertension Network (HAPredCO) on patients diagnosed with PAH. 

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by persistent obstruction and vascular remodeling of the pulmonary arteries following pulmonary thromboembolism (PTE), diagnosed after a minimum of 3 months of therapeutic anticoagulation. 

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.

Segmental pulmonary hypertension (PH) in congenital heart disease remains poorly understood with data limited to case studies. We performed a retrospective, single center study in children treated with PH medications after unifocalization/pulmonary artery reconstruction for major aortopulmonary collaterals (MAPCA). Drug response was determined by hemodynamic changes across at least two cardiac catheterizations.