Transcription factor TBX4 rare variants associate with pulmonary arterial hypertension (PAH), particularly in children, and are the second most common cause of heritable PAH. However, TBX4's down-stream targets and the molecular and cellular pathways these targets regulate remain largely unknown in PAH. 

Pulmonary hypertension (PH) in newborn babies is a relatively rare, heterogeneous condition that has high associated mortality in the neonatal period and beyond. There are limited evidence-based strategies to treat or prevent this condition.

Pulmonary hypertension (PH) constitutes a critical challenge in cardiopulmonary medicine with a pathogenesis that is multifaceted and intricate. Ion channels, crucial determinants of cellular electrochemical gradient modulation, have emerged as significant participants in the pathophysiological progression of PH. 

Female sex increases risk of Group I pulmonary arterial hypertension by roughly threefold, but the mechanism is unclear. Low expression of Cyp1b1, an enzyme that metabolizes estrogens, is associated with disease penetrance, particularly in women. We previously found that lower Pparγ levels in murine PAH models, which may drive disease, are rescued by estrogen blockade.

Resting heart rate has been incorporated in REVEAL risk assessment. Rest and sleep heart rate variability (HRV) measured in the home setting could provide early insight into worsening physiology in patients with pulmonary arterial hypertension (PAH). 

Although current guidelines recommend standard cardiopulmonary exercise testing (CPET) to evaluate symptomatic patients after pulmonary embolism (PE), CPET with simultaneous echocardiography could provide relevant information to evaluate right ventricular–pulmonary arterial coupling.

Treatment patterns of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Finland are unknown. Guidelines now recommend early escalation of treatment for PAH. We evaluated how well Finnish practice follows guidelines, and how treatment initiations and outcomes are related.

My first clinical experience with Pulmonary Hypertension occurred when, as a resident at Duke University Hospital in the late 1970's, a patient with “primary pulmonary hypertension (PPH)” was admitted with right heart failure.

Idiopathic pulmonary fibrosis (IPF) is a leading indication for lung transplantation. Pulmonary hypertension (PH), a common comorbidity in IPF, has gained renewed attention following the updated ESC/ERS guidelines, which redefine diagnostic thresholds for PH.