Learning and research

Although right ventricle (RV) dysfunction drives clinical worsening in pulmonary hypertension (PH), information about RV function has not been well integrated in PH risk assessment. The gold standard for assessing RV function and ventriculo-arterial coupling is the construction of multi-beat pressure–volume (PV) loops.

In the study “Echocardiographic grading of right ventricular afterload in left heart disease: relation to right ventricular function, pulsatile and resistant load, and outcome,” Bech-Hanssen et al. addressed an interesting topic about severity gradation using right ventricular (RV) afterload echocardiographic assessment.

The history of advances in understanding pulmonary circulation is long, dating to the 13th century. Subsequent centuries produced halting progress until the 20th century when investigators applied right heart catheterization and light-microscopy to better understand the physiology and pathology of pulmonary hypertension (PH). The work of Paul Wood, Cornelis Wagenvoort, and others during the first seven decades of the 20th century set the stage for five decades of extraordinary progress.

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.