Normal resting and exercise hemodynamics in pulmonary arterial hypertension: The roadmap to prostacyclin withdrawal?

Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...

Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...

The movement of blood in a human body, commonly referred to as blood flow, is determined by the volume of blood that traverses a certain section of the bodily system per unit time. It is the rhythmic contraction of the heart's ventricles that primarily instigates this movement. As the ventricles contract, blood is forced into the prominent arteries, which then flow from areas of greater pressure to lower pressure areas. This movement continues into smaller arteries and arterioles and...

Assessing blood pressure is a standard procedure executed in virtually all medical environments. The method utilized today was established over a hundred years ago by an innovative Russian doctor, Dr. Nikolai Korotkoff. The soft ticking noise, known as Korotkoff sounds, heard while taking blood pressure readings results from turbulent blood flow within the vessels. The apparatus required for this procedure includes a sphygmomanometer, a blood pressure cuff attached to a gauge, and a...