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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

906
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...
906
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

671
Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
671
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

811
Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
811
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

791
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...
791
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

583
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...
583
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

626
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...
626

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Related Experiment Video

Updated: Apr 19, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
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Portopulmonary hypertension: an update.

Mateo Porres-Aguilar1, Debabrata Mukherjee

  • 1Department of Internal Medicine, Division of Cardiovascular Diseases, Texas Tech University Health Sciences Center/Paul L. Foster School of Medicine, El Paso, Texas, USA.

Respirology (Carlton, Vic.)
|December 20, 2014
PubMed
Summary

Portopulmonary hypertension, a lung vascular disorder, is diagnosed using echocardiography and right heart catheterization. Current therapies show promise for improving patient hemodynamics and survival, especially with timely liver transplantation.

Keywords:
liver diseaseliver transplantationportal hypertensionportopulmonary hypertensionpulmonary arterial hypertension

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Area of Science:

  • Cardiology
  • Pulmonology
  • Hepatology

Background:

  • Portopulmonary hypertension (PoPH) is a severe lung vascular disease linked to portal hypertension.
  • It can occur with or without significant liver disease.
  • Accurate diagnosis and management are crucial for patient outcomes.

Purpose of the Study:

  • To review the diagnostic approaches for PoPH.
  • To discuss current advancements in medical therapies for PoPH.
  • To highlight the importance of identifying liver transplant candidates.

Main Methods:

  • Review of existing literature on PoPH diagnosis and treatment.
  • Focus on echocardiography as an initial diagnostic tool.
  • Emphasis on right heart catheterization as the gold standard.

Main Results:

  • Transthoracic echocardiography is the primary initial diagnostic tool.
  • Right heart catheterization is essential for definitive diagnosis.
  • Case reports and series suggest therapeutic improvements in hemodynamics and RV function.

Conclusions:

  • Early recognition and diagnosis of PoPH are critical.
  • Medical therapies show potential benefits, though lacking large trials.
  • Identifying suitable liver transplant candidates is key to improving survival and quality of life.