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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

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...
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

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, improving...
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

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

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

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 (ECE). Of...
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

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...

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Registry-based estimation of cardiac event-free survival in congenital heart disease complicated by pulmonary hypertension: A nationwide registry study from Japan.

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Mid-term prognosis and risk stratification in patients with post-operative pulmonary hypertension: Insights from the Japanese Association of Congenital Heart Disease Registry (JACPHR).

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Related Experiment Video

Updated: May 7, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

Current challenges in pediatric pulmonary hypertension.

Shinichi Takatsuki1, David Dunbar Ivy

  • 1Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado.

Seminars in Respiratory and Critical Care Medicine
|September 17, 2013
PubMed
Summary

Pediatric pulmonary arterial hypertension (PAH) has various causes, primarily idiopathic or linked to congenital heart disease (CHD). Current treatments rely on adult data, highlighting a need for pediatric-specific research and guidelines.

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Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat

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Last Updated: May 7, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
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Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension

Published on: June 27, 2025

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
08:34

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat

Published on: November 18, 2018

Area of Science:

  • Pediatric Cardiology
  • Pulmonary Hypertension Research
  • Clinical Pediatrics

Background:

  • Pediatric pulmonary arterial hypertension (PAH) presents significant morbidity and mortality.
  • Common causes include idiopathic PAH and PAH associated with congenital heart disease (CHD).
  • Pulmonary hypertension (PH) linked to connective tissue disease is rare in children.

Purpose of the Study:

  • To review the classification, incidence, and treatment of pediatric PAH.
  • To highlight the development of a pediatric-specific PH classification.
  • To discuss the challenges in pediatric PAH treatment due to reliance on adult studies.

Main Methods:

  • Review of existing literature and classification systems for pediatric PH.
  • Analysis of incidence data for idiopathic PAH and PAH-CHD from the Netherlands.
  • Examination of treatment strategies, including selective pulmonary vasodilators.

Main Results:

  • Incidence rates for idiopathic PAH and PAH-CHD per million children were reported.
  • New pediatric-specific PH classification acknowledges developmental and fetal origins.
  • Disparate treatment recommendations exist for sildenafil in the US and Europe.

Conclusions:

  • Pediatric PAH requires tailored approaches due to unique etiologies and developmental factors.
  • Evidence-based treatment for pediatric PAH is evolving, with ongoing research needed.
  • Standardized pediatric guidelines are crucial for consistent and effective management.