Related Experiment Video

Updated: Mar 14, 2026

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

7.8K

Pulmonary arterial hypertension in congenital heart disease: Current perspectives and future challenges

George Giannakoulas1, Michael A Gatzoulis2

  • 1Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Greece.

Hellenic Journal of Cardiology : HJC = Hellenike Kardiologike Epitheorese
|September 20, 2016
PubMed

Insights

Research on pulmonary arterial hypertension (PAH) in adults with congenital heart disease (ACHD) is increasingly collaborative. Education and specialized care in tertiary centers are vital for managing PAH in ACHD patients.

Area of Science:

  • Cardiology
  • Pulmonology
  • Genetics

Background:

  • Pulmonary arterial hypertension (PAH) in adults with congenital heart disease (ACHD) research is becoming globalized and collaborative.
  • Education on congenital heart disease (CHD) and its specific manifestation in PAH is crucial for healthcare professionals and patients.
  • ACHD patients with PAH require specialized care in tertiary centers utilizing a multidisciplinary approach.

Approach:

  • Implementing shared care models between expert tertiary centers and local services.
  • Fostering collaboration among CHD and PAH specialists, geneticists, and epidemiologists.
  • Integrating PAH-CHD patients into national and international registries for comprehensive genotypic and phenotypic data collection.

Key Points:

  • Globalized, inclusive, and collaborative research in PAH-ACHD.
  • Paramount importance of physician, administrator, and patient education on CHD and PAH.
  • Necessity of tertiary center follow-up and multidisciplinary care for ACHD patients with PAH.
  • Shared care models enhance collaboration between expert and local services.
  • Networking among specialists and inclusion in registries with detailed characterization are vital.

Conclusions:

  • Enhanced collaboration and education are key to advancing PAH-ACHD care.
  • Tertiary centers and shared care models improve patient outcomes.
  • Registry inclusion with detailed data is essential for understanding PAH-CHD.
  • A multidisciplinary and collaborative approach is fundamental for managing PAH in ACHD.

Keywords:
Congenital heart diseaseEducationPulmonary arterial hypertensionRegistriesResearch

More Related Videos

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

14.7K
Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension09:23

Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension

Published on: February 11, 2017

17.7K

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...
766
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...
615
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
611
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,...
714
Cardiac Catheterization II: Right Heart Catheterization01:21

Cardiac Catheterization II: Right Heart Catheterization

Right Heart Catheterization: An OverviewRight heart catheterization is an invasive diagnostic procedure that measures right-sided cardiac and pulmonary artery pressures, calculates cardiac output, and identifies intracardiac shunts. It provides detailed hemodynamic data essential for diagnosing and managing various cardiovascular conditions, such as pulmonary hypertension.Access SitesCommon access sites for right heart catheterization include the internal jugular vein in the neck region, the...
1.6K
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...
532