Related Experiment Video
Updated: Jul 18, 2025
08:34
Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
7.2K
Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
Hidekazu Ishida1, Jun Maeda2, Keiko Uchida3,4
1Department of Pediatrics, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita 565-0871, Osaka, Japan.
Journal of Cardiovascular Development and Disease
|August 25, 2023
Summary
Pediatric pulmonary hypertension (PH) has unique subtypes like segmental PH and pediatric pulmonary hypertensive vascular disease (PPHVD), often linked to congenital heart disease. Further research is needed to understand their mechanisms and improve treatments.
Area of Science:
- Cardiology
- Pulmonology
- Pediatric Medicine
Background:
- Pediatric pulmonary hypertension (PH) presents unique challenges compared to adult forms.
- Segmental PH and pediatric pulmonary hypertensive vascular disease (PPHVD) are distinct pediatric subtypes, frequently associated with complex congenital heart disease (CHD).
- Specific genetic factors like TBX4 and SOX17 are implicated in PH related to heart and lung developmental defects.
Purpose of the Study:
- To highlight the unique characteristics of pediatric PH, including segmental PH and PPHVD.
- To emphasize the need for further investigation into the pathophysiology, genetics, and molecular biology of these pediatric conditions.
- To underscore the importance of real-world data analysis for improving treatment strategies and patient outcomes.
Main Methods:
- Review of existing literature on pediatric PH, focusing on unique subtypes and associated conditions.
- Identification of genetic factors implicated in PH associated with developmental defects.
- Discussion of the need for large-scale data analysis using registries.
Main Results:
- Pediatric PH, particularly segmental PH and PPHVD, exhibits distinct pathophysiological mechanisms and associations with complex CHD.
- Genes such as TBX4 and SOX17 have been identified in PH related to developmental heart and lung defects.
- The efficacy of current targeted PAH therapies in pediatric populations with developmental defects remains largely unestablished.
Conclusions:
- Pediatric PH subtypes like segmental PH and PPHVD require specialized understanding due to their unique mechanisms and associations with CHD.
- Targeted therapies for adult pulmonary arterial hypertension (PAH) may not be directly applicable to these pediatric conditions.
- Conducting real-world data analyses using nationwide and international registries is crucial for advancing the treatment and prognosis of pediatric PH.
Keywords:
Fontan circulationcongenital heart diseasedevelopmental disordersgenetic analysismajor aortopulmonary collateral arteriespulmonary hypertensiontetralogy of FallotMore Related Videos
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
222
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...
There are various classifications for PH, each relating to different underlying causes and also...
222
01:29Pulmonary Embolism I: Introduction
7
Pulmonary embolism (PE) occurs when a thrombus, fat or air embolus, amniotic fluid, or tumor tissue blocks one or more pulmonary arteries. These blockages originate in the venous system or the right side of the heart.EtiologyPE primarily arises from deep vein thrombosis (DVT) and other hypercoagulable states, such as inherited thrombophilias. Additional etiological factors include venous stasis, commonly seen in obesity, and endothelial injury from surgery and trauma. Less common causes include...
7
01:17Other Pulmonary Disorders
872
Respiratory disorders encompass a range of conditions with varying levels of severity. Asthma, marked by chronic airway inflammation and hypersensitivity, is one such condition. It can lead to airway obstruction due to factors like bronchial spasms, mucosal edema, increased mucus secretion, or epithelial damage. Asthma triggers are diverse, ranging from allergens to emotional upset, and treatment focuses on both immediate relief through bronchodilators and long-term inflammation suppression.
872
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
201
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...
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
201
01:22Mitral Stenosis I: Introduction
16
Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
16
01:20Chronic Obstructive Pulmonary Disease-I: Introduction
2.8K
Chronic Obstructive Pulmonary Disease (COPD) is a long-lasting respiratory condition requiring continuous attention and care. It is a progressive lung disease that leads to breathing challenges due to airflow obstruction. It manifests as persistent respiratory symptoms and restricted airflow resulting from abnormalities in the airways and alveoli, usually due to long-term exposure to harmful particles or gases. COPD mainly consists of two primary conditions: emphysema and chronic bronchitis.
2.8K