Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects
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- Hidekazu Ishida
- Department of Pediatrics, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita 565-0871, Osaka, Japan
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- Hiroyuki Yamagishi
- Department of Pediatrics, Keio University of Medicine, 35 Shinanomachi, Shinjuku-ku 160-8582, Tokyo, Japan
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- Jun Maeda
- Department of Cardiology, Tokyo Metropolitan Children’s Medical Center, 2-8-29 Musashidai, Fuchu 183-8561, Tokyo, Japan
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- Keiko Uchida
- Department of Pediatrics, Keio University of Medicine, 35 Shinanomachi, Shinjuku-ku 160-8582, Tokyo, Japan
書誌事項
- 公開日
- 2023-08-03
- 資源種別
- journal article
- 権利情報
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- https://creativecommons.org/licenses/by/4.0/
- DOI
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- 10.3390/jcdd10080333
- 公開者
- MDPI AG
説明
<jats:p>Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique pediatric subtype of PH with unclear and/or multifactorial pathophysiological mechanisms, and is often associated with complex congenital heart disease (CHD), pulmonary atresia with ventricular septal defect, and aortopulmonary collateral arteries. Some cases of complex CHD, associated with a single ventricle after Fontan operation, show pathological changes in the small peripheral pulmonary arteries and pulmonary vascular resistance similar to those observed in pulmonary arterial hypertension (PAH). This condition is termed as the pediatric pulmonary hypertensive vascular disease (PPHVD). Recent advances in genetics have identified the genes responsible for PAH associated with developmental defects of the heart and lungs, such as TBX4 and SOX17. Targeted therapies for PAH have been developed; however, their effects on PH associated with developmental heart and lung defects remain to be established. Real-world data analyses on the anatomy, pathophysiology, genetics, and molecular biology of unique PPHVD cases associated with developmental defects of the heart and lungs, using nationwide and/or international registries, should be conducted in order to improve the treatments and prognosis of patients with these types of pediatric PH.</jats:p>
収録刊行物
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- Journal of Cardiovascular Development and Disease
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Journal of Cardiovascular Development and Disease 10 (8), 333-, 2023-08-03
MDPI AG
