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The epidemiology, diagnostic procedures, and management strategy of adult congenital heart disease-pulmonary artery hypertension Makoto TAKEI 1 , Shun KOHSAKA 2 1Department of Cardiology Tokyo Saiseikai Central Hospital 2Department of Cardiology Keio University School of Medicine pp.169-178
Published Date 2021/1/1
DOI https://doi.org/10.11477/mf.3102200844
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Pulmonary artery hypertension associated with adult congenital heart disease (ACHD-PAH) is an important prognostic factor, and is sub-classified into four categories, namely, Eisenmenger syndrome, PAH with persistent systemic to pulmonary shunt, PAH with small or coincidental defects, and PAH after defect closure according to the state of shunt repair and extension of pulmonary vascular disease. In recent years, significant advances have been made in the clinical management of ACHD-PAH. These advances include introduction of pulmonary artery specific vasodilators and minimally invasive shunt repair with novel catheter techniques. In this article, we reviewed the epidemiology, diagnostic procedure, and management strategy of ACHD-PAH according to this sub-classification.


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電子版ISSN 2186-7852 印刷版ISSN 1883-4833 メディカル・サイエンス・インターナショナル

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