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チアノーゼ性心疾患とは,先天性心疾患のなかでも,静脈血が動脈血に混合する右左シャントを有する疾患の総称である。小児期までの外科的治療によりほとんどの患者はチアノーゼのない血行動態となる。しかし,未治療の場合や単心室型循環でのFontan術未到達症例,Eisenmenger症候群などをはじめとして,チアノーゼを有したまま成人年齢に到達する患者も少なくない。
チアノーゼ性心疾患は,解剖学的にも血行動態的にも非常に複雑多岐にわたり,各病態への理解が重要なのは言うまでもない。本稿では,チアノーゼ性心疾患の総論的な病態生理について解説し,各病態へ応用が利くようまとめる。
Cyanotic congenital heart disease (CCHD) is a highly diverse anatomical and hemodynamic entity. Basic physiology can be applied to improve the understanding of the complex hemodynamics. It is not easy to determine the optimal level of SaO2 in patients with CCHD, but it is important to assess whether the oxygen supply is adequate to meet the oxygen demand of the tissues in each condition. Oxygen delivery (DO2) is mainly regulated by arterial oxygen saturation (SaO2), hemoglobin concentration (Hb), and cardiac output (CO). In patients with decreased SaO2, the Hb, and CO can act as compensatory mechanisms to preserve DO2. In single ventricle physiology with parallel circulation, SaO2 is determined by the pulmonary venous oxygen saturation (SpvO2), systemic venous oxygen saturation (SsvO2), and pulmonary to systemic blood flow ratio (Qp/Qs). While an increase of Qp/Qs leads to an increase in SaO2, it may also cause a decrease in DO2 due to decreased systemic blood flow. SsvO2 is an important indicator of DO2. A decrease in SsvO2 is critical, especially for these patients since it directly affects SaO2, thereby further decreasing DO2. Therefore, to maximize DO2, it is necessary to balance Qp/Qs and prevent anemia and low cardiac output.
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