Abstract
The number of adults with congenital heart disease (ACHD), especially those with complex ACHD, is now rapidly increasing owing to the recent medical advances and physicians may have to deal with these ACHD patients. However, most of these complex repaired ACHD patients have unique hemodynamic pathophysiology that is quite different from that in general acquired cardio- vascular diseases. Therefore, understanding of the ACHD-associated heart failure pathophysiology is mandatory to manage these patients. These unique ACHD patients include those with single ventricular physiology, namely Fontan circulation, those with circulatory system supported by non- left ventricle or non-mitral valve, for instance, those with transposition of the great arteries after atrial switch operation, and those with unrepaired circulatory condition with significant intra-cardiac shunting where hypoxia usually exists. In addition, more importantly, surgery-related unique patho- physiology has significant influences on circulatory regulation in these ACHD patients. Because of the limited space, some common ACHD patho- physiology is discussed to help physicians under- stand and manage these patients in their practice.
