成人先心病（48p）.ppt

* * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * Surgical Repair Balloon atrial septostomy Developed by Rashkind (1965) Enlarges the atrial communication Atrial switch Performed first by Senning (1958) and later modified by Mustard (1964) Atrial baffle is created to direct venous return to the contralateral ventricle Adult Presentation RV dysfunction Tricuspid insufficiency Bradyarrhythmias Atrial tachycardias Obstructed/leaky atrial baffle Surgical Repair Arterial switch Performed first by Jatene (1976) Great arteries transected and reattached to appropriate AV valve Coronary ostia also transplanted Surgical treatment of choice Excellent outcomes so far Adult Presentation Coronary perfusion issues Supravalvar aortic and pulmonic stenosis Aortic root dilatation and valvular insufficiency Branch pulmonary stenosis Single Ventricle Physiology Hypoplastic left-heart syndrome Hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum) Tricuspid valve atresia Mitral valve atresia Double-inlet ventricle Atrioventricular canal defects Double outlet right ventricle Surgical Repair Staged rerouting of the systemic venous return Step One Complete mixing of systemic and pulmonary venous return Unobstructed outflow tract to the systemic arterial circulation Reliable source of pulmonary blood flow Adult Presentation RV dysfunction Cyanosis Protein losing enteropathy Key Points The majority of children born with CHD survive into adulthood Infants born with complex CHD are seldom cured; residual problems are common and predictable There are many challenges in the transition of care from pediatric to adult practitioners Adults with moderate and complex CHD should undergo regular evaluation at a regional ACHD center Selected References Bashore TM. Adult congenital heart disease: right ventricular outflow tract lesions. Circulation 2007 115: 1933-1947. Gewillig M. The Fontan circulation. Heart 2005 91: 839-846. Hoffman JIE, Kaplan S. Th