Congenital Heart Disease

Abstract

Congenital heart disease is one of the most common congenital defects in children. A physiological classification of congenital heart disease into four groups is most useful for anesthetists: left-to-right shunts with increased pulmonary blood flow; right-to-left shunts with decreased pulmonary blood flow; complex shunts with mixing of pulmonary and systemic blood flow causing cyanosis, and finally obstructive lesions, such as valvular or major vessel stenosis. During anesthesia in children with cyanotic congenital heart disease, it is critical to maintain a balance between the pulmonary and systemic vascular resistance to avoid hypoxia or inadequate systemic perfusion. Anesthesia in children with a Fontan circulation is particularly challenging and is best performed in a specialist pediatric center. Endocarditis prophylaxis is needed for some children with heart disease having certain procedures. Murmurs are common in children, and most can be identified as innocent at the time of preoperative assessment. Some murmurs may be suspected as being pathological but without significant issues for anesthesia, while others may be suspected as being pathological and require deferral of anesthesia and investigation.

Review Questions

• 1. A 4 year old girl with Trisomy 21 had an VSD repaired while an infant. She is active and well and is scheduled for a dental procedure. Could her procedure be performed in a regional hospital? Does she need endocarditis prophylaxis?

• 2. A 6 month old infant with hypoplastic right ventricle had a modified Blalock Taussig shunt performed soon after birth and is now scheduled for surgery. Why would this child have had a shunt so soon after birth? What factors will affect the child’s SpO₂ during anesthesia?