Anestecia

Anesthetic Management of Pediatric Patients with Hypoplastic Left Heart Syndrome for Non-Cardiac Surgery
Presented to the Faculty of the Gooding Institute of Nurse Anesthesia Bay Medical Center 
in Partial Fulfillment of the Requirements for the Degree 
Master of Science in Nurse Anesthesia 
by Frank A. Jaworski, BSN, RN 
July, 2000
ContentsI. Introduction ............................................................1 
II. Analysis ..................................................................3 
III. Evaluation ..............................................................9 
IV. Conclusions ............................................................9 
V. References .............................................................11 
I. Introduction
Providing safe and effectiveanesthesia for patients with pre-existing health conditions is one of the greatest challenges for the anesthesia provider. A rapidly growing population in need of attention from anesthesia providers is pediatric patients who have survived surgical therapy for congenital heart lesions and present for non-cardiac surgery. These patients will require surgery for problems secondary to their heartdefects such as placement of feeding tubes, tracheostomy or placement of indwelling venous access, as well as common pediatric procedures, such as tonsillectomy, myringotomy and placement of pressure-equalization tubes, appendectomy, circumcision or hernia repair. Patients with congenital heart disease (CHD) have a higher incidence of non-cardiac congenital anomalies, as well as a greater need fordiagnostic procedures which will increase their need for anesthetic services. (17.; 104 and 19.; 183) Survival rate to five years of age for these patients is approximately 70%. (22.; 3) This author has observed his own child, diagnosed with hypoplastic left heart syndrome (HLHS), successfully undergo surgical palliation and reconstruction and anticipates the need for well-informed anesthesiaproviders in the future.
Congenital heart defects occur in 4-8 per 1,000 live births, with hypoplastic left heart syndrome occurring in 0.5 - 2.4 per 10,000 live births, ranking HLHS as high as 5th in frequency of CHDs in some studies. Hypoplastic left heart syndrome is a common and severe malformation.(6.; 5) Hypoplastic left heart syndrome is one of the last forms of congenital heart disease toundergo effective surgical treatment. It is a complex defect which requires either a three-stage surgical reconstruction or transplantation for survival. (10.; 1) Even when these patients have been surgically “repaired” the physiologic consequences of their congenital heart disease and its surgical therapy affect respiratory compliance, heart rate and potential for arrhthymias, pulmonary and systemicvascular pressures, the need for adrenergic agonists and antagonists, the uptake of anesthetic agents, and coagulability. (8.;2) Congenital heart defects were among the first cardiac pathologies repaired surgically, as with the closed heart procedure for ligation of patent ductus arteriosus and the early work of Lillehei in the repair of atrial and ventricular septal defects. (4.; 47-64) This workpredates the now more commonly known coronary artery bypass graft. One of the complex lesions that has evaded effective surgical intervention until relatively recently is hypoplastic left heart syndrome. (10.;1)
The primary criteria for defining HLHS are hypoplastic or absent morphologic left ventricle, aortic valve hypoplasia, stenosis or atresia and mitral valve hypoplasia, stenosis or atresia.Secondary criteria are ductus dependent systemic circulation (with the exception of critical aortic stenosis with hypoplastic left ventricle), ascending aorta hypoplasia and aortic arch hypoplasia. It may also include double outlet right ventricle, interrupted aortic arch, atrioventricular septal defect (canal), anomalous pulmonary venous connection and atrial isomerism. (5.; 30) Because of...