Abstract
Optimal anesthetic management of infants and children with hypoplastic left heart syndrome (HLHS) entails a thorough understanding of the spectrum of anatomic and physiologic findings that can comprise this syndrome. Over the past two decades, a substantial amount has been learned about the unique considerations and challenges posed by patients with HLHS [1]. Some of these new concepts have been beneficial in the management of other forms of critical neonatal congenital heart disease (CHD), while others are nuances that uniquely apply to this malformation, the largest anatomic subset of patients with functional single ventricle.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Mahle WT, Spray TL, Wernovsky G., et al. Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution. Circulation. 102(19Supp 3):III 136–41, 2000.
Rychik J, Bush DM, Spray TL, et al. Assessment of pulmonary/systemic blood flow ratio after first-stage palliation for hypoplastic left heart syndrome: development of a new index with the use of doppler echocardiography. Journal of Thoracic & Cardiovascular Surgery. 102(1): 81–7, 2000.
Rossi AF, Sommer RJ, Lotvin A., et al. Usefulness of intermittent monitoring of mixed venous oxygen saturation after stage I palliation for hypoplastic left heart syndrome. American Journal of Cardiology. 73(15):1118–23, 1994.
Riordan CJ, Locher JP, Santamore WP, et al. Monitoring systemic venous oxygen saturations in hypoplastic left heart syndrome. Annals of Thoracic Surgery. 63(3):83–55, 1997.
Day RW, Barton AJ, Pysher, et al. Pulmonary vascular resistance of children treated with nitrogen during early infancy. Annals of Thoracic Surgery. 65(5):1400–4, 1998.
Jobes DR, Nicolson SC, Steven JM, et al. Carbon dioxide prevents pulmonary overcirculation in hypoplastic left heart syndrome. Annals of Thoracic Surgery. 54(1):150–1, 1992.
Chang AC, Zucker HA, Hickey PR et al. Pulmonary vascular resistance in infants after cardiac surgery: role of carbon dioxide and hydrogen ion. Critical Care Medicine. 23(3):568–74, 1995.
Wessel, DL. Commentary: simple gases and complex single ventricles. [letter; comment]. Journal of Thoracic & Cardiovascular Surgery. 112(3):665–7, 1996.
Gaynor JW, Mahle WT, Cohen MI, et al. Risk factors for mortality after the Norwood procedure. European Journal of Cardiothoracic Surgery, in press.
Hebra A, Brown, MF, Hirschl RB, et al. Mesenteric ischemia in hypoplastic left heart syndrome. Journal of Pediatric Surgery. 28(4):606–11, 1993.
McElhinney DB, Hedrick HL, Bush DM, et al. Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes. Pediatrics. 106(5):1080–7, 2000.
Hansen DD, Hickey PR. Anesthesia for hypoplastic left heart syndrome: use of high-dose fentanyl in 30 neonates. Anesthesia & Analgesia. 65(2):127–32, 1986.
Hickey PR, Hansen DD, Wessel DL, et al. Blunting of stress responses in the pulmonary circulation of infants by fentanyl. Anesthesia & Analgesia. 64(12):1137–42, 1985.
Hickey PR, Hansen DD. High-dose fentanyl reduces intraoperative ventricular fibrillation in neonates with hypoplastic left heart syndrome. Journal of Clinical Anesthesia. 3(4):295–300, 1991.
Anand Kj, Hickey PR. Halothane-morphine compared with high-dose sufentanil for anesthesia and postoperative analgesia in neonatal cardiac surgery. New England Journal of Medicine. 326(1):1–9, 1992.
Gruber EM, Laussen PC, Casta A, et al. Stress response in infants undergoing cardiac surgery: a randomized study of fentanyl bolus, fentanyl infusion, and fentanylmidazolam infusion. Anesthesia & Analgesia. 92(4):882–90, 2001.
Tabbutt S, Ramamoorthy C, Montenegro LM, et al. Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation. 2001;104[suppl 1]:1–159-1–64.
Atz AM, Feinstein JA, Jonas RA, et al. Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect. American Journal of Cardiology. 83(8):1224–8, 1999.
Rychik J, Rome JJ, Collins MH, et al. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. Journal of the American College of Cardiology. 34(2):554–60, 1999.
Naik SK, Knight A, Elliott MJ. A successful modification of ultrafiltration for cardiopulmonary bypass in children. Perfusion. 6(1):41–50, 1991.
Elliott MJ. Ultrafiltration and modified ultrafiltration in pediatric open-heart operations. [Review] [19 refs]. Annals of Thoracic Surgery. 56(6):1518–22, 1993.
Bando K, Turrentine MW, Vijay P, et al. Effect of modified ultrafiltration in high-risk patients undergoing operations for congenital heart disease. Annals of Thoracic Surgery. 66(3):821–7, 1998.
Davies MJ, Nguyen K, Gaynor JW, et al. Modified ultrafiltration improves left ventricular systolic function in infants after cardiopulmonary bypass. Journal of Thoracic & Cardiovascular Surgery. 115(2):361–9, 1998.
Skaryak LA, Kirshbom PM, DiBernardo LR, et al. Modified ultrafiltration improves cerebral metabolic recovery after circulatory arrest. Journal of Thoracic & Cardiovascular Surgery. 109(4):744–51, 1995.
Kern FH, Morana NJ, Sears JJ, et al. Coagulation defects in neonates during cardiopulmonary bypass. Annals of Thoracic Surgery. 54(3):541–6, 1992.
Manno CS, Hedberg KW, Kim HC, et al. Comparison of the hemostatic effects of fresh whole blood, stored whole blood, and components after open-heart surgery in children. Blood. 77(5):930–6, 1991.
Hoffman GM, Ghanayem NS, Kampine JM, et al. Venous saturation and the anaerobic threshold in neonates after the Norwood procedure for hypoplastic left heart syndrome. Annals of Thoracic Surgery. 70(5):1515–20, 2000.
Taeed R, Schwartz SM, Pearl JM, et al. Unrecognized pulmonary venous denaturation early after Norwood palliation confounds Qp:Qs assessment and compromises oxygen delivery. Circulation. 103(22):2699–704, 2001.
Haworth SG. Pulmonary vascular disease in different types of congenital heart disease. Implications for interpretation of lung biopsy findings in early childhood. British Heart Journal. 52(5):557–71, 1984.
Haworth SG, Reid L. Structural study of pulmonary circulation and of heart in total anomalous pulmonary venous return in early infancy. British Heart Journal. 39(1):80–92, 1977.
Better DJ, Apfel HD, Zidere V, et al. Pattern of pulmonary venous blood flow in the hypoplastic left heart syndrome in the fetus. Heart. 81(6):646–9, 1999.
Morray JP, Lynn AM, Mansfield PB. Effect of pH and PCO2 on pulmonary and systemic hemodynamics after surgery in children with congenital heart disease and pulmonary hypertension. Journal of Pediatrics. 113(3):474–9, 1988.
Russell IA, Zwass MS, Finerman JR, et al. The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease. Anesthesia & Analgesia. 87(1):46–51, 1998.
Atz AM, Wessel DL. Inhaled nitric oxide in the neonate with cardiac disease. Seminars in Perinatology. 21(5):441–55, 1997.
Donofrio MT, Jacobs ML, Spray TL, et al. Acute changes in preload, afterload, and systolic function after superior cavopulmonary connection. Annals of Thoracic Surgery. 65(2):503–8, 1998.
Fyfe DA, Kline CH, Sade RM, et al. The utility of transesophageal echocardiography during and after Fontan operations in small children. American Heart Journal. 122(5):1403–15, 1991.
Williams DB, Kiernan PD, Metke MP, et al. Hemodynamic response to positive end-expiratory pressure following right atrium-pulmonary artery bypass (Fontan procedure). Journal of Thoracic & Cardiovascular Surgery. 87(6):856–61, 1984.
Koutlas TC, Gaynor JW, Nicolson SC, et al. Modified ultrafiltration reduces postoperative morbidity after cavopulmonary connect. Annals of Thoracic Surgery. 64(1):37–42, 1997.
Cohen MI, Wernovsky G, Vetter VL, et al. Sinus node function after systemically stage Fontan procedure. Circulation. 98(19 Suppl):11352–8, 1998.
Penny DJ, Redington AN. Doppler echocardiographic evaluation of pulmonary blood flow after the Fontan operation: the role of the lungs. British Heart Journal. 66(5):37–24, 1991.
Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 82(5):1681–9, 1990.
Laks H, Pearl JM, Haas GS, et al. Partial Fontan: advantages of an adjustable interatrial communication. Annals of Thoracic Surgery. 52(5):1084–94, 1991.
Douville EC, Sade RM, Fyfe DA. Hemi-Fontan operation in surgery for single ventricle: a preliminary report. Annals of Thoracic Surgery. 51(6):893–9, 1991.
Frommelt PC, Snider AR, Meliones JN, et al. Doppler assessment of pulmonary artery flow patterns and ventricular function after the Fontan operation. American Journal of Cardiology. 68(11):1211–5, 1991.
Nicolson SC, Steven JM, Kurth CD, et al. Anesthesia for noncardiac surgery infants with hypoplastic left heart syndrome following Hemi-Fontan operation. Journal of Cardiothoracic & Vascular Anesthesia. 8(3):334–6, 1994.
Karl HW, Hensley FA, Cyran SE, et al. Hypoplastic left heart syndrome: anesthesia for elective noncardiac surgery. Anesthesiology. 72(4):753–7, 1990.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2003 Springer Science+Business Media New York
About this chapter
Cite this chapter
Steven, J.M., Nicolson, S.C. (2003). Anesthetic Management for HLHS. In: Rychik, J., Wernovsky, G. (eds) Hypoplastic Left Heart Syndrome. Developments in Cardiovascular Medicine, vol 246. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-0253-1_10
Download citation
DOI: https://doi.org/10.1007/978-1-4615-0253-1_10
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4613-4988-4
Online ISBN: 978-1-4615-0253-1
eBook Packages: Springer Book Archive