Hostname: page-component-586b7cd67f-r5fsc Total loading time: 0 Render date: 2024-11-26T15:25:33.408Z Has data issue: false hasContentIssue false
  • English
  • Français

Neonatal physiology of the functionally univentricular heart

Published online by Cambridge University Press:  21 September 2005

David P. Nelson ,
Steven M. Schwartz  and
Anthony C. Chang
David P. Nelson
Affiliation:
Cardiac Intensive Care Unit, The Heart Center at Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
Steven M. Schwartz
Affiliation:
Cardiac Intensive Care Unit, The Heart Center at Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
Anthony C. Chang
Affiliation:
Cardiac Intensive Care, Texas Children's Hospital, Houston, Texas, USA
Get access Rights & Permissions [Opens in a new window]

Extract

The term “functionally single ventricle” includes a variety of congenital cardiac anomalies where there is only one ventricle pumping blood to the systemic and pulmonary circulations. The physiology in this arrangement is a considerable challenge for the cardiac specialist, because the complexity encountered in patients with these lesions necessitates particularly specialized medical and surgical management. Patients with such functionally univentricular physiology often respond to common interventions, such as supplemental oxygen, mechanical ventilation, and vasoactive drugs, differently than patients with conventional circulations.1 Furthermore, these patients tend to be encountered more frequently by pediatricians and cardiologists because they undergo multiple operations, may be more adversely affected by intercurrent illnesses, or have chronic cardiac problems requiring frequent attention. A thorough understanding of the complexities of the physiology encountered is thus imperative for the pediatric cardiologist. In this review, we will address important physiologic and anatomic principles that influence care of neonates with functionally univentricular hearts. Although the anatomy and physiology of each reconstructive stage of palliation are unique, we will focus upon the pre- and post-operative physiology as encountered in the neonate.

Type
Norwood Procedure and Staged Palliation
Information
Cardiology in the Young , Volume 14 , Issue S1 , February 2004 , pp. 52 - 60
Copyright
© 2004 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Schwartz SM, Taeed R, Nelson DP. Intensive Care of Infants and Children with Hypoplastic Left Heart Syndrome. In: Hennein HA, Bove EL (eds). Hypoplastic Left Heart Syndrome. Armonk, NY: Futura 2002.
Atz AM, Feinstein JA, Jonas RA, Perry SB, Wessel DL. Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect. Am J Cardiol 1999; 83: 1224–1228.Google Scholar
Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 1999; 34: 554–560.Google Scholar
Imoto Y, Kado H, Shiokawa Y, Fukae K, Yasui H. Norwood procedure without circulatory arrest. Ann Thorac Surg 1999; 68: 559–561.Google Scholar
Pearl JM, Nelson DP, Schwartz SM, Manning PB. First-stage palliation for hypoplastic left heart syndrome in the twenty-first century. Ann Thorac Surg 2002; 73: 331–339; discussion 339–340.Google Scholar
Pigula FA, Siewers RD, Nemoto EM. Regional perfusion of the brain during neonatal aortic arch reconstruction. J Thorac Cardiovasc Surg 1999; 117: 1023–1024.Google Scholar
Pigula FA, Nemoto EM, Griffith BP, Siewers RD. Regional low-flow perfusion provides cerebral circulatory support during neonatal aortic arch reconstruction. J Thorac Cardiovasc Surg 2000; 119: 331–339.Google Scholar
Pigula FA. Arch reconstruction without circulatory arrest: Scientific basis for continued use and application to patients with arch anomalies. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 104–115.Google Scholar
Schermerhorn ML, Tofukuji M, Khoury PR, Phillips L, Hickey PR, Sellke FW, Mayer JE, Nelson DP. Sialyl Lewisx Oligosaccharide Preserves Cardiopulmonary and Endothelial Function after Hypothermic Circulatory Arrest in Lambs. J Thorac Cardiovasc Surg 2000; 120: 230–237.Google Scholar
Paparella D, Yau TM, Young E. Cardiopulmonary bypass induced inflammation: pathophysiology and treatment. An update. Eur J Cardiothorac Surg 2002; 21: 232–244.Google Scholar
Wessel DL, Adatia I, Giglia TM, Thompson JE, Kulik TJ. Use of inhaled nitric oxide and acetylcholine in the evaluation of pulmonary hypertension and endothelial function after cardiopulmonary bypass. Circulation 1993; 88: 2128–2138.Google Scholar
Freedom RM, Sondheimer H, Sische R, Rowe RD. Development of “subaortic stenosis” after pulmonary arterial banding for common ventricle. Am J Cardiol 1977; 39: 78–83.Google Scholar
Webber SA, LeBlanc JG, Keeton BR, Salmon AP, Sandor GG, Lamb RK, Monro JL. Pulmonary artery banding is not contraindicated in double inlet left ventricle with transposition and aortic arch obstruction. Eur J Cardiothorac Surg 1995; 9: 515–520.Google Scholar
Rossi AF, Sommer RJ, Lotvin A, Gross RP, Steinberg LG, Kipel G, Golinko RJ, Griepp RB. Usefulness of intermittent monitoring of mixed venous oxygen saturation after stage I palliation for hypoplastic left heart syndrome. Am J Cardiol 1994; 73: 1118–1123.Google Scholar
Tweddell JS, Hoffman GM, Fedderly RT, Ghanayem NS, Kampine JM, Berger S, Mussatto KA, Litwin SB. Patients at risk for low systemic oxygen delivery after the Norwood procedure. Ann Thorac Surg 2000; 69: 1893–1899.Google Scholar
Tweddell JS, Hoffman GM, Mussatto KA, Fedderly RT, Berger S, Jaquiss RD, Ghanayem NS, Frisbee SJ, Litwin SB. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 2002; 106: I82–89.Google Scholar
Tweddell JS, Hoffman GM. Postoperative management in patients with complex congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 187–205.Google Scholar
Tweddell JS, Hoffman GM, Fedderly RT, Berger S, Thomas JP, Jr, Ghanayem NS, Kessel MW, Litwin SB. Phenoxybenzamine improves systemic oxygen delivery after the Norwood procedure. Ann Thorac Surg 1999; 67: 161–167; discussion 167–168.Google Scholar
Taeed R, Schwartz SM, Pearl JM, Raake JL, Beekman RH 3rd, Manning PB, Nelson DP. Unrecognized pulmonary venous desaturation early after Norwood palliation confounds Gp:Gs assessment and compromises oxygen delivery. Circulation 2001; 103: 2699–2704.Google Scholar
Hoffman GM, Ghanayem NS, Kampine JM, Berger S, Mussatto KA, Litwin SB, Tweddell JS. Venous saturation and the anaerobic threshold in neonates after the Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg 2000; 70: 1515–1520; discussion 1521.Google Scholar
Riordan CJ, Locher JP Jr., Santamore WP, Villafane J, Austin EH, 3rd. Monitoring systemic venous oxygen saturations in the hypoplastic left heart syndrome. Ann Thorac Surg 1997; 63: 835–837.Google Scholar
Barnea O, Santamore WP, Rossi A, Salloum E, Chien S, Austin EH. Estimation of oxygen delivery in newborns with a univentricular circulation. Circulation 1998; 98: 1407–1413.Google Scholar
Barnea O, Austin EH, Richman B, Santamore WP. Balancing the circulation: theoretic optimization of pulmonary/systemic flow ratio in hypoplastic left heart syndrome. J Am Coll Cardiol 1994; 24: 1376–1381.Google Scholar
Francis DP, Willson K, Thorne SA, Davies LC, Coats AJ. Oxygenation in patients with a functionally univentricular circulation and complete mixing of blood: are saturation and flow interchangeable? Circulation 1999; 100: 2198–2203.Google Scholar
Donnelly JP, Raffel DM, Shulkin BL, Corbett JR, Bove EL, Mosca RS, Kulik TJ. Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography. J Thorac Cardiovasc Surg 1998; 115: 103–110.Google Scholar
Schwartz SM, Gordon D, Mosca RS, Bove EL, Heidelberger KP, Kulik TJ. Collagen content in normal, pressure, and pressure-volume overloaded developing human hearts. Am J Cardiol 1996; 77: 734–738.Google Scholar
Williams RV, Ritter S, Tani LY, Pagoto LT, Minich LL. Quantitative assessment of ventricular function in children with single ventricles using the Doppler myocardial performance index. Am J Cardiol 2000; 86: 1106–1110.Google Scholar
Beekman RH, Tuuri DT. Acute hemodynamic effects of increasing hemoglobin concentration in children with a right to left ventricular shunt and relative anemia. J Am Coll Cardiol. 1985; 5: 357–362Google Scholar
Lister G, Hellenbrand WE, Kleinman CS, Talner NS. Physiologic effects of increasing hemoglobin concentration in left-to-right shunting in infants with ventricular septal defects. N Engl J Med 1982; 306: 502–506.Google Scholar
Conte S, Hansen PB, Jensen T, Jacobsen JR, Helvind M, Lauridsen P, Pettersson G. Early experience with the Norwood procedure. Cardiovasc Surg 1997; 5: 315–319.Google Scholar
Riordan CJ, Randsbeck F, Storey JH, Montgomery WD, Santamore WP, Austin EH, 3rd. Effects of oxygen, positive end-expiratory pressure, and carbon dioxide on oxygen delivery in an animal model of the univentricular heart. J Thorac Cardiovasc Surg 1996; 112: 644–654.Google Scholar
Tabbutt S, Ramamoorthy C, Montenegro LM, Durning SM, Kurth CD, Steven JM, Godinez RI, Spray TL, Wernovsky G, Nicolson SC. Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation 2001; 104: I159–164.Google Scholar
Ramamoorthy C, Tabbutt S, Kurth CD, Steven JM, Montenegro LM, Durning S, Wernovsky G, Gaynor JW, Spray TL, Nicolson SC. Effects of inspired hypoxic and hypercapnic gas mixtures on cerebral oxygen saturation in neonates with univentricular heart defects. Anesthesiology 2002; 96: 283–288.Google Scholar
Bradley SM, Simsic JM, Atz AM. Hemodynamic effects of inspired carbon dioxide after the Norwood procedure. Ann Thorac Surg 2001; 72: 2088–2093; discussion 2093–2094.Google Scholar
Meliones JN, Bove EL, Dekeon MK, Custer JR, Moler FW, Callow LR, Wilton NC, Rosen DB. High-frequency jet ventilation improves cardiac function after the Fontan procedure. Circulation 1991; 84: III364–III368.Google Scholar
Yahagi N, Kumon K, Tanigami H, Watanabe Y, Haruna M, Hayashi H, Imanaka H, Takeuchi M, Takamoto S. Cardiac surgery and inhaled nitric oxide: indication and follow-up (2–4 years). Artif Organs 1998; 22: 886–891.Google Scholar
Ishino K, Stumper O, De Giovanni JJ, Silove ED, Wright JG, Sethia B, Brawn WJ, de Leval M. The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair. J Thorac Cardiovasc Surg 1999; 117: 920–930.Google Scholar
Stamler A, Wang SY, Li J, Thurer RL, Schoen FJ, Sellke FW. Moderate hypothermia reduces cardiopulmonary bypass-induced impairment of cerebrovascular responses to platelet products. Ann Thorac Surg 1996; 62: 191–198.Google Scholar
Stamler A, Wang SY, Aguirre DE, Johnson RG, Sellke FW. Cardiopulmonary bypass alters vasomotor regulation of the skeletal muscle microcirculation. Ann Thorac Surg 1997; 64: 460–465.Google Scholar
Friedman M, Wang SY, Stahl GL, Johnson RG, Sellke FW. Altered beta-adrenergic and cholinergic pulmonary vascular responses after total cardiopulmonary bypass. J Appl Physiol 1995; 79: 1998–2006.Google Scholar
Riordan CJ, Randsbaek F, Storey JH, Montgomery WD, Santamore WP, Austin EH, 3rd. Inotropes in the hypoplastic left heart syndrome: effects in an animal model. Ann Thorac Surg 1996; 62: 83–90.Google Scholar