Hostname: page-component-8448b6f56d-jr42d Total loading time: 0 Render date: 2024-04-17T22:18:28.942Z Has data issue: false hasContentIssue false
  • English
  • Français

Perioperative management of patients with poorly functioning ventricles in the setting of the functionally univentricular heart

Published online by Cambridge University Press:  10 January 2006

Joseph W. Rossano  and
Anthony C. Chang
Joseph W. Rossano
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas, United States of America
Anthony C. Chang
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas, United States of America
Get access Rights & Permissions [Opens in a new window]

Extract

The patient with a functionally univentricular heart is at increased risk for ventricular dysfunction for a variety of reasons. At birth, the pulmonary and systemic circulations are in parallel, leading to pulmonary overcirculation and a volume-loaded functional ventricle. Significant atrioventricular valvar regurgitation, abnormal ventriculoarterial coupling, diastolic dysfunction, and altered ventricular geometry can also contribute to long-term ventricular dysfunction. These collected circumstances place the patient at increased risk for perioperative morbidity and mortality. We will discuss in this review the pathophysiology that leads to ventricular dysfunction at each stage of surgical palliation, as well as the strategies for perioperative management. In addition, we will highlight novel strategies for management of ventricular dysfunction.

Keywords

Heart failureintensive careFontan circulationhypoplastic left heart syndrome
Type
Research Article
Information
Cardiology in the Young , Volume 16 , Supplement S1: Controversies and Challenges in the Management of the Functionally Univentricular Heart , February 2006 , pp. 47 - 54
Copyright
© 2006 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

Altmann K, Printz BF, Solowiejczyk DE, Gersony WM, Quaegebeur J, Apfel HD. Two-dimensional echocardiographic assessment of right ventricular function as a predictor of outcome in hypoplastic left heart syndrome. Am J Cardiol 2000; 86: 964–968.Google Scholar
Tworetzky W, McElhinney DB, Reddy M, Brook MM, Hanley FL, Silverman NH. Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation 2001; 103: 1269–1273.Google Scholar
Ohye RG, Gomez CA, Goldberg CS, Graves HL, Devaney EJ, Bove EL. Tricuspid valve repair in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2004; 127: 465–472.Google Scholar
Bartram U, Grunenfelder J, Van Praagh R. Causes of death after the modified Norwood procedure: a study of 122 postmortem cases. Ann Thorac Surg 1997; 64: 1795–1802.Google Scholar
Nelson DP, Schwartz SM, Chang AC. Neonatal physiology of the functionally univentricular heart. Cardiol Young 2004; 14: I-52–I-60.Google Scholar
Jobes DR, Nicolson SC, Steven JM, Miller M, Jacobs ML, Norwood Jr WI. Carbon dioxide prevents pulmonary overcirculation in hypoplastic left heart syndrome. Ann Thorac Surg 1992; 54: 150–151.Google Scholar
Reddy VM, Liddicoat JR, Fineman JR, McElhinney DB, Klein JR, Hanley FL. Fetal model of single ventricle physiology: hemodynamic effects of oxygen, nitric oxide, carbon dioxide, and hypoxia in the early postoperative period. J Thorac Cardiovasc Surg 1996; 112: 437–439.Google Scholar
Ramamoorthy C, Tabutt S, Kurth D, et al. 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
Tabutt 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: I-159–I-164.Google Scholar
Hoffman GM, Tweddell JS, Ghanayem NS, et al. Alteration of the critical arteriovenous oxygen saturation relationship by sustained afterload reduction after the Norwood procedure. J Thorac Cardiovasc Surg 2004; 127: 738–745.Google Scholar
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. Ann Thorac Surg 2000; 70: 1515–1521.Google Scholar
De Oliveria NC, Ashburn DA, Khalid F, et al. Prevention of early sudden circulatory collapse after the Norwood operation. Circulation 2004; 100: II-133–II-138.Google Scholar
Chang AC, Atz AM, Wernovsky G, Burke RP, Wessel DL. Milrinone: systemic and pulmonary hemodynamic effects in neonates after cardiac surgery. Crit Care Med 1995; 23: 1907–1914.Google Scholar
Hoffman TM, Wernovsky G, Atz AM, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003; 107: 996–1002.Google Scholar
Mair R, Tulzer G, Sames E, et al. Right ventricular to pulmonary artery conduit instead of modified Blalock–Taussig shunt improves postoperative hemodynamics in newborns after the Norwood operation. J Thorac Cardiovasc Surg 2003; 126: 1378–1384.Google Scholar
Pizarro C, Mroczek T, Malec E, Norwood WI. Right ventricle to pulmonary conduit reduces interim mortality after stage 1 Norwood for hypoplastic left heart syndrome. Ann Thorac Surg 2004; 78: 1959–1964.Google Scholar
Tanoue Y, Kado H, Shikawa Y, Fusazaki N, Ishikawa S. Midterm ventricular performance after Norwood procedure with right ventricular-pulmonary artery conduit. Ann Thorac Surg 2004; 78: 1965–1971.Google Scholar
Riordan CJ, Randsbaek F, Storey JH, Montgomery WD, Santamore WP, Austin III EH. Inotropes in the hypoplastic left heart syndrome: effects in an animal model. Ann Thorac Surg 1996; 62: 83–90.Google Scholar
Reddy VM, Liddicoat JR, McElhinney DB, et al. Hemodynamic effects of epinephrine, bicarbonate and calcium in the early postnatal period in a lamb model of single-ventricle physiology created in utero. J Am Coll Cardiol 1996; 28: 1877–1883.Google Scholar
Shore S, Nelson DP, Pearl JM, et al. Usefulness of corticosteroid therapy in decreasing epinephrine requirements in critically ill infants with congenital heart disease. Am J Cardiol 2001; 88: 591–594.Google Scholar
Kolovos NS, Bratton SL, Moler FW, et al. Outcome of pediatric patients treated with extracorporeal life support after cardiac surgery. Ann Thorac Surg 2003; 76: 1435–1442.Google Scholar
Morris MC, Ittenbach RF, Godinez RI, et al. Risk factors for mortality in 137 pediatric cardiac intensive care unit patients managed with extracorporeal membrane oxygenation. Crit Care Med 2004; 32: 1061–1069.Google Scholar
Hinz SR, Benitz WE, Colby CE, et al. Utilization and outcomes of neonatal cardiac extracorporeal life support: 1996–2000. Pediatr Crit Care Med 2005; 6: 33–38.Google Scholar
Pizarro C, Davis DA, Healy RM, Kerins PJ, Norwood WI. Is there a role for extracorporeal life support after stage I Norwood? Eur J Cardiothorac Surg 2001; 19: 294–301.Google Scholar
Ungerleider RM, Shen I, Yeh T, Schultz J, et al. Routine mechanical ventricular assist following the Norwood procedure-improved neurologic outcome and excellent hospital survival. Ann Thorac Surg 2004; 77: 18–22.Google Scholar
Berman NB, Kimball TR. Systemic ventricular size and performance before and after bidirectional cavopulmonary anastomosis. J Pediatr 1993; 122: S63–S67.Google Scholar
Forbes TJ, Gajarski R, Johnson GL, et al. Influence of age on the effect of bidirectional cavopulmonary anastomosis on left ventricular volume, mass and ejection fraction. J Am Coll Cardiol 1996; 28: 1301–1307.Google Scholar
Fogel MA, Weinberg PM, Chin AJ, Fellows KE, Hoffman EA. Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging. J Am Coll Cardiol 1996; 28: 212–221.Google Scholar
Tanoue Y, Sese A, Ueno Y, Joh K, Hijii T. Bidirectional Glenn procedure improves the mechanical efficiency of a total cavopulmonary connection in high-risk Fontan candidates. Circulation 2001; 103: 2176–2180.Google Scholar
Li J, Hoskote A, Hickey C, et al. Effect of carbon dioxide on systemic oxygenation, oxygen consumption, and blood lactate levels after bidirectional superior cavopulmonary anastomosis. Crit Care Med 2005; 33: 984–989.Google Scholar
Hoskote A, Li J, Hickey C, et al. The effects of carbon dioxide on oxygenation and systemic, cerebral, and pulmonary vascular hemodynamics after the bidirectional superior cavopulmonary anastomosis. J Am Coll Cardiol 2004; 44: 1501–1509.Google Scholar
Valera Martinez FJ, Caffarena Calvar J, Gomez-Ullate JM, et al. Risk factors in the Glenn bi-directional shunt as an intermediate procedure before Fontan correction. Rev Esp Cardiol 1999; 52: 903–909.Google Scholar
Alejos JC, Williams RG, Jarmakani JM, et al. Factors influencing survival in patients undergoing the bi-directional Glenn anastomosis. Am J Cardiol 1995; 75: 1048–1050.Google Scholar
Booth KL, Roth SJ, Thiagarajan RR, Almodovar MC, del Nido PJ, Laussen PC. Extracorporeal membrane oxygenation support of the Fontan and bidirectional Glenn circulations. Ann Thorac Surg 2004; 77: 1341–1348.Google Scholar
Fogel MA. Single ventricle and ventricular performance. Chang and Towbin: heart failure in children and young adults. In press.
Border WL, Syed AU, Michelfelder EC, et al. Impaired systemic ventricular relaxation affects postoperative short-term outcome in Fontan patients. J Thorac Cardiovasc Surg 2003; 126: 1760–1764.Google Scholar
Sluysmans T, Sanders SP, van der Velde M, et al. Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation. Circulation 1992; 86: 1753–1761.Google Scholar
Driscoll DJ, Offork KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85: 469–496.Google Scholar
Redington AN. Right ventricular function. Cardiol Clin 2002; 20: 341–349.Google Scholar
Fogel MA, Weinberg PM, Fellows KE, Hoffman EA. A study in ventricular–ventricular interaction: single right ventricles compared with systemic right ventricles in a dual-chamber circulation. Circulation 1995; 92: 219–230.Google Scholar
Szábo G, Buhmann V, Graf A, et al. Ventricular energetics after the Fontal operation: contractility-afterload mismatch. J Thorac Cardiovasc Surg 2003; 125: 1061–1069.Google Scholar
Cheung YF, Penny DJ, Redington AN. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 2000; 83: 420–424.Google Scholar
Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg 2002; 123: 237–245.Google Scholar
Lemler MS, Scott WA, Leonard SR, Stromberg D, Ramaciotti C. Fenestration improves clinical outcome of the Fontan procedure. Circulation 2002; 105: 207–212.Google Scholar
McBride WT, McMurray. Prolonged high frequency jet ventilation and milrinone therapy following modified Fontan procedure. Anaesthesia 1994; 49: 312–314.Google Scholar
Williams DB, Kiernan PD, Schaff HV, March HM, Danielson GK. The hemodynamic response to dopamine and nitroprusside following right atrium-pulmonary artery bypass (Fontan procedure). Ann Thorac Surg 1982; 34: 51–57.Google Scholar
Kouatli AA, Garcia JA, Zellers TM, Weinstein EM, Mahony L. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation 1997; 96: 1507–1512.Google Scholar
Ohuchi H, Hasegawa S, Yasuda K. Severely impaired cardiac autonomic nervous activity after the Fontan operation. Circulation 2001; 104: 1513–1518.Google Scholar
Abraham WT, Adams KF, Fonarow GC, et al. In-hospital mortality in patients with acute decompensated heart failure requiring intravenous vasoactive medications: an analysis from the Acute Decompensated Heart Failure National Registry (ADHERE). J Am Coll Cardiol 2005; 46: 57–64.Google Scholar
Jeffries JL, Denfield SW, Dreyer WJ, et al. A prospective evaluation of intravenous nesiritide in pediatric heart failure [Abstract]. J Am Coll Cardiol 2005; 45: 315A.Google Scholar
Gwathmey JK, Copelas L, MacKinnon R, et al. Abnormal intracellular calcium handling in myocardium from patients with end-stage heart failure. Circ Res 1987; 61: 70–76.Google Scholar
Katz IS, Loral BH. Regulation of cardiac contraction and relaxation. Circulation 2000; 102: IV69–IV74.Google Scholar
Feeler GM, O'Connor CM. Inotropic therapy for heart failure: an evidence based approach. Am Heart J 2001; 142: 393–401.Google Scholar
Ukkonen H, Saraste M, Akkila J, et al. Myocardial efficiency during levosimendan infusion in congestive heart failure. Clin Pharmacol Ther 2000; 68: 522–531.Google Scholar
Braun JP, Schneider M, Kastrup M, Liu J. Treatment of acute heart failure in an infant after cardiac surgery using levosimendan. Eur J Cardiothorac Surg 2004; 26: 228–230.Google Scholar
Braun JP, Schneider M, Dohmen P, Döpfmer U. Successful treatment of dilative cardiomyopathy in a 12-year-old girl using the calcium sensitizer levosimendan after weaning from mechanical biventricular assist support. J Cardiothorac Vasc Anes 2004; 18: 772–774.Google Scholar
Stevenson LW, Miller LW, Desvigne-Nickens P, et al. Left ventricular assist device as destination for patients undergoing intravenous inotropic therapy: a subset analysis for REMATCH (randomized evaluation of mechanical assistance in treatment of chronic heart failure). Circulation 2004; 110: 975–981.Google Scholar
Rodefeld MD, Boyd JH, Myers CD, et al. Cavopulmonary assist: circulatory support for the univentricular Fontan circulation. Ann Thorac Surg 2003; 76: 1911–1916.Google Scholar