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Acute Pulmonary Hypertension

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Critical Care of Children with Heart Disease

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Abstract

Pulmonary arterial hypertension is a hemodynamic condition defined as a pulmonary artery systolic pressure higher than 35 mm Hg or a pulmonary artery mean pressure higher than 20 mm Hg. However, this may depend on the level of the systemic pressure, and another potential definition could be pulmonary pressure > 60% systemic with signs of low cardiac output. Pulmonary hypertension in the critically ill pediatric cardiac patient remains a clinical challenge. Clinical practitioners still have to face significant problems particularly in the acute postoperative phase. This chapter provides a summary of the main aspects to be taken into account whenever managing acute pulmonary hypertensive crisis.

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References

  1. Gorenflo M, Nelle M, Ullman S. Pulmonary hypertension in infancy and childhood. Cardiol Young. 2003;13:219–227.

    Article  PubMed  Google Scholar 

  2. Landzberg M. Congenital heart disease associated pulmonary arterial hypertension. Clin Chest Med. 2007;28:243–253.

    Article  PubMed  Google Scholar 

  3. Farber H, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004;351:1655–1665.

    Article  CAS  PubMed  Google Scholar 

  4. Rosenzweig E, Widlitz A, Barst R. Pulmonary arterial hypertension in children. Pediatr Pulmonol. 2004;38:2–22.

    Article  PubMed  Google Scholar 

  5. Berger S, Konduri G. Pulmonary hypertension in children: the twenty-first century. Pediatr Clin N Am. 2006;53:961–987.

    Article  Google Scholar 

  6. Donti A., Formigari R, Ragni L, et al. Pulmonary arterial hypertension in the pediatric age. J Cardiovasc Med. 2007;8:72–77.

    Article  Google Scholar 

  7. Bando K, Turrentine MW, Sharp TG, Sekine Y, Aufiero TX, Sun K, Sekine E, Brown JW. Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management. J Thorac Cardiovasc Surg. 1996;112:1600–1607; discussion 1607–1609.

    Article  CAS  PubMed  Google Scholar 

  8. Cooper AL, Beasley D. Hypoxia stimulates proliferation and interleukin-1 alpha production in human vascular smooth muscle cells. Am J Phys. 1999;277:H1326–1337.

    CAS  Google Scholar 

  9. Celermajer DS, Cullen S, Deanfield JE. Impairment of endothelium-dependent pulmonary artery relaxation in children with congenital heart disease and abnormal pulmonary hemodynamics. Circulation. 1993;87:440–446.

    Article  CAS  PubMed  Google Scholar 

  10. Beghetti M, Black SM, Fineman JR. Endothelin-1 in congenital heart disease. Pediatr Res. 2005;57:16R-20R. Review.

    Article  CAS  PubMed  Google Scholar 

  11. Morita K, Ogawa Y, Tobise K. Effect of endothelium of pulmonary artery vasoreactivity in monocrotaline-induced pulmonary hypertensive rats. Jpn Circ J. 1996;60:585–592.

    Article  CAS  PubMed  Google Scholar 

  12. Boehme J, Sun X, Tormos KV, Gong W, Kellner M, Datar SA, Kameny RJ, Yuan JX, Raff GW, Fineman JR, Black SM, Maltepe E. Pulmonary artery smooth muscle cell hyperproliferation and metabolic shift triggered by pulmonary overcirculation. Am J Physiol Heart Circ Physiol. 2016;311:H944-H957.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Levy M, Raisky O, Vouhé PR, Celermajer DS, Bonnet D, Israël-Biet D. Apoptotic deregulation of endothelial cells and intimal proliferation in pulmonary hypertension of congenital heart disease. Arch Mal Coeur Vaiss. 2007;100:365–372.

    CAS  PubMed  Google Scholar 

  14. Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S–12S. Review.

    Article  PubMed  Google Scholar 

  15. Schulze-Neick I, Beghetti M. Classifying pulmonary hypertension in the setting of the congenitally malformed heart--cleaning up a dog’s dinner. Cardiol Young. 2008;18:22–25.

    Article  PubMed  Google Scholar 

  16. Wessel DL, Adatia I, Van Marter LJ, Thompson JE, Kane JW, Stark AR, Kourembanas S. Improved oxygenation in a randomized trial of inhaled nitric oxide for persistent pulmonary hypertension of the newborn. Pediatrics. 1997;100:E7.

    Article  CAS  PubMed  Google Scholar 

  17. Morita K, Ihnken K, Buckberg GD, Sherman MP, Ignarro LJ. Pulmonary vasoconstriction due to impaired nitric oxide production after cardiopulmonary bypass. Ann Thorac Surg. 1996;61:1775–1780.

    Article  CAS  PubMed  Google Scholar 

  18. Celermajer DS, Marwick T. Echocardiographic and right heart catheterization techniques in patients with pulmonary arterial hypertension. Int J Cardiol. 2008;125:294–303. Review.

    Article  PubMed  Google Scholar 

  19. Dincer H, Presberg K. Current management of pulmonary hypertension. Clin Pulm Med. 2004;11:40–53.

    Article  Google Scholar 

  20. Fischer L, Van Aken H, Burkle H. Management of pulmonary hypertension: physiological and pharmacological considerations for anesthesiologists. Anesth Analg. 2003;96:1603–1616.

    Article  PubMed  Google Scholar 

  21. Humbert M, Sitbon O, Simmoneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351:1425–1436.

    Article  CAS  PubMed  Google Scholar 

  22. Ali O, Wharton J, Russell Gibbs JS, et al. Emerging therapies for pulmonary arterial hypertension. Expert Opin Investig Drugs. 2007;16:803–818.

    Article  CAS  PubMed  Google Scholar 

  23. Chang AC, Wernovsky G, Kulik TJ, Jonas RA, Wessel DL. Management of the neonate with transposition of the great arteries and persistent pulmonary hypertension. Am J Cardiol. 1991;68:1253–1255.

    Article  CAS  PubMed  Google Scholar 

  24. Morris K, Beghetti M, Petros A, Adatia I, Bohn D. Comparison of hyperventilation and inhaled nitric oxide for pulmonary hypertension after repair of congenital heart disease. Crit Care Med. 2000;28:2974–2978.

    Article  CAS  PubMed  Google Scholar 

  25. Kinsella JP, et al. Inhaled nitric oxide in preterm infants undergoing mechanical ventilation. NEJM. 2006;355:354–364.

    Article  CAS  PubMed  Google Scholar 

  26. Kinsella JP, et al. Inhaled nitric oxide therapy in premature newborns. Curr Opin Pediatr. 2006;18:107–111.

    Article  PubMed  Google Scholar 

  27. The Neonatal Inhaled Nitric Oxide Study Group. Inhaled nitric oxide in full-term and nearly full-term infants with hypoxic respiratory failure. N Engl J Med. 1997;336:597–604.

    Article  Google Scholar 

  28. 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.

    Article  CAS  PubMed  Google Scholar 

  29. Journois D, Baufreton C, Mauriat P, Pouard P, Vouhé P, Safran D. Effects of inhaled nitric oxide administration on early postoperative mortality in patients operated for correction of atrioventricular canal defects. Chest. 2005;128:3537–3544.

    Article  CAS  PubMed  Google Scholar 

  30. Journois D, Pouard P, Mauriat P, Malhère T, Vouhé P, Safran D. Inhaled nitric oxide as a therapy for pulmonary hypertension after operations for congenital heart defects. J Thorac Cardiovasc Surg. 1994;107:1129–1135.

    Article  CAS  PubMed  Google Scholar 

  31. Miller OI, Celermajer DS, Deanfield JE, Macrae DJ. Very-low-dose inhaled nitric oxide: a selective pulmonary vasodilator after operations for congenital heart disease. J Thorac Cardiovasc Surg. 1994;108:487–494.

    Article  CAS  PubMed  Google Scholar 

  32. Miller OI, Tang SF, Keech A, Pigott NB, Beller E, Celermajer DS. Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: a randomised double-blind study. Lancet. 2000;356:1464–1469.

    Article  CAS  PubMed  Google Scholar 

  33. Beghetti M, Adatia I. Inhaled nitric oxide and congenital cardiac disease. Cardiol Young. 2001;11:142–152. Review.

    Article  CAS  PubMed  Google Scholar 

  34. Beghetti M, Habre W, Friedli B, Berner M. Continuous low dose inhaled nitric oxide for treatment of severe pulmonary hypertension after cardiac surgery in paediatric patients. Br Heart J. 1995;73:65–68.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Klinger J. The nitric oxide/cGMP signaling pathway in pulmonary hypertension. Clin Chest Med. 2007;28:143–167.

    Article  PubMed  Google Scholar 

  36. Atz AM, Wessel DL. Sildenafil ameliorates effects of inhaled nitric oxide withdrawal. Anesthesiology. 1999;91:307–310.

    Article  CAS  PubMed  Google Scholar 

  37. Clark RH, Kueser TJ, Walker MW, et al. Low dose nitric oxide therapy for persistent pulmonary hypertension of the newborn. Clinical Inhaled Nitric Oxide Research Group. N Engl J Med. 2000;342:469–474.

    Article  CAS  PubMed  Google Scholar 

  38. Ivy DD, Doran AK, Smith KJ, Mallory GB Jr, Beghetti M, Barst RJ, Brady D, Law Y, Parker D, Claussen L, Abman SH. Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol. 2008;51:161–169.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Rimensberger PC, Spahr-Schopfer I, Berner M, Jaeggi E, Kalangos A, Friedli B, Beghetti M. Inhaled nitric oxide versus aerosolized iloprost in secondary pulmonary hypertension in children with congenital heart disease: vasodilator capacity and cellular mechanisms. Circulation. 2001;103:544–548.

    Article  CAS  PubMed  Google Scholar 

  40. Winterhalter M, Simon A, Fischer S, Rahe-Meyer N, Chamtzidou N, Hecker H, Zuk J, Piepenbrock S, Strüber M. Comparison of inhaled iloprost and nitric oxide in patients with pulmonary hypertension during weaning from cardiopulmonary bypass in cardiac surgery: a prospective randomized trial. J Cardiothorac Vasc Anesth. 2008;22:406–413.

    Article  CAS  PubMed  Google Scholar 

  41. Sakuma M, Demachi J, Nawata J, Suzuki J, Takahashi T, Matsubara H, Akagi S, Shirato K. Epoprostenol infusion therapy changes angiographic findings of pulmonary arteries in patients with idiopathic pulmonary arterial hypertension. Circ J. 2008;72:1147–1151.

    Article  PubMed  Google Scholar 

  42. Motti A, Tissot C, Rimensberger PC, Prina-Rosso A, Aggoun Y, Beghetti M, Berner M, da Cruz E. Intravenous adenosine for refractory pulmonary hypertension in a low-weight premature newborn: a potential new drug for rescue therapy. Pediatr Crit Care Med. 2006;7:380–382.

    Article  PubMed  Google Scholar 

  43. Rex S, Schaelte G, Metzelder S, Flier S, de Waal EE, Autschbach R, Rossaint R, Buhre W. Inhaled iloprost to control pulmonary artery hypertension in patients undergoing mitral valve surgery: a prospective, randomized-controlled trial. Acta Anaesthesiol Scand. 2008;52:65–72.

    Article  CAS  PubMed  Google Scholar 

  44. Oztekin I, Yazici S, Oztekin DS, Goksel O, Issever H, Canik S. Effects of low-dose milrinone on weaning from cardiopulmonary bypass and after in patients with mitral stenosis and pulmonary hypertension. Yakugaku Zasshi. 2007;127:375–383.

    Article  CAS  PubMed  Google Scholar 

  45. Khazin V, Kaufman Y, Zabeeda D, Medalion B, Sasson L, Schachner A, Ezri T. Milrinone and nitric oxide: combined effect on pulmonary artery pressures after cardiopulmonary bypass in children. J Cardiothorac Vasc Anesth. 2004;18:156–159.

    Article  CAS  PubMed  Google Scholar 

  46. Feneck RO, Sherry KM, Withington PS, Oduro-Dominah A;European Milrinone Multicenter Trial Group. Comparison of the hemodynamic effects of milrinone with dobutamine in patients after cardiac surgery. J Cardiothorac Vasc Anesth. 2001;15:306–315.

    Article  CAS  PubMed  Google Scholar 

  47. Hoffman TM, Wernovsky G, Atz AM, Kulik TJ, Nelson DP, Chang AC, Bailey JM, Akbary A, Kocsis JF, Kaczmarek R, Spray TL, Wessel DL. 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.

    Article  CAS  PubMed  Google Scholar 

  48. 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.

    Article  CAS  PubMed  Google Scholar 

  49. Hoganson DM, Gaszxit AZ, Boston US, Sweet SC, Grady RM, Huddleston CB, Eghtesady P. Paracorporeal lung assist devices as a bridge to recovery or lung transplantation in neonates and young children. J Thorac Cardiovasc Surg. 2014;147:420–426.

    Article  PubMed  Google Scholar 

  50. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary hypertension from the REVEAL Registry. Chest. 2012;142:448–456.

    Article  PubMed  Google Scholar 

  51. De Perrot M, Granton JT, McRae K, Pierre AF, Singer LG, Wadell TK, Keshavjee S. Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience. J Thorac Cardiovasc Surg. 2012;143:910–918.

    Article  PubMed  Google Scholar 

  52. Tsai MT, Hsu CH, Luo CY, Hu YN, Roan JN. Bridge-to-recovery strategy using extracorporeal membrane oxygenation for critical pulmonary hypertension complicated with cardiogenic shock. Interact Cardiovasc Thorac Surg. 2015;21:55–61.

    Article  PubMed  Google Scholar 

  53. Javidfar J, Brodie D, Iribame A, Jurado J, Lavelle M, Brenner K, Arcasoy S, Sonett J, Bacchetta M. Extracorporeal membrane oxygenation as a bridge to lung transplantation and recovery. J Thorac Cardiovasc Surg. 2012;144:716–721.

    Article  PubMed  Google Scholar 

  54. Machuca TN, de Perrot M. Mechanical support for the failing right ventricle in patients with precapillary pulmonary hypertension. Circulation. 2015;132:526–536.

    Article  PubMed  Google Scholar 

  55. Wessel D. Postoperative treatment of pulmonary hypertension. In: Beghetti M, Barst RJ, Naeije R, Rubin LJ, eds. Pulmonary Arterial Hypertension Related to Congenital Heart Disease. Munich: Elsevier GmbH;2006:143–175.

    Google Scholar 

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Author information

Authors and Affiliations

  1. Associate Medical Director, CHCO Heart Institute, Head, Pediatric Cardiac Critical Care Program & Inpatient Services, Director, Cardiac Intensive Care Unit, Children’s Hospital Colorado, Tenured Professor of Pediatrics, Pediatric Cardiology and Intensive Care, University of Colorado Denver, School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

  2. Pulmonary Hypertension Program, Heart Institute, Department of Pediatrics, Children’s Hospital Colorado, University of Colorado Denver School of Medicine, Aurora, CO, USA

    Dunbar Ivy

Authors
  1. Eduardo M. da Cruz
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  2. Dunbar Ivy
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Correspondence to Eduardo M. da Cruz .

Editor information

Editors and Affiliations

  1. Chief, Division of Cardiac Critical Care Medicine Executive Director, Telemedicine Co-director, Children’s National Heart Institute, Professor of Pediatrics, George Washington University School of Medicine, Children’s National Health System, Washington, DC, USA

    Ricardo A. Munoz

  2. Department of Pediatric Cardiothoracic Surgery, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Victor O. Morell

  3. Associate Medical Director, CHCO Heart Institute Head, Pediatric Cardiac Critical Care Program & Inpatient Services Director, Cardiac Intensive Care Unit Children’s Hospital Colorado, Tenured Professor of Pediatrics, Pediatric Cardiology and Intensive Care University of Colorado Denver, School of Medicine, Aurora, CO, USA

    Eduardo M. da Cruz

  4. Department of Pharmacy, Pediatric Critical Care, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Carol G. Vetterly

  5. Department of Pediatric Cardiothoracic Surgery, UPMC – Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Jose Pedro da Silva

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da Cruz, E.M., Ivy, D. (2020). Acute Pulmonary Hypertension. In: Munoz, R., Morell, V., da Cruz, E., Vetterly, C., da Silva, J. (eds) Critical Care of Children with Heart Disease . Springer, Cham. https://doi.org/10.1007/978-3-030-21870-6_42

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  • DOI: https://doi.org/10.1007/978-3-030-21870-6_42

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  • Online ISBN: 978-3-030-21870-6

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