Recognition, Stabilization, and Management of Children with Pulmonary Hypertension in the Emergency Department

Chapter

Abstract

Pulmonary hypertension (PH) is an uncommon but potentially life-threatening condition in children, and improved survival makes it more likely that acute care practitioners will encounter children with PH. This is due in part to better outcomes of extremely preterm infants who develop PH from chronic lung disease and children with congenital heart and lung defects who survive surgery but have residual PH, increased diagnosis of PH due to expanded screening indications, and better diagnostic modalities. This chapter provides an overview of the types of pediatric PH likely to be encountered in the acute care setting and discusses the recognition and initial management of this disease. Specific clinical cases are presented to illustrate various pulmonary hypertensive emergencies in the five categories of PH as classified by the World Health Organization. These consist of (1) pulmonary arterial hypertension, (2) PH with left heart disease, (3) PH associated with lung diseases and/or hypoxemia, (4) PH due to chronic thrombotic and/or embolic disease, and (5) miscellaneous.

Keywords

Pulmonary hypertension Congenital heart disease Thromboembolic pulmonary hypertension Sickle cell disease 

References

  1. 1.
    Ross RD, Daniels SR, Schwartz DC, Hannon DW, Shukla R, Kaplan S. Plasma norepinephrine levels in infants and children with congestive heart failure. Am J Cardiol. 1987;59(8):911–4.CrossRefGoogle Scholar
  2. 2.
    Devaney EJ, Chang AC, Ohye RG, Bove EL. Management of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg. 2006;81(3):992–5; discussion 995–996.CrossRefGoogle Scholar
  3. 3.
    Gowda S, Bhat D, Feng Z, Chang CH, Ross RD. Pulmonary vein stenosis with Down syndrome: a rare and frequently fatal cause of pulmonary hypertension in infants and children. Congenit Heart Dis. 2014;9(3):E90–7.CrossRefGoogle Scholar
  4. 4.
    McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009;119(16):2250–94.CrossRefGoogle Scholar
  5. 5.
    Khemani E, McElhinney DB, Rhein L, Andrade O, Lacro RV, Thomas KC, et al. Pulmonary artery hypertension in formerly premature infants with bronchopulmonary dysplasia: clinical features and outcomes in the surfactant era. Pediatrics. 2007;120(6):1260–9.CrossRefGoogle Scholar
  6. 6.
    Mourani PM, Ivy DD, Gao D, Abman SH. Pulmonary vascular effects of inhaled nitric oxide and oxygen tension in bronchopulmonary dysplasia. Am J Respir Crit Care Med. 2004;170(9):1006–13.CrossRefGoogle Scholar
  7. 7.
    Klok FA, Mos IC, van Kralingen KW, Vahl JE, Huisman MV. Chronic pulmonary embolism and pulmonary hypertension. Semin Respir Crit Care Med. 2012;33(2):199–204.CrossRefGoogle Scholar
  8. 8.
    Raffini L, Huang YS, Witmer C, Feudtner C. Dramatic increase in venous thromboembolism in children’s hospitals in the United States from 2001 to 2007. Pediatrics. 2009;124(4):1001–8.CrossRefGoogle Scholar
  9. 9.
    Tormene D, Gavasso S, Rossetto V, Simioni P. Thrombosis and thrombophilia in children: a systematic review. Semin Thromb Hemost. 2006;32(7):724–8.CrossRefGoogle Scholar
  10. 10.
    Madani MM, Wittine LM, Auger WR, Fedullo PF, Kerr KM, Kim NH, et al. Chronic thromboembolic pulmonary hypertension in pediatric patients. J Thorac Cardiovasc Surg. 2011;141(3):624–30.CrossRefGoogle Scholar
  11. 11.
    Kim JS, McSweeney J, Lee J, Ivy D. Pediatric Cardiac Intensive Care Society 2014 consensus statement: pharmacotherapies in cardiac critical care pulmonary hypertension. Pediatr Crit Care Med. 2016;17(3 Suppl 1):S89–100.CrossRefGoogle Scholar
  12. 12.
    Walton BL, Lehmann M, Skorczewski T, Holle LA, Beckman JD, Cribb JA, et al. Elevated hematocrit enhances platelet accumulation following vascular injury. Blood. 2017;129(18):2537–46.CrossRefGoogle Scholar
  13. 13.
    Guzzetta NA. Benefits and risks of red blood cell transfusion in pediatric patients undergoing cardiac surgery. Paediatr Anaesth. 2011;21(5):504–11.CrossRefGoogle Scholar
  14. 14.
    Carter HF, Lau C, Juma D, Wells B, Applegate RL II. Intraoperative red blood cell transfusion in infant heart transplant patients is not associated with worsened outcomes. Anesth Anal. 2016;122(5):1567–77.CrossRefGoogle Scholar
  15. 15.
    Cholette JM, Swartz MF, Rubenstein J, Henrichs KF, Wang H, Powers KS, et al. Outcomes using a conservative versus liberal red blood cell transfusion strategy in infants requiring cardiac operation. Ann Thorac Surg. 2017;103(1):206–14.CrossRefGoogle Scholar
  16. 16.
    Lacroix J, Tucci M, Du Pont-Thibodeau G. Red blood cell transfusion decision making in critically ill children. Curr Opin Pediatr. 2015;27(3):286–91.CrossRefGoogle Scholar
  17. 17.
    Kuo JA, Maher KO, Kirshbom PM, Mahle WT. Red blood cell transfusion for infants with single-ventricle physiology. Pediatr Cardiol. 2011;32(4):461–8.CrossRefGoogle Scholar
  18. 18.
    de Gast-Bakker DH, de Wilde RB, Hazekamp MG, Sojak V, Zwaginga JJ, Wolterbeek R, et al. Safety and effects of two red blood cell transfusion strategies in pediatric cardiac surgery patients: a randomized controlled trial. Intensive Care Med. 2013;39(11):2011–9.CrossRefGoogle Scholar
  19. 19.
    Linguraru MG, Orandi BJ, Van Uitert RL, Mukherjee N, Summers RM, Gladwin MT, et al. CT and image processing non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc IEEE Eng Med Biol Soc. 2008;2008:859–62.PubMedPubMedCentralGoogle Scholar
  20. 20.
    Robert-Ebadi H, Le Gal G, Righini M. Evolving imaging techniques in diagnostic strategies of pulmonary embolism. Expert Rev Cardiovasc Ther. 2016;14(4):495–503.CrossRefGoogle Scholar
  21. 21.
    Wilkes J. AHA/ATS release guidelines on the diagnosis and treatment of pediatric pulmonary hypertension. Am Fam Physician. 2016;93(7):605–14.PubMedGoogle Scholar
  22. 22.
    Potoka KP, Gladwin MT. Vasculopathy and pulmonary hypertension in sickle cell disease. Am J Physiol Lung Cell Mol Physiol. 2015;308(4):L314–24.CrossRefGoogle Scholar
  23. 23.
    Mekontso Dessap A, Leon R, Habibi A, Nzouakou R, Roudot-Thoraval F, Adnot S, et al. Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2008;177(6):646–53.CrossRefGoogle Scholar
  24. 24.
    Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004;350(9):886–95.CrossRefGoogle Scholar
  25. 25.
    Machado RF, Mack AK, Martyr S, Barnett C, Macarthur P, Sachdev V, et al. Severity of pulmonary hypertension during vaso-occlusive pain crisis and exercise in patients with sickle cell disease. Br J Haematol. 2007;136(2):319–25.CrossRefGoogle Scholar
  26. 26.
    Dham N, Ensing G, Minniti C, Campbell A, Arteta M, Rana S, et al. Prospective echocardiography assessment of pulmonary hypertension and its potential etiologies in children with sickle cell disease. Am J Cardiol. 2009;104(5):713–20.CrossRefGoogle Scholar
  27. 27.
    Nouraie M, Lee JS, Zhang Y, Kanias T, Zhao X, Xiong Z, et al. The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe. Haematologica. 2013;98(3):464–72.CrossRefGoogle Scholar
  28. 28.
    Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med. 2004;350(9):857–9.CrossRefGoogle Scholar
  29. 29.
    De Castro LM, Jonassaint JC, Graham FL, Ashley-Koch A, Telen MJ. Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. Am J Hematol. 2008;83(1):19–25.CrossRefGoogle Scholar
  30. 30.
    Gladwin MT, Sachdev V. Cardiovascular abnormalities in sickle cell disease. J Am Coll Cardiol. 2012;59(13):1123–33.CrossRefGoogle Scholar
  31. 31.
    Kato GJ, McGowan V, Machado RF, Little JA, Taylor JT, Morris CR, et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood. 2006;107(6):2279–85.CrossRefGoogle Scholar
  32. 32.
    Machado RF, Anthi A, Steinberg MH, Bonds D, Sachdev V, Kato GJ, et al. N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease. JAMA. 2006;296(3):310–8.CrossRefGoogle Scholar
  33. 33.
    Minniti CP, Sable C, Campbell A, Rana S, Ensing G, Dham N, et al. Elevated tricuspid regurgitant jet velocity in children and adolescents with sickle cell disease: association with hemolysis and hemoglobin oxygen desaturation. Haematologica. 2009;94(3):340–7.CrossRefGoogle Scholar
  34. 34.
    Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639–44.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatrics, Division of CardiologyChildren’s Hospital of Michigan and Wayne State University School of MedicineDetroitUSA
  2. 2.Division of Critical Care Medicine, Children’s Hospital of MichiganWayne State University School of MedicineDetroitUSA

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