Volume 5, Issue 2, March 2019, Page: 26-30
Pulmonary Hypertension Following Cardiac Surgery in Children with Down Syndrome: A Review
Abubakar Umar, Cardiothoracic Surgery Unit, Department of Surgery, Usmanu Danfodiyo University/Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
Abdullahi A. A., Department of Anaesthesiology and Intensive Care, Usmanu Danfodiyo University/Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
Received: Dec. 13, 2018;       Accepted: Jan. 10, 2019;       Published: May 20, 2019
DOI: 10.11648/j.ijcts.20190502.11      View  176      Downloads  34
Abstract
Pulmonary hypertension is a known complication in children with congenital heart lesions especially those with left-to-right shunts. Children with Down syndrome are known to have certain types of congenital heart lesions like atrioventricular septal defect, ventricular septal defect, patent ductus arteriosus etc. These lesions can cause pulmonary hypertension if left untreated in any child. In those with Down syndrome, pulmonary hypertension is said to be worse. Several factors have been identified. These factors are upper airway obstruction that is common in all children with this syndrome, abnormality in their pulmonary vascular bed, lower levels of nitric oxide production which is a known vasodilator, abnormalities in their immune system which predisposes them to respiratory tract infection, gastroesophageal reflux disease and recently pulmonary hemosiderosis has been reported. Therefore, children with Down syndrome being prepared for cardiac surgery should be thoroughly evaluated and if possible measures should be taken to minimize untoward effects of these factors.
Keywords
Pulmonary Hypertension, Down Syndrome, Cardiac Surgery
To cite this article
Abubakar Umar, Abdullahi A. A., Pulmonary Hypertension Following Cardiac Surgery in Children with Down Syndrome: A Review, International Journal of Cardiovascular and Thoracic Surgery. Vol. 5, No. 2, 2019, pp. 26-30. doi: 10.11648/j.ijcts.20190502.11
Copyright
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Reference
[1]
Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62: D42–D50.
[2]
Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol. 2013;62: D117–D126.
[3]
Ivy DD. Pulmonary hypertension in children. Cardiol clin. 2016;(34(3): 451-471.
[4]
Toth R, Szantos P, Lex DJ, Sapi E, Szatmari A, Gal J, Szantos T, Szekely A. down syndrome and postoperative complications after paediatric cardiac surgery: A propensity-matched analysis. Interactive CardioVascular and Thoracic Surgery. 2013 1–7.
[5]
Anaclerio S, Di Ciommo V, Michielon G, et al. Conotruncal heart defects: impact of genetic syndromes on immediate operative mortality. Ital Heart J. 2004; 5:624–8.
[6]
Calderon-Colmenero J, Flores A, Ramirez S, Patino-Bahena E, Zabal C, Garcia-Montes JA et al. Surgical treatment results of congenital heart defects in children with Down’s syndrome. Arch Cardiol Mex 2004;74: 39–44.
[7]
Newman JH. Pulmonary hypertension. Am J Respir Crit Care Med 2005;172:1072-7.
[8]
Anderson RJ, Malhotra A, Kim NH. Pulmonary hypertension: the evolution of pulmonary hypertension and chronic thromboembolic pulmonary hypertension.J thorac Dis. 2016;8(7):562-565.
[9]
Berger TJ, Blackstone EH, Kirklin JW. Survival and probability of cure without and with surgery in complete atrioventricular canal. Ann Thorac Surg. 1979;27:104–111
[10]
Kumar KR. Left to right shunt with pulmonary vascular disease still an enigma. Heart Asia. 2015; 7(2): 38–39
[11]
Jacobs I, Gray RT, odd W. Upper airway obstruction in children with Down syndrome. Arch Otolaryngol Head Neck Surg.1996;122:945-950.
[12]
Mitchell RB, Call E, Kelly J. diagnosis and therapy for airway obstruction in children with Down syndrome. Arch Otolaryngol Head Neck Surg. 2003;129(6):642-645.
[13]
Chi TPL, Krovetz J (1975) The Pulmonary Vascular bed in Children with Down syndrome. J Pediatr 86: 533-538.
[14]
Banjar H (2009) Down’s Syndrome and Pulmonary Arterial Hypertension. PVRI Review 1: 213-2.
[15]
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. Congenital heart dis 2014;9(3):E90-7.
[16]
Byard RW (2007) Forensic issues in Down syndrome fatalities. J Forensic Leg Med 14: 475-481.
[17]
Holm T, Aukrust P, Aagaard E, Ueland T, Haugstad TS, Kjekshus J, Simonsen S, Froland SS, Gullestad L, Andreassen AK (2002) Hypertension in relation to nitric oxide, asymmetric dimethylarginine, and inflammation: different patterns in heart transplant recipients and individuals with essential hypertension. Transplantation 74:1395–1400.
[18]
Kielstein JT, Bode-Boger SM, Hesse G, Martens-Lobenhoffer J, Takacs A, Fliser D, Hoeper MM (2005) Asymmetrical dimethylarginine in idiopathic pulmonary arterial hypertension. Arterioscler Thromb Vasc Biol 25:1414–1418
[19]
Fukushima H, Kosaki K, Sato R, Yagihashi T, Gatayama R, Kodo K, Hayashi T, Nakazawa M, Tsuchihashi T, Maeda J, Kojima Y, Yamagishi H, Takahashi T (2010) Mechanisms underlying early development of pulmonary vascular obstructive disease in Down syndrome: an imbalance in biosynthesis of thromboxane A(2) and prostacyclin. Am J Med Genet A 152A(8):1919–1924.
[20]
Burgio GR, Ugazio AG, Nespoli L, Marcioni AF, Bottelli AM, Pasquali F. Derangements of immunoglobulin levels, phytohemagglutinin responsiveness and T and B cell markers in Down's syndrome at different ages. Eur J Immunol. 1975;5:600–3.
[21]
Cruz NV, Mahmoud SA, Chen H, Lowery-Nordberg M, Berlin K, Bahna SL. Follow up study of immune defects in patients with dysmorphic disorders. Ann Allergy Asthma Immunol. 2009;102:426–31.
[22]
Burgio GR, Ugazio AG, Nespoli L, Marcioni AF, Bottelli AM, Pasquali F. Derangements of immunoglobulin levels, phytohemagglutinin responsiveness and T and B cell markers in Down's syndrome at different ages. Eur J Immunol. 1975;5:600–3.
[23]
Ram G, Chinen J. infections and immunodeficiency in Down syndrome. Clin Exp Immunol. 2011;164(1):9-16.
[24]
Thompson L, McElhinney D, Jue K, Hodge D. Gastroesophageal reflux after re- 
pair of atrioventricular septal defect in infants with trisomy 21: a comparison of medical and surgical therapy. J Pediatr Surg. 1999;34:1359-1363.
[25]
Alimi A, Taytard J, Abou Taam R, Houdouin V, Forgeron A et al. Pulmonary hemosiderosis in children with Down syndrome: a national experience. Ophanet Journal of Rare Diseases. 2018;60(13);1-8.
[26]
Malouf JF, Enriquez-Sarano M, Pellikka PA, et al. Severe pulmonary hypertension in patients with severe aortic valve stenosis: clinical profile and prognostic implications. J Am Coll Cardiol 2002; 40(4): 789-95.
[27]
Tuman KJ, McCarthy RJ, March RJ, Najafi H, Ivankovich AD. Morbidity and duration of ICU stay after cardiac surgery. A model for preoperative risk assessment. Chest 1992; 102(1): 36-44.
[28]
Garg N, Sharma MK, Sinha N. Role of oral sildenafil in severe pulmonary arterial hypertension: clinical efficacy and dose response relationship. Int J Cardiol. 2007;120:306–313.
[29]
Mukhopadhyay S, Nathani S, Yusuf J, Shrimal D, Tyagi S. Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome–a randomized, placebo-controlled, double-blind crossover study. Congenit Heart Dis. 2011;6:424 – 431
[30]
Chau EMC, Fan KYY, Chow WH. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol. 2007;120:301–305.
[31]
Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62:D42–D50.
[32]
D’Alto M, Romeo E, Argiento P, D’Andrea A, Sarubbi B, Correra A, Scognamiglio G, Papa S, Bossone E, Calabro` R, Vizza CD, Russo MG. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down’s syndrome. Int J Cardiol. 2013;164:323–326.
[33]
Journois D, Pouard P, Mauriat P, Malhere T, Vouhe P, Safran D. Inhaled nitric oxide as a therapy for pulmonary hypertension after operations for congenital heart defects. J Thorac Cardiovasc Surg. 1994;107:1129-35.
[34]
Duffels MGJ, Vis JC, van Loon RLE, Nieuwkerk PT, van Dijk APJ, Hoendermis ES, de Bruin-Bon RHACM, Bouma BJ, Bresser P, Berger RMF, Mulder BJM. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am J Cardiol. 2009;103:1309 – 1315.
[35]
Gurtu V, Michelakis ED. Emerging therapies and future directions in pulmonary arterial hyperten- sion. Can J Cardiol. 2015;31(4):489–501.
[36]
Reitz BA, Wallwork JL, Hunt SA, et al. Heart- lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med 1982;306:557-64.
[37]
Spiekerkoetter E. FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension. J Clin Investig. 2013;123(8):3600–13, Doe Z, et al. Evidence for Rho-kinase activation in patients with pulmonary arterial hypertension. Circ J. 2009;73(9):1731–9.
[38]
Chen SL, et al. Hemodynamic, functional, and clinical responses to pulmonary artery denervation in patients with pulmonary arterial hypertension of different causes: phase II results from the pul- monary artery denervation-1 study. Circ Cardiovasc Interv. 2015;8(11).
[39]
Chiu JS, et al. Balloon atrial septostomy in pul- monary arterial hypertension: effect on survival and associated outcomes. J Heart Lung Transplant. 2015;34(3):376–80.
[40]
Baruteau AE, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg. 2012;94(3): 817–24.
[41]
Kirkby S, Hayes D. Pediatric lung transplantation: indications and outcomes. J Thorac Dis. 2014;6(8):1024–31.
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