Persistent Challenges in Pediatric Pulmonary Hypertension

Chest

Volumn 150, Issue 1, 2016, Pages 226-236

  Hopper, Rachel K ^a   Abman, Steven H ^b   Ivy, D Dunbar ^b  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b Nutrition Obesity Research Center at the University of Colorado   (United States)

Author keywords

pediatric cardiology; pediatric pulmonary; pulmonary hypertension

Indexed keywords

AMBRISENTAN; BOSENTAN; SILDENAFIL; TREPROSTINIL;

CAVOPULMONARY CONNECTION; CHILDHOOD DISEASE; CONGENITAL DIAPHRAGM HERNIA; DISEASE ASSOCIATION; DISEASE COURSE; DISEASE SEVERITY; DRUG EFFICACY; DRUG SAFETY; DRUG TOLERABILITY; HEADACHE; HUMAN; INCIDENCE; LUNG ARTERY PRESSURE; LUNG DYSPLASIA; LUNG TRANSPLANTATION; LUNG VASCULAR RESISTANCE; LUNG WEDGE PRESSURE; MORBIDITY; MORTALITY; OPEN STUDY; OXYGEN CONSUMPTION; PATHOGENESIS; PENIS ERECTION; PERSISTENT PULMONARY HYPERTENSION; PREMATURITY; PREVALENCE; PRIORITY JOURNAL; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SIDE EFFECT; VISUAL DISORDER; ADULT; AGE; CHILD; HYPERTENSION, PULMONARY; LUNG; PATHOLOGY; PATHOPHYSIOLOGY;

ADULT; AGE FACTORS; CHILD; HUMANS; HYPERTENSION, PULMONARY; LUNG;

EID: 84990020992     PISSN: 00123692     EISSN: 19313543     Source Type: Journal    
DOI: 10.1016/j.chest.2016.01.007     Document Type: Review

References (72)

1. 1 Maxwell, B.G., Nies, M.K., Ajuba-Iwuji, C.C., Coulson, J.D., Romer, L.H., Trends in hospitalization for pediatric pulmonary hypertension. Pediatrics 136:2 (2015), 241–250.
2. 2 Frank, D.B., Crystal, M.A., Morales, D.L.S., et al. Trends in pediatric pulmonary hypertension-related hospitalizations in the United States from 2000-2009. Pulm Circ 5:2 (2015), 339–348.
3. 3 Abman, S.H., Hansmann, G., Archer, S.L., et al. Pediatric pulmonary hypertension guidelines from the American Heart Association and American Thoracic Society. Circulation 132:21 (2015), 2037–2099.
4. 4 Jain, A., McNamara, P.J., Persistent pulmonary hypertension of the newborn: advances in diagnosis and treatment. Semin Fetal Neonatal Med 20:4 (2015), 262–271.
5. 5 Simonneau, G., Gatzoulis, M.A., Adatia, I., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62:25 (2013), D34–D41.
6. 6 del Cerro, M., Diaz, G., Freudenthal, F., et al. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 1:2 (2011), 286–298.
7. 7 Ivy, D.D., Abman, S.H., Barst, R.J., et al. Pediatric pulmonary hypertension. J Am Coll Cardiol 62:25 (2013), D117–D126.
8. 8 Simonneau, G., Robbins, I.M., Beghetti, M., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54:1 (2009), S43–S54.
9. 9 Simonneau, G., Galiè, N., Rubin, L.J., et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 43:12 (2004), S5–S12.
10. 10 Drossner, D.M., Kim, D.W., Maher, K.O., Mahle, W.T., Pulmonary vein stenosis: prematurity and associated conditions. Pediatrics 122:3 (2008), e656–e661.
11. 11 Schuuring, M.J., Bouma, B.J., Cordina, R., et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol 164:1 (2013), 106–110.
12. 12 Stoll, B.J., Hansen, N.I., Bell, E.F., et al. Neonatal outcomes of extremely preterm infants from the NICHD Neonatal Research Network. Pediatrics 126:3 (2010), 443–456.
13. 13 An, H.S., Bae, E.J., Kim, G.B., et al. Pulmonary hypertension in preterm infants with bronchopulmonary dysplasia. Korean Circ J, 40(3), 2010, 131.
14. 14 Bhat, R., Salas, A.A., Foster, C., Carlo, W.A., Ambalavanan, N., Prospective analysis of pulmonary hypertension in extremely low birth weight infants. Pediatrics 129:3 (2012), e682–e689.
15. 15 Mourani, P.M., Sontag, M.K., Younoszai, A., et al. Early pulmonary vascular disease in preterm infants at risk for bronchopulmonary dysplasia. Am J Respir Crit Care Med 191:1 (2014), 87–95.
16. 16 Khemani, E., McElhinney, D.B., Rhein, L., et al. Pulmonary artery hypertension in formerly premature infants with bronchopulmonary dysplasia: clinical features and outcomes in the surfactant era. Pediatrics 120:6 (2007), 1260–1269.
17. 17 Kim, D.-H., Kim, H.-S., Choi, C.W., Kim, E.-K., Kim, B.I., Choi, J.-H., Risk factors for pulmonary artery hypertension in preterm infants with moderate or severe bronchopulmonary dysplasia. Neonatology 101:1 (2012), 40–46.
18. 18 Jayet, P.-Y., Rimoldi, S.F., Stuber, T., et al. Pulmonary and systemic vascular dysfunction in young offspring of mothers with preeclampsia. Circulation 122:5 (2010), 488–494.
19. 19 Check, J., Gotteiner, N., Liu, X., et al. Fetal growth restriction and pulmonary hypertension in premature infants with bronchopulmonary dysplasia. J Perinatol 33:7 (2013), 553–557.
20. 20 Tang, J.-R., Karumanchi, S.A., Seedorf, G., Markham, N., Abman, S.H., Excess soluble vascular endothelial growth factor receptor-1 in amniotic fluid impairs lung growth in rats: linking preeclampsia with bronchopulmonary dysplasia. AJP Lung Cell Mol Physiol 302:1 (2012), L36–L46.
21. 21 Mestan, K.K., Check, J., Minturn, L., et al. Placental pathologic changes of maternal vascular underperfusion in bronchopulmonary dysplasia and pulmonary hypertension. Placenta 35:8 (2014), 570–574.
22. 22 Kim, D.-H., Kim, H.-S., Serial changes of serum endostatin and angiopoietin-1 levels in preterm infants with severe bronchopulmonary dysplasia and subsequent pulmonary artery hypertension. Neonatology 106:1 (2014), 55–61.
23. 23 Rozance, P.J., Seedorf, G.J., Brown, A., et al. Intrauterine growth restriction decreases pulmonary alveolar and vessel growth and causes pulmonary artery endothelial cell dysfunction in vitro in fetal sheep. AJP Lung Cell Mol Physiol 301:6 (2011), L860–L871.
24. 24 MacRitchie, A.N., Albertine, K.H., Sun, J., et al. Reduced endothelial nitric oxide synthase in lungs of chronically ventilated preterm lambs. Am J Physiol - Lung Cell Mol Physiol 281:4 (2001), L1011–L1020.
25. 25 Afshar, S., Gibson, L.L., Yuhanna, I.S., et al. Pulmonary NO synthase expression is attenuated in a fetal baboon model of chronic lung disease. Am J Physiol - Lung Cell Mol Physiol 284:5 (2003), L749–L758.
26. 26 O'Reilly, M., Thébaud, B., Stem cells for the prevention of neonatal lung disease. Neonatology 107:4 (2015), 360–364.
27. 27 Stege, G., Fenton, A., Jaffray, B., Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics 112:3 Pt 1 (2003), 532–535.
28. 28 Colvin, J., Bower, C., Dickinson, J.E., Sokol, J., Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia. Pediatrics 116:3 (2005), e356–e363.
29. 29 Dillon, P.W., Cilley, R.E., Mauger, D., Zachary, C., Meier, A., The relationship of pulmonary artery pressure and survival in congenital diaphragmatic hernia. J Pediatr Surg 39:3 (2004), 307–312.
30. 30 Wynn, J., Krishnan, U., Aspelund, G., et al. Outcomes of congenital diaphragmatic hernia in the modern era of management. J Pediatr 163:1 (2013), 114–119.
31. 31 Geggel, R.L., Murphy, J.D., Langleben, D., Crone, R.K., Vacanti, J.P., Reid, L.M., Congenital diaphragmatic hernia: arterial structural changes and persistent pulmonary hypertension after surgical repair. J Pediatr 107:3 (1985), 457–464.
32. 32 Acker, S.N., Seedorf, G.J., Abman, S.H., Nozik-Grayck, E., Partrick, D.A., Gien, J., Pulmonary artery endothelial cell dysfunction and decreased populations of highly proliferative endothelial cells in experimental congenital diaphragmatic hernia. AJP Lung Cell Mol Physiol 305:12 (2013), L943–L952.
33. 33 Makanga, M., Dewachter, C., Maruyama, H., et al. Downregulated bone morphogenetic protein signaling in nitrofen-induced congenital diaphragmatic hernia. Pediatr Surg Int 29:8 (2013), 823–834.
34. 34 Hofmann, A.D., Friedmacher, F., Takahashi, H., Hunziker, M., Gosemann, J.-H., Puri, P., Decreased apelin and apelin-receptor expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia. Pediatr Surg Int 30:2 (2014), 197–203.
35. 35 Keller, R.L., Tacy, T.A., Hendricks-Munoz, K., et al. Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity. Am J Respir Crit Care Med 182:4 (2010), 555–561.
36. 36 Fleck, S., Bautista, G., Keating, S.M., et al. Fetal production of growth factors and inflammatory mediators predicts pulmonary hypertension in congenital diaphragmatic hernia. Pediatr Res 74:3 (2013), 290–298.
37. 37 Schaible, T., Veit, M., Tautz, J., et al. Serum cytokine levels in neonates with congenital diaphragmatic hernia. Klin Pädiatr 223:7 (2011), 414–418.
38. 38 van Loon, R.L.E., Roofthooft, M.T.R., Hillege, H.L., et al. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation 124:16 (2011), 1755–1764.
39. 39 Berger, R.M., Beghetti, M., Humpl, T., et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 379:9815 (2012), 537–546.
40. 40 Abman, S.H., Raj, U., Towards improving the care of children with pulmonary hypertension: the rationale for developing a Pediatric Pulmonary Hypertension Network. Prog Pediatr Cardiol 27:1-2 (2009), 3–6.
41. 41 Barst, R.J., Maislin, G., Fishman, A.P., Vasodilator therapy for primary pulmonary hypertension in children. Circulation 99:9 (1999), 1197–1208.
42. 42 Barst, R.J., McGoon, M.D., Elliott, C.G., Foreman, A.J., Miller, D.P., Ivy, D.D., Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 125:1 (2012), 113–122.
43. 43 Haworth, S.G., Hislop, A.A., Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart 95:4 (2008), 312–317.
44. 44 King, M.E., Braun, H., Goldblatt, A., Liberthson, R., Weyman, A.E., Interventricular septal configuration as a predictor of right ventricular systolic hypertension in children: a cross-sectional echocardiographic study. Circulation 68:1 (1983), 68–75.
45. 45 Mourani, P.M., Sontag, M.K., Younoszai, A., Ivy, D.D., Abman, S.H., Clinical utility of echocardiography for the diagnosis and management of pulmonary vascular disease in young children with chronic lung disease. Pediatrics 121:2 (2008), 317–325.
46. 46 Koestenberger, M., Ravekes, W., Everett, A.D., et al. Right ventricular function in infants, children and adolescents: reference values of the Tricuspid Annular Plane Systolic Excursion (TAPSE) in 640 healthy patients and calculation of z score values. J Am Soc Echocardiogr 22:6 (2009), 715–719.
47. 47 Zakaria, D., Sachdeva, R., Gossett, J.M., Tang, X., O'Connor, M.J., Tricuspid annular plane systolic excursion is reduced in infants with pulmonary hypertension. Echocardiography 32:5 (2015), 834–838.
48. 48 Kassem, E., Humpl, T., Friedberg, M.K., Prognostic significance of 2-dimensional, M-mode, and Doppler echo indices of right ventricular function in children with pulmonary arterial hypertension. Am Heart J 165:6 (2013), 1024–1031.
49. 49 Bernus, A., Brain natriuretic peptide levels in managing pediatric patients with pulmonary arterial hypertension. CHEST J, 135(3), 2009, 745.
50. 50 Steurer, M.A., Moon-Grady, A.J., Fineman, J.R., et al. B-type natriuretic peptide: prognostic marker in congenital diaphragmatic hernia. Pediatr Res 76:6 (2014), 549–554.
51. 51 Cuna, A., Kandasamy, J., Sims, B., B-type natriuretic peptide and mortality in extremely low birth weight infants with pulmonary hypertension: a retrospective cohort analysis. BMC Pediatr, 14(1), 2014, 68.
52. 52 Blalock, S., Chan, F., Rosenthal, D., Ogawa, M., Maxey, D., Feinstein, J., Magnetic resonance imaging of the right ventricle in pediatric pulmonary arterial hypertension. Pulm Circ, 3(2), 2013, 350.
53. 53 Truong, U., Fonseca, B., Dunning, J., et al. Wall shear stress measured by phase contrast cardiovascular magnetic resonance in children and adolescents with pulmonary arterial hypertension. J Cardiovasc Magn Reson, 15(1), 2013, 81.
54. 54 Pandya, B., Quail, M.A., Steeden, J.A., et al. Real-time magnetic resonance assessment of septal curvature accurately tracks acute hemodynamic changes in pediatric pulmonary hypertension. Circ Cardiovasc Imaging 7:4 (2014), 706–713.
55. 55 Sitbon, O., Humbert, M., Jaïs, X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:23 (2005), 3105–3111.
56. 56 Douwes, J.M., van Loon, R.L.E., Hoendermis, E.S., et al. Acute pulmonary vasodilator response in paediatric and adult pulmonary arterial hypertension: occurrence and prognostic value when comparing three response criteria. Eur Heart J 32:24 (2011), 3137–3146.
57. 57 Taylor, C.J., Derrick, G., McEwan, A., Haworth, S.G., Sury, M.R.J., Risk of cardiac catheterization under anaesthesia in children with pulmonary hypertension. Br J Anaesth 98:5 (2007), 657–661.
58. 58 Carmosino, M.J., Friesen, R.H., Doran, A., Ivy, D.D., Perioperative complications in children with pulmonary hypertension undergoing noncardiac surgery or cardiac catheterization. Anesth Analg 104:3 (2007), 521–527.
59. 59 Bobhate, P., Guo, L., Jain, S., et al. Cardiac catheterization in children with pulmonary hypertensive vascular disease. Pediatr Cardiol 36:4 (2015), 873–879.
60. 60 Williams, G.D., Maan, H., Ramamoorthy, C., et al. Perioperative complications in children with pulmonary hypertension undergoing general anesthesia with ketamine. Pediatr Anesth 20:1 (2010), 28–37.
61. 61 Lammers, A., del Cerro, M., Freudenthal, A., et al. Functional classification of pulmonary hypertension in children: report from the PVRI pediatric taskforce, Panama 2011. Pulm Circ, 1(2), 2011, 280.
62. 62 Ploegstra, M.-J., Douwes, J.M., Roofthooft, M.T.R., Zijlstra, W.M.H., Hillege, H.L., Berger, R.M.F., Identification of treatment goals in paediatric pulmonary arterial hypertension. Eur Respir J 44:6 (2014), 1616–1626.
63. 63 Siehr, S.L., McCarthy, E.K., Ogawa, M.T., Feinstein, J.A., Reported sildenafil side effects in pediatric pulmonary hypertension patients. Front Pediatr, 3, 2015, 12.
64. 64 Barst, R.J., Ivy, D., Dingemanse, J., et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther 73:4 (2003), 372–382.
65. 65 Barst, R.J., Beghetti, M., Pulido, T., et al. STARTS-2: Long-Term Survival With Oral Sildenafil Monotherapy in Treatment-Naive Pediatric Pulmonary Arterial Hypertension. Circulation 129:19 (2014), 1914–1923.
66. 66 Trottier-Boucher, M.N., Lapointe, A., Malo, J., et al. Sildenafil for the treatment of pulmonary arterial hypertension in infants with bronchopulmonary dysplasia. Pediatr Cardiol 36:6 (2015), 1255–1260.
67. 67 Takatsuki, S., Rosenzweig, E.B., Zuckerman, W., Brady, D., Calderbank, M., Ivy, D.D., Clinical safety, pharmacokinetics, and efficacy of ambrisentan therapy in children with pulmonary arterial hypertension. Pediatr Pulmonol 48:1 (2013), 27–34.
68. 68 Ivy, D.D., Rosenzweig, E.B., Lemarié, J.-C., Brand, M., Rosenberg, D., Barst, R.J., Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings. Am J Cardiol 106:9 (2010), 1332–1338.
69. 69 Siehr, S.L., Dunbar Ivy, D., Miller-Reed, K., Ogawa, M., Rosenthal, D.N., Feinstein, J.A., Children with pulmonary arterial hypertension and prostanoid therapy: long-term hemodynamics. J Heart Lung Transplant 32:5 (2013), 546–552.
70. 70 Ferdman, D.J., Rosenzweig, E.B., Zuckerman, W.A., Krishnan, U., Subcutaneous treprostinil for pulmonary hypertension in chronic lung disease of infancy. Pediatrics 134:1 (2014), e274–e278.
71. 71 Olson, E., Lusk, L.A., Fineman, J.R., Robertson, L., Keller, R.L., Short-term treprostinil use in infants with congenital diaphragmatic hernia following repair. J Pediatr 167:3 (2015), 762–764.
72. 72 Baruteau, A.E., Belli, E., Boudjemline, Y., et al. Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients. Eur J Cardiothorac Surg 47:3 (2015), e105–e110.