Abstract
Pulmonary hypertension (PH) is a rare condition. Data on adult PH patients is abundant, but data on pediatric PH are scarce. Nevertheless, in the past decade, increasing data concerning pediatric PH are becoming available, enabling this review of the epidemiology, characterization, and outcome of pediatric pulmonary hypertension.
PH is a condition that is associated with a variety of diseases. It is a rare condition in children with a complex and age-specific presentation. Transient forms of pulmonary arterial hypertension (PAH), i.e., potentially reversible PAH, specifically occur in children and form the largest group of pediatric PH. Advanced PAH in children is predominantly idiopathic or associated with congenital heart disease. Other forms of PAH seldom occur in children. WHO PH groups 2–5 are rare in childhood; however, they may be underreported.
The clinical presentation of pediatric PH is relatively nonspecific as it is in adults, with dyspnea on exertion as the most common presenting symptom. However, the clinical presentation of pediatric PH includes symptoms specific for children such as syncope. Pediatric PH frequently co-occurs with genetic and syndromal abnormalities, including Down syndrome. Right heart failure is less common in children despite a severe hemodynamic profile.
PAH is a detrimental disease with a poor prognosis. Outcome of pediatric PAH differs between patients with different types of congenital heart disease. In the last decades, new PAH-targeted therapies have been developed, which have improved outcome in pediatric pulmonary arterial hypertension. However, currently reported survival rates vary and critical appraisal of patient populations, survival data, and proposed treatment approaches is necessary.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Authors/Task Force Members, Galie N, Hoeper MM et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493–2537
van Loon RL, Roofthooft MT, Hillege HL et al (2011) Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation 124:1755–1764
Fishman AP (1998) Etiology and pathogenesis of primary pulmonary hypertension: a perspective. Chest 114:242S–247S
Simonneau G, Galie N, Rubin LJ et al (2004) Clinical classification of pulmonary hypertension. J Am Coll Cardiol 43:5S–12S
Simonneau G, Robbins IM, Beghetti M et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54:S43–S54
Barst RJ, Ertel SI, Beghetti M et al (2011) Pulmonary arterial hypertension: a comparison between children and adults. Eur Respir J 37:665–677
Rosenzweig EB, Widlitz AC, Barst RJ (2004) Pulmonary arterial hypertension in children. Pediatr Pulmonol 38:2–22
van Loon RL, Roofthooft MT, Osch-Gevers M et al (2009) Clinical characterization of pediatric pulmonary hypertension: complex presentation and diagnosis. J Pediatr 155:176–182
Humbert M, Morrell NW, Archer SL et al (2004) Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 43:13S–24S
Jeffery TK, Morrell NW (2002) Molecular and cellular basis of pulmonary vascular remodeling in pulmonary hypertension. Prog Cardiovasc Dis 45:173–202
Pietra GG, Capron F, Stewart S et al (2004) Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol 43:25S–32S
Harrison RE, Berger R, Haworth SG et al (2005) Transforming growth factor-beta receptor mutations and pulmonary arterial hypertension in childhood. Circulation 111:435–441
Nasim MT, Ogo T, Ahmed M et al (2011) Molecular genetic characterization of SMAD signaling molecules in pulmonary arterial hypertension. Hum Mutat 32:1385–1389
Austin ED, Ma L, Leduc C et al (2012) Whole exome sequencing to identify a novel gene (Caveolin-1) associated with human pulmonary arterial hypertension. Circ Cardiovasc Genet 5(3):336–343
Rosenzweig EB, Morse JH, Knowles JA et al (2008) Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension. J Heart Lung Transplant 27:668–674
Schulze-Neick I, Beghetti M (2008) Classifying pulmonary hypertension in the setting of the congenitally malformed heart–cleaning up a dog’s dinner. Cardiol Young 18:22–25
Berger RM, Beghetti M, Humpl T et al (2012) Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 379:537–546
Berner M, Beghetti M, Spahr-Schopfer I et al (1996) Inhaled nitric oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congenital heart disease. Am J Cardiol 77:532–535
Berger RM (2000) Possibilities and impossibilities in the evaluation of pulmonary vascular disease in congenital heart defects. Eur Heart J 21:17–27
van Loon RL, Roofthooft MT, Berger RM (2009) Pulmonary arterial hypertension in children with congenital heart disease. PVRI Rev 1:219–223
Andrews R, Tulloh R, Magee A et al (2002) Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease? Pediatr Cardiol 23:528–530
Tulloh RM (2005) Congenital heart disease in relation to pulmonary hypertension in paediatric practice. Paediatr Respir Rev 6:174–180
Ivy DD, Feinstein JA, Humpl T et al (2009) Non-congenital heart disease associated pediatric pulmonary arterial hypertension. Prog Paediatr Cardiol 27:13–23
Stayer SA, Liu Y (2010) Pulmonary hypertension of the newborn. Best Pract Res Clin Anaesthesiol 24:375–386
Roofthooft MT, Elema A, Bergman KA et al (2011) Patient characteristics in persistent pulmonary hypertension of the newborn. Pulm Med 2011:858154
Duffels MG, Engelfriet PM, Berger RM et al (2007) Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 120:198–204
Stenmark KR, Abman SH (2005) Lung vascular development: implications for the pathogenesis of bronchopulmonary dysplasia. Annu Rev Physiol 67:623–661
Carmosino MJ, Friesen RH, Doran A et al (2007) Perioperative complications in children with pulmonary hypertension undergoing noncardiac surgery or cardiac catheterization. Anesth Analg 104:521–527
Humbert M, Sitbon O, Chaouat A et al (2006) Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 173:1023–1030
Peacock AJ, Murphy NF, McMurray JJ et al (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30:104–109
Moledina S, Hislop AA, Foster H et al (2010) Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart 96:1401–1406
Fraisse A, Jais X, Schleich JM et al (2010) Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France. Arch Cardiovasc Dis 103:66–74
Barst RJ, McGoon MD, Elliott CG et al (2012) Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 125:113–122
Walsh-Sukys MC, Tyson JE, Wright LL et al (2000) Persistent pulmonary hypertension of the newborn in the era before nitric oxide: practice variation and outcomes. Pediatrics 105:14–20
Khemani E, McElhinney DB, Rhein L et al (2007) Pulmonary artery hypertension in formerly premature infants with bronchopulmonary dysplasia: clinical features and outcomes in the surfactant era. Pediatrics 120:1260–1269
Rich S, Dantzker DR, Ayres SM et al (1987) Primary pulmonary hypertension. A national prospective study. Ann Intern Med 107:216–223
Le RJ, Fenstad ER, Maradit-Kremers H et al (2011) Syncope in adults with pulmonary arterial hypertension. J Am Coll Cardiol 58:863–867
Badesch DB, Raskob GE, Elliott CG et al (2010) Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry. Chest 137:376–387
Barst RJ, Maislin G, Fishman AP (1999) Vasodilator therapy for primary pulmonary hypertension in children. Circulation 99:1197–1208
Barst RJ (1986) Pharmacologically induced pulmonary vasodilatation in children and young adults with primary pulmonary hypertension. Chest 89:497–503
Sitbon O, Humbert M, Jais X et al (2005) Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:3105–3111
Douwes JM, van Loon RL, Hoendermis ES et al (2011) Acute pulmonary vasodilator response in paediatric and adult pulmonary arterial hypertension: occurrence and prognostic value when comparing three response criteria. Eur Heart J 32:3137–3146
van Albada ME, Berger RM (2008) Pulmonary arterial hypertension in congenital cardiac disease–the need for refinement of the Evian-Venice classification. Cardiol Young 18:10–17
Cerro MJ, Abman S, Diaz G et al (2011) A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 1:286–298
D’Alonzo GE, Barst RJ, Ayres SM et al (1991) Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 115:343–349
van Loon RL, Roofthooft MT, Delhaas T et al (2010) Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies. Am J Cardiol 106:117–124
Ivy DD, Rosenzweig EB, Lemarie JC et al (2010) Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings. Am J Cardiol 106:1332–1338
Haworth SG, Hislop AA (2009) Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001–2006. Heart 95:312–317
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag London
About this entry
Cite this entry
Douwes, J.M., Berger, R.M.F. (2014). Epidemiology of Pediatric Pulmonary Hypertension. In: Da Cruz, E., Ivy, D., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4619-3_204
Download citation
DOI: https://doi.org/10.1007/978-1-4471-4619-3_204
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-4471-4618-6
Online ISBN: 978-1-4471-4619-3
eBook Packages: MedicineReference Module Medicine