alkhalidy, A. (2025). pulmonary arteial hypertension in children with congenital heart disease. , 24(1), 4-12. doi: 10.52573/ipmj.2025.136886
Atyaf Abdulsattar alkhalidy. "pulmonary arteial hypertension in children with congenital heart disease". , 24, 1, 2025, 4-12. doi: 10.52573/ipmj.2025.136886
alkhalidy, A. (2025). 'pulmonary arteial hypertension in children with congenital heart disease', , 24(1), pp. 4-12. doi: 10.52573/ipmj.2025.136886
alkhalidy, A. pulmonary arteial hypertension in children with congenital heart disease. , 2025; 24(1): 4-12. doi: 10.52573/ipmj.2025.136886

pulmonary arteial hypertension in children with congenital heart disease

Iraqi Postgraduate Medical Journal
Article 2, Volume 24, Issue 1, January 2025, Pages 4-12    PDF (574.65 K)
Document Type: Research Paper
DOI: 10.52573/ipmj.2025.136886
Author
Atyaf Abdulsattar alkhalidy*
Baghdad Iraq
Abstract
ABSTRACT:
BACKGROUND:
Pulmonary arterial hypertensions defined as the mean pulmonary arterial pressures more than 25mmHg at rest or more than 30mmHg with exercise no matter what age, or Tricuspid regurgitation with a Doppler velocity of more than 2.5 m/sec. In pediatric patients, it is defined as systolic pulmonary artery pressure exceeds 50% of systolic systemic pressure.
OBJECTIVE:
To describe the congenital cardiac defects that lead to pulmonary arterial hypertension in children, estimate the severity of pulmonary arterial hypertension associated with congenital heart disease, and to describe and evaluate the modality of diagnosis and treatment of pulmonary arterial hypertension in children with congenital heart disease.
PATIENTS AND METHOD:
Cross sectional study done in Iraqi center for cardiac disease in Gazi Al Hareri Hospital from 14th of December 2016 to 12th of July 2017. The study included 47 child their ages from (1-156 months)who approved to had Pulmonary arterial hypertension with congenital heart disease by echo study and or cardiac catheterization that done by pediatric cardiologist. Information were collected
from their caregivers that included age,sex, time at diagnosis, family history ofcongenital heart disease , mode of diagnosis, type of treatment that child receive. Type ofcongenital heart disease and the severity of pulmonary arterial hypertension were approved by pediatric cardiologist.
RESULTS:
From a total number of 47 child that had congenital heart disease with Pulmonary arterial hypertension, 29 (61.7%) was males and 18( 38.3%) was females. In this study, ventricular septal defect was found as the most common cause of pulmonary arterial hypertension. The mean age of the study group was 56months with range ( 1- 156) month. The mean age at diagnosis of congenital heart disease is 10month and the mean age of diagnosis of is Pulmonary arterial hypertension16month . Where 38 (80.9 % ) patients were diagnosed with pulmonary arterial hypertension at early infancy , 2 ( 4.3 % ) patients were diagnosed during late infancy and 7 ( 14.9 % ) patients were diagnosed after infancy. There was statistically significant difference between the age of the patients and the severity of pulmonary arterial hypertension (P value 0.05) and there was statistical significance between the severity of Pulmonary arterial hypertension that associated with congenital heart disease and family history (P value 0.02).This study show there was no statistical significance between the severity of Pulmonary arterial hypertension and the type of congenital heart disease.
CONCLUSION:
The prevalence of severe pulmonary arterial hypertension in Iraq is high.
Keywords
pulmonary hypertension,,; ,،,؛congenital heart disease,,; ,،,؛ventricular septal defect
References
  1. Petros ,  Pierce  CM.  Review  article:  The  management  of  pulmonary Hypertension. Pediatric Anesthesia 2006;16: 816-21.
  2. Sitbon O, Gressin V, Speich R. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;170: 1212-17.
  3. Beghetti M, Hoeper MM, Kiely DG. Safety experience with bosentan in 146 children 2-11 years with pulmonary arterial hypertension: results from the European post-marketing surveillance program. Pediatr Res 2008;64:200-4.
  4. Rodriguez FH, Moodie DS, Parekh DR. Outcomes of hospitalization in adults in the United States with atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Am J Cardiol. 2011;108:290–93.
  5. Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011; 58:538–46.
  6. Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010; 137:52S–61S.
  7. Duffels MGJ, Engelfriet PM, Berger RMF, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007;120:198–
  8. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62: D34–D41.
  9. S Hatano, T Strasser (Eds.), Primary Pulmonary Hypertension. Report on a WHO Meeting, World Health Organization, Geneva 1975:45:7.
  10. S Rich, D. R Dantzer, S.M Ayres. Primary pulmonary hypertension: a national prospective study Ann Intern Med 1987;107: 216–28.
  11. McLaughlin VV, Archer SL, Badesch DB; American College of Cardiology Foundation Task Force on Expert Consensus Documents; J Am Coll Cardiol 2009;53:1573–619.
  12. Cerro MJ, Abman S, Diaz G. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 2011; 1:286–98.
 

 

11
 

 

  1. Schulze-Neick I, Beghetti Classifying pulmonary hypertension in the setting of the congenitally malformed heart—cleaning up a dog’s dinner. Cardiol Young 2008;18:22–25.
  2. Takatsuki S, Soep JB, Calderbank M, Ivy DD. Connective tissue disease presenting with signs and symptoms of pulmonary hypertension in children. Pediatr Cardiol 2011;32:828–33.
  3. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 2012;125:113–22.
  4. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 2012;379:537–46.
  5. Cabral JE, Belik J. Persistent pulmonary hypertension of the newborn: recent advances in pathophysiology and treatment. J Pediatr Rio J 2013;89:226 ̶ 42.
  6. Lakshminrusimha The pulmonary circulation in neonatal respiratory failure. Clin Perinatol 2012;39:655–83.
  7. Diller GP, Dimopoulos K, Okonko D. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005; 112: 828–835.
  8. Kempny A, Dimopoulos K, Uebing A. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life– single centre experience and review of published data. Eur Heart J 2012;33:1386–96.
  9. Kozlik-Feldmann R, Hansmann G, Bonnet D, Schranz D, Apitz C, Michel- Behnke I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart 2016; 102 Suppl 2:ii42-8.
  10. Kücükoglu SM, Kaymaz C, Alehan D, Kula S, Akcevin A, Celiker A, et al; THALES Registry Investigators. Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES). Pulm Circ 2021; 11:1-10.
 

 

 

  1. Al-Zuhairi Pattern of Congenital Heart Disease In Children Attending Central Teaching Pediatric Hospital, Baghdad. AL-Kindy College Medical Journal 2020;15:82–86. Available from: https://doi.org/10.47723/kcmj.v15i2.17
  2. Hopper RK, Abman SH, Elia EG, Avitabile CM, Yung D, Mullen MP, et al; Pediatric Pulmonary Hypertension Network Investigators. Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry. J Pediatr 2023; 252:131-140.e3.
  3. Hansmann G. Pulmonary Hypertension in Infants, Children, and Young Adults. J Am Coll Cardiol 2017; 69:2551-2569.
  4. Landzberg, M, Daniels, C, Forfia, P. Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights. JACC Adv 2023:2 . Available from: https://doi.org/10.1016/j.jacadv.2023.100649
  5. AlSuwayfee KI, Albu-Dawalah ME, Mohammed QN. Congenital heart diseases and pulmonary hypertension among Down syndrome pediatric patients. Ann Coll Med Mosul June 2020; 42:50-56.
  6. Zhao M, Liu J, Xin M, Yang K, Huang H, Zhang W, et al. Pulmonary arterial hypertension associated with congenital heart disease: An omics study. Front Cardiovasc Med 2023; 10:1037357.
  7. Handoyo, Gunawijaya E, Yantie Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension. Med J Indones 2023;32:38-44. Available from: https://mji.ui.ac.id/journal/index.php/mji/article/view/6582
  8. Cruz-Utrilla A, Gallego N, Segura de la Cal T, Tenorio-Castaño J, Arribas- Ynsaurriaga F, Escribano Subias P. The role of genetics in pulmonary arterial hypertension associated with congenital heart disease. Rev Esp Cardiol (Engl Ed) 2021;74:884-86.
 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

  1. Petros ,  Pierce  CM.  Review  article:  The  management  of  pulmonary Hypertension. Pediatric Anesthesia 2006;16: 816-21.
  2. Sitbon O, Gressin V, Speich R. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;170: 1212-17.
  3. Beghetti M, Hoeper MM, Kiely DG. Safety experience with bosentan in 146 children 2-11 years with pulmonary arterial hypertension: results from the European post-marketing surveillance program. Pediatr Res 2008;64:200-4.
  4. Rodriguez FH, Moodie DS, Parekh DR. Outcomes of hospitalization in adults in the United States with atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Am J Cardiol. 2011;108:290–93.
  5. Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011; 58:538–46.
  6. Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010; 137:52S–61S.
  7. Duffels MGJ, Engelfriet PM, Berger RMF, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007;120:198–
  8. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62: D34–D41.
  9. S Hatano, T Strasser (Eds.), Primary Pulmonary Hypertension. Report on a WHO Meeting, World Health Organization, Geneva 1975:45:7.
  10. S Rich, D. R Dantzer, S.M Ayres. Primary pulmonary hypertension: a national prospective study Ann Intern Med 1987;107: 216–28.
  11. McLaughlin VV, Archer SL, Badesch DB; American College of Cardiology Foundation Task Force on Expert Consensus Documents; J Am Coll Cardiol 2009;53:1573–619.
  12. Cerro MJ, Abman S, Diaz G. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 2011; 1:286–98.
 

 

 
   
 

 

  1. Schulze-Neick I, Beghetti Classifying pulmonary hypertension in the setting of the congenitally malformed heart—cleaning up a dog’s dinner. Cardiol Young 2008;18:22–25.
  2. Takatsuki S, Soep JB, Calderbank M, Ivy DD. Connective tissue disease presenting with signs and symptoms of pulmonary hypertension in children. Pediatr Cardiol 2011;32:828–33.
  3. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 2012;125:113–22.
  4. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 2012;379:537–46.
  5. Cabral JE, Belik J. Persistent pulmonary hypertension of the newborn: recent advances in pathophysiology and treatment. J Pediatr Rio J 2013;89:226 ̶ 42.
  6. Lakshminrusimha The pulmonary circulation in neonatal respiratory failure. Clin Perinatol 2012;39:655–83.
  7. Diller GP, Dimopoulos K, Okonko D. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005; 112: 828–835.
  8. Kempny A, Dimopoulos K, Uebing A. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life– single centre experience and review of published data. Eur Heart J 2012;33:1386–96.
  9. Kozlik-Feldmann R, Hansmann G, Bonnet D, Schranz D, Apitz C, Michel- Behnke I. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart 2016; 102 Suppl 2:ii42-8.
  10. Kücükoglu SM, Kaymaz C, Alehan D, Kula S, Akcevin A, Celiker A, et al; THALES Registry Investigators. Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES). Pulm Circ 2021; 11:1-10.
 

 

 

  1. Al-Zuhairi Pattern of Congenital Heart Disease In Children Attending Central Teaching Pediatric Hospital, Baghdad. AL-Kindy College Medical Journal 2020;15:82–86. Available from: https://doi.org/10.47723/kcmj.v15i2.17
  2. Hopper RK, Abman SH, Elia EG, Avitabile CM, Yung D, Mullen MP, et al; Pediatric Pulmonary Hypertension Network Investigators. Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry. J Pediatr 2023; 252:131-140.e3.
  3. Hansmann G. Pulmonary Hypertension in Infants, Children, and Young Adults. J Am Coll Cardiol 2017; 69:2551-2569.
  4. Landzberg, M, Daniels, C, Forfia, P. Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights. JACC Adv 2023:2 . Available from: https://doi.org/10.1016/j.jacadv.2023.100649
  5. AlSuwayfee KI, Albu-Dawalah ME, Mohammed QN. Congenital heart diseases and pulmonary hypertension among Down syndrome pediatric patients. Ann Coll Med Mosul June 2020; 42:50-56.
  6. Zhao M, Liu J, Xin M, Yang K, Huang H, Zhang W, et al. Pulmonary arterial hypertension associated with congenital heart disease: An omics study. Front Cardiovasc Med 2023; 10:1037357.
  7. Handoyo, Gunawijaya E, Yantie Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension. Med J Indones 2023;32:38-44. Available from: https://mji.ui.ac.id/journal/index.php/mji/article/view/6582
  8. Cruz-Utrilla A, Gallego N, Segura de la Cal T, Tenorio-Castaño J, Arribas- Ynsaurriaga F, Escribano Subias P. The role of genetics in pulmonary arterial hypertension associated with congenital heart disease. Rev Esp Cardiol (Engl Ed) 2021;74:884-86.
 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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