EC Paediatrics

Research Article Volume 12 Issue 3 - 2023

Pediatric Pulmonary Hypertension: Characteristics and Prospective Follow-Up of Children in Algeria

Mohammed Mokhtar Bekkar1,2,3,4*, Amina Tari1,2 and Sakina Niar1,2

1Faculty of Medicine, University Oran 1 “Ahmed Ben Bella”, Algeria
2Marfan Pediatric Department, Oran University Hospital Center, Algeria
3GRAS Research Laboratory, IEC Health Team, Algeria
4Algerian Pulmonary Hypertension Society, Algeria

*Corresponding Author:Mohammed Mokhtar Bekkar, Marfan Pediatric Department, Oran University Hospital Center and Faculty of Medicine, University Oran 1 “Ahmed Ben Bella” and GRAS Research Laboratory, IEC Health Team and Algerian Pulmonary Hypertension Society, Algeria.
Received: February 20, 2023; Published: February 28, 2023



Introduction: Pulmonary hypertension (PH) in children is a serious disease. There is few data describing pediatric PH. PH, long considered to be outstanding, is poorly known in Algeria where the diagnostic means and the supply of care are significantly limited. Our work item on the situation in the West Algerian and is part of a strategy to improve the diagnosis and support.

Objectives: The objective was to describe the clinical features and management of PH in children, as well as its effects on the quality of life and its consequences, excluding patients with persistent pulmonary hypertension of the newborn.

Methods: This prospective non interventional study included children with PH who were prospectively followed for two years in in a pediatric center in Oran. The WHO functional classification, the 6-minute walk distance, were evaluated.

Results: Sixty children with a mean age of 6,4 ± 4,6 years in 12 cities in the West Algerian were included from January 2020 to January 2022. The diagnosis is late (3 years on average). The prevalence of PH was estimated at 2,2 cases per 100.000 children in the wilaya of Oran. The patients had the following types of PH: associated with congenital heart disease (73%), idiopathic (6%), associated with an HIV (3%) or associated with diffuse interstitial lung disease (3%). Although there may be misleading clinical signs, it is therefore imperative that PH is recognised and that its severity is assessed. Echocardiography is the essential examination which is most often used to assess PAH and its effects. During follow-up there was an increase in the number of drugs prescribed specifically for pulmonary arterial hypertension (44% patients versus 22% at inclusion). The clinical status, 6-minute walk test and quality of life of the majority of patients remained stable. The survival at one and at two years was estimated at 95% and 93,3% respectively (95% confidence interval).

Conclusion: The majority of cases of PH in children are secondary to congenital heart disease or idiopathic. The prognosis depends largely on its etiology. The use of specific treatments for PAH may contribute to the stability of the disease and to better survival. Progress in understanding the pathophysiology will open the way to new therapeutic choices.

Keywords: Pediatric Pulmonary Hypertension; Congenital Heart Defect; Epidemiology; Pulmonary Vasodilators

  1. Rubin LJ. “Primary pulmonary hypertension”. The New England Journal of Medicine 36 (1997): 111-117.
  2. D’Alonzo GE., et al. “Survival in patients with primary pulmonary hypertension. Results from a national prospective registry”. Annals of Internal Medicine5: (1991): 343-349.
  3. Cerro MJ., et al. “A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011”. Pulmonary Circulation - SAGE Journals2 (2011): 286-298.
  4. Pietra GG., et al. “Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry”. Circulation5 (1989): 1198-1206.
  5. Adatia I., et al. “Abnormalities in the biosynthesis of thromboxane A2 and prostacyclin in children with cyanotic congenital heart disease”. British Heart Journal 69 (1993): 179-182.
  6. Rosenzweig EB, Barst RJ. “Idiopathic pulmonary arterial hypertension in children”. Current Opinion in Pediatrics 17 (2005): 372-380.
  7. Beghetti M. “Current treatment options in children with pulmonary arterial hypertension and experiences with oral bosentan”. European Journal of Clinical Investigation3 (2006): 16-24.
  8. Galie N., et al. “2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS)”. European Respiratory Journal 46 (2015): 903-975.
  9. Fraisse A., et al. “Characteristics and prospective 2-year follow up of children with pulmonary arterial hypertension in France”. Archives of Cardiovascular Diseases 103 (2010): 66-74.
  10. B Van Loon RL., et al. “Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005”. Circulation16 (2011): 1755-1764.
  11. M Lévy. “Syndrome d'Eisenmenger”. EMC - Cardiologie2 (2014): 1-7.
  12. Beghetti M., et al. “Haemodynamic characterisation and heart catheterisation complications in children with pulmonary hypertension: Insights from the Global TOPP Registry (tracking outcomes and practice in paediatric pulmonary hypertension)”. International Journal of Cardiology 203 (2016): 325-330.
  13. Humbert M., et al. “Pulmonary arterial hypertension in France: results from a national registry”. American Journal of Respiratory and Critical Care Medicine 173 (2006): 1023-1030.
  14. Fasnacht MS., et al. “The Swiss registry for pulmonary arterial hypertension: the paediatric experience”. Swiss Medical Weekly35-36 (2007): 510-513.
  15. Haworth SG and Hislop AA. “Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006”. Heart 95 (2009): 312-317.
  16. Barst RJ., et al. “Pulmonary arterial hypertension: a comparison between children and adults”. European Respiratory Journal 37 (2011): 665-677.
  17. Mocumbi AO., et al. “A global perspective on the epidemiology of pulmonary hypertension”. Canadian Journal of Cardiology4 (2015): 375-381.
  18. Sandoval J., et al. “Primary pulmonary hypertension in children: clinical characterization and survival”. Journal of the American College of Cardiology 25 (1995): 466-474.
  19. RJ Barst., et al. “Survival in childhood pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management”. Circulation 125 (2012): 113-122.
  20. Moledina S., et al. “Childhood idiopathic pulmonary arterial hypertension: a national cohort study”. Heart 96 (2010): 1401-1406.
  21. Galiè N., et al. “2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension”. European Heart Journal 37 (2016): 67-119.
  22. Barst RJ., et al. “Vasodilator therapy for primary pulmonary hypertension in children”. Circulation 99 (1999): 1197-1208.
  23. Rosenzweig EB., et al. “Effects of long-term bosentan in children with pulmonary arterial hypertension”. Journal of the American College of Cardiology 46 (2005): 697-704.
  24. Maiya S., et al. “Response to bosentan in children with pulmonary hypertension”. Heart 92 (2006): 664-670.
  25. Stiebellehner L., et al. “Long-term treatment with oral sildenafil in addition to continuous IV Epoprostenol in patients with pulmonary arterial hypertension”. Chest 123 (2003): 1293-1295.
  26. Mourani PM., et al. “Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease”. The Journal of Pediatrics 154 (2008): 379-384.
  27. Yung D., et al. “Outcomes in children with idiopathic pulmonary arterial hypertension”. Circulation 110 (2004): 660-665.
  28. Ivy DD., et al. “Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension”. Journal of the American College of Cardiology 51 (2008): 161-924.
  29. Humpl T., et al. “Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study”. Circulation 111 (2005): 3274-3280.
  30. Ivy DD., et al. “Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings”. The American Journal of Cardiology 106 (2010): 1332-1338.
  31. Van Loon RL., et al. “Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies”. The American Journal of Cardiology 106 (2010): 117-124.
  32. Houde C., et al. “Profile of paediatric patients with pulmonary hypertension judged by responsiveness to vasodilators”. British Heart Journal 70 (1993): 461-468.

Mohammed Mokhtar Bekkar., et al. Pediatric Pulmonary Hypertension: Characteristics and Prospective Follow-Up of Children in Algeria. EC Paediatrics 12.3 (2023):76-86.