Pulmonary hypertension in congenital heart disease of children


  • Radohery Lovasoa Randriamanga Department of Paediatrics, Antananarivo Faculty of Medicine, Madagascar https://orcid.org/0000-0002-4065-1102
  • Fanjaharimbola Cynthia Rakotoarivelo Department of Paediatrics, Antananarivo Faculty of Medicine, Madagascar
  • Lalaina Andonirina Rakotoarisoa Department of Paediatrics, University Hopsital Joseph Raseta Befelatanana, Madagascar




Congenital heart disease, Atrioventricular canal, Ventricular septal defect, Pulmonary arterial hypertension, Persistent ductus arteriosus


Background: Pulmonary arterial hypertension is the dreaded complication of congenital heart disease with left-right shunt. The aim of this study was to describe the epidemio-clinical aspects of PAH associated with congenital heart disease.

Methods: This was a retrospective descriptive multicentre study over a 2-year period (1 January 2020 to 31 December 2021) conducted in three University Hospitals in Antananarivo. Hospitalized children with congenital left-right shunt heart disease were included.

Results: A total of 74 cases were recruited. The sex ratio was 0.7. The mean age of the children was 10.9 (SD=18.2) months. The mean age of onset of heart disease was 8 (SD=13.6) months. Heart disease was dominated by ventricular septal defect (51.3%), patent ductus arteriosus (39.2%), and atrioventricular canal (8.1%). PAH was encountered in 49 cases (66.2%). The majority of children with PAH were under 12 months of age (71.4%) and malnourished (70.2%). According to the type of heart disease, PAH was encountered in all complete CAVs, in 76.3% of CIVs and in 55.2% of PCAs. Eseinmenger's syndrome was present in 4% of cases, and the death rate was 20.3%.

Conclusions: Improved management of children with congenital heart disease is needed, both in terms of diagnosis and surgical treatment, to prevent the onset of PAH.


Rosenzweig EB, Abman SH, Adatia I, Baghetti M, Bonnet D, Haworth S, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019;53:180.

Begetthi M. Hypertension arterielle des cardiopathies congénitales. Rev Mal Respir. 2006;23 :13S49-59.

Fraissea A, Jaïsb X, Schleichc JM, Di Filippod S, Maragnèse P, Beghettif M, et al. Caractéristiques et suivi prospectif sur deux ans des enfants atteints d’hypertension artérielle pulmonaire en France. Arch Cardiovasc Dis Supp. 2010;2:137-45.

Mulder BJ. Évolution démographique de l'hypertension artérielle pulmonaire dans les cardiopathies congénitales. Rev Resp Eur. 2010; 19(118):308-13.

Duffels MGJ, Engelfriet PM, Berger RMF, Van Loon RLE, Hoendermis E, Vriend JWJ, et al. Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120:198-204.

Beghetti M. Congenital heart disease and pulmonary hypertension. Rev Port Cardiol. 2004;23:273-81.

Bouzas B, Gatzoulis MA. Pulmonary arterial hypertension in adults with congenital heart disease. Rev Esp Cardiol. 2005;58:465-9.

Galié N, Torbicki A, Barst R. Guidelines on diagnosis and treatment of pulmonary arterial hypertension, The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. 2004;25:2243-78.

Bazolo Ba Ngouala GA, Affangala DA, Laye M, Kane A. Prévalence des cardiopathies infantiles symptomatiques au Centre Hospitalier Régional de Louga, Senegal. Cardiovasc J Afr. 2015;26(4):e1-5.

Żuk M, Mazurkiewicz-Antoń K, Migdał A, Jagiełłowicz-Kowalska D, Turska-Kmieć A, Ziółkowska L. Prognosis in children with pulmonary arterial hypertension: 10-year single-center experience. Kardiol Pol. 2016;74(2):159-67.






Original Research Articles