mohammed mokhtar bekkar

Abstract

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