TITLE:
Pulmonary Arterial Hypertension in Congenital Heart Disease with Left-to-Right Shunt: According to a Retrospective Study at Albert Royer’s National Children’s Hospital Center
AUTHORS:
Aliou Mar Coundoul, Khadim Bop, Abdou Aziz Faye, Amadou Lamine Fall, Idrissa Demba Ba, Papa Mactar Faye
KEYWORDS:
PAH, Left-to-Right Shunt, Einsenmenger Syndrome
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.13 No.5,
September
5,
2023
ABSTRACT: Pulmonary
arterial hypertension PAH is a progressive disease characterized by an increase
in pulmonary arterial pressure and resistance. It often complicates congenital
heart disease with a left-to-right shunt. The objective of this study is to
evaluate the evolutionary profile of the pulmonary pressures of children with
congenital heart disease with left-to-right shunt and to identify the factors
favoring an evolution towards PAH. This is a retrospective, descriptive and
analytical study over a period of four years and six months (from January 1,
2016 to June 30, 2020) at the Center National d’Enfant Albert Royer in Dakar on
a series of 87 cases. The hospital frequency was 3.98 per thousand, and the
incidence of PAH was 9.44%. The sex ratio of 0.74. The average age was 44.76
months. The average time between the onset of symptoms and the diagnosis of
heart disease is 78.81 days. Dyspnea was found in 70.11% of cases, the burst of
pulmonary B2 was found in 55%. Global heart failure was noted in 39.08% of
cases. Cardiomegaly was found in 89.66% and pulmonary hypervascularization was
found in 57.72% of cases. CIV was present in 54.02% of cases, followed by PCA
in 21.14% and CAVc in 18.39% of cases. furosemide was used in 97.70% of cases
and Captopril in 74.71% of cases. Sildenafil was used in 10.34% of patients, and
12.64% of patients benefited from surgical management. Eisenmenger syndrome was
found in 12.64% of patients. PAH in these heart diseases is a formidable and
frequent complication, due to an increase in precapillary flow. The major
improvement in mortality and morbidity is early surgical management, in the
first months of life, to prevent pulmonary vascular disease.