Background: The significant pulmonary vascular resistance can result in right-sided heart failure, elevated pulmonary vascular resistance, and even mortality, a progressive condition known as pulmonary arterial hypertension (PAH), which also has a high mortality and morbidity rate. Objectives: we aimed investigate plasma connective tissue growth factor (CTGF) levels in pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) (PAH-CHD) in children and the relationships of CTGF with hemodynamic parameters.  Material and methods: A review of MEDLINE, Cochrane Library, ClinicalTrials.gov, indexed through 31 October 2022, utilizing the following searches: connective tissue growth factor OR/AND one of the following: Biomarker; Children; Congenital heart disease; Pulmonary arterial hypertension [MESH], was conducted. Additional studies were identified through review of references. English-language epidemiological studies, clinical studies, and case reports/series of connective tissue growth factor was included. The authors reached consensus regarding study inclusion after full-text review. The body of literature was assessed for bias qualitatively. Conclusion: Children with PAH-CHD had considerably higher serumconnective tissue growth factor (CTGF) levels, which were inversely linked with oxygen saturation. The parameters of the echocardiograph and CTGF did not correlate. A possible diagnostic biomarker for PAH-CHD in children may be found in serum CTGF levels.