Objective To investigate the evaluation value of the ratio of C-reactive protein(CRP) to albumin(ALB) in the long-term prognosis of patients with congenital heart disease-related pulmonary hypertension. Methods A total of 216 patients with congenital heart disease (CHD) and pulmonary hypertension (PAH) who were treated in our hospital from June 2014 to May 2018 were selected, and the patients were divided into survival group (174 cases) and death group(42 cases) based on the results of the 3-year follow-up. Automatic biochemical analyzer was used to detect the level of CRP and ALB and then calculate CRP/ALB. The receiver operating characteristic curve (ROC) was drawn, and the area under the curve (AUC) was calculated to analyze the predictive value of CRP/ALB on the prognosis of patients with CHD and PAH. Multivariate logistic regression analysis explored the prognostic factors of patients with CHD and PAH. Results Serum creatinine, serum potassium, NT-proBNP, hscTnT, resting heart rate, CRP, and CRP/ALB in the death group were significantly higher than in the survival group, and ALB was significantly lower than in the survival group (P<0.05). CRP/ALB ratio was significantly positively correlated with serum creatinine, NT-proBNP, hs-cTnT, NYHA cardiac function classification (r=0.413, 0.583, 0.621, 0.677, P<0.05). The area under the ROC curve (AUC) of CRP and ALB in predicting the prognosis of patients with CHD and PAH were 0.720 (95%CI: 0.656~0.798) and 0.662 (95%CI: 0.608~0.713), respectively. Under the critical value of 85.3 mg/L, the sensitivity of CRP prediction was 74.4%, and the specificity was 71.2%. Under the critical value of 27.5 g/L, the sensitivity of ALB prediction was 66.4%, and the specificity was 83.2%. The AUC of CRP/ALB was 0.837 (95%CI: 0.768~0.903), the sensitivity was 85.1%, and the specificity was 84.5%. The results of multivariate logistic regression analysis showed that: the OR and 95% CI of the CRP/ALB ratio of Model 1 (before adjustment), Model 2 (adjusted for gender, age, systolic blood pressure, diastolic blood pressure, resting heart rate, serum potassium), Model 3 (based on model 2 and then adjusted for NT-proBNP, hs-cTnT, blood creatinine, NYHA cardiac function classification) were 2.036 (1.284~3.227), 1.944 (1.558~2.426), 1.868 (1.594~2.189), and CRP/ALB ratio was an independent predictor of poor prognosis (death) in patients with CHD and PAH(P<0.05). Conclusion The CRP/ALB ratio is an independent predictor of the prognostic death risk of patients with CHD and PAH and is related to the NYHA cardiac function classification. It is expected to become a biomarker for screening early complications of CHD. An increase in the CRP/ALB ratio predicts a high prognostic risk of death.