Abstract
Patients with adult congenital heart disease (ACHD) are at increased risk of developing late cardiovascular complication. However, little is known about the predictive factors for long-term outcome. The Model for End-Stage Liver Disease eXcluding INR (MELD-XI) score was originally developed to assess cirrhotic patients and has the prognostic value for heart failure (HF) patients. In the present study, we examined whether the score also has the prognostic value in this population. We retrospectively examined 637 ACHD patients (mean age 31.0 years) who visited our Tohoku University hospital from 1995 to 2015. MELD-XI score was calculated as follows; 11.76 x ln(serum creatinine) + 5.11 x ln(serum total bilirubin) + 9.44. We compared the long-term outcomes between the high (≥10.4) and the low (<10.4) score groups. The cutoff value of MELD-XI score was determined based on the survival classification and regression tree (CART) analysis. The major adverse cardiac event (MACE) was defined as a composite of cardiac death, HF hospitalization, and lethal ventricular arrhythmias. During a mean follow-up period of 8.6 years (interquartile range 4.4–11.4 years), MACE was noted in 51 patients, including HF hospitalization in 37, cardiac death in 8, and lethal ventricular arrhythmias in 6. In Kaplan-Meier analysis, the high score group had significantly worse MACE-free survival compared with the low score group (log-rank, P<0.001). Multivariable Cox regression analysis showed that the MELD-XI score remained a significant predictor of MACE (hazard ratio 1.36, confidence interval 1.17–1.58, P<0.001) even after adjusting for patient characteristics, such as sex, functional status, estimated glomerular filtration rate, and cardiac function. Furthermore, CART analysis revealed that the MELD-XI score was the most important variable for predicting MACE. These results demonstrate that the MELD-XI score can effectively predict MACE in ACHD patients, indicating that ACHD patients with high MELD-XI score need to be closely followed.
Highlights
Along with the advances in the treatment of congenital heart disease (CHD), more than 90% of CHD patients are expected to reach adulthood [1]
Compared with the low score group, the high score group was characterized by a male predominance, increased γ-glutamyl transpeptidase (GGT) and aspartate aminotransferase (AST), a lower platelet count, higher prevalence of great complexity of CHD, New York Heart Association (NYHA) functional class II, Fontan circulation, renal insufficiency, Systemic ventricular ejection fraction (SVEF)
These interactions are known as cardio-renal and cardio-hepatic syndromes [17]. Both increased serum total bilirubin and decreased Estimated glomerular filtration rate (eGFR) have been reported as risk factors for adverse cardiovascular outcomes in heart failure (HF) patients [18, 19] and in adult congenital heart disease (ACHD) patients [20, 21]
Summary
Along with the advances in the treatment of congenital heart disease (CHD), more than 90% of CHD patients are expected to reach adulthood [1]. We have recently demonstrated that HCV antibody positivity predicts cardiovascular outcomes in selected ACHD patients [7]. The Model for End-Stage Liver Disease (MELD) score was originally developed to assess the short-term survival of patients with liver cirrhosis undergoing transjugular intrahepatic portosystemic shunt (TIPS) procedures [8]. This score uses 3 objective biochemical values, including serum total bilirubin, creatinine, and international normalized ratio for prothrombin time (INR), and effectively reflects hepatic and renal dysfunction after TIPS procedures [8]. The MELD-XI score has been reported to predict poor outcomes in HF patients [11,12,13]
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