Korean Circ J.  2024 Oct;54(10):653-668. 10.4070/kcj.2023.0211.

The Long-term Outcomes and Risk Factors of Complications After Fontan Surgery: From the Korean Fontan Registry (KFR)

• Lee SY
• Kim SJ
• Lee CH
• Park CS
• Choi ES
• Ko H
• An HS
• Kang IS
• Yoon JK
• Baek JS
• Lee JY
• Song J
• Lee J
• Huh J
• Ahn KJ
• Jung SY
• Cha SG
• Kim YH
• Lee Y
• Cho S

• KMID: 2560648
• DOI: http://doi.org/10.4070/kcj.2023.0211

Abstract

Background and Objectives
This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries.
Methods
The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed.
Results
Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration.
Conclusions
In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Keyword

Fontan procedure; Republic of Korea

Figure

• 

  Figure 1 Summary of patients who underwent Fontan surgery in the Republic of Korea.AP = atriopulmonary; BCPS = bidirectional cavopulmonary shunt; BT = Blalock-Taussig; ECC = extracardiac conduit; FU = follow-up; LT = lateral tunnel; TPL = transplantation.

• 

  Figure 2 The number of Fontan surgery. (A) The number of Fontan surgeries according to type and (B) cumulative number of Fontan surgeries according to year. The blue color indicates AP; red, LT; and green, ECC type.AP = atriopulmonary; ECC = extracardiac conduit; LT = lateral tunnel.

• 

  Figure 3 Kaplan–Meier curve of (A) the overall survival curve, (B) survival curve according to Fontan type, and (C) Fontan survival curve according to Fontan operation period. Overall, the 10-, 20-, and 30-year survival rates were 91.7%, 87.1%, and 74.4%, respectively. AP-type (p<0.0001) and period before the 2000 of Fontan surgery (p<0.0001) were associated with a significantly lower overall survival rate.AP = atriopulmonary; ECC = extracardiac conduit; LT = lateral tunnel.

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