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The Long-term Outcomes and Risk Factors of Complications After Fontan Surgery: From the Korean Fontan Registry (KFR)
Sang-Yun Lee1, Soo-Jin Kim2, Chang-Ha Lee3
1Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Insights
Fontan surgery outcomes were analyzed in Asian patients. Heterotaxy syndrome and high pulmonary artery pressure were poor prognostic factors, while low pulmonary artery pressure and less atrioventricular valve regurgitation improved survival.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Outcomes Research
Background:
- Limited data exists on Fontan surgery outcomes in Asian populations.
- This study addresses the need for regional data from the Republic of Korea.
Purpose of the Study:
- To analyze the outcomes of Fontan surgery in a large cohort of Korean patients.
- To identify risk factors for early and late mortality after Fontan surgery in this population.
Main Methods:
- Retrospective review of medical records from 1,732 patients who underwent Fontan surgery across 10 cardiac centers.
- Data collected included patient demographics, surgical type, pre-operative conditions, and survival rates.
Main Results:
- Overall survival rates at 10, 20, and 30 years were 91.7%, 87.1%, and 74.4%, respectively.
- Risk factors for early mortality included male sex, heterotaxy syndrome, atriopulmonary (AP) type surgery, and high pre-operative mean pulmonary artery pressure (mPAP).
- Risk factors for late mortality included heterotaxy syndrome, genetic disorders, significant atrioventricular valve regurgitation (AVVR), high pre-operative mPAP, and absence of fenestration.
Conclusions:
- Heterotaxy syndrome is a significant poor prognostic factor in Asian patients undergoing Fontan surgery.
- Preoperative low mPAP and less AVVR are associated with improved early and long-term outcomes.
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.
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