Two Decades of Single Ventricle Palliation: Survival Improvement and the Persistent Burden of Fontan Morbidity

Q: What are the key findings of this study?

Later surgical era (era 4 vs era 1) after Stage I single ventricle palliation was associated with reduced mortality, while 36-month Fontan completion was unchanged.

Q: What is the clinical evidence from this study?

Study design: Cohort. Population: Single ventricle congenital heart disease undergoing Stage I palliation (including hypoplastic left heart syndrome) (n=1732). Intervention: Later calendar-period surgical era (Era 4 vs Era 1) vs. Earliest surgical era (Era 1). Primary outcome: All-cause mortality (HR 0.35, 95% CI 0.26-0.48, p=<.001).

Q: What does this research mean for the field?

Survival after single ventricle palliation has improved substantially over the past two decades—especially for hypoplastic left heart syndrome—while Fontan completion rates have remained stagnant and the risk of heart failure and thromboembolism has increased. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Q: What question did this study set out to answer?

To examine era-specific trends in survival, Fontan completion, and long-term morbidity after single ventricle staged palliation in a nationwide Korean cohort over two decades.

Jae Hong Lim; Min Ji Kim; Jihye You

doi:10.1016/j.athoracsur.2026.10.036

November 1, 2026The Annals of Thoracic Surgery

Two Decades of Single Ventricle Palliation: Survival Improvement and the Persistent Burden of Fontan Morbidity

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Key result

Later surgical era linked to ~65% lower mortality after Stage I single-ventricle palliation.

HR 0.35
95% CI 0.26-0.48
P<.001
n=1,732

Why the study?

Population-level data on long-term outcomes after single ventricle staged palliation in East Asian cohorts were lacking.

Does treatment in more recent surgical eras improve survival, Fontan completion, and morbidity in patients undergoing Stage I single ventricle palliation in Korea?

Key Points

To examine era-specific trends in survival, Fontan completion, and long-term morbidity after single ventricle staged palliation in a nationwide Korean cohort over two decades.
Retrospective cohort study analyzing Korean National Health Insurance Service claims data (2002–2023) for 1,732 patients undergoing Stage I palliation across four calendar eras (median follow-up 20.0–199.9 months).
Evaluated overall survival and post-palliation morbidities using Cox proportional hazards regression, and assessed Fontan completion using Fine-Gray competing risk methods.
Adjusted mortality hazards declined markedly in era 3 (HR 0.54; 95% CI, 0.43–0.69; P < .001) and era 4 (HR 0.35; 95% CI, 0.26–0.48; P < .001) compared with era 1, with the greatest gains seen in hypoplastic left heart syndrome.
Fontan completion did not differ across eras, with cumulative incidence at 36 months remaining between 17.3% and 19.4% (P = .80).
Hazards for heart failure (HR 2.12), thromboembolism (HR 2.37), and reintervention rose in era 4 while arrhythmia declined (HR 0.26); female sex, preterm birth, hypoplastic left heart syndrome, and lower socioeconomic status independently predicted mortality.

Study Design

Type

Cohort (n=1,732)

Multicenter

Yes

Structured PICO

Does treatment in more recent surgical eras improve survival, Fontan completion, and morbidity in patients undergoing Stage I single ventricle palliation in Korea?

P

Population

1,732 patients in South Korea undergoing Stage I single ventricle palliation between 2002 and 2023, followed for a median of 20.0 to 199.9 months.

E

Exposure

Later calendar-period surgical eras (Era 3 and Era 4) of Stage I single ventricle staged palliation

C

Comparator

Earliest calendar-period surgical era (Era 1) of Stage I single ventricle staged palliation

O

Outcome

Mortality after Stage I single ventricle palliation across calendar-period eras (followed through December 2023, median follow-up 20.0-199.9 months)hard clinical

Main Result

Hazard Ratio: 0.35 (95% CI 0.26–0.48)

p-value: p=<.001

Over two decades in South Korea, survival after Stage I single ventricle palliation improved substantially—especially in hypoplastic left heart syndrome—without an increase in 36-month Fontan completion, while rising heart failure and thromboembolism hazards highlight a growing survivor population needing lifelong surveillance.

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Implication

Improved survival in later eras necessitates lifelong morbidity surveillance in survivors; leaves open whether gains reflect care advances or selection.

Abstract

Population-level data on long-term outcomes after single ventricle staged palliation in East Asian cohorts are lacking. We examined era-specific trends in survival, Fontan completion, and morbidity in a Korean nationwide cohort. This retrospective cohort study used Korean National Health Insurance Service claims data (2002-2023). Patients undergoing Stage I palliation were stratified into four calendar-period eras and followed through December 2023. Survival and morbidity were assessed by Cox regression; Fontan completion was analyzed by Fine-Gray competing risk methods and at fixed post-palliation horizons. Among 1,732 patients (median follow-up 20.0-199.9 months), adjusted mortality hazard ratios (HRs) were 0.54 (95% confidence interval [CI], 0.43-0.69) and 0.35 (95% CI, 0.26-0.48) in eras 3 and 4 versus era 1 (both P<.001), with the largest gains in hypoplastic left heart syndrome. Fontan completion did not differ across eras; at 36 months, cumulative incidence was 17.3%-19.4% (P=.80). Heart failure (HR, 2.12), thromboembolism (HR, 2.37), and reintervention hazards rose in era 4, whereas arrhythmia declined (HR, 0.26). Female sex, preterm birth, and hypoplastic left heart syndrome independently predicted mortality, as did socioeconomic position below the highest stratum in a categorical specification. Over two decades, survival after single ventricle palliation improved substantially in Korea, most strikingly in hypoplastic left heart syndrome, whereas Fontan attainment did not improve at a common horizon. The rise in heart failure and thromboembolism, together with higher mortality below the highest socioeconomic stratum, identifies a growing population requiring lifelong surveillance and targeted support.

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Cite This Study

Lim et al. (2026) conducted a cohort in Single ventricle congenital heart disease undergoing Stage I palliation (including hypoplastic left heart syndrome) (n=1,732). Later calendar-period surgical era (Era 4 vs Era 1) vs. Earliest surgical era (Era 1) was evaluated on All-cause mortality (HR 0.35, 95% CI 0.26-0.48, p=<.001). Later surgical era (era 4 vs era 1) after Stage I single ventricle palliation was associated with reduced mortality, while 36-month Fontan completion was unchanged.

synapsesocial.com/papers/6af88dda0e4d8f4b089c1c86 https://doi.org/10.1016/j.athoracsur.2026.10.036

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Two Decades of Single Ventricle Palliation: Survival Improvement and the Persistent Burden of Fontan Morbidity

Key Points

Study Design

Structured PICO

Main Result

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Two Decades of Single Ventricle Palliation: Survival Improvement and the Persistent Burden of Fontan Morbidity

Key Points

Study Design

Structured PICO

Main Result

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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