Outcomes of Hypoplastic Left Heart Syndrome by Subtype and Presence of Ventriculocoronary Connections: A Fetal Heart Society Multicenter Retrospective Study
Authors' conclusion
In a large, multicenter cohort of HLHS patients, the MS/AA subtype with VCCs had the lowest TFS. Prenatal diagnosis of VCCs was associated with a lower TFS. This study underscores the prognostic value of assessing for VCCs in patients with HLHS both before and after birth.
Study context
- Population
- Fetuses and infants <2 months old with hypoplastic left heart syndrome (HLHS) admitted between January 2012 and December 2016 to participating Fetal Heart Society institutionspatients born alive with intention to treat, among 16 centers, prenatal diagnosis of HLHS n=532 · MA/AA 261 · MS/AA 200 · MS/AS 130
- Participants
- 591 fetuses
- Exposure
- MA/AA, MS/AS, and MS/AA with and without ventriculocoronary connections (VCCs)
- MA/AAn = 261
- MS/AA with VCCsn = 115
- MS/ASn = 130
- Outcome
- Overall transplant-free survival (TFS)
- Follow-up
- 5 and 10 years
Methodological detailsFetal Heart Society institutions, Cox proportional hazards analysis controlling for cardiac center (random effect), birth weight, genetic diagnosis, extracardiac malformation, and restricted/intact atrial septum
- Fetal Heart Society institutions
- Cox proportional hazards analysis controlling for cardiac center (random effect), birth weight, genetic diagnosis, extracardiac malformation, and restricted/intact atrial septum
Main finding
As reported in the abstract
5-year transplant-free survival (TFS)
55.6%
10-year TFS
49.4%
Observational data: associations, not effects.
Further findings
| Outcome | MA/AA | MS/AA with VCCs | MS/AA without VCCs | MS/AS | HR (CI) | |
|---|---|---|---|---|---|---|
| Transplant-free survival (TFS) by subtype and VCCs · 5 and 10 yearsMS/AA with VCCsvs MS/AA without VCCsLowest TFS | 1.69 (1.12–2.57) | |||||
| 5-year TFS | 54.1% | 44.7% | 60.4% | 65.0% | ||
| 10-year TFS | 46.6% | 40.3% | 56.9% | 58.3% | ||
| Transplant-free survival (TFS) by subtype and VCCs · 5 and 10 yearsMA/AAvs MS/AA without VCCs | 1.40 (0.95–2.05) | |||||
| Transplant-free survival (TFS) by subtype and VCCs · 5 and 10 yearsMS/ASvs MS/AA without VCCsSurvival similar | 0.91 (0.59–1.41) | |||||
| TFS, MS/AA with VCCs suspected on fetal echocardiogramvs MS/AA without fetal suspicion for VCCs (among those with a prenatal diagnosis of HLHS, n=532)Lower TFS with fetal suspicion of VCCs | 1.79 (1.12–2.85) | |||||
| 0.20.5125Hazard ratio (log scale) |
- TFS, MS/AA with VCCs suspected on fetal echocardiogram vs MS/AA without fetal suspicion for VCCs (among those with a prenatal diagnosis of HLHS, n=532): subgroup with prenatal diagnosis of HLHS (n=532)
Fetal echocardiographic diagnosis of VCCs among MS/AA cases with VCCs suspected postnatally
- VCCs diagnosed by fetal echocardiography in cases with MS/AA and VCCs suspected postnatally
- 33 of 103 (32%)
TFS by type of stage I palliation, among patients with MS/AA and VCCs
- Type of stage I palliation not associated with TFS
- P = 0.784
Original publication
Outcomes of Hypoplastic Left Heart Syndrome by Subtype and Presence of Ventriculocoronary Connections: A Fetal Heart Society Multicenter Retrospective Study. Circulation. Published 26 October 2026. doi:10.
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