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Peer-reviewedCC BY 4.0Interdisciplinary Cardiovascular and Thoracic Surgery

Long-Term Outcomes After Supravalvar Aortic Stenosis Repair: Impact of Residual Haemodynamics on Reintervention

Abstract

Objectives: Surgical repair of supravalvar aortic stenosis (SVAS) provides excellent early outcomes; however, late reintervention remains common. The determinants of left ventricular outflow tract (LVOT) failure, particularly the role of residual haemodynamics, remain incompletely defined. We sought to identify predictors of LVOT reintervention in a contemporary cohort.

Methods: A retrospective study of consecutive patients aged <18 years undergoing SVAS repair between 2005 and 2024 was performed. Primary end-points were overall survival and freedom from LVOT reintervention. Survival and freedom from reintervention were assessed using Kaplan-Meier analysis. Multivariable Cox regression was used to identify independent predictors of LVOT reintervention.

Results: Seventy-seven patients underwent repair at a median age of 3 (1-8) years, with median follow-up of 9.3 (6-14) years. There was no early or late mortality; 19 patients (25%) required reintervention, including 16 (21%) LVOT-related procedures. Freedom from LVOT reintervention was 74% at 10 years and 71% at 15-20 years. Discharge LVOT velocity >3 metres per second (m/s) was independently associated with LVOT reintervention (hazard ratio [HR] 11.66, 95% confidence interval [CI] 3.56-38.18; P < .001). Younger age was also independently associated with increased risk (HR per year 0.74, 95% CI, 0.59-0.94; P = .013) for LVOT reintervention. Coronary abnormalities were documented in 17% of patients, with one early coronary reintervention.

Conclusions: Surgical repair of supravalvar aortic stenosis is associated with excellent long-term survival. Residual discharge velocity emerged as an independent predictor of late LVOT reintervention, supporting the importance of achieving optimal haemodynamic relief at the index operation and structured lifelong surveillance.

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BibTeX
@article{hussein2026longterm,
  title = {{Long-Term Outcomes After Supravalvar Aortic Stenosis Repair: Impact of Residual Haemodynamics on Reintervention}},
  author = {Nabil Hussein and Branko Mimic and Mats Synnergren and Nagarajan Muthialu and Victor Tsang and Martin Kostolny},
  journal = {Interdisciplinary Cardiovascular and Thoracic Surgery},
  year = {2026},
  month = aug,
  doi = {10.1093/icvts/ivag230},
  url = {https://doi.org/10.1093/icvts/ivag230},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Long-Term Outcomes After Supravalvar Aortic Stenosis Repair: Impact of Residual Haemodynamics on Reintervention
AU  - Nabil Hussein
AU  - Branko Mimic
AU  - Mats Synnergren
AU  - Nagarajan Muthialu
AU  - Victor Tsang
AU  - Martin Kostolny
PY  - 2026
DA  - 2026/08/17
JO  - Interdisciplinary Cardiovascular and Thoracic Surgery
DO  - 10.1093/icvts/ivag230
UR  - https://doi.org/10.1093/icvts/ivag230
AB  - Objectives: Surgical repair of supravalvar aortic stenosis (SVAS) provides excellent early outcomes; however, late reintervention remains common. The determinants of left ventricular outflow tract (LVOT) failure, particularly the role of residual haemodynamics, remain incompletely defined. We sought to identify predictors of LVOT reintervention in a contemporary cohort.  Methods: A retrospective study of consecutive patients aged <18 years undergoing SVAS repair between 2005 and 2024 was performed. Primary end-points were overall survival and freedom from LVOT reintervention. Survival and freedom from reintervention were assessed using Kaplan-Meier analysis. Multivariable Cox regression was used to identify independent predictors of LVOT reintervention.  Results: Seventy-seven patients underwent repair at a median age of 3 (1-8) years, with median follow-up of 9.3 (6-14) years. There was no early or late mortality; 19 patients (25%) required reintervention, including 16 (21%) LVOT-related procedures. Freedom from LVOT reintervention was 74% at 10 years and 71% at 15-20 years. Discharge LVOT velocity >3 metres per second (m/s) was independently associated with LVOT reintervention (hazard ratio [HR] 11.66, 95% confidence interval [CI] 3.56-38.18; P < .001). Younger age was also independently associated with increased risk (HR per year 0.74, 95% CI, 0.59-0.94; P = .013) for LVOT reintervention. Coronary abnormalities were documented in 17% of patients, with one early coronary reintervention.  Conclusions: Surgical repair of supravalvar aortic stenosis is associated with excellent long-term survival. Residual discharge velocity emerged as an independent predictor of late LVOT reintervention, supporting the importance of achieving optimal haemodynamic relief at the index operation and structured lifelong surveillance.
SN  - 2753-670X
ER  -