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Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis

European Journal of Medical Research volume 30, Article number: 26 (2025) Cite this article

Abstract

Background

An effective prognostic nomogram to predict the prognosis for supravalvular aortic stenosis (SVAS) patients is lacking.

Methods

A multi-center retrospective study of consecutive SVAS patients with surgery between 2002 and 2020 was conducted. Patients underwent McGoon repairs, Doty repairs, and other repairs. The primary outcome was the re-operation or restenosis at follow-up. The nomogram based on Cox regression and Kaplan–Meier method was used to show the risk factors of the primary outcome. The predictive accuracy was determined by the concordance index (C-index) and calibration curve. The results were validated using the bootstrap resampling method.

Results

Of the 291 SVAS patients, 143 (49.1%) used McGoon repairs, 118 (40.5%) used Doty repairs and 30 (10.3%) used other repairs. The median age at operation was 4.9 years (IQR 2.3–9.9). After a median follow-up of 24 months (IQR 6.0–54.0), no difference in re-operation or restenosis was found between McGoon repairs and Doty repairs. Age, gender, SVAS type, pulmonary artery stenosis, aortic valve stenosis, sinotubular junction z-score and gradient were considered independent risk factors by Lasso regression and were included in the nomogram. The C-index of the nomogram was 0.71 (95% CI 0.61 to 0.81). The calibration curve for the probability of re-operation or restenosis showed good agreement between prediction by nomogram and actual observation.

Conclusions

McGoon repairs and Doty repairs had no difference in re-operation and restenosis risk. The proposed nomogram gave an accurate prediction of re-operation or restenosis for patients with SVAS after surgery.

Trial registration http://www.chictr.org.cn, ChiCTR2300067851, 2023.01.29 (retrospectively registered).

Background

Supravalvular aortic stenosis (SVAS) is an uncommon left ventricular outflow tract obstructive congenital cardiac malformation, accounting for approximately 0.05% of all congenital heart diseases and characterized by an hourglass-shaped narrowing of the sinotubular junction (STJ) [1]. Depending on the extent of aortic arch involvement, SVAS is classified as discrete (type I) or diffuse (type II) [2]. In various studies in the literature, the prevalence of type II disease is 14–35% [1]. Patients with type II lesions have more severe symptoms and present earlier compared to those with type I [3, 4]. In addition, almost half of the patients with SVAS have aortic valve lesions, with possible causes being congenital anomalies or secondary to the high pressure in the proximal aortic root [5].

Several surgical techniques have been described for the repair of SVAS, but the rarity of this lesion results in a small patient cohort and makes it difficult to reach a consensus on the optimal approach [6]. Among them, McGoon repair, Doty repair, Brom repair, and sliding aortoplasty have been reported successively [7,8,9,10]. Although favorable early and mid-term outcomes have been reported, the higher re-operation rate in the long term cannot be ignored [11,12,13]. Several studies have reported re-operation rates of 0–32% after surgical treatment of SVAS [4, 14,15,16,17]. Gender [12], age at surgery [11], type of SVAS [4], and pulmonary artery disease [18] were the independent risk factors of re-operation, but a comprehensive predictive model was lacking.

Therefore, we aimed to compare the surgical outcomes of different surgical techniques and develop a nomogram of re-operation and restenosis based on the patients from two centers.

Methods

Patients

This registered study included 330 consecutive SVAS patients from Beijing FuWai and Yunnan FuWai Hospital between May 2002 and January 2020. The diagnosis of SVAS was documented by an echocardiogram. Patients without surgical treatment and patients with secondary postoperative SVAS were excluded (n = 39). And finally, 291 patients were included in our cohort (Fig. 1). Follow-up echocardiography was performed at 1 month, 3 months, 6 months, and 1 year after surgery. After the first year, echocardiography is performed annually. This retrospective study was approved by our institutional ethics committee of Fuwai Hospital (no.2021–1578). Informed consent was waived for retrospective collection and analysis of deidentified demographic and medical data.

Fig. 1
figure 1

Flow chart of patient selection and follow-up. FU follow-up. SVAS supravalvular aortic stenosis

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Surgical technique

A standard aortic and bicaval cardiopulmonary bypass setup was established. Cardiac arrest was accomplished by cardioplegia, moderate hypothermia, and cross-clamping. For McGoon repairs, the stenosis section was incised longitudinally from the ascending aorta to the non-coronary sinus of Valsalva, and then it was enlarged with a teardrop-shaped patch (Fig. 2A, B). For Doty repairs, the incision was made in an inverted Y-shape from the ascending aorta through the non-coronary and the right coronary sinus of Valsalva. A pantaloon-shaped patch was placed in the enlarged aortic segment (Fig. 2C, D). Autologous pericardium, bovine or glutaraldehyde-treated autologous pericardium was used in the technique. After the reconstruction of the aorta, the patient was disconnected from the bypass in the usual manner. The associated lesion was repaired contemporaneously with the cross-clamp time (details of other repairs were in the supplementary material).

Fig. 2
figure 2

McGoon repair (A&B) and Doty repair (C&D) of supravalvular aortic stenosis. A Incision from ascending aorta to non-coronary sinus; B teardrop-patch augmentation of the supravalvular aortic segment; C reverse “Y”-shaped incision from ascending aorta to non-coronary and right coronary aortic sinuses; D pantaloon-patch augmentation of supravalvular aortic segment. Note: This is an original work by the authors, created for this study, and based on standard surgical protocols

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Variables

The demographic characters, operative, post-operative, and follow-up data were obtained from hospital records. The primary outcome was the re-operation or restenosis at follow-up. Restenosis was defined as peak supravalvular velocity > 4 m/s or mean supravalvular aortic gradients over 40 mmHg [1] by transthoracic echocardiogram. The z-score of STJ, and ascending aorta were calculated according to the Boston Children's Hospital echocardiography calculation tool (https://zscore.chboston.org/). The z-score of pulmonary artery < −2 was identified as pulmonary artery stenosis (PS). Aortic valve stenosis (AVS) was identified and defined as any mild or more AVS (peak flow velocity across the aortic valve > 2 m/s, or mean gradient pressures across the aortic valve > 20mmhg). Each patient’s pre-operative and post-operative cardiac anatomy was evaluated by transthoracic echocardiography. If echocardiography findings were equivocal, an angiographic evaluation was performed. All patients completed echocardiographic evaluation preoperatively, before discharge, and at the most recent follow-up visit.

Statistics analysis

Continuous variables were reported as mean ± SD and median (inter-quartile range (IQR)). Dichotomous variables were reported as the frequency (percentage). Analysis of variance was used to compare normally continuous variables, and the Kruskal–Wallis H test was to compare non-normally distributed continuous variables. The Pearson chi-squared test or Fisher’s exact test was used to compare categorical data. Kaplan–Meier plot was used to depict the cumulative events of the primary outcome. Hazard ratios (HR) were calculated using Cox proportional hazards regression models. Variable selection considered Cox regression models. Variables with a p value < 0.1 in the univariate Cox regression model were selected in the multivariate model. The least absolute shrinkage and selection operator (LASSO) regression was used as an alternative way of variable selection. A final model selection for the nomogram was based on the multivariate Cox regression, LASSO regression model, and clinical experience. The performance of the nomogram was assessed by the concordance index (C-index), the area under the curve (AUC), and the calibration plot. Bootstraps with 1000 resamples were used to validate the model. Missing data were imputed using multiple imputation methods. A two-sided p value < 0.05 was considered to be significant. All analyses were conducted using R (version 3.5.1).

Results

Baseline information

Among 291 SVAS patients, 143 (49.1%) used McGoon repairs, 118 (40.5%) used Doty repairs and 30 (10.3%) used other repairs (Brom repair, and sliding aortoplasty). The median age at operation was 4.9 years (IQR: 2.3–9.9). Detailed demographics, Concomitant cardiovascular anomaly, and surgery-related characteristics are summarized in Table 1. 33.7% of the included patients were female, 34.4% had diffuse SVAS, 22.3% combined with Williams–Beuren syndrome (WBS), 18.6% with PS, and 3.1% with AVS. Patients with Doty repairs had a lower STJ z-score compared with other groups. No other difference was found among the three surgical techniques (Tables 1, S1).

Table 1 Baseline characteristics and intra-operative information of patients with SVAS
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Postoperative and follow-up information

Five patients (1.7%) died in the hospital and no difference existed among the three groups (P = 0.573). Three dead patients were treated with the McGoon repairs, one with Doty repair, and one with sliding aortoplasty. Four of them were caused by heart failure and the remaining one was caused by pulmonary arterial hypertension. During the hospitalization, twelve patients had re-operation, one patient underwent a re-correction of SVAS, two patients underwent diaphragmatic plication, two patients underwent surgical hemostasis, six patients underwent chest closure, and one patient underwent subaortic membrane resection (Table 2).

Table 2 Post-operative and follow-up information of the patients with SVAS
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The Echocardiographic follow-up was conducted in 82.5% (240/291) of patients. The baseline information of patients with follow-up did not differ from patients without follow-up. The median follow-up duration was 24.0 months (IQR: 6.0–54.0).

At follow-up, no death occurred and eight patients (4.2%) had re-operation. Four patients underwent re-correction of SVAS, two of whom underwent aortic arch surgery and aortic valvuloplasty at the same time. Two patients underwent Ross surgery, and two patients underwent aortic valvuloplasty. 33 (15.0%) patients had restenosis. The re-operation or restenosis rates were 5.1% (2.1%, 8.0%), 10.7% (5.6%, 15.4%), and 19.2% (11.3%, 26.5%) at 1-, 3-, and 5-year follow-ups, respectively (Fig. 3A). Kaplan–Meier curves showed no difference in re-operation or restenosis between McGoon repairs and Doty repairs (Fig. 3B).

Fig. 3
figure 3

Kaplan–Meier curves of the composite outcome (re-operation or restenosis). A All patients; B McGoon repair vs. Doty repair groups. p values were obtained by the log-rank test

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Prognostic nomogram for a composite outcome of re-operation and restenosis

The univariate analyses and lasso regression demonstrated that age, SVAS type, PS, AVS, STJ z-score, and STJ gradient were independent risk factors for the composite outcome of re-operation and restenosis. The multivariate cox model identified that age < 1 year (compared with ≧18 years, HR = 1.23, 95% CI 1.02 to 1.54; p < 0.05), STJ mean gradients ≧60 mmHg (compared with < 40 mmHg, HR = 1.10, 95% CI 1.02 to 1.25; p < 0.045), STJ z-score < −4 (compared with ≧2, HR = 1.35, 95% CI 1.16 to 1.56; p < 0.001), and PS (HR = 1.15, 95% CI 1.02 to 1.30; p < 0.021) had more risk on re-operation or restenosis (Tables S2, S3, Figure S1).

The prognostic nomogram integrated all significant independent factors for re-operation and restenosis (Fig. 4A). The STJ z-score showed the best prediction ability and an STJ z-score less than −4 showed 100 points risk. Patients with PS had 55 points, STJ mean gradient over 60 mmHg gave 50 points, and ages younger than 1-year-old gave 44 points. The C-index for the composite outcome prediction was 0.71 (95% CI 0.61 to 0.81). The AUC of the model was 0.842, 0.783, and 0.691 at 1-, 3- or 5-year follow-up, separately (Figure S2). The calibration plot for the risk probability at 1-, 3- or 5-year follow-up indicated an optimal agreement between the prediction and actual observation (Fig. 4B). The result was validated by bootstrap resampling 1000 times, and 80 patients per time.

Fig. 4
figure 4

Nomogram to predict re-operation or restenosis of SVAS patients after surgery at 1 year, 3 years, and 5 years. A Nomogram of the composite outcome. For each individual, a vertical line is drawn through each feature status according to the patient's profile towards the ‘points’ line. This assigns a point to the individual. All points are summed to generate a total point. A vertical line is then drawn at the ‘total point’ axis to the ‘risk probability’ axis for prediction of the re-operation or restenosis at a certain timepoint. B Calibration curves for the nomogram at 1 year, 3 years, and 5 years. The x-axis represents the nomogram-predicted probability and the y-axis represents the actual probability of re-operation or restenosis. The perfect prediction would correspond to the 45°dashed line. The red line represents the entire cohort (n = 240) with the blue error bar. The bias-corrected points were marked by asterisk through bootstrap resampling (B = 1000 repetitions, n = 80 per time), indicating observed nomogram performance. AVS Aortic valve stenosis, PS Pulmonary artery stenosis, STJ Sinotubular junction, SVAS Supravalvular aortic stenosis

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Discussion

This multi-center retrospective clinical study for congenital SVAS patients showed a good overall survival rate and acceptable surgical risk compared to other studies [4, 13, 19]. McGoon and Doty repairs had no significant differences in survival, re-operation, and restenosis rates. In addition, with the relatively large sample size, we identified key risk factors (age, gender, SVAS type, PS, AVS, STJ z-score and mean gradient) and developed the first nomogram to predict risks of re-operation and restenosis after surgery. The model had satisfactory and robust performance, which was of great significance for postoperative monitoring in high-risk patients.

The optimal surgical technique is currently unknown. Like other Asian hospitals [20], McGoon repair and Doty repair were used more in our centers, while Brom repair and sliding aortoplasty were used less. Our findings showed no statistically significant difference between McGoon repair and Doty repair for in-hospital death, late re-operation and restenosis. It is worth noting that patients with Doty repairs had a worse baseline STJ z-score (− 1.5 ± 2.0) compared with McGoon repairs (− 0.5 ± 2.8). Ibarra [21] et al. also showed a similar baseline STJ z-score of Doty and McGoon repairs, and they found a lower re-operation rate of Doty repairs. In addition, a meta-analysis indicated that Doty repairs had a lower rate of late re-operation compared to McGoon repairs and Brom repairs [20]. Considering that Doty repairs have a greater increase in the STJ, we suggest using Doty repairs for patients with severely obstructed lesions, but the choice of surgical technique is more dependent on the operator's habits and preferences.

This study found that preoperative combined AVS or PS had a higher risk of re-operation and restenosis, which was consistent with previous studies. In our cohort, 22.3% of patients had WBS, an elastin artery lesion that could accumulate in the aorta and pulmonary arteries [5]. Even though genetic testing was not a standard practice at our center, leading to a lower detection rate of WBS compared to Western nations (58.5%) [13], this study reported the distribution of all the combined cardiac abnormalities. For AVS, several studies have shown that preoperative combined aortic valve disease (aortic valve stenosis, aortic valve regurgitation, bicuspid aortic valve) was a predictor of distant mortality and re-operation, and there was a consensus to manage aortic valve disease concurrently [4, 12, 22,23,24]. For PS, it is generally defined as the narrowing of the left or right pulmonary arteries resulting in a pressure gradient over 30 mmHg. However, the treatment strategy for PS was uncertain. Some researchers considered that the natural history of the PS might decrease spontaneously over time, making the indications for surgery in these patients limited [23]. Some advocated catheter intervention alone, some preferred surgical treatment, while others combined these two approaches [5, 25, 26]. Our strategy was to conduct either unilateral or bilateral pulmonary arterioplasty for patients with preoperative PS. However, regardless of the treatment approach, close follow-up was necessary.

It was not clear whether gender and age at surgery were the risks of re-operation and restenosis at follow-up. Previous studies found that male was a risk factor for residual aortic stenosis [5, 12]. The possible reason was the higher percentage of male SVAS patients and the greater growth potential in males than females. For the age of surgery, various studies have focused on patients with a mean age of 1.9–15.8 years, but the optimal age for repair remains unknown [4, 14, 17, 22, 27,28,29,30]. Surgical repair in early childhood was challenging, and patients < 1 year were often at higher risk of re-intervention [22]. Previous studies also demonstrated that most surgical deaths occurred in children < 2 years [3, 11]. The smaller aortic root size and smaller coronary lumen size in younger patients might make SVAS repair more difficult, but there was no evidence directly suggesting that these factors affect early and late outcomes. This study found that patients < 1 year had the highest risk of re-operation. To minimize the risk of re-operation, it was recommended that children with asymptomatic SVAS and no other cardiovascular abnormalities consider delaying surgical intervention until 1 year of age.

Several studies have confirmed diffuse SVAS, excessive pressure gradient and z-score in the STJ as risk factors for the development of re-operation and restenosis in the long-term follow-up [4, 12, 14, 30, 31].Diffuse SVAS is not a localized disease and may extend to the aortic arch in addition to stenosis at the STJ [32]. The overall poorer vascular development in these patients may contribute to the poorer prognosis. We also found that higher pressure gradients and z-score at the STJ are risk factors for re-operation and restenosis, suggesting that the severity of the original obstruction may determine the prognosis of SVAS. A study showed that the z-score was higher in patients after multi-sinus repair than in those after single-sinus repair [21]. In patients with severe obstruction at the STJ and distal stenosis, adequate relief of the obstruction and correction of the distal arterial obstruction with multi-sinus repair, preserving the function of the aortic valve as much as possible, maybe an appropriate strategy for such patients.

Strengths and limitations

This study had the second-largest multi-center sample size to date, with uniform diagnostic criteria and homogeneous treatments. In addition, we provided the first nomogram to predict the prognosis of patients with SVAS. However, this study still had some limitations. First, we only included Chinese patients, which would limit the generalizability of our results. However, the risk factors provided in this study have been separately demonstrated in other studies, so the results of this study could also benefit patients worldwide as a way to assess prognosis. Another limitation was that our model was only validated by resampling due to the rarity of the disease. Therefore, it still needs to be validated in external multi-center studies. Furthermore, the follow-up period is relatively short and the follow-up rate is not complete, which may be a source of potential bias. Future studies should aim for longer follow-up period with higher patient follow-up rates.

Conclusion

Surgical repair of congenital SVAS is an effective treatment with low surgical risk, but late adverse events require focused attention. In this study, there were no significant differences in surgical outcomes between McGoon repairs and Doty repairs. We then identified key prognostic variables that predicted the risk of restenosis and re-operation after surgical treatment of SVAS. A nomogram with good predictive abilities for the occurrence of re-operation and restenosis in SVAS patients was established, which could be used as a practical method for individualized risk evaluation for patients with SVAS after surgery. Future prospective, multi-center, long follow-up studies are needed to externally validate the nomogram.

Availability of data and materials

The data sets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Abbreviations

AVS:

Aortic valve stenosis

PS:

Pulmonary artery stenosis

STJ:

Sinotubular junction

SVAS:

Supravalvular aortic stenosis

WBS:

Williams–Beuren syndrome

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Acknowledgements

None.

Funding

This study was funded by the National Key Research and Development Plan during the fourteenth Five-Year Plan Period (2022YFC2503400, 2022YFC2503402); the Clinical Study on Integrated Management of Prenatal and Postpartum, and Intrapartum Surgery to Improve the Prognosis of Critical Congenital Heart Disease in Newborns (No. KCZD202202); the Noncommunicable Chronic Diseases–National Science and Technology Major Project (2023ZD0514400); and the Major Science and Technology Special Plan Project of Yunnan Province (202302AA310045).

Author information

Author notes

  1. Yuekun Sun and Lizhi Lv have contributed equally to this article.

Authors and Affiliations

  1. Department of Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, China

    Yuekun Sun, Lizhi Lv & Qiang Wang

  2. Department of Cardiac Surgery, Yunnan Fuwai Cardiovascular Hospital, Kunming, 650102, China

    Lizhi Lv & Qiang Wang

  3. Department of Pharmacy and Clinical Trial Unit, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, China

    Xinyue Lang

  4. Department of Radiology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, China

    Aihua Zhi

  5. Department of Radiology, Yunnan Fuwai Cardiovascular Hospital, Kunming, 650102, China

    Aihua Zhi

  6. Department of Cardiac Surgery, Peking University People’s Hospital, Beijing, 100044, China

    Simeng Zhang

  7. Center for Pediatric Cardiac Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, China

    Cheng Wang

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Contributions

Y.-K.S. and L.-Z.L. designed the study, collected and analyzed the data, wrote and revised the manuscript. X.-Y.L.: Analyzed the data and wrote the manuscript. A.-H.Z., S.-M.Z., C.-W., and Q.-W. Analyzed the data and revised the manuscript. All authors have read and approved the publication of the manuscript.

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Correspondence to Cheng Wang or Qiang Wang.

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The study protocol was approved by the local ethics committee who waived the need for obtaining informed consent of patients for this retrospective analysis.

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Sun, Y., Lv, L., Lang, X. et al. Surgical techniques and prognostic nomogram for patients with supravalvular aortic stenosis. Eur J Med Res 30, 26 (2025). https://doiorg.publicaciones.saludcastillayleon.es/10.1186/s40001-024-02257-w

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  • DOI: https://doiorg.publicaciones.saludcastillayleon.es/10.1186/s40001-024-02257-w

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European Journal of Medical Research

ISSN: 2047-783X

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