Introduction
CHDs are common in children, with coarctation of the aorta representing a significant subset, constituting approximately 6–8% of CHD cases. Reference Setty, Patil, Ramegowda, Vijaykumar and Manjunath1 This condition presents typically as stenosis of the proximal thoracic aorta, leading to distinct anatomical, pathophysiological, and clinical characteristics that require diverse treatment approaches and result in various outcomes. Reference Setty, Patil, Ramegowda, Vijaykumar and Manjunath1,Reference Allen, Driscoll, Shaddy and Feltes2
The pathophysiology of coarctation of the aorta is influenced by the severity of stenosis and the presence of associated lesions, such as patent ductus arteriosus, ventricular septal defect, or left ventricular outflow obstruction. Reference Allen, Driscoll, Shaddy and Feltes2,Reference Anderson, Baker, Penny, Redington, Rigby and Wernovsky3 The prevalence disparity between boys and girls is noteworthy in this condition, with a higher prevalence observed in boys, as observed in several left-sided obstructions. Reference Yokoyama, Ichikawa, Minamisawa and Ishikawa4–Reference Kenny and Hijazi6 Mechanical models suggest that coarctation of the aorta arises from irregular blood flow patterns, endothelial cell migration disruptions, and excessive tissue accumulation in the aortic isthmus. Reference Allen, Driscoll, Shaddy and Feltes2 Fetal adaptation mechanisms allow the fetus to tolerate the coarctation of the aorta. After birth, closure of the ductus arteriosus and foramen ovale can increase blood flow through the contracted segment, exacerbating haemodynamic disturbances. Reference Allen, Driscoll, Shaddy and Feltes2,Reference Yokoyama, Ichikawa, Minamisawa and Ishikawa4,Reference Rosenthal7
Clinical presentation is highly variable. In critical coarctation of the aorta, newborns may develop severe heart failure following patent ductus arteriosus closure. In older children and adults, coarctation of the aorta is often detected incidentally during evaluation of a cardiac murmur or hypertension in adolescents and adults. Reference Allen, Driscoll, Shaddy and Feltes2,Reference Doshi and Chikkabyrappa8 Although diagnosis can be suspected in the absence or weakness of femoral pulses and may be confirmed by echocardiography, additional imaging tools such as MRI, CT angiography, and catheter angiography play crucial roles in accurate assessment and envisioning interventions. Reference Darabian, Zeb, Rezaeian, Razipour and Budoff9–Reference Karaosmanoglu, Khawaja, Onur and Kalra11
In the absence of treatment, coarctation carries a poor prognosis. Reference Torok, Campbell, Fleming and Hill12,Reference Feltes, Bacha and Beekman13 Timing of the treatment hinges on individual factors, lesion morphology, presence of associated cardiac lesions, age, and clinical presentation. Reference Feltes, Bacha and Beekman13,Reference Suradi and Hijazi14 We conducted this retrospective analysis to compare the outcome of surgical treatment and percutaneous interventions in the treatment of coarctation of the aorta and to identify the most suitable treatment approach.
Materials and methods
Patient selection and data collection
This study was conducted in Ankara University Faculty of Medicine, Department of Pediatric Cardiology. The study was approved by the Ethics Committee of Ankara University Faculty of Medicine. The medical records of all patients treated for coarctation of the aorta in our institution over a 12-year period were evaluated retrospectively. A total of 179 patients were diagnosed and treated for coarctation of the aorta in this period, but 59 patients with missing data were excluded from the study; as a result, 120 patients were enrolled in the study. The study population was stratified into two age groups: early infancy (0–4 months) and post-early infancy (>4 months). Patient demographics, including age, gender, age at diagnosis, presenting symptoms, and comorbidities, were reviewed. The choice of treatment, acute success, and recoarctation rate were evaluated. Treatment modalities and recoarctation rates were also compared according to the timing of intervention: at 0–4 months of age or ≥ 4 months.
Echocardiographic parameters of all patients were evaluated. All the echocardiographic examinations were performed in accordance with American College of Cardiology/American Heart Association guidelines by paediatric cardiologists highly experienced in CHD. Reference Cheitlin, Alpert and Armstrong15 The localisation of aortic coarctation was classified based on its proximity to the ductus as preductal, juxtaductal, or postductal. Reference Allen, Driscoll, Shaddy and Feltes2 Pre- and post-procedural pressure gradients were assessed using echocardiographic and, when available, angiographic measurements.
The treatment decision was determined by a multidisciplinary team and was done according to the 2011 American Heart Association paediatric guidelines for percutaneous intervention for CHD. Reference Feltes, Bacha and Beekman13 Briefly, surgical repair was the choice of treatment in children <4 months old due to a lower risk of reintervention, but in critically ill neonates unsuitable for surgical repair, balloon angioplasty was used as a palliative option. Surgical techniques included extended end-to-end anastomosis, subclavian flap aortoplasty, and patch aortoplasty that were selected according to the patient’s age, coarctation anatomy, and associated cardiac lesions. For infants with coarctation of the aorta who are ≥4 months old and for children whose weight is <25 kg, the choice of treatment was determined according to the underlying morphology, and balloon angioplasty was the preferred procedure. For patients with discrete coarctation of the aorta and with a weight of ≥25 kg (i.e., large enough to allow use of an adult-size stent), percutaneous stent implantation was the preferred treatment. While balloon angioplasty was performed using low-profile balloon catheters of appropriate size that are resistant to low pressures, balloon catheters resistant to high pressures were generally used during stent implantation. In case of recoarctation, percutaneous stent implantation was the choice of treatment in children with a weight ≥ 25 kg, and if the patient was not suitable for percutaneous stent implantation, balloon angioplasty was the preferred choice of treatment. Reference Feltes, Bacha and Beekman13
The acute success of the intervention was assessed based on the resolution of stenosis, achieving over 75% widening of the aortic isthmus, maintaining a post-intervention pressure gradient below 20 mmHg, ensuring normal left ventricular function, and confirming the absence of procedure-related aneurysm formation. Reference Feltes, Bacha and Beekman13,Reference Cheitlin, Alpert and Armstrong15 Early and late complications associated with these treatment approaches were also reviewed.
Statistical analysis
The SPSS version 21 (IBM) software package was used for statistical analysis. Frequency, percentage, minimum and maximum values, mean, median, and standard deviation were used for descriptive statistics. Pearson’s chi-square and McNemar tests were used for comparison of categorical variables. The normality of numerical variables was evaluated both visually (using histograms) and analytically (via the Shapiro–Wilk test). Paired t-tests were used for comparisons of normally distributed variables, and non-parametric tests, such as the Mann–Whitney U test and Kruskal–Wallis test, were used for variables that did not conform to a normal distribution. A p-value of <0.05 was considered statistically significant.
Results
A total of 120 patients with a diagnosis of coarctation of the aorta were reviewed; 62% (n = 74) were male, and 38% (n = 46) were female. The mean age at diagnosis was 23.7 ± 48.7 months, with a median age of 1.1 months (Table 1). The youngest patient was diagnosed on the first postnatal day, while the oldest patient was an adolescent pregnancy aged 17 years and 3 months. A total of 66.7% (n = 80) of the patients were diagnosed between 0 and 4 months, whereas 33.3% of the patients (n = 40) were diagnosed after 4 months of age. There was no statistically significant difference between the age at diagnosis and gender (p > 0.05).
Demographic characteristics of patients
The most common presenting complaints were cardiac murmur 62.5% (n = 75), respiratory distress 10% (n = 12), and an antenatal diagnosis 8.3% (n = 10). Additional presentations included failure to obtain femoral pulses (7.5%, n = 9), hypertension detected during routine follow-up (5.8%, n = 7), and diagnosis during the course of another disease (3.3%, n = 4). The distribution of presenting complaints according to age groups is shown in Figure 1.
Distribution of complaints by age groups at the time of diagnosis.
The most common cardiac abnormality was bicuspid aorta. In order of frequency, the aortic valve was tricuspid (49.2 %, n = 59), bicuspid (47.5 %, n = 57), monocuspid (2.5%, n = 3), and quadricuspid (0.8%, n = 1). The most common CHDs associated with coarctation of the aorta were ventricular septal defect (30%, n = 36), patent ductus arteriosus (14.1%, n = 17), and atrial septal defect (10%, n = 12).
The mean echocardiographic gradient at the coarctation site before intervention was 35 ± 16 mmHg (median: 30 mmHg; range: 10–80 mmHg). Of the patients, 73.3% (n = 88) had juxtaductal coarctation, 14.2 % (n = 17) had preductal coarctation, and 12.5 % (n = 15) had postductal coarctation. Preductal coarctation was significantly more frequent in patients diagnosed before 4 months of age (p < 0.01).
The most common primary intervention was balloon angioplasty (50.0%, n = 60), followed by surgical repair (45.0%, n = 54) and percutaneous stent implantation (5.0%, n = 6). When evaluated according to the age at the time of intervention, 57.5% (n = 69) of the patients underwent intervention during the 0–4-month period, while 42.5% (n = 51) received therapeutic intervention ≥4 months of age. Among patients aged 0–4 months, 50.7 % (n = 35) and 49.3 % (n = 34) underwent surgery and balloon angioplasty, respectively. In the ≥ 4-month age group, 37.2% (n = 19) underwent surgery, 51 % (n = 26) underwent balloon angioplasty, and 11.8 % (n = 6) underwent percutaneous stent implantation (Figure 2).
Characteristics of initial cardiac interventions in patients according to age groups.
The intervention (percutaneous and surgery) was successful in all patients except 2 patients, and the acute success rate, as defined before, was 98.3 % (n = 118). Both of these two patients died, and both were in the 0–4-month age group. One patient was referred for surgery due to failure of balloon angioplasty and subsequently died, while the other patient died due to sepsis in the early postoperative period. Intervention-associated complications were observed in 13.3% of patients, including arterial thrombosis (7.5%, n = 9), aortic aneurysm (2.5%, n = 3), mortality (1.6%, n = 2), haematoma (0.8%, n = 1), and paraplegia (0.8%, n = 1), while 86.7% of patients (n = 104) experienced no complications. Four additional patients were lost to follow-up after the initial intervention, all of whom had undergone surgery in the 0–4 months group.
Recoarctation occurred in 49 (43%) of 114 patients. The mean time to recoarctation was 13.7 ± 25.6 months (median: 3 months; range: 1–108 months). The time to recoarctation was shorter in the 0–4-month age group (7 ± 12 months) in comparison to patients ≥ 4 months old (42 ± 42 months), although this difference was not statistically significant (p = 0.178).
Recoarctation occurred in 29.6 % (n = 16/54) and 55% (n = 33/60) of patients who underwent surgery and balloon angioplasty, respectively, and recoarctation was significantly higher after balloon angioplasty (p = 0.004). When analysed according to the age of intervention, 52.2 % (n = 36/69) and 25.5 % (n = 13/51) of patients in the 0–4 months age and ≥ 4 months old groups had recoarctation, respectively, and recoarctation was significantly higher in the 0–4 months age group (p = 0.014). Although recoarctation rate did not differ between surgery and balloon angioplasty after ≥ 4 months of age, it was significantly higher after balloon angioplasty in comparison to surgery in the 0–4-month age group (p < 0.01) (Table 2). None of the patients who underwent transcatheter percutaneous stent implantation developed recoarctation.
Comparison of recoarctation rates according to age and initial intervention
* Chi-Square Test, f Fisher’s Exact Test, ϒ Transcatheter stenting cases were excluded due to low numbers. δ Percentages are given by recoarctation status.
Forty-one patients (36%) required secondary intervention for recoarctation, with surgery being the most common (53.6 %, n = 22/41) procedure, followed by balloon angioplasty (41.5%, n = 17/41) and percutaneous stent placement (4.9%, n = 2/41). Six patients had recoarctation after the second intervention, with a mean time of 10.3 ± .18.5 months (median: 3 months; range: 2–48 months). The third interventions for recoarctation were balloon angioplasty (50%, n = 3/6), percutaneous stent implantation (16.7%, n = 1/6), and surgery (33.3%, n = 2/6). Recoarctation developed in 2 patients after the third intervention, and one of the patients underwent surgery while the other one had percutaneous stent implantation. The procedures and outcome of patients according to age groups are presented in Figure 3.
Long-term follow-up outcomes according to age groups.
Discussion
Coarctation of the aorta is the stenosis of the proximal thoracic aorta and represents a common congenital heart defect in children. Although the precise aetiology of coarctation of the aorta remains incompletely understood, accumulating evidence suggests that its pathogenesis results from a complex interplay of genetic predisposition, environmental influences, and abnormal haemodynamic forces during fetal development. Reference Allen, Driscoll, Shaddy and Feltes2,Reference Carr, Curtis and Marek16 The clinical presentation of coarctation of the aorta varies widely, ranging from severe heart failure and cardiogenic shock in the neonatal period to incidentally detected asymptomatic hypertension in adolescents and young adults. The management of coarctation of the aorta encompasses both surgical and percutaneous intervention strategies, with the choice of treatment modality being determined by a combination of the patient’s haemodynamic status, anatomical considerations, and institutional expertise. Reference Torok, Campbell, Fleming and Hill12,Reference Park17 Despite advances in therapeutic interventions, the long-term prognosis of patients with coarctation of the aorta remains heterogeneous. Outcomes are influenced by factors including the timing of treatment and the selected intervention modality. Moreover, post-treatment complications, including recoarctation, persistent hypertension, and vascular dysfunction, are relatively common and necessitate lifelong surveillance and management. Reference Anderson, Baker, Penny, Redington, Rigby and Wernovsky3
In our single-centre study conducted over a 12-year period, the most notable finding was that recoarctation represented the most common post-intervention complication, occurring in 43% of patients, with a significantly higher incidence among those younger than four months. Balloon angioplasty was associated with a markedly higher recoarctation rate compared to surgical repair in this age group. Additionally, the median time to recoarctation was significantly shorter in younger infants, underscoring the challenges of early interventions. Our findings emphasise the importance of individualised treatment planning, careful intervention selection, and long-term follow-up to improve patient outcomes.
Among patients with CHD, coarctation of the aorta is observed in 6–8%, with an approximate incidence of 4 per 10,000 live births, and it is more common in males, with a male-to-female ratio of 1.27–1.74. Reference Allen, Driscoll, Shaddy and Feltes2,Reference Yokoyama, Ichikawa, Minamisawa and Ishikawa4,Reference Vigneswaran, Sinha, Valverde, Simpson and Charakida18 Coarctation of aorta has a widespread distribution in means of age at diagnosis, ranging from the neonatal period to adulthood, while some patients are diagnosed antenatally and some remain undiagnosed until adulthood. Clinical presentation and physical findings vary with age. Similar to previous reports, in our study, there was a male predominance with a ratio of 1.6/1. but the mean age at diagnosis (23.7 ± 48.7 months) was lower than previously reported, probably due to referral of patients with suspected coarctation of the aorta to our institution, as it is a tertiary health care centre. Reference Vigneswaran, Sinha, Valverde, Simpson and Charakida18 The most common presentation was a cardiac murmur, followed by respiratory distress and antenatal diagnosis among patients aged 0–4 months. For patients aged ≥4 months, hypertension was the most common presentation, as expected. Reference Allen, Driscoll, Shaddy and Feltes2 In our study, hypertension was identified in 44.1% of patients prior to intervention, a frequency lower than reported in the literature, probably attributable to the lower mean age at diagnosis. Reference Rajbanshi, Joshi and Pradhan19
Treatment recommendations by the American Heart Association lack high-level evidence (mostly Level B or C), resulting in variability among centres. Reference Torok, Campbell, Fleming and Hill12,Reference Feltes, Bacha and Beekman13,Reference Cheitlin, Alpert and Armstrong15 Surgery is generally the preferred choice of treatment for patients <1 year. Fiore et al. evaluated 57 neonates with neonatal aortic coarctation and reported surgery as the first intervention in 60% of patients <40 days old, while balloon angioplasty was performed in 40%. Reference Fiore, Fischer and Schwartz20 Similarly, He et al. evaluated the results of balloon angioplasty for the treatment of critical coarctation of the aorta in 37 infants aged 6–60 days, and all patients initially underwent successful angioplasty. During a 7-year follow-up, six patients developed recoarctation and required repeat balloon angioplasty, while two aortic aneurysms resolved spontaneously. None of the patients required subsequent surgical intervention for coarctation. Reference He, Liu, Wu, Qi, Zhang and Huang21 In a non-randomised study, Forbes et al. reported that among 350 patients—enrolled from 36 institutions—217 underwent stent implantation, 61 underwent balloon angioplasty, and 72 underwent surgical repair for native coarctation of the aorta. They concluded that patients treated with percutaneous stent implantation had significantly lower acute complications in comparison to surgery or balloon angioplasty patients. At short-term and intermediate follow-up, patients who underwent percutaneous stent implantation or surgery achieved a superior haemodynamic outcome than the balloon angioplasty patients; however, a planned reintervention was more likely after percutaneous stent implantation. Reference Forbes, Kim and Du22 In our cohort, initially balloon angioplasty was performed in 50%, surgical repair in 45%, and stent implantation in 5% of patients. Acute success was achieved in 97.4% of cases, comparable to the 100% success rate reported by He et al. and the 95% success rate by Rao et al. Reference He, Liu, Wu, Qi, Zhang and Huang21,Reference Rao and Chopra23
Recoarctation was the most common complication in our study, affecting 43% of patients. Its incidence was significantly higher in patients <4 months compared to older patients (p = 0.014). Among intervention types, balloon angioplasty was associated with significantly higher recoarctation rates compared to surgery in the younger age group (p = 0.004). The higher rate of recoarctation after balloon angioplasty in neonates s may be explained by the elastic recoil which can’t be prevented by means of balloon angioplasty in contrast to surgery and percutaneous stent implantation, in which the radial forces of the stent prevent elastic recoil. Reference Dikmen, Ozcinar and Eyileten24,Reference Brzezinska-Rajszys25 These findings align with the report of Sen et al., who reported a 63.6% recoarctation rate with balloon angioplasty compared to 17% with surgery, further supporting that while surgical coarctoplasty provides superior immediate and midterm outcomes, particularly in neonates, balloon angioplasty remains a viable alternative in older infants, offering acceptable reintervention rates and serving as a less invasive option for high-risk surgical candidates. Reference Sen, Garg, Rao and Kulkarni26 Hu et al. performed a meta-analysis of surgical versus balloon angioplasty treatment for native coarctation of the aorta and evaluated a total of 623 patients, of whom 378 and 245 were assigned to surgery and balloon angioplasty, respectively. Similarly to our study, they reported that balloon angioplasty reduces the risk of severe complications but has a higher risk of short-term recoarctation in comparison to surgery. Reference Hu, Wang and Dai27 As a result, it may be concluded that even though balloon angioplasty offers reduced procedural time and intensive care needs, it has a higher recoarctation rate, particularly in neonates and infants due to elastic recoil. Reference Dikmen, Ozcinar and Eyileten24,Reference Brzezinska-Rajszys25,Reference Hu, Wang and Dai27 The most common secondary intervention for recoarctation was surgery (53.6%), followed by balloon angioplasty (41.5%) and percutaneous stent implantation (4.9%). Recoarctation necessitating a third intervention occurred in 6 patients, and among these, stent implantation demonstrated the best outcomes with no subsequent recoarctation. Percutaneous stent implantation for coarctation is an effective and safe procedure, but it is limited by the patient’s weight. This underscores the need for advancements in stent technology. Reference Brzezinska-Rajszys25 In our study, the median time to recoarctation was significantly shorter in the younger age group (2.8 months vs. 24 months; p < 0.01), consistent with findings by Lee et al., who reported a higher incidence of restenosis median time of 1.5–4 months in infants under 3 months. Reference Lee, Lin, Hsieh, Lin and Huang28
Study limitations: This study is subject to the usual limitations of a retrospective study, and due to the wide range of years over which the patients were collected, there were data losses both in the digital records and in the written files of patients. Furthermore, 12 patients did not return for follow-up after their initial intervention, which further limited the completeness of outcome data. In addition, the number of patients that underwent percutaneous stent implantation was very few (n = 6), which limited the strength and generalisability of conclusions regarding this treatment modality. Additionally, the follow-up duration for recoarctation varied widely among patients (1–108 months) and some of the patients had other associated cardiac anomalies which may have influenced the comparison of outcomes.
In conclusion, coarctation of the aorta has high mortality and morbidity when left untreated. The choice of intervention varies according to the patient’s age, clinical presentation, and overall condition. Each patient should be evaluated individually, and management should involve a multidisciplinary team to ensure the most appropriate approach. Although surgical intervention is more invasive than balloon angioplasty, its lower rates of recoarctation make it a preferable option in the neonatal and early infancy period under current conditions. Further multicentre studies are warranted to establish more definitive conclusions.
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S1047951125110093.
Financial support
No specific funding was received from any bodies in the public, commercial, or not-for-profit sectors to carry out the work described in this article.
Competing interests
All authors declare that they have no conflict of interest.
Ethical standard
The present study was approved by the local ethics committee of the Ankara University Faculty of Medicine.
Consent to participate
All participants consented to participate in the survey study.
Data availability
The data that support the findings of this study are not openly available due to reasons of sensitivity and are available from the corresponding author upon reasonable request.
Open access
