Significant effort has been put into the optimization of the antenatal diagnosis of coarctation of the aorta (CoA). However, although left-sided cardiac lesions are known to cluster, the necessity to intervene postnatally for other left-sided cardiac lesions has not been reported in a cohort of fetuses with suspected CoA. We report a study of all 89 fetuses with antenatally suspected and postnatally confirmed diagnosis of CoA who underwent CoA repair as the primary procedure at a single tertiary congenital heart disease center over 10 years (January 1, 2010, to December 31, 2019). Almost 1 in 5 patients (18%) had to undergo surgery and/or transcatheter intervention on additional left-sided cardiac lesions (14%) and/or reintervention on the aortic arch (12%) during follow-up to median age of 2.85 years. Freedom from intervention at 5 years was 78% (95% confidence interval [CI] 67 to 88%) if reintervention on CoA was excluded, and 72% (95% CI 60 to 82%) if this was included. Five-year survival was 95% (95% CI 90 to 100%). Furthermore, 20% of affected infants had genetic (10%) and/or extracardiac (16%) abnormalities. Our study highlights the need for comprehensive antenatal counseling, including the prognosis of primary repair of CoA and the potential development of additional left-sided cardiac lesions, which may be difficult to diagnose prenatally even in expert hands or impossible to diagnose because of the physiology of the fetal circulation.
Coarctation of the aorta (CoA) accounts for around 5% of congenital heart disease and is diagnosed in about 5 in every 10,000 live births. Antenatal diagnosis of CoA has been shown to reduce preoperative morbidity. However, difficulty in accurate antenatal diagnosis of CoA continues to pose antenatal counseling challenges to fetal cardiologists and parents. The short-term outcome of fetuses with suspected CoA has been previously reported , but this predominantly relates to surgical repair of CoA in the neonatal period and the association with hypertension and vascular dysfunction in adulthood. However, left-sided cardiac lesions have long been recognized to cluster and the frequency and necessity to intervene postnatally for other left-sided cardiac lesions have not been reported in a cohort of fetuses with suspected antenatal CoA. Such data would assist in prognostication for expectant parents in this setting. This study aims to report the medium-term outcome of fetuses with antenatally suspected and postnatally confirmed CoA, with emphasis on the requirement for intervention not only for CoA but also for associated left-sided cardiac lesions.
Methods
This was a retrospective single-center cohort study at the Evelina London Children’s Hospital (London, United Kingdom), a tertiary congenital heart disease center. Fetuses with suspected CoA who proved to have CoA after birth were included. All infants (up to 1 year of age) with coding for the repair of CoA procedure during the period January 1, 2010, to December 31, 2019, on a background of antenatally suspected CoA, were retrieved and included in the study. Patients who had associated ventricular septal defects (VSDs) were included, but those with abnormal atrioventricular or ventriculoarterial connections were excluded. Patients whose primary management was toward a functionally univentricular circulation, such as Norwood operation, were excluded. The typical approach to repair of CoA diagnosed during infancy at our institution is surgical, either by way of a lateral thoracotomy or more extensive repair of the aortic arch on bypass by way of a median sternotomy.
We retrieved this study group from our submissions to the mandatory United Kingdom National Congenital Heart Disease Audit database, which is externally audited for accuracy. Data were further collected from our electronic patient record system, fetal cardiology electronic database (Filemaker Pro, Claris Corp, California.), and digital imaging archive (available since 2013) (Philips ISCV V2.3, Philips Zoetermeer, Netherlands). The presence of antenatal or postnatal cardiac, extracardiac and genetic abnormalities was recorded. For this study, the term ‘additional left-sided cardiac lesions’ included supramitral membrane, parachute mitral valve (MV), MV stenosis, subaortic stenosis, bicuspid aortic valve (BAV), and supravalvar aortic stenosis. The study was registered and approved as per our Institutional Policy (registration number: 10,878).
Statistical analysis was performed using IBM SPSS Statistics for Windows Version 25.0 (IBM Corp., Armonk, New York, 2017). Survival analysis was performed by Kaplan-Meier estimate.
Results
Between January 1, 2010, and December 31, 2019, the initial search identified 119 patients ( Figure 1 ). A total of 25 patients were excluded because of additional cardiac lesions, including atrio VSD (n = 7), hypoplastic left-sided cardiac complex (n = 6), double outlet right ventricle (n = 5), ventriculoarterial discordance (n = 4), partial anomalous pulmonary venous drainage (n = 2) and interrupted inferior vena cava (n = 1). A total of 5 patients were excluded because of severe aortic valve stenosis, which was the dominant prenatal consideration. This left a total of 89 infants, 48 male (54%), who met the inclusion criteria and underwent surgery for CoA following an antenatal suspicion of CoA. All 89 patients had electronic records including echocardiography and surgical reports, but our digital echocardiographic archive has only been in place since 2013, so antenatal echocardiographic images were available for 75 of the 89 patients.
A VSD was detected in 20 of 75 (27%) fetuses. MV abnormalities were suspected prenatally in 6 of 75 (8%) cases; in 3 cases suspicion of a dominant or single papillary muscle was described and in another 3 cases restricted MV opening or dysplasia was noted. In 13 of 75 patients (17%), there were bilateral superior vena cavas with left superior vena cava drainage to the coronary sinus. Antenatal diagnosis of a BAV had been confidently made in 3 patients, with a strong suspicion of BAV in 8 more fetuses (postnatally confirmed in 7 of 8). There was 1 case of antenatally suspected and postnatally confirmed mild aortic valve stenosis. Extracardiac abnormalities were present in 13 of 75 (17%) cases. Invasive antenatal genetic testing was performed in 26 of the total 89 pregnancies and was abnormal in 3 of 26 (12% of those tested).
The median gestational age at birth was 38.4 weeks (interquartile range [IQR] 37.6 to 39.0) and the median weight was 3,000 g (IQR 2,648 to 3,411). The median age at the initial operation for CoA was 7 days (IQR 4 to 13) with all but 2 patients operated within the first 3 months. Median postoperative follow-up was 2.76 years (IQR 1.38 to 6.49) and the median age at follow-up was 2.85 years (IQR 1.40 to 6.55). A total of 4 patients (4.6%) died during the follow-up period, with a Kaplan-Meier 5-year survival of 95% (95% confidence interval [CI] 90.1% to 99.9%) ( Figure 2 ). Early postoperative mortality (≤ 30 days after the initial operation) was limited to 1 patient who suffered from seizures and hypoxic-ischemic injury after cardiopulmonary bypass. A total of 3 other deaths were related to a lower respiratory tract infection, MV stenosis and pulmonary hypertension (n = 1), respiratory syncytial virus bronchiolitis (n = 1), and aspiration (n = 1). The initial approach to coarctation repair was by median sternotomy in 44 cases (49%) and by left thoracotomy in 45 cases (51%) ( Figure 3 ). A pulmonary artery band was placed within the first 2 months after birth in 2 cases, to restrict pulmonary blood flow because of concomitant interventricular communication; VSD closure was performed at a second stage in 1 of the cases, whereas the VSD became of negligible size in the second case.
Additional left-sided cardiac lesions were present in 64 of 89 patients (72%) ( Table 1 ). Although in most cases (50 patients), 1 such lesion was present, there were 5 patients with 2 lesions and 9 patients with 3 lesions. Interventions for left-sided cardiac lesions were surgical in 11 and transcatheter in 1 patient. Survival analysis with Kaplan-Meier estimate showed 5-year survival free of intervention for additional left-sided cardiac lesions of 77.8% (95% CI 67.2 to 88.4%) ( Figure 4 ).
| Cardiac Finding | Postnatal Detection or Confirmation | Surgery or Transcatheter Intervention | Age at Diagnosis | Age at First Intervention |
|---|---|---|---|---|
| Total patients with additional left-sided cardiac lesions, including bicuspid aortic valve | 64 (72%) | 12 (14%) | ||
| Total patients with additional left-sided cardiac lesions other than bicuspid aortic valve | 17 (19%) | 10 (11%) | ||
| Additional left-sided cardiac lesions | ||||
| Supramitral membrane | 4 (5%) | 4 (5%) | 0.38 (0.07 to 0.67) | 0.81 (0.71 to 1.18) |
| Total patients operated for mitral valve stenosis | 6 (7%) | 0.98 (0.4 to 2.36) | ||
| Parachute mitral valve | 9 (10%) | 2 (2%) | ||
| Surgery for mitral stenosis without a parachute mitral valve | 4 (5%) | |||
| Mitral valve replacement | 2 (2%) | |||
| Subaortic membrane | 9 (10%) | 6 (7%) | 1.17 (0.07 to 2.44) | 2.22 (0.71 to 8.73) |
| Bicuspid aortic valve | 61 (69%) | 4 (5%) | 2.46 (0.53 to 4.96) | |
| Bicuspid aortic valve without other additional left-sided cardiac lesions | 47 (53%) | 2 (2%) | ||
| Supravalvar aortic stenosis | 1 (1%) | 1 (1%) | 2.8 | 3.4 |
| Total patients with surgical/transcatheter reintervention on the aortic arch | 11 (12%) | 0.32 (0.02 to 0.90) | ||
| Surgical | 7 (8%) | |||
| Transcatheter | 4 (5%) | |||
| Total patients with surgical/transcatheter intervention on additional left-sided cardiac lesions and/or reintervention on the aortic arch | 16 (18%) | |||
| Ventricular septal defect(s) | 30 (34%) | 12 (14%) |
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