Coarctation of the aorta (CofA) has been associated with an increased risk of intracranial aneurysm (IA). This magnetic resonance angiography (MRA) study investigates the prevalence of IAs in 80 children treated in early life for CofA. MRA was performed at mean age of 15.7 ± 7.1 years, and surgical or endovascular treatment for CofA occurred at a mean age of 2.6 ± 4.4 years. No IA was found. In contrast with earlier findings in adult patients with late treatment for CofA, this first systematic study of very early treated patients for CofA failed to confirm the association between CofA and IAs. Our results call the abnormal developmental relation between CofA and IAs into question and suggest that modifiable risk factors like hypertension may be responsible for IA development in patients with CofA with adult diagnosis and treatment. In conclusion, our data suggest that early treatment of CofA can reduce the formation of IAs in children so as to make MRA screening less valuable in this young population.
To evaluate the prevalence of intracranial aneurysms (IAs) in early repaired coarctation of the aorta (CofA) patients and to test the hypothesis that CofA and IAs may share a common neurodevelopmental alteration, we evaluated by magnetic resonance angiography (MRA) the intracranial arteries in a large cohort of young patients with CofA. Opposite to previous studies, all patients had early diagnosis and treatment of CofA, thus minimizing the role of potentially associated modifiable risk factors for the development of IAs.
Methods
Patients were enrolled on the basis of a diagnosis of CofA treated by surgery or balloon angioplasty by the age of 18 years. Exclusion criteria were the presence of acquired or congenital factors potentially involved in the pathogenesis of IAs, such as head trauma, cerebral infections, smoking or alcohol abuse, positive family history for IA, and connective tissue disease.
After approval from the Policlinico S. Orsola-Malpighi Hospital Ethics Committee, 80 consecutive patients (65% were men; mean age at MRA screening was 15.7 ± 7.1 years) were recruited from 2004 to 2011. Baseline demographic, clinical, surgical, and interventional data were systematically collected for each patient ( Table 1 ). Hypertension was defined according to the recent European Society of Hypertension and Cardiology guidelines: systolic blood pressure (BP) >140 mm Hg and/or diastolic BP >90 mm Hg or treatment with antihypertensive medications for adults and systolic BP and/or diastolic BP persistently more than the ninety-fifth percentile for children. MRA was performed in all patients using a 1.5 T superconducting system (Signa, GE Medical Systems, Milwaukee, Wisconsin) with a 16-channel HNS FP by GE (posterior-head face) head phased array coil. All patients underwent MRA using a time-of-flight spoiled gradient recalled sequence (TR 20 ms, TE 4.7 ms, 20° flip angle, 384 × 256 matrix; 22.7 field of view, 1 number of excitations, 1.4-mm effective thickness of sections acquired with 3 slabs oversampled by 10 slices) with postprocessing volume rendering and maximum intensity projection reconstructions. The study was completed with a standard magnetic resonance imaging of the head performed with sagittal fluid-attenuated inversion recovery T1, coronal T2 fast spin echo, and axial fluid-attenuated inversion recovery T2 sequences.
| Variable | Present study | Connolly et al. | Cook et al. | Curtis et al. |
|---|---|---|---|---|
| Type of study | Prospective | Prospective | Prospective | Retrospective |
| Patients | 80 | 100 | 43 | 117 |
| Male | 52 (65%) | 70 (70%) | 18 (42%) | 71 (61%) |
| Age (years) at CofA diagnosis [Mean ± SD] | 2.3 ± 4.2 | 17.3 ± 16.3 [97 patients] | – | – |
| Age (years) at MRA/CTA [Mean ± SD] | 15.7 ± 7.1 | 41.6 ± 16.5 | 33.5 ± 10.2 | 29 ± 11 |
| IAs on neuroimaging | 0 | 10 (10%) | 5 (11%) | 12 (10.3%) |
| BAV | 38 (47.5%) | 75 (77%) [98 patients] | 32 (74%) | 47 (40%) |
| Previous CofA intervention | 80 (100%) | 86 (86%) | – | 107 (91%) |
| Age (years) at first CofA intervention [Mean ± SD] | 2.6 ± 4.4 | – | 9.8 ± 11.3 | – |
| Treated for reCofA | 13 (16%) | 19 (19%) | 13 (30%) | – |
| Personal history of IA | 0 | 1 (1%) [98 patients] | – | – |
| Family history of IA | 0 | 3 (5%) [64 patients] | – | – |
| Hypertension | 6 (7.5%) | 63 (63%) | – | 53 (45%) |
| Smokers | 0 | 41 (43%) [96 patients] | 4 (9%) | 19 (17%) [110 patients] |
| Neurological symptoms | 9 (11.2%) | 35 (36%) [98 patients] | – | – |
Images were evaluated in blind by 2 neuroradiologists (LS and LF both with >5 years of experience) and by an interventional neuroradiologist (MPT with >20 years of experience in endovascular treatment of IAs). When an evaluation was discordant, a consensus judgment was reached.
Results
Demographic and clinical patients’ data, compared with previous relevant studies, are reported in Table 1 . Of the 80 patients (mean age at CofA diagnosis 2.3 ± 4.2 years), 22 had isolated CofA, whereas the remaining patients had several associated congenital anomalies ( Table 2 ). Moreover 5 patients had hypoplasia of the aortic arch: 4 with Shone’s complex and 1 with an associated ventricular septal defect. Overall, 38 patients (47.5%) had bicuspid aortic valve (BAV): 28 CofA + BAV alone and 10 with other associated defects. Four patients had syndromic diseases: 3 Turner’s syndrome and 1 DiGeorge’s syndrome.
| Type of associated cardiac defects | No. |
|---|---|
| Bicuspid aortic valve | 28 (35%) |
| Ventricular septal defect | 14 (17%) |
| Bicuspid aortic valve + ventricular septal defect | 5 (6%) |
| Shone complex ∗ | 4 (5%) |
| Ventricular septal defect + atrial septal defect | 2 (2%) |
| Ventricular septal defect + aortic stenosis | 1 (1%) |
| Bicuspid aortic valve + partial anomalous pulmonary venous return | 1 |
| Persistent left superior vena cava | 1 |
| Atrial septal defect | 1 |
| Pulmonary stenosis | 1 |
| No associated cardiac defects | 22 (27%) |
∗ Coarctation of the aorta + bicuspid aortic valve + mitral stenosis.
Mean age at CofA treatment was 2.6 ± 4.4 years. Sixty patients were treated by surgical resection and end-to-end anastomosis, 5 by reverse subclavian flap angioplasty, 13 with balloon angioplasty, and 2 with balloon angioplasty and stent implantation. Thirteen underwent a second treatment for reCofA (8 with balloon angioplasty and 5 with balloon angioplasty plus stent implantation).
Of the whole population, 11 patients (13.7%) required antihypertensive therapy: 8 treated with end-to-end anastomosis and no residual CofA, 2 with residual CofA after end-to-end anastomosis subsequently treated with balloon angioplasty plus stent implantation, and 1 with residual CofA after balloon angioplasty at 14 years. Of these patients, 6 (7.5%; 3 adults and 3 children) were still hypertensive despite medical therapy: 2 children had systolic BP more than the ninety-ninth percentile and 1 had systolic BP more than the ninety-fifth and less than the ninety-ninth percentile, whereas 2 adults had grade 1 hypertension and 1 had grade 2 hypertension. Nine patients complained of neurologic symptoms: occasional headache in 8 and episodes of seizures in 1. MRA screening (mean age at neuroimaging 15.7 ± 7.1 years) showed no evidence of IA in any of our patients. One patient showed left common carotid and subclavian artery hypoplasia and left extracranial internal carotid artery agenesis.
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