Abstract
Background and Introduction
Interrupted aortic arch (IAA) is a very rare congenital anomaly carrying high neonatal mortality rate if left untreated. Rarer still, is its presentation in teenage or adulthood. This condition has been found to be complicated with cerebral aneurysms, which is a consequence of hemodynamic stress and hypertension secondary to arch interruption. Cerebral aneurysms can further complicate the clinical course and lead to poor clinical outcomes, especially if ruptured.
Clinical Presentation
A 17-year-old female presented with ruptured basilar top aneurysm and was considered for endovascular coiling. Transfemoral access was chosen but the catheter could not be negotiated beyond proximal thoracic aorta. A computed tomographic angiography (CTA) of thorax and abdomen was performed, which showed isolated interruption of aortic arch. Subsequently, transradial route was used for coiling of the aneurysm.
Conclusion
To the best of our knowledge, the index case is one of the only seven cases of IAA with cerebral aneurysm that have been reported till date in medical literature. It also holds the unique distinction of being the first case of IAA with cerebral aneurysm treated by endovascular approach. Our case highlights the importance of transradial access in such pathological conditions.
Keywords: Cerebral aneurysm, endovascular coiling, interrupted aortic arch, subarachnoid hemorrhage, transradial
Background and Introduction
Interrupted aortic arch (IAA) is the complete absence of anatomical continuity of aortic arch. Steidele gave the first description of this entity in 1778 and an association with DiGeorge syndrome has also been described.1,2 It is very rare accounting for <1% of all congenital cardiac anomalies. 3 In >97% cases, this entity is associated with other cardiac anomalies, isolated IAA being a rare proposition.2,3 This condition is usually diagnosed in prenatal period or infancy and carries a very high mortality rate with median age of death being 4–10 days in untreated patients.1,4 Extremely selective reports have described its presentation in adulthood with secondary hypertension or cerebral aneurysm rupture.1,2,4 We report a case of ruptured intracranial basilar top aneurysm associated with isolated IAA, which was managed with transradial coiling. A written informed consent from the patient’s attendant was obtained for reporting it.
Clinical Presentation
A 17-year-old female with no prior known medical condition presented with history of sudden onset severe headache and neck pain. On examination, she was conscious but confused with Glasgow Coma Scale (GCS) of 13/15 (E3V4M6). Bilateral lower limb and left upper limb weakness was present. Blood pressure (BP) in left upper limb was 145/90 mm Hg. Non-contrast computed tomography (CT) of head revealed Fisher grade IV subarachnoid hemorrhage. Basilar top aneurysm was demonstrated on a subsequent cerebral CT angiography (CTA) and she was advised endovascular coiling. Through the right transfemoral access, the catheter was navigated to the thoracic aorta with the intent of performing a prior diagnostic vertebral angiogram. However, despite repeated attempts, the catheter could not be passed beyond proximal thoracic aorta. Careful on-table evaluation suggested bilateral rib notching on fluoroscopy (Figure 1(a)) alongwith relatively feeble femoral pulse, which had warranted an ultrasound-guided puncture in the first place. Contrast injection in thoracic aorta showed its blind ending proximal end (Figure 1(b)). CTA of the chest and abdomen was then performed which revealed interruption of the aortic arch just distal to left subclavian artery origin with reformation of distal aorta by collateral circulation from bilateral posterior intercostal, periscapular and internal mammary arteries (Figure 2(a) and (b)). Echocardiography did not show any other associated cardiac anomaly except for mild left ventricular wall hypertrophy. Patient was again taken for endovascular coiling through the transradial route. After injecting the radial cocktail, right vertebral artery (VA) angiogram was obtained which showed severe vasospasm in the posterior circulation (Figure 3(a)). Chemodilatation with 3 mg nimodipine infusion was employed followed by positioning of a 6F-guiding catheter (Neuron, Penumbra Inc.) in V3 segment of right VA. Unassisted coiling was subsequently performed resulting in complete occlusion of the aneurysm (Figure 3(b)). Subsequent bilateral common carotid artery (CCA) angiograms indicated diffuse severe vasospasm in anterior circulation (Figure 3(c) and (d)), which was partially reversed with intra-arterial nimodipine instillation. She was extubated and retained the preoperative GCS (13/15). The following day, the patient started gasping and required emergent intubation and ventilation. Her GCS continued to deteriorate and she reached E1V1M1 status after a few hours. MRI showed multiple patchy bilateral cerebral and brainstem infarcts. There was progressive hemodynamic deterioration, which prompted the attendants to withdraw life-support. She ultimately succumbed to severe vasospasm.
Figure 1.
(a) Chest X-ray showing absence of aortic knuckle with bilateral rib notching (arrows). (b) Angiographic run of descending thoracic aorta showing blind ending proximal end of thoracic aorta.
Figure 2.
(a) Interruption of aortic arch distal to left subclavian artery origin (arrow) with markedly dilated and tortuous intercostal arteries (arrowhead) due to collateral circulation and (b) markedly dilated internal mammary artery (curved arrow) due to collateralization to descending aorta.
Figure 3.
(a) Right VA angiography showing marked vasospasm involving distal BA and bilateral superior cerebellar and posterior cerebral arteries with basilar top aneurysm. (Inset: right brachial angiogram demonstrating the radial access) (b) Post coiling right VA angiography showing complete occlusion of aneurysm. Right (c) and left (d) CCA runs showing severe vasospasm involving bilateral distal ICA (arrows), middle and anterior cerebral arteries. Note: Fetal PCA on left side.
Discussion
IAA was classified into 3 types by Celoria and Patton in 1959 on the basis of site of interruption. The interruption in type A is located distal to left subclavian artery, and it is between left common carotid and left subclavian arteries in type B and between brachiocephalic and left common carotid arteries in type C. 3 Complete absence of luminal and anatomic continuity between adjacent aortic segments is the key feature of IAA, in contrast to coarctation, where there is stenosis at the insertion of ductus arteriosus. Anatomic resemblance of coarctation with type A IAA makes these two entities a part of a continuum with isolated IAA being an extreme form of coarctation. 5 In isolated IAA (without associated cardiac anomalies), patient survival is longer because of effective collateralization, similar to coarctation of aorta. 4
All the reported adult cases in literature belong to type A as in our case.2,6 To the best of our knowledge, only seven cases of ruptured cerebral aneurysms with IAA are published in English literature till date, ours being the eighth one in the series (Table 1).1,2,4,7–10 All these aneurysms, including our case, were saccular, and subarachnoid hemorrhage (SAH) was the presenting feature in all, except the one reported by Borcek et al., in which sentinel headache was present but CT head did not show SAH. Six out of these total eight cases of cerebral aneurysm with IAA had isolated IAA; one patient had associated cardiac anomalies while no mention of the same was made in one case. Type A IAA was present in 75% (6/8) cases while two cases had type B IAA. Twenty-five percent (2/8) of these cases had multiple (>1) cerebral aneurysms while all others had a single aneurysm. In terms of age, 12.5% (1/8) was a neonate while the rest [87.5% (7/8)] were adolescents or adults at the time of presentation with male preponderance (six males and two females). 11 Another case of SAH with isolated IAA has been described by Zhang et al., which however did not reveal any source of hemorrhage on magnetic resonance angiography (MRA). 12 All the previously reported cases of IAA with aneurysm were treated by clipping, except for the one reported by Ling et al., in which no treatment for cerebral aneurysm was done. The index case is the first such case treated by endovascular coiling.
Table 1.
Summary of all described cases of IAA with cerebral aneurysm (IAA = interrupted aortic arch, VA = vertebral artery, NA = not available, AComA = anterior communicating artery, DACA = distal ACA, MCA = middle cerebral artery, PCA = posterior cerebral artery, VSD = ventricular septal defect).
| S. No | Year | Authors | Age/sex | Associated cardiac anomalies | Type of IAA | Number of aneurysm/s | Site of aneurysm/s | Type of aneurysm | Treatment modality for cerebral aneurysm |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 1994 | Ling et al. 7 | 14 yr/M | Isolated IAA | Type B | 1 | Rt VA (ruptured) | Saccular | Not treated |
| 2 | 1996 | Hu et al. 8 | 26 yr/M | NA | Type A | 1 | AcomA (ruptured) | Saccular | Surgical clipping |
| 3 | 2000 | Baysal et al. 4 | 16 yr/M | Isolated IAA | Type A | 1 | AcomA (ruptured) | Saccular | Surgical clipping |
| 4 | 2002 | Varghese et al. 9 | 26 yr/M | Isolated IAA | Type A | 1 | AcomA (ruptured) | Saccular | Surgical clipping |
| 5 | 2013 | Borcek et al. 1 | 17 yr/M | Isolated IAA | Type A | 2 | DACA, Lt MCA (?ruptured) | Saccular | Surgical clipping |
| 6 | 2014 | Eren et al. 2 | 20 yr/M | Isolated IAA | Type A | 3 | ACA, Lt PCA origin, Lt M1 MCA (ruptured MCA) | Saccular | Surgical clipping |
| 7 | 2017 | Hidalgo et al. 10 | 26 days/F | VSD with bi directional shunt and hypoplastic right ventricle | Type B | 1 | Lt MCA (ruptured) | Saccular | Surgical clipping |
| 8 | 2020 | Present case | 17 yr/F | Isolated IAA | Type A | 1 | Basilar top (ruptured) | Saccular | Endovascular (transradial) coiling |
The approach to treatment of cerebral aneurysm needs to be customized in these cases. In the era of endovascular expertise, surgical clipping in posterior circulation aneurysms has fallen into disrepute due to high morbidity and mortality rates. It becomes imperative that in suspected cases of difficult vascular access in a patient in whom an endovascular approach is being considered, a complete cervicocranial CT angiography should be performed. This would help one in mapping the aortic arch and assist in planning of the procedure through an alternate route. Also, transradial route has proved to be effective for obtaining access to such sites. We could achieve technical success of securing the aneurysm in the present case, but however, hit a roadblock with extreme vasospasm resulting in eventual patient demise.
Conclusion
IAA is a very rare anomaly, bearing resemblance to coarctation of aorta, but usually associated with severe congenital cardiac anomalies, which is fatal in early age if not timely treated. Isolated IAA with cerebral aneurysm (especially in adolescents and adults) is an extremely uncommon phenomenon, associated with high risk of rupture and complicated clinical course. A personalized treatment approach for cerebral aneurysms is required in these cases with the radial artery serving as an excellent access route.
Footnotes
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
Informed consent: Consent sought from patient attendant.
Consent for publication: All the authors have given consent for possible publication of the manuscript.
Ethics approval: The study has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
ORCID iD
Chirag K Ahuja https://orcid.org/0000-0003-0734-3252
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