Interrupted Aortic Arch with Multiple Vascular Malformations
Interrupted aortic arch (IAA) is a rare congenital cardiovascular malformation characterized by the complete discontinuity of the aortic lumen. It is typically diagnosed prenatally or during infancy, with a high mortality rate if not surgically corrected. However, this case report presents a unique instance of a 66-year-old male patient with type B IAA, who survived to adulthood without surgical intervention. This case highlights the role of collateral vessels in maintaining blood flow and the diagnostic utility of computed tomography angiography (CTA) in identifying complex vascular malformations.
The patient, a 66-year-old male with a 40-year history of heavy smoking, presented with sudden dizziness, nausea, and emesis. His medical history was otherwise unremarkable. CTA revealed a type B IAA, with complete discontinuity of the aortic lumen distal to the origin of the left common carotid artery (CCA). The ascending aorta (AA) and descending aorta (DA) exhibited different densities. Both CCAs and a dilated, tortuous left subclavian artery (SCA) originated from the distal AA and DA, respectively. The right SCA originated from the DA, posterior to the trachea and esophagus. Abundant systemic collaterals formed pathways among the cervical vessels, and a persistent left-sided superior vena cava (PLSVC) was observed.
A cerebral CTA was performed to investigate the dizziness, revealing that part of the dominant left vertebral artery was not in the intervertebral foramen but originated from a collateral vessel of the left CCA. Both internal carotid siphons were slender, and a fetal posterior cerebral artery was present. Based on these findings, the patient was diagnosed with type B IAA associated with PLSVC and an aberrant right subclavian artery (ARSA).
The patient’s condition improved after a week of medication. Although surgical interventions such as stent placement are effective for IAA, the patient opted for conservative management due to the risks associated with surgery in elderly adults.
IAA is a congenital cardiovascular malformation that occurs in approximately 3 per 1,000,000 live births annually and accounts for 1% of congenital heart disease cases. It is classified into three types based on the location of the interruption: type A, where the interruption is distal to the origin of the left SCA; type B, where the interruption is between the origin of the left CCA and SCA; and type C, where the interruption is between the origin of the brachiocephalic artery and left CCA. This case report focuses on type B IAA concurrent with PLSVC and ARSA.
The survival of this patient to adulthood is attributed to the abundant collateral vessels that provided sufficient blood flow to the distal aorta. These collaterals likely represent compensatory growth during the fetal stage, although further research is needed to understand the mechanisms underlying their formation.
The patient’s dizziness may be related to cardiovascular malformations. While central nervous system diseases are the primary cause of dizziness in the elderly, cardiovascular factors should be considered when episodes of dizziness are accompanied by abnormal cardiac rhythm, heart rate, blood pressure, or electrocardiogram changes. In this case, the patient may have experienced transient ischemia due to vertebrobasilar artery malformation and vascular aging.
The diagnosis of IAA relies on various imaging techniques. Echocardiography is economical and efficient, often used for prenatal diagnosis and to identify hypoplasia and associated cardiac abnormalities. Angiography, though invasive, is considered the gold standard for IAA diagnosis, providing clear visualization of the aortic arch interruption and collateral vessel branches. Magnetic resonance angiography (MRA) and CTA are reliable non-invasive examinations with multi-planar capabilities and three-dimensional imaging. MRA is superior due to the use of hypo-toxic contrast agents and non-ionizing radiation, while CTA is cheaper, less time-consuming, and offers higher spatial resolution with fewer artifacts.
In this case, CTA provided detailed visual information, enabling better identification of the anatomic relationships between IAA and associated malformations. This led to a deeper understanding and description of the patient’s condition.
In conclusion, this case report illustrates the survival of a 66-year-old male with type B IAA associated with PLSVC and ARSA, attributed to the presence of abundant collateral vessels. The patient’s dizziness may be linked to vertebrobasilar artery malformation and vascular aging. CTA played a crucial role in the diagnosis and evaluation of the patient’s cardiovascular malformations, highlighting the importance of combined diagnostic imaging in identifying IAA and guiding surgical approaches.
doi.org/10.1097/CM9.0000000000000438
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