Tetralogy of Fallot with vertebral defect and left aberrant subclavian artery: a rare occurrence

This case exemplifies the intricate nature of TOF, which can manifest in diverse ways, posing a diagnostic challenge. The co-occurrence of several anomalies, as highlighted in this case, underscores the importance of comprehensive imaging techniques, such as echocardiography and chest CT, to provide a holistic understanding of the condition.

Furthermore, the absence of a family history of congenital heart disease serves as a reminder that these conditions can occur in isolation without prior familial predisposition. The case also highlights the need for a high index of suspicion in cases with unusual or atypical symptoms, especially in infants and neonates.

The right aortic arch is an infrequent congenital anomaly, with an estimated occurrence rate of roughly 0.1%. This condition is characterized by the positioning of the aortic arch on the right side of the trachea. This developmental aberration manifests during the period of embryogenesis spanning from weeks 4 to 5. It is characterized by the migration and formation of the six pairs of aortic arch arteries, which connect the ventral aorta to both dorsal aortae, resulting in the development of their respective structures [4].

The prevalence of an anomalous left subclavian artery in persons with a right aortic arch is estimated to be around 0.04–0.1% based on observations from postmortem specimens. Radiological examinations have yielded similar findings, with a reported incidence of approximately 0.05% [5]. Moreover, there have been a limited number of reports documenting its correlation with an uncommon anomaly known as the congenital absence of the left pulmonary artery [6]. Individuals who possess a right aortic arch with an anomalous left subclavian artery frequently exhibit no symptoms. Nevertheless, it is typically the symptoms related to the compression of neighboring structures, such as the esophagus and trachea, that provide indications of concomitant vascular anomalies in conjunction with tetralogy of Fallot (TOF) [7].

The utilization of chest radiography is advantageous in the evaluation of the right aortic arch, while barium enema can be employed for the identification of esophageal compression. The identification of an anomalous left subclavian artery is confirmed through the utilization of medical imaging techniques such as CT scan, MRI, or 2D echocardiography with color Doppler [8, 9]. Patients presenting with a right aortic arch and an abnormal subclavian artery necessitate surgical surgery.

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