Other Congenital Heart and Vascular Malformations

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Chapter 426 Other Congenital Heart and Vascular Malformations

426.1 Anomalies of the Aortic Arch

Vascular Rings

Congenital abnormalities of the aortic arch and its major branches result in the formation of vascular rings around the trachea and esophagus with varying degrees of compression (Table 426-1). The origin of these lesions can best be appreciated by reviewing the embryology of the aortic arch (Fig. 414-1). The most common anomalies include (1) double aortic arch (Fig. 426-1A), (2) right aortic arch with a left ligamentum arteriosum, (3) anomalous innominate artery arising farther to the left on the arch than usual, (4) anomalous left carotid artery arising farther to the right than usual and passing anterior to the trachea, and (5) anomalous left pulmonary artery (vascular sling). In the latter anomaly, the abnormal vessel arises from an elongated main pulmonary artery or from the right pulmonary artery. It courses between and compresses the trachea and the esophagus. Associated congenital heart disease may be present in 5-50% of patients, depending on the vascular anomaly.

Table 426-1 VASCULAR RINGS

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Diagnosis

Standard roentgenographic examination is not usually helpful, however, in the past, performing a barium esophagogram was the standard method of diagnosis (Fig. 426-2). Echocardiography in combination with either MRI or CT will usually define the lesion. Cardiac catheterization is reserved for cases with associated anomalies or in rare cases where these other modalities are not diagnostic. Bronchoscopy may be helpful in more severe cases to determine the extent of airway narrowing.

Treatment

Surgery is advised for symptomatic patients who have evidence of tracheal compression. The anterior vessel is usually divided in patients with a double aortic arch (see Fig. 426-1B). Compression produced by a right aortic arch and left ligamentum arteriosum is relieved by division of the latter. Anomalous innominate or carotid arteries cannot be divided; attaching the adventitia of these vessels to the sternum usually relieves the tracheal compression. An anomalous left pulmonary artery is corrected by division at its origin and re-anastomosis to the main pulmonary artery after it has been brought in front of the trachea. Severe tracheomalacia, if present, may require reconstruction of the trachea as well.