Vascular Lesions, Congenital

• • 2 main processes: AV malformations and vascular rings

AV Malformations

• • Uncommon, abnormal capillary formation during canalicular phase of development, mostly from pulmonary artery, rarely coronary artery

  • Coronary AV fistulas drain into RV (40%) and RA (25%) of time

Vascular Rings

• • Abnormal development of aortic arches and branches with compression of trachea and esophagus

  • Normal fetal development: A dual system of 6 aortic arches regresses so that the left fourth arch persists as main left aorta; the left sixth arch remains as the ductus arteriosus; and the right fourth arch becomes the right innominate and subclavian arteries

  • Most associated with right-sided aortic arch

  • Classified as complete or incomplete (arterial sling)

  • Common complete rings: Double aortic arch (67%), right aortic arch with left subclavian and ductus arteriosus (30%)

  • Incomplete rings: Aberrant right subclavian originating from left side, and left pulmonary artery arising from right pulmonary artery (pulmonary artery sling)

Symptoms and Signs

AV Malformations

• • Asymptomatic, otherwise dyspnea, congestive heart failure, angina

  • Continuous murmur and signs of rapid aortic runoff on physical exam

Vascular Rings

• • Symptoms of tracheal or esophageal compression

  • Dysphagia lusoria: Swallowing symptoms from anomalous right subclavian

  • Complete ring and pulmonary artery slings: Present within 6 mos with respiratory distress, poor feeding

Imaging Findings

AV Malformation

• • Echocardiogram with Doppler often diagnostic

Vascular Rings

• • Barium esophagogram may be diagnostic

  • -Bilateral indentations imply double aortic arch

    -Posterior indentation implies aberrant right subclavian artery

    -Right indentation implies right aortic arch

    -Anterior impression: Pulmonary artery sling

  • Echocardiography can confirm diagnosis

  • CT and aortography may be required for diagnosis

• • Shunt fraction calculation via angiography is definitive for diagnosis

• • Physical exam

  • Echocardiography

  • Barium esophagogram for vascular rings

  • Occasionally angiography

• • Once diagnosed, symptomatic vascular rings should be repaired

  • Vascular rings: If double aortic arch, divide smaller of 2 arches

  • Complete rings: Divide ligamentum arteriosum

  • Pulmonary artery slings: Reimplantation of left pulmonary artery

Surgery

Indications

• • AV malformations: Symptomatic patients or if large shunts

  • Vascular rings: Should be repaired via left thoracotomy

References