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  • • 2 main processes: AV malformations and vascular rings

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AV Malformations

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  • • 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

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Vascular Rings

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  • • 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)

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Symptoms and Signs

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AV Malformations

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

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

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Vascular Rings

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  • • 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

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Imaging Findings

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AV Malformation

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  • • Echocardiogram with Doppler often diagnostic

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Vascular Rings

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  • • 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

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  • • Shunt fraction calculation via angiography is definitive for diagnosis

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  • • Physical exam

    • Echocardiography

    • Barium esophagogram for vascular rings

    • Occasionally angiography

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  • • 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

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Surgery

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Indications

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  • • AV malformations: Symptomatic patients or if large shunts

    • Vascular rings: Should be repaired via left thoracotomy

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References

Jacobowitz GR et al: Transcatheter embolization of complex pelvic vascular malformations: results and long-term follow-up. J Vasc Surg 2001;33:51.  [PubMed: 11137923]
Mattle HP et al: Dilemmas in the management of patients with arteriovenous malformations. J Neurol 2000;247:917.  [PubMed: 11200683]
Mulliken JB et al: Vascular anomalies. Curr Probl Surg 2000;37:517.  [PubMed: 10955029]
White RI Jr et al: Long-term outcome of embolotherapy and surgery for high-flow extremity arteriovenous malformations. ...

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