Anomalous origin of a pulmonary artery from the ascending aorta: Associated anomalies and pathogenesis

https://doi.org/10.1016/0002-9149(88)91078-8Get rights and content

Abstract

The pathologic anatomic features and associated cardiovascular anomalies of 9 patients with anomalous origin of 1 pulmonary artery (PA) from the ascending aorta seen at the University of Florida and 99 previously reported cases were analyzed. Anomalous origin of the right PA was much more common than anomalous origin of the left PA. The anomalous right PA usually arose from the posterior aspect of the ascending aorta close to the aortic valve. Less commonly, it originated from the lateral ascending aorta just proximal to the innominate artery. Patent ductus arteriosus and aorticopulmonary septal defect were commonly associated with anomalous origin of the right PA; other cardiovascular anomalies were rare. In contrast, tetralogy of Fallot and aortic arch anomalies, e.g., right aortic arch and anomalous origin of the subclavian artery, were common in anomalous origin of the left PA. An association with DiGeorge syndrome, frequently noted with persistent truncus arteriosus, was not seen with anomalous origin of a PA from the ascending aorta. The observations indicate that anomalous origin of the right PA and anomalous origin of the left PA are pathogenetically unrelated to each other and both are unrelated to persistent truncus arteriosus. Moreover, anomalous origin of the right PA arising close to the innominate artery is thought to be pathogenetically distinct from the type that arises close to the aortic valve. These pathogenetic relations should be considered in epidemiologic studies of congenital heart disease.

References (70)

  • RC Bahler et al.

    Absent right pulmonary artery. Problems in diagnosis and management

    Am J Med

    (1969)
  • DL Bricker et al.

    Anomalous aortic origin of the right and left pulmonary arteries in a normally septated truncus arteriosus

    Chest

    (1975)
  • AL Calder et al.

    Variant of tetralogy of Fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

    Am J Cardiol

    (1980)
  • GR Cumming et al.

    Aortic origin of the right pulmonary artery

    Am J Cardiol

    (1972)
  • PO Daily et al.

    Correction of absence of the aortopulmonary septum by creation of concentric great vessels

    Ann Thorac Surg

    (1975)
  • WJ Duncan et al.

    Two dimensional echocardiographic identification of hemitruncus: anomalous origin of one pulmonary artery from ascending aorta

    Am Heart J

    (1981)
  • JW DuShane et al.
  • JB Flege et al.

    Aortic origin of the right pulmonary artery and ventricular septal defect. A case report

    J Thorac Cardiovasc Surg

    (1970)
  • WH Herbert et al.

    Anomalous origin of left pulmonary artery from ascending aorta, right aortic arch, and right patent ductus arteriosus

    Chest

    (1973)
  • PFW Kuers et al.

    Tetralogy of Fallot with aortic origin of the right pulmonary artery. Surgical implications

    J Thorac Cardiovasc Surg

    (1973)
  • PJ Kuypers et al.

    Origin of the right pulmonary artery from the aorta with patent ductus arteriosus

    J Thorac Cardiovasc Surg

    (1969)
  • H Matsuda et al.

    Aortic origin of the right pulmonary artery

    Ann Thorac Surg

    (1977)
  • E Robin et al.

    Aortic origin of the left pulmonary artery. Variant of tetralogy of Fallot

    Am J Cardiol

    (1975)
  • PW Sanger et al.

    Aortic origin of the right pulmonary artery with patent ductus arteriosus

    Ann Thorac Surg

    (1965)
  • HH Sievers et al.

    Repair of anomalous origin of the right pulmonary artery from the ascending aorta associated with subtotal left cor triatriatum

    Ann Thorac Surg

    (1985)
  • BR Wilcox et al.

    Aortic origin of the right pulmonary artery

    Ann Thorac Surg

    (1968)
  • R Van Praagh et al.

    Persistent fifth arterial arch in man. Congenital double-lumen aortic arch

    Am J Cardiol

    (1969)
  • LM Kutsche et al.

    Anatomy and pathogenesis of aorticopulmonary septal defect

    Am J Cardiol

    (1987)
  • LM Kutsche et al.

    Cervical origin of the right subclavian artery in aortic arch interruption: pathogenesis and significance

    Am J Cardiol

    (1984)
  • GE Cucci et al.

    Absence of a primary division of the pulmonary trunk. An ontogenetic theory

    Circulation

    (1964)
  • JG Mudd et al.

    Origin of one pulmonary artery from the aorta

    Am Rev Respir Dis

    (1964)
  • G Ambrus

    Congenital absence of the right pulmonary artery with bleeding into the right lung

    J Tech Methods

    (1936)
  • EW Jew et al.

    Aortic origin of the right pulmonary artery and absence of the transverse aortic arch

    Arch Pathol

    (1952)
  • JE Odell et al.

    Right pulmonary artery arising from ascending aorta

    Am J Dis Child

    (1963)
  • CA Wagenvoort et al.

    Origin of right pulmonary artery from ascending aorta

    Circulation

    (1961)
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    1

    Dr. Kutsche is the recipient of Clinical Investigator Award 5K08 HL00885 from the National Heart, Lung, and Blood Institute, Bethesda, Maryland.

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