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The spectrum of double-outlet right atrium including hearts with three atrioventricular valves.

Abstract

Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.

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  • Publisher Full Text
  • Authors

    Edwin F, Kinsley RH, Mamorare HM, Govendrageloo K

    Source

    European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 41:4 2012 Apr pg 947-9

    MeSH

    Adolescent
    Child
    Child, Preschool
    Double Outlet Right Ventricle
    Female
    Heart Atria
    Heart Septal Defects, Atrial
    Heart Septal Defects, Ventricular
    Humans
    Male
    Mitral Valve
    Tricuspid Valve

    Pub Type(s)

    Case Reports
    Journal Article

    Language

    eng

    PubMed ID

    22423064