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.

Links

Publisher Full Text

Authors

Edwin F, Kinsley RH, Mamorare HM, Govendrageloo K

Institution

Walter Sisulu Pediatric Cardiac Center for Africa, Sunninghill Hospital, Johannesburg, South Africa. fedwin68@yahoo.com

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