Author + information
- Received May 2, 1983
- Revision received July 18, 1983
- Accepted July 22, 1983
- Published online February 1, 1984.
- Victor M. Pérez-Martínez, MD, FACC1,*,
- Francisco García-Fernandez, MD1,
- José Oliver-Ruiz, MD1 and
- Luis Nuñez-Gonzalez, MD1
- ↵*Address for reprints: Victor M. Pérez-Martínez, MD, Servicio de Cardiología Pediatrica, Clinica Infantil de la Ciudad Sanitaria de la Seguridad Social, “La Paz”, Paseo de la Castellana 261, Madrid-34, Spain.
In double-outlet atrium either the right or the left atrium empties into both ventricles while the other atrium remains disconnected from the ventricular cavities. Cases of double-outlet atrium with a single atrioventricular (AV) valve have been reported but no cases of double-outlet left atrium and only two of double-outlet right atrium (one with three valves) have been reported. A description of the second case of double-outlet right atrium with two AV valves and a review of the other reported case are presented.
Common anatomic characteristics were: two AV valves in the right atrium that connected with two normal ventricles; disconnection of the left atrium from the ventricular cavities; an atrial septal defect in a malpositioned atrial septum; and drainage of a left superior vena cava into the left atrium. Mitral incompetence was found in both cases. Clinical findings were similar to those seen in ostium primum atrial septal defect.
Two-dimensional echocardiography and angiocardiography are able to accurately demonstrate this entity. Successful surgical correction was performed in both cases. Two morphogenetic hypotheses are proposed: abnormal development of the septum primum or primitive malposition of the common atrioventricular canal.
- Received May 2, 1983.
- Revision received July 18, 1983.
- Accepted July 22, 1983.
- American College of Cardiology Foundation