The Journal of Thoracic and Cardiovascular Surgery, Vol 81, 127-130, Copyright © 1981 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
Syndrome of single ventricle without pulmonary stenosis but with left atrioventricular valve atresia and interatrial obstruction. Palliative management with simultaneous atrial septostomy and pulmonary artery banding
P. Syamasundar Rao, RJ Kulangara, HV Moore and WB Strong
Case histories of four infants with single ventricle and left
atrioventricular (AV) valve atresia, with interatrial obstruction but
without pulmonic stenosis, are presented. Relief of interatrial obstruction
by surgical or balloon atrial septostomy resulted in a marked fall in the
left atrial pressure and the pulmonary artery mean pressure and resistance
as well as a marked increase in the left-to- right interatrial shunt,
pulmonary blood flow, and systemic arterial oxygen saturation. Because of
this predictable fall in pulmonary vascular resistance, concomitant
pulmonary artery banding was performed in each case with good results.
There are only a few previously reported cases of single ventricle with
left AV valve atresia. Interatrial obstruction is quite common in this
lesion. The concept that relief of interatrial obstruction produces a rapid
and predictable fall in the pulmonary vascular resistance and that
concomitant pulmonary artery banding is necessary in these infants with
complex congenital heart disease is emphasized.
