Postmortem echocardiography and tomographic anatomy of hypoplastic left heart syndrome after palliative surgery

A study was undertaken to improve the understanding of the 3-dimensional (3-D) topology of a complex surgical reconstruction. The pathologic anatomy was investigated by first fixing postmortem heart specimens in such a way as to preserve the 3-D relations. Next, a technique for postmortem 2-D echocardiography was developed to aid in selection of tomographic planes for sectioning the specimens. Subsequent adjustment of planes of section was made to better show particular facets of the anatomy. The material for this investigation was drawn from cases of hypoplastic left heart syndrome after surgical palliation by the Norwood procedure. Three potential hemodynamic sequelae--restrictive inter atrial communication, aortic obstruction at any level and distortion of the pulmonary artery confluence--served as the anatomic focus for this study. Careful preservation of 3-D topology and postmortem 2-D echocardiographic imaging coupled with tomographic sectioning of specimens led to development of new, clinically relevant echocardiographic views for imaging specific atrial septal and aortic arch anomalies. These techniques offer insight into the spectrum of anatomic sequelae of this type of surgery and may be applied to echocardiographic imaging of patients and refinement of surgical technique for other forms of complex congenital heart disease.