Intermediate septal accessory pathways are located in close proximity to the atrioventricular (AV) node and His bundle, have unique features that distinguish them from typical anterior and posterior accessory pathways and have been associated with a high risk for unsuccessful pathway division and the production of complete AV block after surgery. Between July 1986 and May 1990, 4 of 70 patients (3 men and 1 woman; mean age 33 ± 13 years) undergoing surgery for accessory pathway division were found to have an intermediate septal accessory pathway. The presenting arrhythmia was atrial fibrillation with rapid anterograde conduction over the accessory pathway in two patients and recurrent orthodromic reciprocating tachycardia in two patients. In all patients, the delta wave on the electrocardiogram (ECG) was inversed in lead V1, but two patterns of delta wave configuration were observed. In three patients (type 1 intermediate septal accessory pathway), the delta wave was upright in lead II, inverted in lead III and isoelectric in lead aVF; the transition from a negative to an upright delta wave occurred in lead V2. The fourth patient exhibited a different delta wave pattern (type 2 intermediate septal accessory pathway). The delta wave was upright in each of leads II, III and aVF; the transition from a negative to an upright delta wave occurred at lead V3. Intraoperative electrophysiologic study localized the atrial insertion of type 1 pathways to the midpoint of Koch's triangle close to the AV node. In the one patient with a type 1 pathway in which both anterograde and retrograde accessory pathway conduction was present, preoperative catheter mapping demonstrated that earliest retrograde atrial activation occurred near the foramen ovale. Intraoperative mapping during anterograde conduction over the type 1 pathway demonstrated earliest epicardial ventricular activation to occur simultaneously at the crux and the base of the aorta. The atrial insertion of the type 2 intermediate septal accessory pathway was localized to the apex of Koch's triangle in close proximity to the bundle of His. Preoperative catheter mapping revealed that earliest retrograde atrial activation occurred on the His bundle electrogram. Intraoperative mapping during anterograde conduction over the type 2 pathway demonstrated that earliest epicardial ventricular activation occurred anteriorly at the base of the aorta. Intraoperative ablation of the intermediate septal accessory pathway was accomplished by cooling the endocardium at the site of pathway insertion on the atrial side of the tricuspid anulus with a 5 mm cryoprobe. Patients with a type 1 intermediate septal accessory pathway had preservation of AV conduction, but the patient with the type 2 pathway did not and required permanent pacing. At late follow-up study, no patient has had return of intermediate septal accessory pathway conduction. Distinguishing an intermediate septal accessory pathway close to the AV node (type 1) from one close to the His bundle (type 2) is useful to predict both surgical success and success without the production of permanent complete AV block. © 1991.