Background: Hypertensive lower esophageal sphincter (HLES) is an uncommon manometric abnormality found in patients with dysphagia, chest pain, gastroesophageal reflux (GERD), and/or hiatal hernia. Preventing reflux or repairing the hernia by performing a fundoplication raises concerns of inducing or worsening dysphagia. The role of myotomy of the HLES is also unclear. Aim: To define the surgical treatment of patients with HLES associated with dysphagia, chest pain, GERD and/or hiatal hernia. Methods: Sixteen patients (age range: 39-89 years; M:F=5:11) with HLES (>26 mmHg, i.e.>95th percentile of our control population) who had surgical therapy between 1996 and 1999 were reviewed. Patients with a diagnosis of achalasia and diffuse esophageal spasm were excluded. All patients had dysphagia or chest pain. In addition, 8 patients had GERD symptoms (HLES + GERD), 4 had a paraesophageal hernia (HLES + paraesophageal hernia), and 4 had isolated dysphagia or chest pain (HLES only) (see table). Patients with HLES and GERD had Nissen fundoplication, those with HLES and paraesophageal hernia had repair and Nissen fundoplication, and those with HLES only had myotomy of the sphincter with partial fundoplication. Outcome was considered excellent if the patient was asymptomatic, good if symptoms were present but no treatment was required, fair if symptoms were present and required treatment and poor if symptoms were unimproved or worsened. All patients were contacted by telephone for symptom assessment at a median of 3.6 years (IQR 3.2-5.0) after surgery. Results: Dysphagia and chest pain were relieved in all patients at long term follow up. Outcome was excellent in 10/16, good in 3/16, and fair in 3/16. All, but one patient, were satisfied with their outcome. Conclusion: Treatment of patients with HLES should be individualized. Concern about inducing or worsening dysphagia by Nissen fundoplication in patients with GERD or paraesophageal hernia is unwarranted. When the indication for operation is dysphagia alone, myotomy with partial fundoplication is effective.