Phenotypes and clinical context of hypercontractility in high-resolution Esophageal Pressure Topography (EPT)

Objectives: This study aimed to refine the criteria for esophageal hypercontractility in high-resolution esophageal pressure topography (EPT) and to examine the clinical context in which it occurs. Methods: A total of 72 control subjects were used to define the threshold for hypercontractility as a distal contractile integral (DCI) greater than observed in normals. In all, 2,000 consecutive EPT studies were reviewed to find patients exceeding this threshold. Concomitant EPT and clinical variables were explored. Results: The greatest DCI value observed in any swallow among the control subjects was 7,732 mm Hg-s-cm; the threshold for hypercontractility was established as a swallow with DCI >8,000 mm Hg-s-cm. A total of 44 patients were identified with a median maximal DCI of 11,077 mm Hg-s-cm, all with normal contractile propagation and normal distal contractile latency, thereby excluding achalasia and distal esophageal spasm. Hypercontractility was associated with multipeaked contractions in 82% of instances, leading to the name Jackhammer Esophagus. Dysphagia was the dominant symptom, although subsets of patients had hypercontractility in the context of esophagogastric junction (EGJ) outflow obstruction, reflux disease, or as an apparent primary motility disorder. Conclusions: We describe an extreme phenotype of hypercontractility characterized in EPT by the occurrence of at least a single contraction with DCI >8,000 mm Hg-s-cm, a value not encountered in control subjects. This phenomenon, branded Jackhammer Esophagus, was usually accompanied by dysphagia and occurred both in association with other esophageal pathology (EGJ outflow obstruction, reflux disease) or as an isolated motility disturbance. Further studies are required to define the pathophysiology and treatment of this disorder.