Objective: To examine the quality of life of adults with congenital heart disease. Design and setting: Observational, cross sectional study conducted at one general hospital in Birmingham, UK. Patients: All 471 patients registered at the adult congenital heart disease clinic were sent the 36 item short form health survey (SF-36) to assess their quality of life. Questionnaires were completed by 276 (58.6%) patients (41.7% men; median (interquartile range) age 31.0 (26.3-36.0) years, range 16-85 years). Results: Surprisingly, patients deemed surgically cured (for example, atrial septal defect repair) had significantly poorer quality of life in all domains (all p < 0.05), except for pain, than the general population, as determined from population normative data. Patients who had received palliative treatment reported quality of life scores similar to those who had never required cardiac surgery and to the general population, although both patient groups had significantly poorer physical functioning and overall general health perception than the general population (all p < 0.01). Patients with inoperable conditions had significantly poorer physical functioning (all p < 0.01) and overall general health perception (all p < 0.05) than all other patients, and significantly worse quality of life in all domains than the similarly aged general population. Patients with cyanotic conditions had significantly worse quality of life than age and sex matched acyanotic patients (all p < 0.01). Conclusions: Patients with inoperable or cyanotic conditions and, paradoxically, those deemed surgically cured, had the poorest quality of life among adults with congenital heart disease. However, all adults with congenital heart disease had significantly poorer levels of physical functioning and overall general health perception then similarly aged people in the general population.

Reversible and fixed perfusion defects with concordant regional wall motion abnormalities occur in the right (systemic) ventricle 10 to 20 years after Mustard repair for transposition of the great arteries; this may be important in the pathogenesis of late right ventricular dysfunction in this group.

Patients with unexplained chest pain and occult GERD have esophageal pain hypersensitivity that is PPI responsive. The increase in resting PT and secondary allodynia only following PPI therapy suggests that pain hypersensitivity in these GERD patients may partially be the result of central sensitization.