Introduction A follow-up study1 of 770 PBC patients found that their survival was much poorer than an age- and sex-matched population (standardised mortality ratio for PBC patients was 2.87). Excluding deaths from hepatic causes, the standardised mortality ratio for PBC patients was 1.73: the balance of the risk was of a cardiac-related death the mechanism by which the disease could affect cardiac tissue was unclear. 50% of PBC patients suffer systemic fatigue, and in these the risk of cardiac-related death has been shown to be higher than in non-fatigued patients.2

Method 15 proven PBC stage I–II patients were recruited (age: 48±6 y) and 8 age, weight and height- matched female subjects were recruited as controls (age: 52±11 y). MRI short axis cardiac tagging was used to measure circumferential strain and torsion throughout the cardiac cycle.3 Patient and control subject fatigue severity was assessed by means of a validated questionnaire, the Fatigue Impact Score (FIS), where 0 indicates no fatigue to a maximum of 160. Patients were divided into two groups: those without significant fatigue (defined as FIS<25) and those with severe fatigue (FIS>50). All controls had FIS <25.

Results In fatigued PBC patients, cardiac torsion was increased and circumferential strain significantly decreased compared to controls and non-fatigued PBC patients, indicating impaired subendocardial myofibre function, which is characteristic of advanced ageing.3 Anatomical MRI showed no morphological or functional alteration in the hearts of either group of PBC patients compared to controls.

Conclusion In PBC patients with severe fatigue we have found changes in the relationship between peak torsion and circumferential strain indicating that these patients may have suffered effective ageing of their hearts. This is in alignment with previous findings that PBC patients with substantial fatigue had a greater risk of cardiac-related death.