Three patients with Emery Dreifuss muscular dystrophy are reported. Emery Dreifuss muscular dystrophy is an X linked muscular dystrophy, in which locomotor involvement is characteristically mild and slowly progressive. The eVect on the heart becomes apparent in the teenage years and is characterised by cardiac conduction defects and infiltration of the myocardium by fibrous and adipose tissue. It first aVects the atria, which results in atrial paralysis; treatment with ventricular pacing is usually needed. Female carriers can develop heart problems and are at risk of sudden death. Relatives of aVected patients should be oVered screening with electrocardiography and echocardiography. (Heart 1999;82:105-108)

3. Above a cuff pressure of 20 mmHg, capillary pulse pressure amplitude (CPPA) tended to decline. At 50 mmHg cuff pressure, CPPA was lower than at baseline for all subjects. At baseline, CPPA was 4-2 + 2-0 mmHg (mean + S.D.) and at 50 mmHg it was 2-3 + 1.1 mmHg (P = 0-02).4. In the period between 1 and 6 min following cuff release, both P" and CPPA were lower than at baseline. (At baseline, Pc was 16-1 + 2-3 mmHg and following cuff release it was 11-2 + 1-5 mmHg (P = 002). At baseline, CPPA was 4-2 + 2-0 mmHg and following cuff release it was 1-8 + 1.1 mmHg (P = 0 03).)5. Estimated changes in the ratio of pre-to postcapillary resistance (Ra/Rv), using arterial blood pressure (P.) measured in the contralateral arm, and taking (P -P)/(PC-Pv) to approximate to Ra/R,, closely mirrored changes in CPPA.6. The results support an increase in precapillary resistance occurring in response to elevation in postcapillary pressure, and suggest that this increase persists into the recovery period, but the results may also be consistent with early changes in Ra/RV resulting from a fall in postcapillary resistance.Of the factors determining fluid movement across the capillary wall, capillary hydrostatic pressure is likely to be the most variable, and is thus a major determinant of transcapillary fluid exchange. Capillary pressure is determined by arterial inflow and venous outflow pressures, and by the levels of pre-and postcapillary resistance.

Objective-Severe impairment of left ventricular (LV) contraction is associated with an adverse prognosis in patients with ischaemic heart disease. Revascularisation may improve the impaired LV contraction if hibernating myocardium is present. The proportion of patients likely to benefit from this intervention is unknown. Therefore, the prevalence of hibernating myocardium in patients with ischaemic heart disease and severe impairment of LV contraction was assessed. Design-From a consecutive series of patients undergoing coronary angiography for the investigation of chest pain or LV impairment, all patients with ischaemic heart disease and an LV ejection fraction (LVEF) < 30% were identified. These patients underwent positron emission tomography (PET) to detect hibernating myocardium, identified by perfusion metabolism mismatch. Setting-A teaching hospital directly serving 500 000 people. Results-Of a total of 301 patients, 36 had ischaemic heart disease and an LVEF < 30%. Twenty-seven patients had PET images, while nine patients were not imaged because of emergency revascularisation (three), loss to follow up (one), inability to give consent (four), and age < 50 years (one, ethics committee guidelines). Imaged and non-imaged groups were similar in LV impairment, demographic characteristics, and risk factor profile. Fourteen patients (52% of the imaged or 39% of all patients with ischaemic heart disease and LVEF < 30%) had significant areas of hibernating myocardium on PET. Conclusion-It is possible that up to 50% of patients with ischaemic heart disease and severely impaired left ventricles have hibernating myocardium. (Heart 1998;80:559-564)

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