We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.

Aims Heart failure chiefly affects the elderly, with frequent emergency admissions. Telemonitoring can identify worsening heart failure but previous randomized trials have enrolled selected patient populations. The Home‐HF study examined the impact of home telemonitoring on typical heart failure patients discharged from three acute hospitals in North West London, UK. Methods and results Patients hospitalized with heart failure were randomized to telemonitoring or usual specialist care. Primary outcome measures were days alive and out of hospital. Secondary outcome measures were number and duration of heart failure hospitalizations, clinic visits, and quality of life. We recruited 182 patients. There was no difference in the primary outcome measure in the two groups, but there were significantly fewer unplanned hospitalizations for heart failure decompensation, and a reduction in clinic and emergency room visits in the telemonitoring group. There was no statistically significant difference in the mean direct health service costs. Conclusion Home telemonitoring in a typical elderly population of heart failure patients produces a similar outcome to ‘usual’ specialist care, but reduces clinic and emergency room visits and unplanned heart failure rehospitalizations at little additional cost. This method of disease monitoring may allow specialist services to increase the number of patients under their care.

The symptoms and signs of heart failure can occur in the setting of an increased cardiac output and has been termed 'high output heart failure'. An elevated cardiac output with clinical heart failure is associated with several diseases including chronic anaemia, systemic arterio-venous fistulae, sepsis, hypercapnia and hyperthyroidism. The underlying primary physiological problem is of reduced systemic vascular resistance either due to arterio-venous shunting or peripheral vasodilatation. Both scenarios can lead to a fall in systemic arterial blood pressure and neurohormonal activation leading to overt clinical heart failure. In contrast to low output heart failure, clinical trial data in this area are lacking. The use of conventional therapies for heart failure, such as angiotensin converting enzyme inhibitors, angiotensin receptor blockers and certain beta-blockers with vasodilatory properties, is likely to further reduce systemic vascular resistance resulting in deterioration. The condition, although uncommon, is often associated with a potentially correctable aetiology. In the absence of a remediable cause, therapeutic options are very limited but include dietary restriction of salt and water combined with judicious use of diuretics. Vasodilators and beta-adrenoceptor positive inotropes are not recommended.

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After liver transplantation, patients with FAP should have regular clinical evaluations including electrocardiographic and echocardiographic examinations to look for continued deterioration in heart structure or function.

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