Cardiac transplantation in a patient with hereditary hemochromatosis: role of adjunctive phlebotomy and erythropoietin.

We describe the case of a 36-year-old woman with hereditary hemochromatosis (HH) resulting in end-stage cardiomyopathy and treated successfully with orthotopic cardiac transplantation. Before and after transplantation, the patient underwent aggressive treatment with frequent phlebotomy. We used erythropoietin concomitantly to maintain adequate hematocrit to support continued phlebotomy. We believe that aggressive use of phlebotomy provided the patient hemodynamic benefit and hastened the return of endocrine function post-transplantation. We also believe that the patient's history of high-dose vitamin C usage may have accelerated iron deposition in the heart and other vital organs.

Role of metabolically active drugs in the management of ischemic heart disease.

This article reviews the fundamentals of myocardial energy metabolism and selectively outlines the use of several metabolically active drug therapies in the treatment of ischemic heart disease. These drugs - ranolazine, trimetazidine, dichloroacetate (DCA), glucose-insulin-potassium (GIK) solutions, and L-carnitine - have mechanisms of action distinct from traditional anti-ischemic drugs. These agents work by shifting myocardial energy metabolism away from fatty acids toward glucose as a source of fuel. Because these agents are well tolerated and do not affect heart rate or blood pressure, they conceivably could supplement traditional anti-ischemic drug therapy with little risk. The background, rationale for use, and published literature on each agent is reviewed, and the outcomes of pertinent clinical trials are discussed. In the case of ranolazine, data suggest benefit in the treatment of stable angina pectoris, particularly with sustained release formulations. Trimetazidine appears to have similar physiologic effects to ranolazine, and it is effective as monotherapy and as additive therapy in patients with chronic ischemic heart disease. DCA improves acidosis in critically ill patients and, likewise, improves myocardial hemodynamics in those with chronic coronary artery disease and congestive heart failure; however, its metabolism is variable and clinical data on its use in chronic ischemic heart disease are limited. GIK solutions have been shown to be beneficial in animal and human models of ischemia and acute myocardial infarction, and they offer an inexpensive means by which to improve the oxidation of glucose in the heart. Lastly, a large body of literature suggests a benefit with L-carnitine in a number of cardiovascular illnesses, including ischemic heart disease. Clinical trial data in acute myocardial infarction are promising and have prompted the initiation of a large-scale mortality trial.

Cardiac transplantation: the University of Florida experience.

Heart transplantation offered satisfactory outcomes in carefully selected patients with end stage congestive heart failure treated at our institution from 1990-1996. At the University of Florida, our survival rates involving 196 heart transplants were 86%, 78% and 74%, respectively, at one, 3 and 5 years. This data compares favorably with international results. Our typical transplant recipient was a 50-60 year old caucasian male with ischemic cardiomyopathy. Based on this population and the severe shortage of donor hearts available, aggressive attempts must continue to identify patients with ischemic cardiomyopathy that may benefit (i.e. adequate target vessel, viable myocardium) from high-risk coronary artery bypass surgery. Infection, rejection and graft vasculopathy continue to influence morbidity and mortality after transplantation. Continued efforts aimed at the development of improved immunosuppression as well as prevention and containment of coronary vasculopathy are needed if these results are to be significantly improved.

Age at baptism in pre-industrial England.

Abstract The adequacy of English parish registers as demographic sources has been a subject for much debate.(1) Most attention has been directed to the problem of how far the population at large continued to use the sacraments ofthe Established Church in the late eighteenth and early nineteenth centuries, especially in areas affected by urban growth or Nonconformity. But the more general problem of how far the ecclesiastical registers of ceremonies are acceptable substitutes for registers of vital events also deserves some attention.