digoxin and Endocardial-Fibroelastosis

This is the first report in the forensic literature of a combination of fatal digoxin poisoning with endocardial fibroelastosis (EFE). Typical morphological features of EFE as the cause of clinically diagnosed cardiomyopathy were present in the autopsy of a 3-year-old girl, including cardiac hypertrophy and marked thickening of the left-sided endocardium, consisting of numerous elastic and collagenic fibres. After exclusion of cardiac and cerebral causes of death, accidental digoxin intoxication was proved. Postmortem toxicological analyses by fluorescence polarization immunoassay (FPIA) disclosed digoxin levels of 71 micrograms/kg (femoral vein blood), 77 micrograms/kg (cardiac blood), 255 and 221 micrograms/kg (cardiac muscle of the right and left chamber), 163 micrograms/kg (psoas muscle), 91 micrograms/kg (lung), 222 micrograms/kg (liver) and 520 micrograms/kg (kidney). The results are compared with the antemortem digoxin concentration of 39 ng/ml serum. The case is discussed from its unusual morphological and toxicological aspects, with special consideration of possible medical malpractice.

Topics: Cardiotonic Agents; Child, Preschool; Digoxin; Endocardial Fibroelastosis; Fatal Outcome; Female; Humans; Poisoning

The diagnosis, treatment and etiology of primary infantile endocardial fibroelastosis were studied in 33 patients from 1989 to 1992. Two dimensional echocardiography with Doppler examination showed that the thickness of the endocardium obviously increased and systolic and diastolic functions of the left ventricles were abnormal. Endomyocardial biopsy proved that the endocardium and myocardium under went pathological changes. The virus probe examination of biopsy tissue showed negative results and the effectiveness of immunosuppressive therapy (total effective rate 96%) indicated that this disease is probably of autoimmune etiology. The facts led us to nominate this disease as infantile endomyocardial disease. Besides, the classification, diagnostic criteria, principles of treatment and prognosis of this disease were discussed.

Topics: Child, Preschool; Digoxin; Echocardiography, Doppler; Endocardial Fibroelastosis; Endocardium; Female; Humans; Infant; Male; Myocardium; Prednisone

Two related families with 15 children, seven of whom developed endocardial fibroelastosis (EFE) are described. Three of the children died during infancy, and the disease was confirmed in one of them at autopsy. The survivors, two sisters age 5 years and 15 months (Family A) and two sisters age 17 and 14 years (Family B), are now symptomless and show a decrease in left ventricular hypertrophy. The mode of inheritance of EFE in our two families appears to be either autosomal or X-linked dominant with reduced penetrance.

Topics: Carnitine; Digoxin; Endocardial Fibroelastosis; Female; HLA Antigens; Humans; Infant; Infant, Newborn; Male; Pedigree

Nine children with endocardial fibroelastosis were followed from the time of admission with congestive heart failure until either death or discharge. Review of multiple clinical features showed that only the electrocardiographic pattern could be correlated with death or survival. The presence of a delayed transition zone with anterior force loss on the initial electrocardiogram ('infarct pattern') was noted in all the children who died. Progression of these changes with a pattern of anterolateral 'infarct' in two and inferior wall 'infarct' in two occurred before death. Necropsy on three of the four children confirmed the diagnosis of endocardial fibroelastosis. There was extensive fibrosis and thinning of the left ventricular myocardium as well as involvement of the mitral valve structures. Review of published cases supports the view that an 'infarct' pattern in a child with endocardial fibroelastosis is usually associated with death and that this pattern is a negative prognostic sign for survival.

Topics: Cardiomegaly; Child; Child, Preschool; Digoxin; Electrocardiography; Endocardial Fibroelastosis; Female; Follow-Up Studies; Heart Failure; Humans; Infant; Male; Myocardial Infarction; Prognosis

Topics: Angiocardiography; Cardiac Catheterization; Child, Preschool; Digitalis Glycosides; Digoxin; Electrocardiography; Endocardial Fibroelastosis; Female; Hemodynamics; Humans; Infant; Male; Radiography, Thoracic

Topics: Anti-Bacterial Agents; Anticoagulants; Aortic Coarctation; Bradycardia; Chlorothiazide; Diet, Sodium-Restricted; Digitalis Glycosides; Digitoxin; Digoxin; Drug Tolerance; Electrocardiography; Endocardial Fibroelastosis; Furosemide; Heart Block; Heart Defects, Congenital; Heart Failure; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Lanatosides; Myocarditis; Organomercury Compounds; Oxygen Inhalation Therapy; Potassium Chloride; Pulmonary Edema; Tachycardia; Tachycardia, Paroxysmal; Transposition of Great Vessels

Topics: Angiocardiography; Cardiomegaly; Child; Coronary Vessel Anomalies; Diagnosis, Differential; Digitalis; Digoxin; Drug Therapy; Electrocardiography; Endocardial Fibroelastosis; Glycogen Storage Disease; Humans; Infant; Mumps; Myocarditis; Skin Tests