1/1443. Hereditary dysrhythmic congestive cardiomyopathy. A patient with hereditary congestive cardiomyopathy, who presented with recurrent episodes of life-threatening ventricular arrhythmias most often precipitated by exercise, is described. The condition is marked by either a progressive course, in which case congestive cardiac failure may set in towards the end, or by unexpected sudden death. The family tree could be traced for 10 generations. The information about the tenth generation firmly established that 4 members, 2 of whom had died, were affected. Other evidence suggests that the condition was the cause of death in 3 members of the eighth generation. ( info) |
2/1443. Tc-99m HDP uptake in cardiac amyloidosis. amyloidosis is characterized by the soft-tissue deposition of amyloid protein. It may occur as a primary disorder but more often is seen as a manifestation of chronic illness. Scattered reports of the affinity of amyloid for bone scanning agents have appeared over many years. Isolated cardiac uptake of Tc-99m HDP is described in a patient with biopsy-proved cardiac amyloidosis on a background of tuberculosis, prostate cancer, and coronary artery disease. ( info) |
3/1443. Cardiac involvement in proximal myotonic myopathy. Proximal myotonic myopathy (PROMM) is a recently described autosomal dominantly inherited disorder resulting in proximal muscles weakness, myotonia, and cataracts. A few patients with cardiac involvement (sinus bradycardia, supraventricular bigeminy, conduction abnormalities) have been reported. The cases of three relatives with PROMM (weakness of neck flexors and proximal extremity muscles, calf hypertrophy, myotonia, cataracts) are reported: a 54 year old man, his 73 year old mother, and 66 year old aunt. All three presented with conduction abnormalities and one had repeated, life threatening, sustained monomorphic ventricular tachycardia. This illustrates that severe cardiac involvement may occur in PROMM. ( info) |
4/1443. Long-term follow-up of a true contractile left ventricular diverticulum. The natural history of contractile left ventricular diverticulum in the adult is not known. Serial left ventricular angiography in an adult revealed that a left ventricular diverticulum did not increase in size over a 13-year period, suggesting that the clinical course may be benign. ( info) |
5/1443. Evidence of cell-mediated cardiac myocyte injury involved in the heart failure of a patient with progressive systemic sclerosis. A 54-year-old woman with progressive systemic sclerosis (PSS) was admitted to hospital because of dyspnea and chest pain. Echocardiogram revealed diffuse hypokinesis of the left ventricle (ejection fraction 24%). methylprednisolone, heparin, and diuretics were administered, without benefit. anemia, thrombocytopenia, and renal dysfunction rapidly progressed, and she died of heart failure on the 14th hospital day. Immunohistochemical study of the myocardial tissue showed mild to moderate cell infiltration, mainly consisting of natural killer (NK) cells, macrophages, cytotoxic T lymphocytes (CTLs), and T helper cells. perforin, a cytolytic factor, was expressed in the infiltrating CTLs and NK cells, indicating that these cells were activated killer cells. Furthermore, human leukocyte antigen classes I and II, intercellular adhesion molecule-1, as well as costimulatory molecules B7-1, B7-2, and CD40, all of which are known not to be expressed in cardiac myocytes under normal conditions, were moderately to strongly expressed in cardiac myocytes. There was no detectable level of enterovirus genomes in the polymerase chain reaction products from the myocardial tissue of this patient. These findings strongly suggest that the infiltrating killer cells recognized cardiac myocytes as target cells and directly damaged them by releasing perforin. Enhanced expression of these antigens may have played an important role in the activation and cytotoxicity of the infiltrating killer cells. Absence of enterovirus genomes in the myocardial tissue may suggest that this autoimmune process is primarily induced by PSS. ( info) |
6/1443. Thrombolysis with resolution of pulmonary hypertension in a heart transplant candidate. We report a patient with idiopathic cardiomyopathy and high pulmonary resistance due to pulmonary emboli of unknown age. Successful thrombolytic therapy returned his pulmonary resistance to normal, allowing orthotopic cardiac transplantation. This case underscores the need to aggressively diagnose and treat pulmonary emboli in potential transplant candidates. ( info) |
7/1443. Sudden death in right ventricular dysplasia with minimal gross abnormalities. Arrhythmogenic right ventricular cardiomyopathy is emerging as a relatively common cause of exercise-induced sudden death in the young. The diagnostic criteria at autopsy are, however, not fully established, leading to both over- and underdiagnosis. We report a young man and a young woman dying suddenly of right ventricular dysplasia during exercise, in whom the gross autopsy findings in the right ventricle were minimal or even absent. However, the histologic features in both right and left ventricles were typical of the disease, and consisted of fibrofatty infiltrates with typical myocyte degeneration of the right ventricle and subepicardial regions of the left ventricle. These cases illustrate that microscopic findings are diagnostic and may be present in the absence of gross findings. Marked fat replacement is not essential for the diagnosis of right ventricular dysplasia, and the right ventricle should be extensively sampled histologically in all cases of sudden unexpected death, especially those that are exercise related. ( info) |
8/1443. Severe cardiac dysrhythmia in patients using bromocriptine postpartum. Used worldwide since 1980 for the prevention of breast engorgement in the puerperium, in 1994 bromocriptine mesylate was withdrawn from the American market as an agent suitable for ablactation. The relevant recommendation of the food and Drug Administration rested on case reports that described severe vasospastic reactions among users of the drug. Some patients so affected suffered stroke, intracranial bleeding, cerebral edema, convulsions, myocardial infarction, and puerperal psychosis. More recently, it has been suggested that the side effects of the drug may also include circulatory collapse secondary to cardiac dysrhythmia. This report describes two additional cases in this category. The antepartum clinical evaluation of these women suggested that they were predisposed to arrhythmias. ( info) |
| Sudden death is the most common manifestation of myocardial sarcoidosis, which is often clinically silent. The disease is rarely encountered in forensic medicine, but the diagnosis is important because it may change the interpretation of the manner of death and thus have insurance implications. Six cases of myocardial sarcoidosis diagnosed at autopsy are reported, and the significance of the findings is discussed. ( info) |
10/1443. Progression of cardiomyopathy and neuropathy after liver transplantation in a patient with familial amyloidotic polyneuropathy caused by tyrosine-77 transthyretin variant. Familial amyloidotic polyneuropathy is an inherited form of amyloidosis associated with a mutant form of a protein called transthyretin. The methionine-30 variant is the most frequent mutation observed. This disorder is caused by deposition of this protein as amyloid in several organs, such as the heart, kidneys, and peripheral nervous system. The disease is always progressive and fatal, and patients die 7 to 10 years after the onset of symptoms. liver transplantation is at present the only choice for these patients because it provides improvement of symptoms and/or stops progression of the disease in most patients. We report the case of a patient who showed clear progression of cardiomyopathy and neuropathy after liver transplantation. ( info) |