1/148. 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.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
2/148. DDD-pacing-induced cardiomyopathy following AV node ablation for persistent atrial tachycardia.Ventricular rate control by catheter ablation of the AV node and pacing in patients with persistent atrial tachycardia has been reported to improve left ventricular function. However, this approach requires careful selection of the pacing mode. We report a patient who underwent AV node ablation for persistent multiple atrial tachycardias, and who then had a non-mode-switching pacemaker implanted. Because of an inappropriately programmed relatively high upper rate limit, the patient developed left ventricular dysfunction after 6 years. This resolved after programming the pacemaker to VVI at 70 bpm.- - - - - - - - - - ranking = 236.50305368585keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
3/148. Dramatic improvement of left ventricular function after cytotoxic therapy in lupus patients with acute cardiomyopathy: report of 6 cases.Although lupus cardiomyopathy is thought to be clinically uncommon, we encountered 6 patients with systemic lupus erythematosus (SLE) over a 10 year period who had severe left ventricular dysfunction and showed remarkable improvement in their cardiac function after cytotoxic therapy. All patients met the American College of rheumatology criteria for classification of SLE and presented with signs of severe biventricular failure relatively early in their disease. Concurrent manifestations of SLE at the time of cardiomyopathy included rash, arthritis, myalgias, pleuritis, pericarditis, and nephritis. Four of the 6 patients were taking prednisone 20 mg/day at the time heart failure developed. In all cases the CPK were normal. Evaluation of cardiac function by echocardiogram and/or radionuclide gated blood pool scintigraphy revealed a severe depression of ventricular function with initial left ventricular ejection fraction (LVEF) ranging from 11 to 34% (mean 19%). Within 6 months of initiation of cytotoxic treatment all patients showed a dramatic response: the post-treatment LVEF ranged from 25 to 55%. This series of patients suggests that cardiomyopathy may be a more common complication of SLE than previously reported. Cardiomyopathy occurs relatively early in the course of SLE, may lead to severe cardiac dysfunction despite corticosteroid therapy, and appears to be responsive to cytotoxic therapy.- - - - - - - - - - ranking = 237.50305368585keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
4/148. Transient myocardial dysfunction associated with angiotensin-converting enzyme inhibitor-induced angioedema: recognition by serial echocardiographic studies.We report a case of a 58-year-old woman who had angiotensin converting enzyme inhibitor-induced angioedema after she underwent a biopsy of a hypopharyngeal mass. The angioedema was associated with severe transient myocardial dysfunction documented on echocardiography. She did not have anaphylaxis or coronary artery disease. To our knowledge this is the first reported case of transient myocardial dysfunction in the setting of angiotensin converting enzyme inhibitor-induced angioedema without anaphylaxis.- - - - - - - - - - ranking = 6keywords = dysfunction (Clic here for more details about this article) |
5/148. An Italian family with Ala-47 transthyretin mutation associated with cardiomyopathy and polyneuropathy.We describe two Italian first cousins with familial amyloidotic polyneuropathy associated with transthyretin variant consisting of the substitution of alanine for glycine at codon 47 (TTR Ala-47), from a family with a history of cardiac failure. The 40-year-old patient presented with autonomic dysfunction and the 44-year-old cousin with congestive heart failure. Both developed sensorimotor and autonomic polyneuropathy. Since a similar clinical picture has been described in another Italian family, the cardiac involvement must be regarded as a salient and early feature of the TTR Ala-47 mutation.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
6/148. Reversible left ventricular dysfunction associated with raised troponin i after subarachnoid haemorrhage does not preclude successful heart transplantation.Transient abnormalities in ECGs, echocardiograms, and cardiac enzymes have been described in the acute setting of subarachnoid haemorrhage. In addition, left ventricular dysfunction has been reported at the time of brain death. A patient with an acute subarachnoid haemorrhage who presented with raised troponin i (TnI) concentrations and diffuse left ventricular dysfunction is described. After declaration of brain death 32 hours later, the heart was felt initially not suitable for transplantation. A normal cardiac catheterisation, however, lead to successful transplantation of the donor heart. Raised catecholamine concentrations and metabolic perturbations have been proposed as the mechanisms leading to the cardiac dysfunction seen with brain death. This may be a biphasic process, allowing time for myocardial recovery and reversal of the left ventricular dysfunction. awareness of this phenomenon in the acutely ill neurologic population needs to be raised in order to prevent the unnecessary rejection of donor hearts.- - - - - - - - - - ranking = 1656.521375801keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
7/148. A new off-pump technique for thoratec right ventricular assist device insertion.The need for right ventricular support as an adjunct to left ventricular assistance is uncommon. When required, the insertion of a right ventricular assist device may be complicated by preexisting hepatic dysfunction, coagulation abnormalities, and renal failure, all of which are exacerbated by cardiopulmonary bypass. We report a technique for insertion of a right ventricular assist device without the need for cardiopulmonary bypass.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
8/148. Distinguishing cardiac features of a novel form of congenital muscular dystrophy (Salih cmd).The cardiac features of a novel form of congenital muscular dystrophy (Salih CMD) are described in two adolescent siblings. The patients presented with severe hypotonia at birth, associated with delayed development. They could walk independently and managed to maintain walking after 13 years of age. Their muscle immunohistochemistry differed from that seen in Duchenne and Becher muscular dystrophy (DMD and BMD), severe childhood autosomal recessive muscular dystrophy (SCARMD) due to sarcoglycan deficiency (sarcoglycanopathies), and lamininalpha2 (merosin)-deficient CMD. However, both patients had associated cardiomyopathy. electrocardiography (ECG) in Salih CMD was characterized by delayed atrioventricular (AV) conduction, left anterior fascicular block (left axis deviation), and left atrial enlargement without evidence of atrial dysarrhythmia. echocardiography showed features of severe left ventricular dysfunction with estimated left ventricle ejection fraction (LVEF) of 25% at 16 years-of-age in the older patient. A year later, multigated aquisition MUGA scan showed LVEF of 21% and dilatation of the right ventricle. echocardiography and MUGA scan were normal in the younger patient at 15 years-of-age. ECG, echocardiography, and MUGA scan are effective techniques for diagnosing and monitoring the cardiomyopathy in Salih CMD. They can also distinguish it from features seen in the other common forms of MD, including DMD, BMD, and sarcoglycanopathies.- - - - - - - - - - ranking = 236.50305368585keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
9/148. Isolated non-compaction of the left ventricle: a rare indication for transplantation.This report describes the diagnostic difficulty encountered in a young female patient presenting with neurologic symptoms, atrial fibrillation and severe left ventricular systolic dysfunction, eventually leading to cardiac transplantation. The scrutiny used in the evaluation of the particular aspect of the left ventricle, and the integration of the information obtained from echocardiography, angiography and magnetic resonance imaging, led to the diagnosis of a rare and mostly unknown cause of cardiac failure. The correct identification of this entity is mandatory because enhanced risk of thromboembolism and malignant arrhythmia should be anticipated. A review of the literature revealed only 6 patients in whom isolated non-compaction of the left ventricle was treated by heart transplantation.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
10/148. Papillary muscle calcification after inferoposterior myocardial infarction.Extensive papillary muscle calcification is quite a rare finding in echocardiographic examinations. A case of a 71 year old man with isolated calcification of the papillary muscles, detected by fluoroscopy and confirmed by echocardiography, is presented. Intracardiac calcifications in patients with prior right coronary artery occlusion and mitral regurgitation should suggest the possibility of posteromedial papillary muscle calcification and dysfunction.- - - - - - - - - - ranking = 1keywords = dysfunction (Clic here for more details about this article) |
| | Next -> |