1/101. 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.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/101. Spurious redetection of sinus rhythm by an implantable cardioverter defibrillator during spontaneous ventricular fibrillation.Implantable cardioverter defibrillator undersensing leading to delayed or aborted therapy delivery has been reported with induced arrhythmias and following failed defibrillator shocks. We describe a case in which spurious redetection of sinus rhythm during a spontaneous episode of ventricular fibrillation resulted in aborted device therapy.- - - - - - - - - - ranking = 0.2keywords = liver (Clic here for more details about this article) |
3/101. A case of late onset cardiac amyloidosis with a new transthyretin variant (lysine 92).A new transthyretin (TTR) variant (lysine 92), which causes late onset cardiac amyloidosis, is described in a 71-year-old man. The patient at first had syncope due to ventricular tachycardia and was admitted our hospital. Typical findings of cardiac amyloidosis were observed by echocardiography, and a diagnosis of systemic amyloidosis was made by rectal biopsy. The man died approximately 3 years and 6 months after first admission, with gradually worsening congestive heart failure. Pathological examination showed prominent amyloid deposits in the heart and the vascular wall of many organs including the liver, pancreas, kidney, lung, and gastrointestinal tracts. Amyloid protein of transthyretin type was indicated by immunohistochemical study, and dna sequencing identified a novel mutation in the transthyretin gene encoding 92 glutamine --> lysine. A polymerase chain reaction-induced mutation restriction analysis with a mismatched antisense primer showed that the patient was heterozygous for the TTR Lys92 allele.- - - - - - - - - - ranking = 0.2keywords = liver (Clic here for more details about this article) |
4/101. Recurrent cerebral embolism in a familial amyloid polyneuropathy patient who received partial liver transplantation from a living donor.Cerebral embolism in relation to cardiac amyloidosis has not been widely noted. A 47-year-old woman who had been suffering from familial amyloid polyneuropathy (FAP) for 7 years was treated with partial liver transplantation from a living donor and her early postoperative course was uneventful. During the 391st to 613th postoperative day she experienced recurrent cerebral infarctions, but clinical examinations revealed no disorders capable of producing cerebral embolism. At autopsy splenic infarction and intracardiac thrombi adhering to the mitral valve and left atrium were found, and these areas showed severe amyloid deposition. Amyloid heart is considered to be one possible cause of systemic embolism.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
5/101. A case study of Jerry: emphasizing team communication through use of the Discipline.After the chaplain is well acquainted with The Discipline and has begun to implement it in daily pastoral practice, half of the work is done. The further work concerns how and what to communicate to the care team regarding the chaplain's observations. This article begins by offering a pastoral reflection on the chaplain's identity and pastoral practice within a multi-disciplinary care team. The pastoral reflection highlights key theological assertions used by The Discipline. The author then identifies the particular problems facing care teams and their communication that the chaplain can anticipate when using The Discipline. Thirdly, the author suggests workable, theologically based tools for the resolution of these problems. Lastly, through the case study of "Jerry," the author illustrates both the "how" and "what" components of care team communication using the working elements of The Discipline. The "how" component describes the informal and formal relational processes that have contributed to a working partnership. The case study also illustrates the "what" part of care team communication--the structure and delivery of observable and discernible content to physicians and nurses. These materials can ease the transition towards effective pastoral presence on the interdisciplinary care team regarding patient and family/support partner care.- - - - - - - - - - ranking = 0.2keywords = liver (Clic here for more details about this article) |
6/101. 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 = 0.091130735009518keywords = hepatic (Clic here for more details about this article) |
7/101. Unusual recurrence of hydatid cysts of the heart: report of two cases and review of the clinical and surgical aspects of the disease.In cardiac echinococcosis, a hydatid cyst most frequently forms either solely in the heart or in the pericardium, but there are several reports of cysts forming in the liver or lung or in both. In two cases reported here, both patients developed cysts in new sites after one or more previous surgeries for hydatid cyst removal. In Case 1, the patient first underwent spleenectomy and resection of multiple cysts with no evidence of a cyst in the heart; 3 years later, there was no sign of echinococcus in the liver, but a large inframyocardial cyst had damaged the left ventricle. In Case 2, the patient first underwent surgery to remove cysts from the pericardium, 2 years later from the anterior wall of the left ventricle, and, finally, 8 months after this second operation, from the left atrium also with no evidence of cyst formation anywhere else in the heart at the time of surgery. These cases emphasize the need for thorough and frequent reevaluation to detect new hydatid cyst formation in the heart and elsewhere caused by the echinococcus organism.- - - - - - - - - - ranking = 0.4keywords = liver (Clic here for more details about this article) |
8/101. Reversal of oxalosis cardiomyopathy after combined liver and kidney transplantation.Few data have been published on the course of oxalosis cardiomyopathy after combined liver and kidney transplantation in hyperoxaluria patients with myocardial involvement. We report the case of a primary hyperoxaluria type 1 patient with renal failure who developed end-stage cardiomyopathy. Left venticulography showed severe diffuse hypokinesia and left ventricular ejection fraction was calculated at 12%. Endomyocardial biopsy demonstrated platelike calcium oxalate crystals within the myocardium and the connective tissue, and mild perivascular fibrosis. The patient was first considered for combined liver-heart-kidney transplantation, but as his cardiac function improved slightly with an intensive dialysis program, combined liver and kidney transplantation was performed. Normal cardiac function was demonstrated at 1-year follow-up, and comparative endomyocardial biopsy showed regression of the myocardial oxalate deposits. This case adds stronger clinical, hemodynamic, and histopathological evidence that severe oxalosis cardiomyopathy may be reversed after combined liver and kidney transplantation.- - - - - - - - - - ranking = 1.6keywords = liver (Clic here for more details about this article) |
9/101. MR findings in cardiac hydatid cyst.We report a case of a 23-year-old man with a cardiac hydatid cyst involving the left ventricle wall. The diagnosis of the cyst was obtained by magnetic resonance (MR) imaging. He was operated on for cardiac hydatid cyst using enucleation and capitonnage procedure under extracorporeal circulation. Histopathologic study confirmed hydatid cyst diagnosis.- - - - - - - - - - ranking = 0.98761236500499keywords = circulation (Clic here for more details about this article) |
10/101. Thrombocytopenic purpura and cardiomyopathy in pregnancy reversed by combined plasma exchange and infusion.thrombocytopenia and hemolytic anemia have been seen with thrombotic thrombocytopenic purpura (TTP), hellp syndrome (hemolysis, elevated liver enzymes, and low platelets), and hemolytic uremic syndrome (HUS). Differentiating between TTP, HUS, and hellp syndrome is often difficult. Coexistence of TTP and HELLP is possible. Cardiomyopathy occurring in pregnancy can be idiopathic or associated with TTP. We describe a previously healthy woman who developed thrombocytopenia and hemolysis at 34 wk gestation. The patient underwent delivery after transfusion with platelets and RBCs. The suspicion of TTP was raised, and plasma exchange was begun by the third hospital day. On the seventh day of treatment, the patient developed shortness of breath, and an echocardiogram showed global hypokinesis with an ejection fraction of 25%. Plasma infusion, one unit q 4 h, was initiated in addition to the daily plasma exchange. The patient improved and her ejection fraction normalized. plasma exchange and infusion and corticosteroids were gradually tapered off. von willebrand factor (vWF) protease activity in the plasma upon transfer was completely deficient with the presence of inhibitor. This case illustrates that vWF protease assay and detection of inhibitor can be used for the diagnosis of TTP during pregnancy; and a severe cardiomyopathy in TTP can be reversed rapidly with combined plasma exchange and infusion.- - - - - - - - - - ranking = 0.4keywords = liver (Clic here for more details about this article) |
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