A Case of Cardiac Amyloidosis Following Familial Mediterranean Fever

A Case of Cardiac Amyloidosis Following Familial Mediterranean Fever

S194 Journal of Cardiac Failure Vol. 21 No. 10S October 2015 who developed infections during hospitalization of AHF. Methods: We enrolled 217 patients...

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S194 Journal of Cardiac Failure Vol. 21 No. 10S October 2015 who developed infections during hospitalization of AHF. Methods: We enrolled 217 patients (Age: 78612 years old, Male/Female: 115/102) hospitalized for AHF without signs of infection on admission (from Jan 2011 through Dec 2012). Results: 26 patients (12.0%) developed nosocomial infections during hospitalization (infectious group). 11 patients of them had respiratory infections and 9 patients had urinary tract infections. Compared with AHF patients without infections (non-infectious group), infectious group had a tendency of high hospital mortality (15.4% vs 5.8%, P50.088) and significantly longer length of hospital stay (31(26-45) vs 19(12-27) days, P!0.001, data given as median (interquartile range)). In multivariate analysis, aging (Odds ratio (OR) 1.070, P50.015), the history of cerebral vascular disorder (CVD) (OR 3.300, P50.032), high glucose level (OR 1.010, P50.006), and lack of ACE inhibitor or ARB (OR 0.312, P50.026) on admission were independent factors for nosocomial infection. Conclusions: This study suggested negative impact of the nosocomial infection on AHF. It may be necessary to be careful for the treatment of AHF patients with these infectious risks.

OP42-4 Clinical Features of Patients with Acute Decompensated Heart Failure Admitted in Winter Season HITOSHI SUSAWA, SHINSUKE MIKAMI, HAJIME TAKEMOTO, KENGO KOBAYASHI, HARUKI TANAKA, KOUICHI TANAKA Department of Cradioligy, Miyoshi City Central Hospital Background: Cardiovascular events have variations depends on the season. Clinical features of heart failure (HF) patients in the winter season were not fully understood. Methods: We used a cohort from our hospital 2010-2013, comprising a total of 273 patients who were admitted owing to HF. Winter was defined as the period between December and March. Results: Of these patients, 103 (38%) were admitted in winter. The HF patients admitted in winter were older, and had a higher prevalence of cognitive decline, hypertension and diabetes mellitus than the patients admitted in other seasons. Patients with conditions categorized as clinical scenario one tended to be admitted more commonly in winter. There were no differences in left ventricular ejection fraction, but significant increase in e/e’ between patients who admitted in winter and in other seasons. Readmission within one year was similar in HF patients admitted in the winter than in other seasons. Conclusions: HF admission was often observed in the winter season and these patients were older, and had higher prevalence of hypertension and diabetes mellitus, but had similar readmission within one year than other seasons.

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precise mechanisms of trastuzumab-related cardiotoxicity as well as its frequency and clinical course in Japanese patients are still unclear. In this study, we investigated the frequency and extent of trastuzumab-induced cardiotoxicity. Methods: We enrolled sequential 176 breast cancer patients treated with trastuzumab, and evaluated their LVEF by echocardiography before and after the treatment in our hospital from January 2008 to March 2012. Diagnosis of CTRCD was defined as a reduction of LVEF greater than 10% or as less than 53% of LVEF. Results: LVEF after trastuzumab treatment was significantly reduced rather than that before the treatment (63.768.4% vs. 69.064.9%, p!0.001), and average reduction of LVEF was 5.266.7%. Among them, 13 patients (7.4%) showed LVEF less than 53% after the treatment, 42 patients (23.9%) showed more than 10% reduction in LVEF. Many patients recovered their LVEF after the treatment, however, patients with reduced LVEF (LVEF!50%) showed sustained cardiac dysfunction and received. Conclusions: Trastuzumab-related myocardial dysfunction was frequently observed in breast cancer patients. For severely damaged patients, it is necessary for cardiologists to prevent its deterioration promptly.

OP43-2 A Case of Dilated Cardiomyopathy Associated with CD36 Deficiency SHIN KAWASOE1, TAKURO KUBOZONO1, MASAAKI MIYATA1, SHOJI FUJITA2, TSUYOSHI YAMAGUCHI2, HIROYUKI TORII2, MITSURU OHISHI1 1 Department of Cardiovascular Medicine and Hypertension, Graduate School of Medical and Dental Sciences, Kagoshima University; 2Department of Cardiovascular Medicine, Kagoshima Association Hospital The CD 36 molecule is cell surface antigen and associated with transport of longchain fatty acid on the myocardial membrane. It is reported that the CD36 deficiency exists in 39% of patients with hypertrophic cardiomyopathy. However, the patients with dilated cardiomyopathy associated with CD36 deficiency are rare. We report a 36-year-old male patient. He was born healthy, and had not been pointed out the abnormality in medical checkup. In May 2014, he felt palpitation and shortness of breath even in mild exercise. He admitted to the hospital for treatment of heart failure due to left ventricular dysfunction. Heart failure was improved by administration of dopamine and furosemide. He transferred to our hospital for further examination and treatment because the scintigraphy showed complete loss of myocardial accumulation of 123I-BMIPP. The echocardiography demonstrated marked dilation and reduced contractility of left ventricle (LVDd 75.8 mm, EF 31%), and the coronary angiography did not reveal organic stenosis and spasm of coronary artery. In addition, the flow cytometric analysis of CD36 in granulocute, lymphocyte and platelet identified a type1 CD36 deficiency. We treated with increasing dose of ACE-inhibitor (enalapril 5 mg/day) and beta-blocker (carvedilol 20 mg/day), and left ventricular systolic function was gradually improving (LVDd 65 mm, EF 47%).

The Effect of Regular Carvedilol Therapy before Admission for New-onset Acute Heart Failure KOJI MURAI, KUNIYA ASAI, YOSHIAKI KUBOTA, YAYOI TUKADA, WATARU SHIMIZU Nippon Medical School Hospital

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Background: Although carvedilol reduces mortality in patients with chronic heart failure (HF), it has not investigated whether carvedilol, which has been prescribed before onset of HF, has beneficial effects on acute phase of de novo acute heart failure (AHF). Methods: Continuous patients who admitted for AHF in our hospital were prospectively registered (N5320). Nine patients treated with endotracheal intubation for non-cardiac reason and 22 patients who were unknown about beta blocker therapy (6 patients) and who were prescribed beta blockers other than carvedilol before admission (16 patients) were excluded. One hundred five patients were excluded for having a history of admission for HF. Finally, we compared the baseline characteristics and in-hospital mortality between new-onset AHF patients with carvedilol before admission (carvedilol group, N534) and those without carvedilol (non-carvedilol group, N5150). Results: Patients with carvedilol group were higher proportion of ischemic cardiomyopathy (P!0.01), and higher heart rate (P50.016), and treated more with furosemide (P!0.01), and lower proportion of endotracheal intubation for acute cardiogenic pulmonary edema (P50.02). In-hospital mortality was not different between patients with carvedilol group and those with non-carvedilol group. Conclusions: It is possible that carvedilol treatment before onset of HF had a beneficial effect in management of respiratory support in AHF patients without histories of HF admission.

A 67-years-old man with a history of kidney transplantation, was admitted with heart failure. Echocardiography showed the diffuse left ventricular hypertrophy and contractile dysfunction with left ventricular ejection fraction (LVEF) of 46%. Over the next few days, LVEF decreased more. Although we had suspected myocarditis at first, the pathological findings of endomyocardial biopsy revealed cardiac amyloidosis, in which immunostaining examination confirmed AA type amyloidosis. His recurrent episodes of high-grade fever of unknown origin promoted us to perform gene analysis. The results of genetic analysis showed a mutation in exon 10 (p.M694I) of the Mediterranean fever gene. In Japan, several Familial Mediterranean fever patients with M694I mutation who had complicated with renal amyloidosis have been reported, however, the case complicated with cardiac amyloidosis is rare. Despite intensive treatment including maximum inotropic support, his hemodynamic balance was broken, resulting in death.

OP43-1 Cardiotoxicity Associated with Trastuzumab in Breast Cancer Patients: Definition of Cancer Therapeutics-Related Cardiac Dysfunction (CTRCD) WATARU SHIOYAMA, MIKIO MUKAI, TORU OKA, EIROU TONE, NOBUHISA AWATA, MASATSUGU HORI Department of Cardiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka, Japan Background: Trastuzumab is a monoclonal antibody targeting human epidermal growth factor receptor 2 (HER2), and is used for the treatment of breast cancer overexpressing HER2 receptors. Trastuzumab occasionally induces cardiotoxicity as asymptomatic left ventricular ejection fraction (LVEF) reduction. However, the

A Case of Cardiac Amyloidosis Following Familial Mediterranean Fever SHIN MAKABE, HIROYUKI WATANABE, HIROSHI ITO Department of Internal Medicine Division of Cardiovascular and Respiratory Medicine, Akita University School of Medicine, Akita, Japan

OP43-5 Marked Improvement of Left Ventricular Function after Calcium Supplementation in a Patient with Severe Hypocalcemia Secondly to Idiopathic Hypoparathyroidism TAKURO SHINSATO, TAKEKO KAWABATA, YUKI HAMAMOTO, TOMOYOSHI ARIKAWA, HIROKAZI SHIMONO Department of Cardiology, Sendai Medical Association Hospital Hypocalcemia is a rare cause of cardiomyopathy. We report a case of cardiomyopathy associated with hypocalcemia secondary to idiopathic hypoparathyroidism. A 82-year-old woman was admitted, because of exertional dyspnea and leg edema. Chest X-ray showed pleural effusion and cardiomegaly. Echocardiography revealed global hypokinesia with mild dilatation of the left ventricle, moderate mitral regurgitation, moderate tricuspid regurgitation, and left ventricular (LV) systolic dysfunction with an ejection fraction (EF) of 32%. On physical examination,