Mitral valve replacement due to mitral valve regurgitation in patient with Fabry disease

Mitral valve replacement due to mitral valve regurgitation in patient with Fabry disease

The 12th Annual Scientific Meeting NS) after Ringer injection. The increase of ANP/BNP had a negative correlation with LAVI (r 5 -0.30, p ! 0.05), sugg...

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The 12th Annual Scientific Meeting NS) after Ringer injection. The increase of ANP/BNP had a negative correlation with LAVI (r 5 -0.30, p ! 0.05), suggesting that the secretion reserve of ANP after volume-overload is smaller in patients with enlarged left atrium. In conclusion, the change of ANP/BNP may be an index to reflect atrial functional reserve of natriuretic peptide secretion in patients with SR.

006 Mitral valve replacement due to mitral valve regurgitation in patient with Fabry disease MAKIKO MIYATA, TAKAMASA SATO, MASUMI IWAI-TAKANO, HITOSHI SUZUKI, TOSHIYUKI ISHIBASHI, YASUCHIKA TAKEISHI First Department of Internal Medicine, Fukushima Medical University, Fukushima, Japan Mitral regurgitaion (MR) in patients with Fabry disease is one of precipitating factors that induce heart failure (HF), however, there is no documented optimal treatment for MR in such patients.We report a case of severe MR treated by mitral valve replacement (MVR) in a 54-year-old man with Fabry disease. He was admitted by renal failure and diagnosed as Fabry disease at 52-year-old. For past 2 years, he has repeatedly hospitalized for HF. In October 2007, he admitted because of HF. Echocardiogram showed severe left ventricular (LV) hypertrophy and dilatation, but LVEF was 72%. The mitral valve and papillary muscles were hypertrophic and shortened, and these morphological changes caused severe MR. Estimated right ventricular systolic pressure by Doppler echocardiography was 48 mmHg. We considered the indication of surgical treatment for severe MR, however, the medical treatment was selected because of his renal dysfunction. In December 2007, he admitted for HF again. Echocardiogram showed the worsening of LV function (LVDd 77 mm, LVDs 42 mm, and LVEF 66 %). Because it was difficult to control HF by medical treatment, he underwent MVR and tricuspid valve plasty. The hypertrophy in anterior and posterior leaflets of mitral valve was revealed during operation, and pathologic diagnosis of mital valve was compatible for Fabry disease. His symptoms and functional status of HF were improved after MVR.

007 Assessment of Cardiac Function by Intraventricular Time-intensity Curve Analysis Using Magnetic Resonance Perfusion Imaging SATOSHI OKAYAMA, SHIRO UEMURA, SATOSHI SOMEKAWA, YUKIJI TAKEDA, YOSHIHIKO SAITO The First Department of Internal Medicine, Nara Medical University, Nara, Japan Objective: To examine whether cardiac functuon can be assessed by intraventricular time-intensity-curve (TIC) analysis obtained from magnetic resonance (MR) perfusion imaging. Subjects and Methods: Cardiac MR imaging was performed in 25 subjects without arrhythmia: 2 healthy volunteers, 10 patients with ischemic heart disease, 3 with valvular disease, and 10 with cardiomyopathy. MR perfusion images were acquired during a total of 40 cardiac cycle, at the end-diastolic phase, with a frame rate of 1 image/heartbeat. Each 1 ml bolus injection of Gd-DTPA at a rate of 4 ml/s was followed by an injection of 15 ml of normal saline at the same injection rate. Perfusion was quantified using TIC analysis. A region of interest (ROI) was placed in the area of right and left ventricle. Time interval between right and left intraventricular peak signal intensity (peak interval) was measured in TIC, and represented by heartbeat. Results: TIC showed bimodal patten in 19 patients, whereas monomodal patten in 6 patients with severe heart failure or misplacement of ROIs because of poor breath-hold. Peak interval was significantly longer (p ! 0.01) in 11 patients with poor LVEF (! 40%) or valvular disease (13.4 6 6.5) than with 14 good LVEF (O 60%) and no valvular disease (8.3 6 1.5). Conclusion: Intraventricular TIC analysis may be useful for the assessment of cardiac function.

008 Diabetes Mellitus Exacerbates Diastolic Dysfunction through Activation of Renin-Angiotensin System in Hypertensive Rats SHIGEFUMI FUKUI, YOSHIHIRO FUKUMOTO, JUN SUZUKI, KENYA SAJI, JUN NAWATA, KOICHIRO SUGIMURA, YUTAKA MIURA, TSUYOSHI SHINOZAKI, YUTAKA KAGAYA, HIROAKI SHIMOKAWA Department of Cardiovascular Medicine Tohoku University Graduate School of Medicine, Sendai, Japan Background: We have recently demonstrated that diabetes mellitus (DM) is an independent predictor in patients with diastolic heart failure in our cohort study. However, its precise mechanisms of DM on diastolic dysfunction remain to be fully elucidated. Methods: We made 5 groups of Dahl salt-sensitive rats; normal diet with or without streptozotocin (STZ) injection, a high-salt (5% NaCl) diet with or without STZ injection (HT + DM and HT), and HT + DM treated with olmesartan



(1 mg/kg/day, no blood pressure-lowering dose) (n 5 12e20 each). Results: At week 17, cardiac diastolic dysfunction with preserved systolic function was noted in the HT group and was most prominently noted in the HT + DM group (E/A, 1.2 6 0.4 vs. 1.6 6 0.4 vs. 2.7 6 0.5, P ! 0.05; tau, 18 6 1 vs. 17 6 2 vs. 13 6 1 msec, P ! 0.05; HT + DM vs. HT vs. control), characterized by enhanced cardiac fibrosis. Myocardial expressions of collagen III, transforming growth factorbeta2, and angiotensin-converting enzyme (ACE) and myocardial oxidative stress (evaluated by 4-hydroxy-2-nonenal-modified protein) were mostly enhanced in the HT + DM group. Importantly, there was a positive correlation between the extent of diastolic dysfunction (tau) and that of myocardial ACE expression (n 5 28, R 5 0.46, P ! 0.05). All these cardiac abnormalities by DM and HT were significantly ameliorated in the olmesartan group, independent of blood pressure. Conclusions: These results suggest that DM exacerbates diastolic dysfunction through cardiac fibrosis, activation of cardiac RAS, and increased oxidative stress.

009 Clinical outcome of Fontan operation in the patients with impaired cardiac function YASUHIRO KOTANI1, SHINGO KASAHARA1, YASUHIRO FUJII1, KO YOSHIZUMI1, YU OSHIMA1, SHIN-ICHI OTSUKI2, TEIJI AKAGI3, SHUNJI SANO1 1 Department of Cardiovascular Surgery, Okayama University, Okayama, Japan, 2 Department of Pediatrics, Okayama University, Okayama, Japan, 3Cardiac Care Unit, Okayaka University Hospital, Okayama, Japan Background: Impaired cardiac function has been an increasing factor of early and late mortality after Fontan operation. We evaluated the clinical outcome of Fontan operation in patients with impaired cardiac function. Methods and Results: A retrospective review was performed on 217 patients who had undergone Fontan operation between 1991 and 2007. Twenty-nine (13%) of the patients had impaired cardiac function (EF of less than 50%). Median age at operation was 3 (range, 1e31 years) years. Ventricular morphology was right in 20 patients and others (left and biventricle) in 9 patients. Heterotaxy syndrome was present in 8 patients. Previous palliation included bidirectional Glenn anastomosis in 24, BT shunt in 2, and pulmonary artery banding in 2. Pre-operative EF was 42.8 6 5.6 (range, 30e50) %. Significant atrioventricular valve regurgitation was noted in 4 patients. Percutaneous oxgen saturation (SaO2) was 82.1 6 5.2%. Pulmonary artery index and mean pulmonary artery resistance were 296 6 102 mm2/m3 and 11.1 6 3.2 mmHg, respectively. All 29 patients were tolerated the Fontan operation with no early death. There were 2 late deaths and 2 re-operations. At median follow up of 7.5 (1e19) years, EF was 59 6 15 (35e82)%. SaO2 increased to 92 6 2%. Cardiothoracic ratio and BNP were 51 6 8% and 22 (9e382)pg/ml, respectively. Three patients had heart failure. Conclusions: Acceptable clinical outcomes have been observed at intermediate follow-up of the Fontan operation in patients with impaired cardiac function.

010 Prognostic value of combination of NT-proBNP and cardiac troponin T in patients admitted for CHF with preserved LV systolic function HIROYUKI NARUSE1, JUNNICHI ISHII2, KOUSUKE HATTORI1, MAKOTO ISHIKAWA1, MASANORI OKUMURA1, SHINO KAN1, SHIGERU MATSUI1, SHINNICHIRO MORIMOTO1, HITOSHI HISHIDA1, YUKIO OZAKI1 1 Division of Cardiology Department of Internal Medicine Fujita Health University, Aichi, Japan, 2Department of Joint Research Laboratory of Clinical Medicine, Fujita Health University, Aichi, Japan We prospectively investigated the prognostic value of NT-proBNP and cardiac troponin T (TnT) on admission in 219 patients (NYHA class OII) hospitalized for worsening chronic heart failure (CHF) with preserved left ventricular (LV) systolic function (LV ejection fraction O40%). Results: During a mean follow-up period of 933 days, there were 37(16.9%) cardiac deaths. On a multivariate Cox regression analysis including 7 clinical variables, NT-proBNP (per 10-fold increase) and TnT (per 1 ng/ml) were independently associated with cardiac mortality (RR 5 2.78, P 5 0.001; and RR 5 3.35, P 5 0.01). Cardiac mortality rates according to NT-proBNP O 6462 pg/ml (determined by ROC curve) and/or TnT O 0.029ng/ml (determined by ROC curve) were shown in Table. Conclusion: The combination of NT-proBNP and TnT may be a highly effective means for risk stratification in patients hospitalized for worsening CHF with preserved LV systolic function.

NT-proBNP O6462pg/ml TnT O0.029ng/ml Cardiac death, n(%)

I (n595)

II (n531))

III (n529)

IV (n564)

(e) (e) 6 (6.3%)

(þ) (e) 4 (12.9%)

(e) (þ) 5 (17.2%)

(þ) (þ) 22 (34.4%)