Mechanism and Clinical Significance of Mitral Regurgitation in Cardiac Sarcoidosis

S22 Journal of Cardiac Failure Vol. 23 No. 10S October 2017

Oral Session O1-1 A Paradoxical Association between Valvular Heart Disease and Coronary Artery Disease: A Study by Covariance Structure Analysis Risa Fukumoto, Makoto Kawai, Yasunori Inoue, Michihiro Yoshimura; Division of Cardiology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan Background: Pathogenesis of valvular heart disease (VHD) must have been modifying by lifestyle change and alteration in the environment. It is thus conceivable that contemporary VHD is affected largely by atherosclerotic and calcific process, which is similar to that observed in coronary artery disease (CAD). This study was designed to examine a possible comorbid condition of VHD and CAD in the context of aging, gender, and dyslipidemia. Method: Covariance structure analysis were proposed to evaluate a possible comorbid condition of VHD and CAD regarding the effects of aging, gender, and the serum low-density lipoprotein cholesterol (LDL-C) levels in 3,457 inpatients. Result: The path models revealed that aging was associated with VHD and CAD severity (P < .001 for each). There was, however, an inverse association regarding the comorbid condition between VHD and CAD (P < .001). To prove this inverse association, there was a gender difference; females were associated with VHD and males with CAD. In addition, the LDL-C levels were not associated with VHD, whereas those were highly associated with CAD (P < .001). Conclusion: Aging was generally a promoting factor of VHD and CAD; however, the incidence of respective diseases showed a significant conflicting relationship. The differences in gender and the LDL-C levels between VHD and CAD support likely presence of unknown diverse mechanisms on top of the common cascade of atherosclerosis and calcification.

O1-2 Balloon Aortic Valvuloplasty Might Be a Safe Therapeutic Option for Acute Decompensated Heart Failure Due to Severe Aortic Valve Stenosis Saaya Fuda, Tsuyoshi Takada, Hiroki Saito, Hiroyuki Satake, Hironori Kanemitsu, Koji Fukuda, Morihiko Takeda, Nobuyuki Shiba; Department of Cardiovascular Medicine, International University of Health and Welfare Hospital Background: Acute decompensated heart failure (ADHF) due to atrial valve stenosis (AS) has been increasing in highly aging society as Japan. However, little is known regarding the usefulness of balloon aortic valvuloplasty (BAV) in AS patients with ADHF. We report the initial results of BAV in our hospital, which started from October 2016. Methods: We retrospectively analyzed the characteristics, in-hospital death and hospitalization days of AS patients admitted due to ADHF. We included 11 patients who were medically treated in October 2015 to May 2016 (G1), and 8 patients who underwent BAV in October 2016 to May 2017 (G2). Results: Female in G1 and G2 accounted for 72% and 100%, respectively. Mean age was 89.1 y.o. in G1 and 88.6 y.o. in G2. Mean aortic valve area, mean max velocity, and median BNP level in G1 and G2 were, 0.88 cm2 and 0.46 cm2, 3.3m/sec and 4.5m/sec, and 568 pg/ml and 513 pg/ ml, respectively. Three patients in G1 died and two of them died at 8 days after admission, but no patients died in G2. Mean change of pressure gradient after BAV was 25.2 mmHg. Median hospitalization days of G1 and G2 were 19 days and 26.5 days, respectively. Conclusion: We had no in-hospital deaths in patients underwent BAV, suggesting that BAV could be a safe and effective therapeutic option for ADHF due to AS.

O1-3 Worsening Heart Failure after Transcatheter Aortic Valve Implantation in Patients with Severe Aortic Stenosis Nobuhiko Haruki1, Takeshi Ohnohara2, Yoshiharu Kinugasa1, Shingo Harada2, Koichi Matsubara1, Kiyotaka Yanagihara1, Yoshinobu Nakamura2, Masahiko Kato1, Motonobu Nishimura2, Kazuhiro Yamamoto1; 1Division of Cardiovascular Medicine, Department of Molecular Medicine and Thrapeutics, Faculty of Medicine, Tottori University, Yonago, Japan; 2Department of Surgery Division of Organ Regeneration Surgery, Faculty of Medicine, Tottori University, Yonago, Tottori Background: Worsening heart failure (WHF) after transcatheter aortic valve implantation (TAVI) is occasionally observed. The aim of this study was to determine the characteristics of the patients who worsened HF after TAVI. Methods: A total of 44 patients with symptomatic severe aortic stenosis (AS) who underwent TAVI (age: 85 ± 5years) were included for this study. All patients were performed transthoracic echocardiography before and 1 week after TAVI. WHF was defined as a case of unplanned re-hospitalization or required treatment intensification for HF later than 1 month after the procedure. We clarified the association between WHF and clinical and echocardiographic parameters. Results: Ten patients had experienced WHF after TAVI. Baseline characteristics such as HF severity, BNP level, operative risk score and comorbidities were similar between the two groups. Compared to non-WHF patients, those with WHF were older (84 ± 5 vs. 88 ± 5years, P < .05), high E/A ratio (0.7 ± 0.3 vs. 1.1 ± 0.6, P < .05), and lower trans-aortic velocity and pressure gradient before TAVI (5.1 ± 0.6 vs. 4.6 ± 0.6m/sec, 62 ± 16 vs. 49 ± 14 mmHg, respectively, P < .05). In addition, changes in left atrial (LA) longitudinal strain value before and 1week after TAVI were lower in WHF patients compared to non-WHF patients (−1.5 ± 2.1 vs. 3.1 ± 4.3%,

P < .01). Conclusions: WHF was observed in one out of five patients later than 1 month after TAVI, and was associated with LV diastolic function and changes in LA function.

O1-4 Clinical Outcomes of TAVI for Aortic Stenosis Patients with Impaired Cardiac Function Kei Torikai1, Toru Kuratani2, Koichi Maeda1, Isamu Mizote3, Toshinari Ohnishi3, Takayoshi Ueno2, Koichi Toda1, Satoshi Nakatani3, Yasushi Sakata3, Yoshiki Sawa1; 1 Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan; 2Department of Minimally Invasive Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Osaka, Japan; 3Department of Cardiology, Osaka University Graduate School of Medicine, Suita, Osaka, Japan The subject of TAVI involves high-surgical risk patients including low EF. However, efficacy of this treatment for such patients is still unknown. So, we reviewed clinical outcomes of TAVI for patients with severe aortic stenosis (AS), complicated with impaired cardiac function. We have experiences of 50 cases with low EF equal or less than 40% in all 440 TAVI cases. In the cohort, the median of age was 83 years old. Twenty cases (40%) was female, and STS-PROM was calculated as 11.05% at average. Urgent or emergency cases were 14(28%). Used TAVI devices were SAPIEN in 37 patients (74%), CoreValve in 10 (20%), Acurate in 3 (6%). Trans-femoral approach was chosen in 24 cases (48%), and alternative approach in 26 cases (52%) Twenty patients (40%) required intended ECMO support during the procedure. As a result, in all cases, procedural success was achieved without any conversion to open heart surgery. Not-intended ECMO support was needed in only one case because of instable hemodynamics. Thirty day mortality was only one case (2%), and the reason for death was acute coronary syndrome. The incidence of stroke was 0%. Kaplan-Meire curve of overall survival showed 87, 75, 75% at 1, 3, and 5 year postoperatively. TAVI for AS patients with cardiac dysfunction was very feasible treatment option with acceptable early and mid-term outcomes.

O1-5 Efficacy and Safety of Transcatheter Mitral Valve Repair Using MitraClip System for Extra Low Left Ventricular Function Patients Yoshifumi Nakajima, Sam Dawkins, Krissada Meemook, Mamoo Nakamura, Asma Hussaini, Saibal Kar; Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA Background: Percutaneous mitral valve repair (MVR) using MitraClip system is a novel technique to treat patients with severe mitral regurgitation (MR) with surgical high risk. In the United States, trials were started in 2003 and the device was approved in 2013. In trials, patients left ventricle ejection fraction (LVEF) less than 20% were excluded. Little is known of the procedure for those patients. This study serves to evaluate clinical outcomes of extra low LVEF MR patients treated with the MitraClip system. Methods: From 2005 to 2016, we treated total 547 patients with symptomatic moderateto-severe or severe MR using the MitraClip system in our institute. Among those patients, 29 patients with LVEF less than 20%, were evaluated. Results: Mean age at the procedure was 66.6 ± 12.6 years, 76% of patients were male, and mean EF was 15.6 ± 2.5%. The Society of Thoracic Surgeons risk of mortality for replacement was 11.6 ± 12.1. Reason of MR were degenerative: 3%, ischemic: 45%, and non-ischemic: 52%. The MitraClip implant rate was 97%, procedure success rate was 90%, and there was no procedure related complication. Mean number of clips was 1.97 (range 0–4). One patient died and 1 patient needed hospitalization due to heart failure in 30 days. Conclusion: Percutaneous MVR showed effectiveness and safety in extra low LVEF group in short term.

O2-1 Mechanism and Clinical Significance of Mitral Regurgitation in Cardiac Sarcoidosis Makoto Takamatsu1, Hiromi Tsuchiya1, Hikaru Kimura1, Hiroki Kasai2, Yoshikazu Yazaki1; 1Department of Cardiovascular Medicine, Saku Central Hospital Advanced Care Center, Saku, Japan; 2Department of Cardiovascular Medicine, NHO Mastmoto Medical Center Matsumoto Hospital, Matsumoto, Japan Backgrounds: Mitral regurgitation (MR) is sometimes observed in cardiac sarcoidosis (CS), and might be related to worsening heart failure. However, mechanism and clinical significance of MR associated with CS remains unknown. Methods and Results: To clarify this issue, we retrospectively analyzed consecutive 41 CS patients, and identified 14 patients with moderate to severe MR. We compared clinical findings, treatment and outcome between patients with and without MR. Mechanism of MR consists of prolapse in 5, and tethering in 9 including 6 symmetric and 3 asymmetric leaflet tethering. Incidence of complete AV block was significantly greater in patients with tethering MR (P < .05), whereas the incidence of thinning in the basal septum was similar. Significantly reduced left ventricular (LV) ejection fraction (P < .01) and increased LV end-diastolic volume index (P < .01) were observed in patients with tethering MR. Cardiac resynchronization therapy in 4 and mitral valve replacement in 2 of the 14 with MR

The 21st Annual Scientific Meeting

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were received before or after corticosteroid therapy. A cardiac event including cardiac death and arrhythmias or heart failure requiring hospitalization occurred 9 patients during the mean follow-up of 79 months. Cardiac-event free survival was significantly worse in patients with MR due to tethering (P < .001) Conclusions: MR associated with CS has variety of mechanism. Existence of tethering MR is strongly related to worsening heart failure and poor outcome in CS.

757 pg/ml, diagnostic accuracy for increased PCWP was as follows: sensitivity 0.889, specificity 0.867, positive predictive value 0.973, negative predictive value 0.591. Conclusion: Compared to conventional echocardiographic parameters, BNP is more useful to predict the increase of PCWP in AS patients.

O2-2

Surgical Outcome of Severe Tricuspid Valve Regurgitation Developed Long after Left-sided Valvular Surgeries Takeshi Kashimura1, Hiromi Kayamori2, Shinya Fujiki2, Takayuki Wakasugi2, Yuka Hayashi2, Tohru Minamino2; 1Department of Advanced Cardiopulmonary Vascular Therapeutics, Niigata University Graduate School of Medicine and Dental Sciences, Niigata, Japan; 2Department of Cadiovascular Biology and Medicine, Niigata University Graduate School of Medicine and Dental Sciences, Niigata, Japan

Strategy for the Management of Unrepaired Ebstein’s Disease in Adult Patients Toshimi Koitabashi1, Teppei Fujita1, Mayu Yazaki2, Toyoji Kaida1, Yuuichirou Iida1, Syunsuke Ishii1, Emi Maekawa1, Yoshiyasu Minami1, Takayuki Inomata2, Junnya Ako1,3; 1 Department of Cardiovascular Medicine, Kitasato University School of Medicine, Sagamihara, Japan; 2Shizuoka City Shimizu Hospital, Shizuoka, Japan; 3Kitasato University Kitasato Institute Hospital, Tokyo, Japan Background: Ebstein’s Disease (ED) is a rare congenital heart disease with the tricuspid valve dysfunction. Little is known about the clinical course and the optimal strategy for the management of adult ED patients. Method and Result: The clinical course of six consecutive adult patients of unrepaired ED was investigated. Among them, four cases including three asymptomatic pregnant women (32, 36 and 37 y.o.) and one elderly patient (72 y.o.) with severe tricuspid regurgitation (TR) and chronic atrial fibrillation (Af) had no cardiac event. On the other hand, two cases required surgical intervention. One younger patient (32 y.o.) with atrial septal defect and severe TR had surgical repair due to progressive polycythemia by hypoxia. Another elder patient (64 y.o.) with severe TR had tricuspid valve repair due to exacerbated heart failure induced by new onset of Af. Conclusion: The clinical course of unrepaired ED in adult patients is varied. Careful follow-up is important not to miss the optimal timing of surgical intervention in adult ED patients irrespective of their symptom.

O2-3 The Impact of Diastolic Dysfunction on Postoperative Tricuspid Regurgitation and Heart Failure Readmission in Patients with Severe Aortic Stenosis Keigo Yamashita, Takehisa Abe, Nobuoki Tabayashi, Yoshihiro Hayata, Tomoaki Hirose, Yoshio Kaniwa, Rei Tonomura, Hiroshi Nishikawa, Shinya Yokoyama, Shigeki Taniguchi; The Department of Thoracic and Cardiovascular Surgery, Nara Medical University, Nara, Japan Objectives: A clinical significance of left ventricular diastolic dysfunction (LVDD) in patients with severe aortic stenosis (AS) is not fully elucidated. This study sought to evaluate the impact of LVDD on postoperative tricuspid regurgitation and heart failure readmission after aortic valve replacement (AVR) for severe AS. Methods and Results: From January 2007 to December 2016, we consecutively enrolled 101 patients undergoing isolated AVR (+CABG) for severe AS. Patients with LVDD (E/E’ ≥15) were compared with those without LVDD (E/E’ <15). Patients with LVDD showed significant difference in the frequency of postoperative tricuspid regurgitation more than mild 1, 3, and 5 years after surgery (P = .023, 0.046, and 0.025, respectively). KaplanMeier analysis showed that patients with LVDD had a significantly higher rate of heart failure readmission (P = .023) compared with those without LVDD. A multivariate Cox hazards model showed that the independent predictors of heart failure readmission were dialysis (adjusted hazard ratio 5.094, 95% confidence interval 1.654 to 15.690, P = .005) and persistent atrial fibrillation (adjusted hazard ratio 19.158, 95% confidence interval 1.677 to 218.822, P = .017). Conclusions: Presence of preoperative diastolic dysfunction was associated with postoperative tricuspid regurgitation and heart failure readmission. Especially, patients with dialysis or atrial fibrillation have a higher risk of heart failure readmission.

O2-4 Plasma BNP Level Is a Strong Parameter for Detecting Increased Pulmonary Capillary Wedge Pressure in Patients with Severe Aortic Stenosis Masayoshi Oikawa, Atsushi Kobayashi, Satoshi Suzuki, Takamasa Sato, Akiomi Yoshihisa, Hiroyuki Kunii, Kazuhiko Nakazato, Shu-Ichi Saitoh, Takafumi Ishida, Yasuchika Takeishi; Department of Cardiovascular Medicine, Fukushima Medical University, Fukushima, Japan Background: An increased pulmonary capillary wedge pressure (PCWP) is a strong risk factor for the development of congestive heart failure in patients with aortic stenosis (AS). We investigated whether conventional parameters are useful to detect the elevation of PCWP in AS patients. Methods: Ninety-six patients with severe AS (aortic valve area <1.0 cm 2 ) were analyzed in this study. Cardiac catheterization, echocardiography, and plasma B-type natriuretic peptide (BNP) analysis were performed in all patients. Results: PCWP positively correlated with log BNP (r = 0.563) and left ventricular (LV) end-systolic volume index (LVESVI, r = 0.484), but weakly correlated with E/A ratio (r = 0.284), E/E’ ratio (r = 0.243), LV ejection fraction (r = 0.384) and left atrial volume index (LAVI, r = 0.386). Multivariate logistic regression analysis revealed that both log BNP (OR 9.94, 95% CI 2.349–103.565, P = .013) and LAVI (OR 1.063, 95% CI 1.010–1.137, P = .035) were independent predictors of increased PCWP (>18 mmHg). Receiver operating characteristic curve analysis revealed that log BNP showed larger area under the curve for detecting increased PCWP compared to LAVI (0.897 vs. 0.753, P = .07). When the cutoff value of BNP was determined as

O2-5

Background: Early surgical treatment for severe tricuspid regurgitation (TR) is recommended before right or left heart failure develops, however those who have developed severe TR late after left-sided valvular surgery are prone to be treated noninvasively because the risk of repeated surgery is concerned. Methods: Nine patients with severe symptomatic TR treated surgically in our hospital long after left-sided valvular disease were reviewed retrospectively. Results: Average age of the patients were 67 ± 4 years. Prior surgeries had been performed at their age of 37 ± 11 years, and heart failure symptoms had appeared 11 ± 8 years before subsequent surgeries. The second surgeries were performed with aortic valve replacement in 3, with mitral valve replacement in 4, and without left-sided valve surgery were in 2. None of the nine patients died perioperatively, however 4 patients died during a mean follow up period of 2.3 ± 1.7 years. Right heart catheterization was performed after surgery in 6 patients. Systolic regurgitant pressure of right atrium were prone to decrease (P = .66) and cardiac index increased significantly (P = .033), however mean pressure of right atrium and pulmonary artery wedge pressure increased considerably in some patients. Conclusion: Operative risk of surgical treatment for TR late after left-sided valvular surgery is not high, however long-term prognosis is poor with incomplete hemodynamic improvement in some patients.

O3-1 Antegrade Diastolic Pulmonary Artery Flow Implies Right Ventricular Restrictionin Adult Cases Yuri Umeta, Hiroyuki Watanabe, Takako Iino, Mai Shimbo, Wakana Sato, Kumiko Watanabe, Shin Makabe, Hiroshi Ito; Depertment of Cardiovascular Medicine, Akita University Graduate School of Medicine Background: Premature opening of pulmonary valve and subsequent antegrade diastolic pulmonary artery flow (ADPAF) reflect restrictive right ventricular (RV) physiology in children. However, the study of ADPAF has been strangely neglected in adult cases. Purpose: To clarify the clinical implication of ADPAF in adult cases. Methods and Results: We studied 23049 consecutive adult patients who underwent echocardiography in our hospital from 2008 to 2015. ADPAF was found in 17 patients. Causative diseases included congenital heart diseases, cardiomyopathy, severe pulmonary valvular regurgitation, and pericardiac diseases. On cardiac catheterization, simultaneous recording of RV and PA pressures revealed the dip-and-plateau configuration of RV diastolic pressure which exceeded PA pressure during atrial systole. These data suggest that ADPAF reflects the inversion phenomenon between right ventricle and pulmonary artery pressures under the condition of right ventricular restrictive physiology. All patients were divided into two groups based on TAPSE. During a mean follow-up time of 692 days (range 15–2324), five patients died and one patient underwent implant of left ventricular assist system; overall mortality rate was 35.3%. Notably, patients with ADPAF and a TAPSE less than 16 mm had worse prognosis (P = .04). Conclusion: ADPAF could be a sign of right ventricular restriction in adult cases, and suggest a less favorable prognosis in patients with right ventricular dysfunction.

O3-2 Diagnostic Usefulness of Plasma Level of Globotriaosylsphingosine in Patients with Anderson-Fabry Disease Koichiro Sugimura, Saori Yamamoto, Tatsuo Aoki, Shunsuke Tatebe, Nobuhiro Yaoita, Haruka Sato, Katsuya Kozu, Ryo Konno, Kimio Satoh, Hiroaki Shimokawa; Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan Background: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase(α-Gal A). Currently, genetic sequencing is the only valid tool for the diagnosis of female AFD patients, since enzyme activity in female heterozygotes can be in the normal range. In this study, we thus examined whether plasma level of globotriaosylsphingosine (Lyso-Gb3) is useful for diagnosis of AFD. Methods and Results: Among 351 patients with left ventricular hypertrophy(LVH) from January 2004 to July 2016 (64 ± 14[SD] year-old, 117 female), 9 (2.5%) (54 ± 5 year-old, 6 females) were diagnosed as having AFD by cardiac biopsy, and other 6 patients without LVH by gene sequencing. Thus, we examined a total of 15 AFD patients (11 females, 7 families, 4 mutations). All AFD patients showed increased Lyso-Gb3 level (11.6 ± 3.1 ng/mL, n = 15) compared with non-AFD controls (0.4 ± 0.04 ng/mL, n = 46) (P < .0001). In 2 cases of female heterozygotes with normal α-Gal activity and elevated Lyso-Gb3, Lyso-Gb3 level was a reliable marker for the