利用心臟組織相位對比磁共振影像分析法布瑞氏症於心臟的影響

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研究生：	莊柏彥 Chuang, Bo-Yan
論文名稱：	利用心臟組織相位對比磁共振影像分析法布瑞氏症於心臟的影響 Analysis of Fabry Disease with Cardiac Manifestation by Cardiac Magnetic Resonance Tissue Phase Mapping
指導教授：	彭旭霞 Peng, Hsu-Hsia
口試委員:	黃騰毅 Huang, Teng-Yi 王福年 Wang, Fu-Nien
學位類別：	碩士 Master
系所名稱：	原子科學院 - 生醫工程與環境科學系 Department of Biomedical Engineering and Environmental Sciences
論文出版年：	2017
畢業學年度：	105
語文別：	英文
論文頁數：	131
中文關鍵詞：	相位對比磁振造影、組織相位對比技術、法布瑞氏症、酶替代療法
外文關鍵詞：	Phase Contrast MRI, Fabry disease, Myocardium, Enzyme Replacement Therapy
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本篇研究的目的是分析法布瑞氏患者在射血分數尚未影響前觀察左右心室心肌與正常受試者之間的差異，利用組織相位對比技術量化心肌相關參數的變化。另外一個目的則是追蹤法布瑞氏症患者經過酶替代療法後心肌是否有所改善。針對十四位法布瑞氏症患者及三十四位年齡相符的正常受試者進行比較，追蹤組的部分則是原先十四位當中的七位患者進行追蹤評估。
法布瑞氏症患者在許多心肌量化參數揭露出顯著降下，特別是在左心室的側邊，如收縮期的速度峰值在縱向及環向都有明顯下降，在患者中還有些心肌量化參數呈現顯著增加，像是不論在收縮期或舒張期的達峰時間在放射向、縱向以及環向皆有明顯延後的情況，組織相位對比技術提供整體及局部的心肌資訊，在本篇研究當中，法布瑞氏症患者不管在整體及局部的心肌相較於受試者皆有許多參數有顯著差異，法布瑞氏症患者經過酶替代療法後，對於部分的心肌有所改善。
本篇研究法布瑞氏症患者在射血分數尚未影響前，左右心室心肌皆有所影響，組織相位對比技術量化的參數提供良好的診斷依據，有助於評估患者心肌受損即早接受治療，患者經過酶替代療法後，心肌功能亦有所改善。

Our purpose is to observe the left and right myocardium in the early stage before left ventricular ejection fraction reduced in patients with Fabry disease by tissue phase mapping. Another purpose is to observe whether the myocardium improved after enzyme replacement therapy. We collected 14 were FD patients and 34 were age-matched normal volunteers without any cardiac disease, and related cardiac surgery. There are seven cases of fourteen FD patients for the assessment of the follow-up group.
The FD group find that some quantifications revealed significantly reduced in the patients with preserved LVEF, especially in the lateral segment. Such as Vz, Vϕ, and VϕPTP in the systole. Some quantifications of FD group presented significantly increased in the patients. Such as TTPr, TTPz, and TTPϕ in the systole and diastole. Tissue phase mapping provides global and regional myocardial information. We can find that some findings were significantly different in the global and inter-segmental slice. The follow-up FD group revealed improved in some segments after enzyme replacement therapy.
In conclusion, the quantification of the LV and RV myocardium may provide useful information to comprehend the impaired cardiac manifestation and dysfunction in early stage before left ventricular ejection fraction reduced in patients with Fabry disease by tissue phase mapping. The patients of Fabry disease possible improved after enzyme replacement therapy.

Chapter 1 Introduction 1
1 Fabry Disease 1
1.1 Genetic Basis and Pathogenesis 1
1.2 Cardiac Complications 3
1.3 Treatment in Fabry Disease 5
2 Diagnosis of Fabry Disease 6
3 Cardiac Involvement of Fabry Disease Approach 8
3.1 Echocardiography 8
3.2 Tissue Doppler Imaging 10
3.3 Cardiac Magnetic Resonance Imaging 11
4 Motivation 13
5 Orientation of Dissertation 14
Chapter 2 Theory 15
1 Phase Contrast MRI 15
2 Tissue Phase Mapping 17
3 Myocardial Region of Interest 18
4 Bull’s eye 19
Chapter 3 Materials and Methods 20
1 Study Cohort 20
2 MRI Acquisition 23
3 Data Analysis Process 24
4 Myocardial Radial and Longitudinal Indices 26
4.1 Peak Velocity 26
4.2 Time to Peak 27
4.3 Dyssynchrony Index 28
4.4 Action Time 30
5 Enzyme Replacement Therapy Follow-Up 31
6 Myocardial Circumferential Indices 32
6.1 Five Peak Velocity 32
6.2 Five Peaks Time to Peak    33
6.3 Peak to Peak 34
6.4 Dyssynchrony Index 35
6.5 Twist 36
7 Statistical Analysis 37
Chapter 4 Results: Myocardial Radial and Longitudinal Indices 38
1 Myocardial Velocity-Time Course in Radial and Longitudinal
Directions 38
2 Myocardial Radial and Longitudinal Indices 43
2.1 Peak Velocity 43
2.2 Time to Peak 45
2.3 Dyssynchrony Index 47
2.4 Action Time 56
3 Enzyme Replacement Therapy Follow-Up 65
3.1 Left Ventricular Peak Velocity 65
3.2 Right Ventricular Peak Velocity 68
3.3 Left Ventricular Time to Peak 70
3.4 Right Ventricular Time to Peak 74
Chapter 5 Results: Myocardial Circumferential Indices 77
1 Myocardial Velocity-Time Course in Circumferential Direction 77
2 Myocardial Circumferential Indices 80
2.1 Five Peak Velocity 80
2.2 Five Peak Time to Peak 82
2.3 Peak to Peak 84
2.4 Dyssynchrony Index 85
2.5 Twist 93
Chapter 6 Discussion 95
1 Major Findings 95
2 Myocardial Radial and Longitudinal Indices 98
2.1 Peak Velocity 98
2.2 Time to Peak 99
2.3 Dyssynchrony Index 99
2.4 Action Time 100
3 Enzyme Replacement Therapy Follow-Up 101
3.1 Slice Location 101
3.2 Peak Velocity 103
3.3 Time to Peak 104
4 Myocardial Circumferential Indices 105
4.1 Five Peak Velocity 105
4.2 Five Peak Time to Peak 105
4.3 Peak to Peak 106
4.4 Dyssynchrony Index 106
4.5 Twist 107
5 Multiple Comparisons 108
5.1 Statistical analysis 108
5.2 Myocardial Radial and Longitudinal Indices 109
5.3 Myocardial Circumferential Indices 113
6 ROC Curve 118
7 Limitation 125
Chapter 7 Conclusions 126
1 Conclusions 126
2 Future work 127
Chapter 8 References 128

                                

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