This document provides biographical information about Jianyuan Pan and summarizes his research interests in hypertrophic cardiomyopathy (HCM). It notes that he received his graduate education from Anhui Medical University and Heidelberg University. His main research area is HCM, focusing on both clinical and basic science research. His clinical research involves analyzing risk factors for sudden cardiac death in HCM patients and outcomes of different treatment approaches. His basic science research examines the molecular mechanisms underlying HCM pathogenesis.

1. TheassociationbetweenHCMandSCD,Myconcern
Name：Jianyuan Pan
Birthday：1986.04.01
Graduate school: Anhui medical university/Heidelberg university
Supervisor: 徐岩、Thomas Wieland
Main area：Hypertrophic Cardiomyopathy

2. The content of my address

3. • 1929y-
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History of Medicine or Netflix
Drama?

4. Definition and Epidemiology
Hypertrophic cardiomyopathy (HCM) is a primary
myocardial disease characterized by left ventricular
hypertrophy in the absence of other etiologies such as
aortic valve stenosis or systemic arterial hypertension.
Bas
al
Normal
Mid-ventricular Apical
Diffuse types
1. The most common genetic cardiovascular disorder
2. More than 1500 gene mutations are related to the clinical expression of
HCM
3. 1 in 500 people within general population
4. HCM affects 0.2% of the global population.
5. The most-common cause of sudden cardiac death in individuals
younger than 35 years of age
Maron, B. J. et al. Sudden death in young competitive athletes. Clinical, demographic, and
pathological profiles. JAMA 276, 199–204 (1996).

5. Definition and Epidemiology
Hypertrophic cardiomyopathy, Lancet 2013; 381: 242–55
Diffuse Basal Apical
Posterior Basal + Posterior MidVs + Free wall
Transmural infarction after ASA
Fibrosis with gadolinium
enhancement
End stage and systolic
dysfunction
Extensive transmural
scarring
23 years patients
37 years patients
46 years patients

6. Clinical Course and Management of Hypertrophic Cardiomyopathy, The New
England Journal of Medicine. n engl j med 379;7 nejm.org August 16, 2018

7. The mechanism of HCM
1. Disease of the sarcomere
2. MYH7 and MYBPC3 (cardiac myosin-
binding protein C)—3/4 total patients
3. Up to 5% of patients carry at least two
independent mutations
4. Double and triple mutations associated
with an early onset and severe course
of the disease.
Genetic testing for risk prediction is an even
more-controversial
MYH7 mutations cause earlier-onset and more-severe left ventricular
hypertrophy than other mutations
Basic research

8. The mechanism of HCM
2014 ESC Guidelines on diagnosis and management of hypertrophic
cardiomyopathy, European Heart Journal (2014) 35, 2733–2779

9. The mechanism of HCM
1. Disturbed biomechanical stress sensing (Z-disc to the M-band,
mutation lead to a disturbance in transduction of biomechanical stress. )
2. Impaired calcium cycling and sensitivity (calcium fluxes are perturbed ,
which was also associated with SCD.)
3. Altered energy homeostasis (Inefficient energy utilization and increased
energy demand by the sarcomere, HCM-associated mutations )
4. Increased myocardial fibrosis (Arrhythmias attributed to left ventricular
muscle mass, myocyte disarray, or fibrosis. ).
Impaired myofibrillar contractile function
Compensatory hypertrophy and Diastolic dysfunction
Microvascular dysfunction, impairs systolic and diastolic
function, SCD, and the occurrence of arrhythmias

12. CONCLUSIONS: Myosin conformations establish
work-energy equipoise that is essential for life-long
cellular homeostasis and heart function.
Destabilization of myosin energy-conserving states
promotes contractile abnormalities, morphological and
metabolic remodeling, and adverse clinical outcomes
in patients with HCM. Therapeutic restabilization
corrects cellular contractile and metabolic phenotypes
and may limit these adverse clinical outcomes in
patients with HCM.

13. The protocol for diagnosis of HCM
2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic
Cardiomyopathy: Executive Summary, JOURNAL OF THE AMERICAN COLLEGE OF
CARDIOLOGY

14. Therapies for Treatment
2014 ESC Guidelines on diagnosis and management of hypertrophic
cardiomyopathy, European Heart Journal (2014) 35, 2733–2779

16. https://doc2do.com/hcm/webHCM.html

17. SCD risk and ICD implantation
2014 ESC Guidelines on diagnosis and management of hypertrophic
cardiomyopathy, European Heart Journal (2014) 35, 2733–2779

18. Conclusion: The Euro-ASA registry demonstrated low peri-procedural and long-term
mortality after ASA. This intervention provided durable relief of symptoms and a
reduction of LV outflow tract obstruction in selected and highly symptomatic patients
with obstructive HCM. As the post-procedural obstruction seems to be associated with
both worse functional status and prognosis, optimal therapy should be focused on the
elimination of LV outflow tract gradient.

19. Conclusion: In this study, PTSMA could be
proofed as a safe procedure with ongoing
symptomatic improvement and excellent long-
term survival. Therefore, PTSMA is a reasonable
alternative to surgical myectomy in HOCM.

20. Conclusion: Both procedures improved
functional capacity; however, myectomy better-
resolved classes III–IV of heart failure. Septal
ablation was associated with higher reoperation
rates. Myectomy demonstrated benefits in
gradient relief and mitral regurgitation
elimination. The results suggest that decreasing
rates of myectomy procedures need to be
investigated.

21. Conclusion: In patients with obstructive HCM,
earlier versus surgery for Class I indication had a
better long-term survival, similar to the age-sex-
matched US population

23. Mavacamten（化学名为 MYK-461）是一种口 服的小分子药
物，是 β 肌球蛋白的异构体，对肌 动蛋白 - 肌球蛋白交联的
过程起可逆性的调节作 用，可抑制心肌肌球蛋白重链 ATP 酶
活性，从而 调节心肌收缩力，还可以改善心肌细胞能量代谢
异常

24. My Clinical research area

25. My Clinical research area
Conclusions： Patients with a smaller TG index showed a huge advantage in
long-term survival ( 8 years survival from all-cause death 16.53% in TG index≤1683
group Vs. 84.18% in TG index>1683 group). Thus, the TG index should be a perfect
indicator in the evaluation of HOCM patients whether suitable for the PTSMA and to
be used to predict long-term survival.

26. My basic research area

27. My basic research area

28. My basic research area
Still under writing

29. My Clinical research area
指标 皖北 皖中 皖南
均值 标准差 均值 标准差 均值 标准差 F 显著性
脂蛋白a 278.230 205.259 257.809 176.252 252.166 177.254 17.657 0.000
载脂蛋白B 0.858 0.215 0.863 0.208 0.863 0.204 0.920 0.399
载脂蛋白A1 1.236 0.191 1.260 0.202 1.263 0.194 20.838 0.000
极低密度脂蛋白胆固醇 0.858 0.349 0.861 0.350 0.873 0.353 0.774 0.461
高密度脂蛋白胆固醇 1.007 0.244 1.036 0.263 1.063 0.260 27.217 0.000
低密度脂蛋白胆固醇 2.212 0.818 2.300 0.846 2.318 0.824 16.144 0.000
总胆固醇（入院） 4.077 1.098 4.196 1.107 4.253 1.094 19.388 0.000
血糖（入院） 7.385 3.302 7.274 3.014 6.948 2.817 8.575 0.000
甘油三酯（入院） 1.586 1.071 1.618 1.107 1.613 1.092 1.067 0.344
讯飞抓取2018-2021共13006例CHD患者（我院行CAG或CAG+PCI）
拟结合大数据分析冠心病发病率与气候因素的相关性研究
0.816
0.852
0.88
0.184
0.148
0.12
0% 20% 40% 60% 80% 100%
皖北(n=2145)
皖中(n=2372)
皖南(n=382)
PCI患者合并钙化占比
无钙化 有钙化

30. My Clinical research area
指标
CAG CAG+PCI
均值 标准差 均值 标准差 t Sig.(双侧)
脂蛋白a 252.1788 173.01004 281.2705 201.3408 -7.843 0.000
载脂蛋白B 0.8573 0.20266 0.8635 0.21746 -1.492 0.136
载脂蛋白A1 1.266 0.19678 1.2305 0.18409 9.442 0.000
VLDL 0.8404 0.31628 0.8925 0.39454 -7.365 0.000
HDL 1.0479 0.26219 0.9943 0.23946 10.874 0.000
LDL 2.2471 0.80485 2.2748 0.88267 -1.659 0.097
总胆固醇（入院） 4.1353 1.05849 4.1615 1.17299 -1.190 0.234
血糖（入院） 7.0291 2.77285 7.8004 3.48134 -12.378 0.000
甘油三酯（入院） 1.5784 1.00941 1.6791 1.22361 -4.538 0.000
指标
无钙化 有钙化
t Sig.(双侧)
均值 标准差 均值 标准差
脂蛋白a 263.049 187.798 280.896 192.008 -3.281 0.001
载脂蛋白B 0.861 0.211 0.862 0.206 -0.168 0.866
载脂蛋白A1 1.252 0.197 1.241 0.197 1.967 0.049
极低密度脂蛋白胆固醇 0.864 0.357 0.839 0.292 2.831 0.005
高密度脂蛋白胆固醇 1.028 0.255 1.021 0.262 0.925 0.355
低密度脂蛋白胆固醇 2.273 0.837 2.234 0.809 1.618 0.106
总胆固醇（入院） 4.164 1.110 4.094 1.057 2.196 0.028
血糖（入院） 7.249 3.063 7.565 3.447 -3.222 0.001
甘油三酯（入院） 1.625 1.126 1.458 0.777 6.978 0.000
拟进一步通过冠脉CTA得到冠脉钙化的精确评分
回顾性断面分析Lpa和冠脉钙化严重程度
的术后MACE事件的相关性研究
PCSK9干预Lpa对冠脉钙化进展的相关性研究
及早干预Lpa对主动脉瓣二叶瓣畸形
患者主动脉瓣钙化的进展研究