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【PPT】心肌疾病

  • 2026-06-30 13:42:38
【PPT】心肌疾病

【考点笔记】

【考点笔记】心肌疾病

注：如需考点笔记版PDF（方便打印/做笔记），发送关键词“心肌疾病”。

【考点大纲】

总论：心肌病概述

考点
核心内容
星级
心肌病定义
排除负荷增加和缺血后的心肌疾病
★★★☆☆
2023 ESC分类
5个表型：HCM、DCM、NDLVC、ARVC、RCM
★★★★☆
NDLVC
2023年新增类型
★★★★☆
遗传模式
常染色体显性遗传为主
★★★☆☆
基因-表型对应
肌节→HCM，骨架→DCM，桥粒→ARVC
★★★☆☆
诊疗径路
超声→CMR→基因/病理→管理→猝死预防
★★★☆☆

第一节 肥厚型心肌病（HCM）

考点
核心内容
星级
流行病学
发病率2‰，青少年运动猝死主因
★★★★☆
基因突变
60%阳性，MYH7+MYBPC3占70%，8个一线基因
★★★★☆
病理特征
心肌细胞排列紊乱（特征性）
★★★☆☆
LVOTO机制
结构性+功能性（SAM征，Venturi效应）
★★★★☆
杂音增强因素
正性肌力药、前负荷↓、后负荷↓
★★★★★
五大症状
呼吸困难、胸痛、心律失常、心衰、晕厥/猝死
★★★★☆
ECG特征
固定性ST-T、深T波（心尖）、非梗死q波
★★★★☆
TTE表现
室间隔不对称肥厚、SAM征、LVOTO
★★★★★
CMR价值
LGE≥15%→猝死风险高
★★★☆☆
诊断标准
成人15mm/13mm，儿童z值2.5/2
★★★★★
梗阻标准
30mmHg（诊断）/ 50mmHg（激发）
★★★★☆
鉴别诊断
高血压、主狭、运动员心脏；淀粉样变、Fabry病
★★★★☆
β受体拮抗剂
一线，目标心率55-60次/分
★★★★★
玛伐凯泰
肌球蛋白抑制剂，LVEF<55%禁用
★★★★☆
猝死高危因素
家族史、晕厥、肥厚≥30mm、NSVT、血压异常、LGE≥15%
★★★★★

第二节 扩张型心肌病（DCM）

考点
核心内容
星级
流行病学
心脏移植最常见适应证
★★★★☆
最常见基因
TTN截短突变（家族性25%，散发性18%）
★★★☆☆
病理特点
弥漫性室壁运动减弱（非节段性）
★★★★★
体征
一大一低一奔一杂
★★★★☆
CMR鉴别
DCM：中层/心外膜LGE；缺血性：心内膜下/透壁LGE
★★★★☆
诊断标准
LVEDD增大 + LVEF < 50%
★★★★☆
心衰新四联
RAS抑制剂+β受体拮抗剂+SGLT2i+MRA
★★★★★
ICD指征
LVEF ≤ 35% + 优化药物治疗≥3个月
★★★★★
CRT指征
LVEF ≤ 35% + LBBB + QRS ≥ 150ms
★★★★☆
酒精性DCM
>80g/天×>5年，戒酒可逆
★★★☆☆
围生期心肌病
妊娠末月至产后5个月，孕期避用ACEI/ARB
★★★☆☆

第三节 非扩张型左心室心肌病（NDLVC）

考点
核心内容
星级
定义
无左室扩张 + 非缺血性心肌瘢痕/脂肪替代
★★★★☆
诊断核心
CMR-LGE（非缺血性分布）
★★★★☆
高危基因
LMNA、FLNC
★★★☆☆
ICD指征
高危基因者即使LVEF>35%也考虑
★★★☆☆

第四节 致心律失常性右心室心肌病（ARVC）

考点
核心内容
星级
流行病学
35岁以下运动员猝死主因之一
★★★★☆
核心基因
PKP2（桥粒蛋白，占40-50%）
★★★☆☆
病理特征
纤维脂肪组织替代，好发右室三角区
★★★★☆
典型心律失常
LBBB型VT
★★★★★
ECG特征
Epsilon波（特异但敏感性低）、右胸导联T波倒置
★★★★★
诊断六大维度
结构+组织+复极+除极+心律失常+家族史
★★★★☆
核心治疗
避免剧烈运动 + ICD + 抗心律失常药物
★★★★☆

第五节 限制型心肌病（RCM）

考点
核心内容
星级
核心病理生理
舒张功能障碍，收缩功能正常
★★★★☆
最常见病因
淀粉样变（AL型/ATTR型）
★★★★☆
ECG特征
低电压 + 心肌肥厚（矛盾表现）
★★★★★
超声特征
心尖保留征、双房扩大、限制性充盈障碍
★★★★☆
核素扫描
⁹⁹ᵐTc-PYP阳性→ATTR-CA
★★★★☆
鉴别诊断
RCM vs 缩窄性心包炎（心导管+呼吸影响）
★★★★★
靶向治疗
氯苯唑酸（ATTR-CA）、siRNA
★★★☆☆

第六节 心肌炎

考点
核心内容
星级
最常见病因
病毒（柯萨奇病毒B组）
★★★★☆
发病机制
病毒直接损害 + T细胞免疫损伤
★★★☆☆
诊断必要条件
肌钙蛋白升高
★★★★★
CMR诊断
Lake Louise标准（水肿+充血+坏死，满足≥2项）
★★★★★
确诊金标准
EMB（Dallas标准）
★★★☆☆
抗心律失常
胺碘酮首选，避免Ⅰ类抗心律失常药
★★★★☆
暴发性重症
需IABP/ECMO支持，度过急性期预后好
★★★★☆
核心治疗
卧床休息 + 心肌保护 + 心肺支持
★★★★☆

【PPT-预览】-108页

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  1. CONNECT:[ UseTime:0.000507s ] mysql:host=127.0.0.1;port=3306;dbname=h_sjds;charset=utf8mb4
  2. SHOW FULL COLUMNS FROM `fenlei` [ RunTime:0.000824s ]
  3. SELECT * FROM `fenlei` WHERE `fid` = 0 [ RunTime:0.000333s ]
  4. SELECT * FROM `fenlei` WHERE `fid` = 63 [ RunTime:0.001720s ]
  5. SHOW FULL COLUMNS FROM `set` [ RunTime:0.000499s ]
  6. SELECT * FROM `set` [ RunTime:0.000198s ]
  7. SHOW FULL COLUMNS FROM `article` [ RunTime:0.000508s ]
  8. SELECT * FROM `article` WHERE `id` = 530294 LIMIT 1 [ RunTime:0.000447s ]
  9. UPDATE `article` SET `lasttime` = 1782890723 WHERE `id` = 530294 [ RunTime:0.013471s ]
  10. SELECT * FROM `fenlei` WHERE `id` = 64 LIMIT 1 [ RunTime:0.000277s ]
  11. SELECT * FROM `article` WHERE `id` < 530294 ORDER BY `id` DESC LIMIT 1 [ RunTime:0.000464s ]
  12. SELECT * FROM `article` WHERE `id` > 530294 ORDER BY `id` ASC LIMIT 1 [ RunTime:0.000362s ]
  13. SELECT * FROM `article` WHERE `id` < 530294 ORDER BY `id` DESC LIMIT 10 [ RunTime:0.001521s ]
  14. SELECT * FROM `article` WHERE `id` < 530294 ORDER BY `id` DESC LIMIT 10,10 [ RunTime:0.002147s ]
  15. SELECT * FROM `article` WHERE `id` < 530294 ORDER BY `id` DESC LIMIT 20,10 [ RunTime:0.000896s ]
0.096579s