先心病各论

先心病各论，分享了 室间隔缺损 VSD、动脉导管未闭PDA、房间隔缺损ASD、法洛四联症TOF的知识，一起来看看。

先心病各论

• 室间隔缺损 VSD
  • 流行病学
    • 最常见的CHD 30%-40%的小缺陷会在2岁内自发闭合
  • 分类
    • Perimembranous defects 75 % 膜周缺损
    • Muscular defects 15% 肌肉缺损
    • Supracristal (subpulmonary or subarterial) defects 8 % 干下（肺下或动脉下）缺损
  • 病生总结
    • 左向右分流，室间隔收缩期
    • 肺循环充血➡️肺动脉高压➡️功能型肺动脉高压➡️器质性肺动脉高压
    • 取决于分流量
      • 缺损大小
      • 左右心室压力梯度
      • 肺循环阻力
  • 临床表现： 震颤，杂音（经常在胸骨左缘三四肋） 哭闹时短暂发绀 运动耐力降低 生长延迟 肺动脉高压 反复肺炎 充血性心力衰竭CHF
  • 辅助检查
    • Chest X-ray Cardiomegaly of varying degree with enlargement of LA, LV, and RV A prominent MPA segment Increased pulmonary vascular markings PVOD : MPA and hilar PAs enlarge, but lung fields ischemic ， heart size normal
    • Electrocardiography Small VSD Normal Moderate VSD LVH and occasional LAH Large VSD CVH with or without LAH PVOD RVH only
    • Echocardiography超声心动图
    • Cardiac Catheterization心脏插管（非必要
    • Angiography血管造影
  • 并发症
    • Infective endocarditis ( IE )感染性心内膜炎
    • Pneumonia肺炎（最常见
    • Pulmonary hypertention ( PH )
    • Congestive heart failure ( CHF )
  • 治疗
    • Exercise restriction unnecessary without PH Prevention and treatment of complications Monitoring of PA pressure Medical management for Pneumonia or CHF Prophylaxis for IE
    • Radical treatment Observe its natural closure Surgery Intervention
    • Catheter closure Device: Amplatzer septal occluder Nitinol wire mesh Self-expandable Indication and timing muscular and perimembranous defect More than 2 years
• 动脉导管未闭PDA
  • Definition and prevalence
    • Definition: open ductus with pathophysiological changes
    • 15% of all CHDs, excluding premature infants M:F = 1:3 Function closure: 80% born 24 hours Anatomical closure: 80% 3 m and 95% 1 year
  • Summary
    • 在大动脉之间从左向右分流
    • 肺循环充血➡️肺动脉高压➡️ Eisenmenger syndrome器质性肺动脉高压
    • 升主动脉多血，降主动脉缺血
    • 左心房，心室，升主动脉增大
  • Pathophysiology & hemodynamics
    • 管道直径
    • Pressure gradient between Ao and PA
    • Pulmonary vascular resistance
  • 临床表现 取决于缺损大小
    • Symptoms Small: asymptomatic Large: early and severe （Recurrent pneumonia，CHF
    • Physical signs Slender, differential cyanosis Cardiac signs Circumference vessel signs Diastolic depression Pulse pressure increased Water-hammer pulse Capillary pulsation sign Femoral artery shooting sound
    • Chest X-ray Similar to those of VSD Cardiomegaly with LA, LV, ascending aorta A prominent MPA segment Increased pulmonary vascular markings PVOD
    • Electrocardiography ECG findings for PDA are similar to VSD Small：normal ECG Moderate：LVH and occasional LAH Large：CVH with or without LAH PVOD：RVH only
    • Echocardiography
    • Cardiac Catheterization
    • Angiography
  • 并发症
    • 肺炎
    • 充血性心力衰竭
    • 肺动脉高压
    • 感染性心内膜炎IE
  • 治疗
    • Exercise restriction unnecessary
    • 预防和治疗并发症
    • 基础治疗
      • 观察自然闭合
      • 药物促进闭合
      • 介入
      • 手术
        • Indications Difficult to close by catheter Contraindication PVOD
• 房间隔缺损ASD
  • 流行病学
    • Atrial septal congenital defect 5 ~10% CHDs M:F=1:2 Secundum ASD < 8mm spontaneous closure (39%) mostly under 1 year
  • Pathological anatomy
    • ASD 根据位置分为四种类型 （1） 原发孔缺损 ， 15 % （2） 中央型（最常见，75%） （3） 静脉窦缺损 ， 5% 上腔静脉型 下腔静脉型 （4 ）冠状窦 ， 2%
    • summary
      • AS水平上的左向右分流
      • 肺循环充血➡️肺动脉高压
      • 体循环缺血
      • RA，RV扩大
  • 临床表现
    • 无杂音，剧烈活动时短暂紫绀
    • 运动耐力降低
    • 生长延迟
    • 肺动脉高压
    • 反复肺炎
    • 充血性心力衰竭
  • 并发症
    • 肺炎 充血性心力衰竭 肺动脉高压 感染性心内膜炎
  • Surgical. Indications L to R shunt, Qp/Qs ratio of ≥2:1 not appropriate for intervention Timing 1. Usually 3 to 4 y of age 2. Infancy if big ASD, refractory HF, PH trend
• 法洛四联症TOF
  • 定义和流行病学
    • 定义：导致泵送到体内的氧合血液不足的四种异常
    • 婴幼儿先心病最常见的青紫型先心病
  • 病理解剖
    • Right ventricular outflow tract obstruction (RVOTO)右室流出道梗阻
    • VSD室间隔缺损
    • An overriding of aorta主动脉骑跨
    • Right ventricular hypertrophy, RVH右室肥大
  • 病理生理
    • summery： 在心室水平存在双向分流 体循环为混合血 肺循环减少 RVH右室肥厚
    • 取决于： 右室流出道的梗阻
      • 🌟决定： 症状出现时间 紫绀严重程度 右室肥厚程度
  • 临床表现
    • 症状
      • Cyanosis青紫：3-6m内出现（3m内动脉导管未闭，可出现侧支循环）
      • Growth dysplasia生长发育不良
      • Dyspnea on exertion ( 劳力性呼吸困难 )
      • Hypercyanotic spells （缺氧发作) Rapid increase in cyanosis Irritability or inconsolable crying Breathlessness and pallor
      • Squatting 蹲踞现象：减少回心血量
    • 体征
      • Varying degree of cyanosis lips and mouth fingernails and toenails
      • Tachypnea呼吸困难
      • Clubbing of fingers and toes
      • Growth dysplasia
      • 心脏杂音：收缩期杂音L2～4
  • 辅助检查
    • 胸片
      • Heart size normal or smaller PVMs decreased Concave MPA segment with upturned apex (“boot-shaped” heart) is characteristic RV enlargement may be present 若肺纹理增多和左心室增大 考虑合并 PDA 或侧支循环形成
    • 心电图
      • 靴型心，右室肥厚
    • 超声心动图
      • 主动脉覆盖的程度 右心室流出道梗阻的位置和程度 室间隔缺损的数量和大小 近端 PA 的大小
    • 心脏插管
      • 非必要 除非需要明确：外周 PA 和侧支循环，冠状动脉
    • 血管造影
      • Right and left ventricular injection Aortic root injections Occasionally selective PA or CA injection
  • 鉴别诊断
    • 大动脉转位
    • 肺静脉异位引流
    • 右心室双出口
  • 治疗
    • Medical
      • 识别，治疗缺氧发作 Picked up and held over shoulder, or held in a knee-chest position Morphine sulfate Oxygen Bicarbonate (NaHCO3) to correct acidosis Propranolol for relieving subpulmonary muscualr spasm
    • Surgical
      • Complete repair at 6 m to 1y Severely cyanotic pts < 3 m are usually treated with a shunt (palliative procedure), and then full repair at 12 m Complete correction involves  closing VSD  relieving RVOTO