CONGENITAL HEART DISEASE

1
??????

• CONGENITAL HEART DISEASE

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• DEFINITION
• ????????????????????????
• An abnormality in cardiocirculatory structure
  or function that is present at birth , even if it
  is discovered much later .
• ____Heart Disease, Brauwnwald

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• INCIDENCE
• ???????????
• ?????????0.67 0.8
• 25 30??????
• ?????66.6????
• ???????????91?
• ??????, ?????????

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ETIOLOGY
????????,??????2 8???????????,??????????(?????)
??????????,????????
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• ????????(Genetic Aspects)
• 1.?????
• 21-?????Trisomy 21( Down Syndrome)
• 18-?????
• Turner??? 45, XO
• 2. ????? CATCH-22 Syndrome 22q11
• 3. ????? Noonan Syndr., Holt-Oram Syndr. Long
  QT Syndr., Marfan Syndr.

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• ????????(Environmental Aspects)
• ???? ?????,???,??????
• ???
• ???? ???,?????
• ???? ????????
• ????? ??????????
• ???????????????

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CLASSIFICATION

• Left-to-right shunt (??????, ?????)
• Right-to-left shunt (??????,???)
• No shunt (????,????)

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• Left-to-right shunt
• ????, ???????????????????,
  ???????????????????????????.
• ??????????
• ?????(ventricular septal defect, VSD)
• ?????(atrial septal defect, ASD)
• ??????(patent ductus arteriosus, PDA)

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• Common features
• ?????,???????????(????)
• ??????, ??????????????
• ??????,????????,???????,????????,????????,???????,
  (Eisenmenger syndrome)
• ?????????

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• Right-to-left shunt
• ????????????????, ????????????????????
• ???????????(tetralogy of Fallot),?????,???????
• ??????????????,?????, ??????????,???,???,?????.

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????????? ??cyanosis hypoxemia and
acidosis ?????polycythemia ????,
???? ????clubbing digits ??????, ??????. ???
????????, ?????. ??? ?????????.
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No shunt ???? ?????, ?????
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Diagnostic Methods

• ??
• ????
• ????
• X???
• ???
• ?????
• CT?MRI
• ?????
• ?????

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HISTORY
1. ???? 2. ??????lt3? 3. ???? ????,??????
??,???? ????, ?? ???? ??
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• ?? ?????????????????,????????,???????????
• ?????????
• ???????? ?????? CNS??
• ?????? ???????

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PHYSICAL EXAMINATION

• ????
• ???? impaired growth and physical development
• ??????? tachypnea, dyspnea
• ??????????

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• ??cyanosis
• ??????????????????? gt50g/L,??????????????,
  ????????????

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• ???(?)clubbing digitS ?????? 12 ?
• 
  ????.

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• ????cardiac examination
• ?? ???????, ??????
• ?? ?????, ??thrill( ??, ??)
• ?? ????, ??
• ?? ??, ??, S1, S2 ??, P2??, ??,
• ??murmur ??, ??, ??, ??, ?
• ???

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?????systolic murmur ???(ejective)
????????????? ???? ??, ??, ??? ??? ????
(??)?????????????? ???(Regurgitative)
??????????? ???? ??, ?????? ??????
??VSD, ?????????
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• ?????blood pressure and pulse
• ???????????
• ????? ???????, ???????

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Echocardiography

• M??????
• ????????(???????)
• ????????
• ?????????
• ???????
• ???????
• ???????

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Cardiac Catheterization

• ??????????
• ???????????
• ?????????????
• ????????????
• ????????????
• ????????

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?????????/????????????? ???? ???? ????
????? ?? RA-VC gt2vol
gt8 ?? RV-RA gt1vol
gt3 ??? PA-RV gt0.5vol
gt2 ???????????? 95100
25
???????(mmHg)
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????? atrial septal defect,ASD

• ??????????26.
• ???????????.

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• ??????????????????????
• 1.???(????),???
• 2.???
• 3.???
• 4.????

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• Pathophysiology
  
• LA-RA??
• ??????
• ????????
• ?????(pulmonary hypertension),?????????????,?????,
  
• ??????,???Eisenmenger???
• ????????

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• Clinical Manifestations
• ????????????????????,????????,?????,??
• ????????
• ??????,???????
• ?????????,?????
• ??????????,????????????IIIII?????????
• ??????????,?????
• ??????????????

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• Laboratory Examinations
• ?? ECG
• ????,??????????????,????????,??????
• ? ?Chest X-ray
• ????,??????,???????,??????????

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• ? ?Echocardiography
• M?????????????????????,????????
• ??????????????????????????????????????
  ???

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• ?? Cardiac Catheterization
• ??????????????????
• ?????????????????????2vol,???????

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• Management
• ?? Interventional therapy
• ???Sideris ????????Rashkind
  ????????Amplatzer???????????,???????????????Amplat
  zer???????? gt3?, ??gt9??, ????gt4mm??.

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• ? ?Surgical Correction
• ???????????,???????????????????,???????
  ??,?????????? ???? ????.

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• ?????
• ventricular septal defect,VSD
• ???????????2050?

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• ???????,??????????1.????
  ??????78??????????????????????????????2.????????
  ???20,??????????????????3.???? ??????2?

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• ???????,????????
• ????(????lt0.5cm),
• ????(????0.51cm),
• ????(????gt1cm)?

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• Pathophysiology
• LV-RV??
• ??????
• ????????
• ?????,Eisenmenger???
• ???????????????

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• Clinical Manifestations
• ????,?????,???????
• ????,??????????????, ???????pneumonia
• ?????,???????,Eisenmenger?????????
• ?????infective endocarditis ?heart failure ?

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• ????
• ????34?????III-IV???????????????,????,????????
  
• ???????????????
• ???????????

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• Laboratory Examinations
• ?? EEG
• ??????,?????,???????,?????????

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• ? ?Chest X-ray
• ????????????,????????,??????????,??????
  ?
• ??????????,?????????,???????,???????,???
  ???

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• ? ?Echocardiography
• ???????????
• ???????????????????????????????????????
  ??

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• ? ?Cardiac Catheterization
• ??????????????????????????1vol??,?????
  ??????????????????????????

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• Management
• ?????????????????????????,??????2163,
  ??5??????,??????
• ???????????????????????????????????????
  ???????gt21???????

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• ?????????????????????????????????,?????
  ??????????
• ?????VSD???????????

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• ??????
• patent ductus arteriosus, PDA
• ??????????20?

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• ?????????,?????1. ??
  ??????????????????,??????2.???
  ????????,??????,?????????3.?? ????,????,?????,???
  ?,?????4.??? ?????????,?????5.??
  ?????????????,?????,?????

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• Pathophysiology
• ????????
• ??????
• ??????
• ?????, Eisenmenger syndrome LA, LV??????
• ???????????????????(1)???????(2)?????????(3)?
  ??????????????.

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• ??????????,????????,???Eisenmenger???,?
  ???????????????,???????????differential cyanosis.
• ??????????????????,,????????????????????,????
  ??????????, ????????, ????????

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• Clinical Manifestations
• ??????????,??,??
• ????????
• ?????????,???????????????????

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• ????
• ????23?????????,?????????23??????????,?????
  ,??????
• ?????????,???????????,?????????????
• ????????????????
• ?

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• ????????,?????????,???,????,????????,??????
• Eisenmenger???
• differential cyanosis.

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• Laboratory Examinations
• ? ?ECG
• ????,????, ???????, ?????

?? Chest X-ray ?????,?????,??????,???????

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• ?? Echocardiography
• ?????????
• ?????????
• ?????????????????? ????????????????

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• ?. Cardiac
• Catheterization Angiocardiography
• ?????????????????????,?????????????
  ?????????????????????0.5vol,??????????????

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• Management
• ???? ???, ???PDA, ?????.
• ???0.1-0.2mg/kg/d, ???
• ???? Porstmann??Rash
• -kind ? Amplatzer???????
• ??????(??1.70.8mm)
• ?????,??????????
• ??????????????
• ???????

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• 3. Surgical Correction
• ??????????????????????????????????????????????
  ?????????????1.0cm?,?????????????????????

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• ?????
• tetralogy of Fallot, TOF
• ????????1014,
• ?????????.

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???????? ????? ?????
????? ?????
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• Pathophysiology
• ?????????
• 1)????,
• 2) Right-to- Left shunt??
• ?? ????, ???
• ?????? ???????,?????, ?????

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• Clinical manifestations
• ?? cyanosis ?????
• ??squatting ??TOF???,?????,??????????,???????????
  

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• squatting???????
• 1.?????????, ???????,????????, ???????,???????.
• 2. ?????????, ?????????,??????,???????,?????????
  ???

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• ????hypoxic spells
• ?????????????????,????????????????????????????
  ???????????????????????,??????????????????????????
  

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???(?)clubbing digits ????24???????????????
? ???????????????
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• Laboratory Examinations
• ?? ECG
• ????,??????????
• ? ?X-RAY
• ????,????,?????,????,
  ??????,???????????????

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• ??Echocardiography
• ?????????????,????????,?????????,???????
  ???

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• ?. Cardiac
• Catheterization Angiocardiography
• ????????????????????,?????????,??????,?
  ??????????,???????????????????????????????/???????
  ?????

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• Management
• ????????????36?.
• ?6????.
• Total Surgical Correction
• Palliative Surgery

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• Hypoxic spells???
• (1)??????????(????)(2)?????????,?????????
• (3)????ß??????????????

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• (4)?????(0.10.2mg/kg),????)?????????????
• (5)?????(NaHCO3)?????,?????????????
• (6)????????,????,????????????????,???????????,????
  ??????