先心病外科治疗 3623.ppt

Surgery for Congenital Heart Diseases
Seoul National University Children’s Hospital
Yong Jin Kim,.
Etiologic Basis of Congenital Heart Diseases
1. Primary ic factors (10%)
1) Chromosomal ; 5-10%
2) Single mutant gene ; 3%
Recessive
Dominant
2. ic-environmental interaction (90%)
1) Multifactorial inheritance ; majority
2) Risks to offspring of an affected parent
3) Environmental contribution
Drugs
Infections
Maternal conditions
Potential Cardiovascular Teratogens
1. Drugs
Alcohol
Amphetamines
Anticonvulsants
Chemotherapy
Sex hormone
Thalidomide
Retinoic acid
2. Infections
Rubella
Coxsakie virus
3. Maternal conditions
Old age
Diabetes
Lupus
Phenylketonuria
4. Others
Maternal Risk Factors
Factors Malformation
Advanced age Trisomy 21
Maternal CHD Various
Diabetes mellitus VSD, TGA, cardiomyopathy
SLE Heart block
Phenylketonuria TOF, VSD, COA, HLHS
Viruses Teratogenic, myocarditis
(*cytomegalovirus,
herpes, coxsacki B,
parvovirus)
Maternal Drug Exposures
Drug Malformation
Diphenylhydantoin PS, AS
Trimetadione VSD, TOF, TGA, HLHS
Thalidomode TOF, Truncus arteriosus
Lithium Ebstein anomalies
Alcohol VSD, ASD, PDA, TOF
Amphetamine VSD, ASD, PDA, TGA
Birth control pills VSD, TOF, TGA
Incidence of Congenital Heart Diseases
1. Lt to Rt Shunt ( 53 % )
PDA 17 %
ASD %
VSD 13 %
AVSD %
Abn. PV return 3 %
2. Rt to Lt Shunt (11 % )
TOF %
TA 3 %
PA+VSD %
PA+IVS %
3. Admixture Lesion ( 15 % )
TGA 5 %
Univ. Ht. 5 %
Atrial isomerism < 2 %
DORV < 2 %
Truncus %
Corrected TGA < %
4. Obstructive Lesion ( 15 % )
Coarctation %
PS 2 %
MS etc. %
LVOTO %
HLHS %
IAA %
5. Valvular Lesion
Ebstein < 1 %
AR < %
MR < %
SV aneurysm < %
6. Miscellaneous
Arrhythmia 5 %
Vascular ring %
Evaluation of CHD by History Taking
1. Infants
1) Murmur
2) Symptoms of CHF
poor feeding,
low weight gain,
tachypnea, tachycardia,
sweating, anxiety,
irritability, freq