Coarctation
9.5 %
PS
2%
MS etc.
1.5 %
LVOTO
1.3 %
HLHS
0.9 %
IAA
0.6 %
5. Valvular Lesion
Ebstein
<1 %
Hale Waihona Puke AR< 0.5 %
MR
< 0.5 %
SV aneurysm
< 0.5 %
6. Miscellaneous
Arrhythmia
low weight gain, tachypnea, tachycardia, sweating, anxiety, irritability, frequent URI 3) Symptoms of hypoxemia cyanosis, hypoxic spell
dyspnea on exertion, frequent URI, palpitation 3) Syncope, chest pain 4) Symptoms of Hypoxemia cyanosis, hypoxic spell,clubbing
1. Early blood vessel formation
1) Intraembryonic blood vessel at 13 days 2) Extraembryonic blood vessels at 17 days
2. Development of heart
1) Position and cardiac tube at 23 days Heart beating at 26 days.
2. Infections
Rubella Coxsakie virus
3. Maternal conditions Old age
Diabetes Lupus Phenylketonuria
4. Others
Maternal Risk Factors
Factors
Malformation
Advanced age
3) Environmental contribution
Drugs
Infections
Maternal conditions
Potential Cardiovascular Teratogens
1. Drugs
Alcohol Amphetamines Anticonvulsants Chemotherapy Sex hormone Thalidomide Retinoic acid
Trisomy 21
Maternal CHD
Various
Diabetes mellitus VSD, TGA, cardiomyopathy
SLE
Heart block
Phenylketonuria
TOF, VSD, COA, HLHS
Viruses
Teratogenic, myocarditis
5%
Vascular ring
0.5 %
Evaluation of CHD by History Taking
1. Infants
2. Children
1) Murmur
1) Murmur
2) Symptoms of CHF
2) Symptoms of CHF
poor feeding,
exercise intolerance,
2) Formation of heart loop at 8 somites 3) Formation of ventricle 4) Development of sinus venosus
3. Formation of cardiac septa 4. Formation of cardiac valves 5. Formation of arterial system 6. Formation of great systemic veins
Incidence of Congenital Heart Diseases
1. Lt to Rt Shunt
( 53 % )
PDA
17 %
ASD
16.5 %
VSD
13 %
AVSD
3.5 %
Abn. PV return
3%
2. Rt to Lt Shunt
(11 % )
TOF
4.5 %
TA
3%
PA+VSD
(*cytomegalovirus,
herpes, coxsacki B,
parvovirus)
Maternal Drug Exposures
Drug
• Diphenylhydantoin • Trimetadione • Thalidomode • Lithium • Alcohol • Amphetamine • Birth control pills
(10%)
1) Chromosomal
;
5-10%
2) Single mutant gene
;
3%
Recessive
Dominant
2. Genetic-environmental interaction (90%)
1) Multifactorial inheritance
;
majority
2) Risks to offspring of an affected parent
Surgery for Congenital Heart Diseases
Seoul National University Children’s Hospital Yong Jin Kim,M.D.
Etiologic Basis of Congenital Heart Diseases
1. Primary genetic factors
2.5 %
PA+IVS
0.5 %
3. Admixture Lesion ( 15 % )
TGA
5%
Univ. Ht.
5%
Atrial isomerism < 2 %
DORV
<2 %
Truncus
0.8 %
Corrected TGA < 0.5 %
4. Obstructive Lesion ( 15 % )
Malformation
PS, AS VSD, TOF, TGA, HLHS TOF, Truncus arteriosus Ebstein anomalies VSD, ASD, PDA, TOF VSD, ASD, PDA, TGA VSD, TOF, TGA
Stages of Heart Formation