复旦大学儿科医院
Congenital Heart Diseases
(CHD)先天性心脏病
General concept
? CHD occurs in approximately 6-8‰ live births
? In China,alone 250000 children with CHD are
born each year
? 30% of cases may die within first year of life
? The etiology remains unsolved
? Secondary prevention is the strategy at this
stage
Congenital Heart Diseases
(CHD)先天性心脏病
Schematic Diagram of
Cardiogenesis
?Bilaterally symmetrical cardiac progenitor
cells form distinct regions of the heart
?The precardiac mesodermal cells give
rise to a linear heart tube
?Forming a rightward loop
?Establishing the spatial orientation of the
four-chambered mature heart
Schematic Diagram of
Cardiogenesis
LVRV
LA
PA
AOconotruncus
RV=right ventricle; LV=left ventricle; RA=right atrium
Ao=aortic; LA=left atrium; PA=pulmonary artery
RA
Fetal development
One month 5th week 6th week
8th week
Cardiac development
Formation of a single
Heart tube
Formation of the heart
loop
Cardiac development
Out flow tract septation Septation of fourchambers
原发房隔
原发孔
继发房隔
原发房隔
继发孔
继发房隔
Atrial septation
Molecular understanding of CHD
CHD Gene
Gene CHD
? 10％－ 15％ may caused by single gene
? 85％ may caused by polygenic disorders
Fetal and postnatal circulations
胎儿血循环与出生后改变
Diagrammatic representation
of fetal circulation
? Gas and nutritional requirements exchange occurs
in the placenta
? Pulmonary blood flow is very low
? Foramen ovale and ductus arteriosus are opened
? The blood saturation is higher in up limbs and
head
Fetal Circulation
? Pulmonary ventilation,and respiration,pulmonary
vascular resistance decreases rapidly,systemic blood
saturation increased
? The ductus arteriosus closed by its contraction of
muscular wall,which resulted by bradykinin
? By increasing in pulmonary blood flow,pulmonary
venous return increased,combined with a decrease in
pressure within the right atrium,resulted in a closing of
foramen ovale
Postnatal Circulations
? Ductus arterious closure
? full term baby,80% closed within 24 hours after
birth,95％ anatomic closure within the first year
? Total obliteration of the umbilical arteries in
accomplished by 2-3 months
Postnatal circulation
? 1,left to right shunt －左向右分流型（潜伏紫绀型）
VSD,ASD,PDA
? 2,right to left shunt －右向左分流型（紫绀型）
TOF D-TGA
? 3,without shunt －无分流型（无紫绀型）
PS,AS
Classification of CHD
Ventricular Septal Defects
RA LA
RV LV
PA Ao
上下腔 V
右心房
PA dilation肺动
脉扩张
PA congestion
肺循环充血
Ao blood flow
decreased主动脉血
量减少
Systemic blood flow
decreased 体循环血
量不足
LV over load左心室血量
增加右心室
VSD
Pulmonary
hypertension L-R shuntHemodynamic changes in VSD
LA overload 左心房 血量多
Pulmonary blood
overload肺血量多Superior/Inferior
Vena Cava
RA
RV
1,L Rshunt PA blood ?
systemic blood ?
? 2,Volum overload ? LA,LV,RV dilated
? 3,Pulmonary hypertension ?Eisenmenger
syndrome 艾森曼格综合征
VSD
Hemodynamic changes in VSD
Clinical presentations
Depends on size,PVR(肺静脉阻力 ),associated
lesions
Small-moderate VSD
Grade II-VI harsh pansystolic murmur (PSM
全收缩期杂音 )
At lower left sternal border
Normal P2 (pulmonic second sound)
Clinical presentations
Large shut VSD without PH (pulmonary
Hypertension肺动脉高压 )
P2 is normal,grade III-VI PSM,congestive heart
Failure may present
In present of PH
P2 is loud,shot systolic ejection(喷射性 ) murmur
left sternal bord,may be cyanotic if reversal
of shunt
Laboratory findings
In moderate- large VSD
CXR,cardiac enlargement and increased PA flow
ECG,may variable
In large defect
CXR:marked cardiomegaly,and increased pulmonary
Vascularity
ECG,RVH,LVH,or both
Echocardiography,defining position and size
Doppler echo,estimate the pulmonary systolic pressure
Echocardiography
Parasternal short axis view
胸骨旁短轴切面
Parasternal long axis view
胸骨旁长轴轴切面
室上嵴
Cardiac catheterization
angiographies
Treatment
Small defect may rarely need medical
management other than prophylaxis for SBE
Significant spontaneous closure, 25%
Surgical management:
Refractory CHF; Failure to thrive; repeated
pneumonia ; reversible PH
Atrial septal defect
RA LA
RV LV
PA Ao
IVC,SVC
RA overload
RV dialated
PA dialated
Pulmonary
congestion
Pulmonary
veins
LA
LV volume decreased
Aortic volume
decreased
Systemic volume
decreased
ASD
Hemodynamic changes in ASD
? 1,L R shunt
Pa volume ? systemic volume?
? 2,Cardiac load ? RA,RV,LA大
? 3,Pulmonary hypertension Eisenmenger
syndrome
ＡＳＤ
Late
stage
Hemodynamic changes in ASD
Grade II-VI systolic ejection murmur at left
upper sternal bord,
follow by a mid-diastolic flow rumble in tricuspid
valve region (relative tricuspid valve stenosis)
P2 widely split and fixed(肺动脉固定分裂 )
Clinical presentations
临床表现
ECG show RA dilated,mild RV hypertrophy
CXR show cardiomegaly (RA,RV),increased
PVM
Prominent MPA segment
Echo:reveals a dilated RA,RV plus paradoxical
septal motion (矛盾运动 )
Clinical presentations
CXR,cardiomegaly with increased
pulmonary markings
ECG,RVH,
Clinical presentations
Echocardiography
Subcostal four chamber view
尖突下四腔心切面
Anti congestive medications for CHF
Surgical options
Large L-R shunt; CHF; Pulmonary congestion
Transvenous closure with an occluding device is
now available in some medical centers
Treatment
Interventional therapy
Patent Ductus Arteriosus 动脉导管未闭
RA LA
RV LV
PA Ao
RA
RV PAcongestion
PA congestion
LA
idalated
LV
dialatedAo
Systemic volume
decreased
PA dialated Descending
Ao
Ascending
Ao dialatd
PH
Diastolic BP
decreased
Hemodynamic changes in PDA
PDA
1,L R shunt Pulmonary circulation
Systemic circulation
2,Cardiac volume LA,LV RV
3,Pulmonary Hypertension
differential cyanosis
PDA
Hemodynamic Changes in PDA
clinical presentations
? A symptematic when the ductus is mall,when
defect is large,CHF may develop
? A grade I-VI continuous (machinery) murmur
audible at ULSB or left infraclavical
? An apical diastolic rumble is audible (large
\shunt)
? Bounding peripheral pulses with wide pulse
pressure
Clinical presentation
? EKG findings are similar to those of VSD
? CXR,normal in small PDA;
Cardiomegaly in large PDA
? Echocardiograph can directly determine
the hemodynamic significance
PDA Echo image
PDA Echo image
Parasternal short axis view
治 疗
?Indomethacin is effective in infant within
one week of age
?Prophylaxis should be used when SBE arised
?Surgical ligation and division without
cardiopulmonary bypass is indicated for all
PDAs,regardless of size
?Catheter closure of PDA with various
devices has been approved
Interventional therapy
Surgical procedure for PDA
Tetralogy of Fallot 法洛四联症
hemodynamic changes in TOF
1,R L shunt
RV cyanosis
Pa blood flow decreased
2,RV presure volume LV relatively
small
3,Pa flow hypoximia
VSD LV
Ao
Hypoxic spell 缺氧发作
Treatment
Keep in a knee-chest
Position
Morphine sulfate
suppress the respiratory Center
Treat acidosis
Oxygen inhalation has only limited Calue
Oral propranolol to prevent the attack
Squatting
蹲踞
clinical presentation
Cimplications including:
Polycythemia is common
Brain abscess,cerebrovascular accident
are sometime seen
Thrombosis
Clubbing
Normal heart size,decreased PVMs,boot-
shape heart with a concave MPA
segment,right aortic arch is present
Echo shows a large subaortic VSD,overriding
of aorta,
Clinical presentation
ECG show RA dilated and RV hypertrophy
Blalock-Taussig
The anastomosis between
Subclavian artery and
Ipsilateral PA
Gor-tex interposition shunt
between Subclavian artery
and Ipsilateral PA
Conventional
repair surgery
谢 谢
Congenital Heart Diseases
(CHD)先天性心脏病
General concept
? CHD occurs in approximately 6-8‰ live births
? In China,alone 250000 children with CHD are
born each year
? 30% of cases may die within first year of life
? The etiology remains unsolved
? Secondary prevention is the strategy at this
stage
Congenital Heart Diseases
(CHD)先天性心脏病
Schematic Diagram of
Cardiogenesis
?Bilaterally symmetrical cardiac progenitor
cells form distinct regions of the heart
?The precardiac mesodermal cells give
rise to a linear heart tube
?Forming a rightward loop
?Establishing the spatial orientation of the
four-chambered mature heart
Schematic Diagram of
Cardiogenesis
LVRV
LA
PA
AOconotruncus
RV=right ventricle; LV=left ventricle; RA=right atrium
Ao=aortic; LA=left atrium; PA=pulmonary artery
RA
Fetal development
One month 5th week 6th week
8th week
Cardiac development
Formation of a single
Heart tube
Formation of the heart
loop
Cardiac development
Out flow tract septation Septation of fourchambers
原发房隔
原发孔
继发房隔
原发房隔
继发孔
继发房隔
Atrial septation
Molecular understanding of CHD
CHD Gene
Gene CHD
? 10％－ 15％ may caused by single gene
? 85％ may caused by polygenic disorders
Fetal and postnatal circulations
胎儿血循环与出生后改变
Diagrammatic representation
of fetal circulation
? Gas and nutritional requirements exchange occurs
in the placenta
? Pulmonary blood flow is very low
? Foramen ovale and ductus arteriosus are opened
? The blood saturation is higher in up limbs and
head
Fetal Circulation
? Pulmonary ventilation,and respiration,pulmonary
vascular resistance decreases rapidly,systemic blood
saturation increased
? The ductus arteriosus closed by its contraction of
muscular wall,which resulted by bradykinin
? By increasing in pulmonary blood flow,pulmonary
venous return increased,combined with a decrease in
pressure within the right atrium,resulted in a closing of
foramen ovale
Postnatal Circulations
? Ductus arterious closure
? full term baby,80% closed within 24 hours after
birth,95％ anatomic closure within the first year
? Total obliteration of the umbilical arteries in
accomplished by 2-3 months
Postnatal circulation
? 1,left to right shunt －左向右分流型（潜伏紫绀型）
VSD,ASD,PDA
? 2,right to left shunt －右向左分流型（紫绀型）
TOF D-TGA
? 3,without shunt －无分流型（无紫绀型）
PS,AS
Classification of CHD
Ventricular Septal Defects
RA LA
RV LV
PA Ao
上下腔 V
右心房
PA dilation肺动
脉扩张
PA congestion
肺循环充血
Ao blood flow
decreased主动脉血
量减少
Systemic blood flow
decreased 体循环血
量不足
LV over load左心室血量
增加右心室
VSD
Pulmonary
hypertension L-R shuntHemodynamic changes in VSD
LA overload 左心房 血量多
Pulmonary blood
overload肺血量多Superior/Inferior
Vena Cava
RA
RV
1,L Rshunt PA blood ?
systemic blood ?
? 2,Volum overload ? LA,LV,RV dilated
? 3,Pulmonary hypertension ?Eisenmenger
syndrome 艾森曼格综合征
VSD
Hemodynamic changes in VSD
Clinical presentations
Depends on size,PVR(肺静脉阻力 ),associated
lesions
Small-moderate VSD
Grade II-VI harsh pansystolic murmur (PSM
全收缩期杂音 )
At lower left sternal border
Normal P2 (pulmonic second sound)
Clinical presentations
Large shut VSD without PH (pulmonary
Hypertension肺动脉高压 )
P2 is normal,grade III-VI PSM,congestive heart
Failure may present
In present of PH
P2 is loud,shot systolic ejection(喷射性 ) murmur
left sternal bord,may be cyanotic if reversal
of shunt
Laboratory findings
In moderate- large VSD
CXR,cardiac enlargement and increased PA flow
ECG,may variable
In large defect
CXR:marked cardiomegaly,and increased pulmonary
Vascularity
ECG,RVH,LVH,or both
Echocardiography,defining position and size
Doppler echo,estimate the pulmonary systolic pressure
Echocardiography
Parasternal short axis view
胸骨旁短轴切面
Parasternal long axis view
胸骨旁长轴轴切面
室上嵴
Cardiac catheterization
angiographies
Treatment
Small defect may rarely need medical
management other than prophylaxis for SBE
Significant spontaneous closure, 25%
Surgical management:
Refractory CHF; Failure to thrive; repeated
pneumonia ; reversible PH
Atrial septal defect
RA LA
RV LV
PA Ao
IVC,SVC
RA overload
RV dialated
PA dialated
Pulmonary
congestion
Pulmonary
veins
LA
LV volume decreased
Aortic volume
decreased
Systemic volume
decreased
ASD
Hemodynamic changes in ASD
? 1,L R shunt
Pa volume ? systemic volume?
? 2,Cardiac load ? RA,RV,LA大
? 3,Pulmonary hypertension Eisenmenger
syndrome
ＡＳＤ
Late
stage
Hemodynamic changes in ASD
Grade II-VI systolic ejection murmur at left
upper sternal bord,
follow by a mid-diastolic flow rumble in tricuspid
valve region (relative tricuspid valve stenosis)
P2 widely split and fixed(肺动脉固定分裂 )
Clinical presentations
临床表现
ECG show RA dilated,mild RV hypertrophy
CXR show cardiomegaly (RA,RV),increased
PVM
Prominent MPA segment
Echo:reveals a dilated RA,RV plus paradoxical
septal motion (矛盾运动 )
Clinical presentations
CXR,cardiomegaly with increased
pulmonary markings
ECG,RVH,
Clinical presentations
Echocardiography
Subcostal four chamber view
尖突下四腔心切面
Anti congestive medications for CHF
Surgical options
Large L-R shunt; CHF; Pulmonary congestion
Transvenous closure with an occluding device is
now available in some medical centers
Treatment
Interventional therapy
Patent Ductus Arteriosus 动脉导管未闭
RA LA
RV LV
PA Ao
RA
RV PAcongestion
PA congestion
LA
idalated
LV
dialatedAo
Systemic volume
decreased
PA dialated Descending
Ao
Ascending
Ao dialatd
PH
Diastolic BP
decreased
Hemodynamic changes in PDA
PDA
1,L R shunt Pulmonary circulation
Systemic circulation
2,Cardiac volume LA,LV RV
3,Pulmonary Hypertension
differential cyanosis
PDA
Hemodynamic Changes in PDA
clinical presentations
? A symptematic when the ductus is mall,when
defect is large,CHF may develop
? A grade I-VI continuous (machinery) murmur
audible at ULSB or left infraclavical
? An apical diastolic rumble is audible (large
\shunt)
? Bounding peripheral pulses with wide pulse
pressure
Clinical presentation
? EKG findings are similar to those of VSD
? CXR,normal in small PDA;
Cardiomegaly in large PDA
? Echocardiograph can directly determine
the hemodynamic significance
PDA Echo image
PDA Echo image
Parasternal short axis view
治 疗
?Indomethacin is effective in infant within
one week of age
?Prophylaxis should be used when SBE arised
?Surgical ligation and division without
cardiopulmonary bypass is indicated for all
PDAs,regardless of size
?Catheter closure of PDA with various
devices has been approved
Interventional therapy
Surgical procedure for PDA
Tetralogy of Fallot 法洛四联症
hemodynamic changes in TOF
1,R L shunt
RV cyanosis
Pa blood flow decreased
2,RV presure volume LV relatively
small
3,Pa flow hypoximia
VSD LV
Ao
Hypoxic spell 缺氧发作
Treatment
Keep in a knee-chest
Position
Morphine sulfate
suppress the respiratory Center
Treat acidosis
Oxygen inhalation has only limited Calue
Oral propranolol to prevent the attack
Squatting
蹲踞
clinical presentation
Cimplications including:
Polycythemia is common
Brain abscess,cerebrovascular accident
are sometime seen
Thrombosis
Clubbing
Normal heart size,decreased PVMs,boot-
shape heart with a concave MPA
segment,right aortic arch is present
Echo shows a large subaortic VSD,overriding
of aorta,
Clinical presentation
ECG show RA dilated and RV hypertrophy
Blalock-Taussig
The anastomosis between
Subclavian artery and
Ipsilateral PA
Gor-tex interposition shunt
between Subclavian artery
and Ipsilateral PA
Conventional
repair surgery
谢 谢
