HUANG Guoying（黄国英）
Department of Pediatrics
Fudan University Shanghai Medical School
Children’s Hospital of Fudan University
Cardiovascular Diseases
in Children
（ Conspectus ）
The Aim of This Class
? To understand the classification of CHD
? To know well the embryological development
of heart,causes of CHD and its pathogenesis
? To know well the approaches of evaluation of
CHD
? To know the characteristics of fetal
circulation & changes after birth
? To have a general idea about cardiovascular
diseases in children,and the principle of
treatment of CHD
Children’s Hospital
Founded in 1952 Professor CHEN Cuizhen
Cardiovascular Diseases in Children
? Congenital heart disease
? Viral myocarditis
? Rheumatic heart disease
? Cardiomyopathy
? Kawasaki Disease
? Arrhythmia
? Congestive heart failure
? Pericarditis
? Infective endocarditis
? Etc….
Embyological Development of Heart
? Formation of primitive heart tube
? Looping of primitive heart tube
? Formation of endocardial cushion
? Formation of interatrial septum
? Formation of interventricular septum
? Septation of aortic and pulmonary arteries
Formation and Looping of
Primitive Heart Tube
Formation of Endocardial Cushion
AV canal posterior right AV orifice
anterior left AV orifice
Formation of Endocardial Cushion
Septation of Atria and Ventricles
ostium primum
1＝ LA
胚胎第 3周 胚胎第 4周 胚胎第 5-6周 胚胎第 7-8周
2＝ LV 3＝ RA 4＝ RV 5＝ septum primum
6＝ septum secundum 7＝ endocardial cushion
ostium secundum foramen ovale
8,9＝ ventricular septa
Interventricular
foramen
Septation of Atria
Septation of Ventricles
IVS is composed of
three parts：
① muscular septum
② cushion component
③ A-P septum
component
Septation of Great Arteries
AO
PA
PA
AO
PA
PA AO
AO
PA AO
P
A
P
A PA
P
A
Malposition of Great Arteries
TOF TGA
DORV Taussig-Bing
Evolvement of Arterial Arches
obsolete
soon after birth
carotid arteries
subclavian A.
brachiocephalic A.
ductus arteriosus
branches of
pulmonary artery
1st pair
2nd pair
3rd pair
4th pair
5th pair
6th pair
Fetal Circulation
& Changes after Birth
after
birth
before
birth
Congenital Heart Diseases
? Definition：
Cardiovascular malformations present
during embryonic stages
（胚胎期发生的心血管畸形）
? Prevalence：
－ 7~8 / 1000 newborns（ reported data）
－ 6.87 / 1000 newborns in Shanghai
（ epidemiologic survey during 1989～ 1991）
Etiologic Considerations
Hereditary factors
? Trisomies of chromosome 21,18,13
? CATCH22 syndrome （ deletion of 22 q11 )
（ 22号染色体长臂 q11区域微小缺失）
? Gene defects：
－ Mutations of Fibrillin in William’s syndrome
－ Defects of Elastin in Marfan’s syndrome
－ Mutations of Cx43 in Hypoplastic left heart
Etiologic Considerations
Environmental factors
? Exposed to the following factors during
the first trimester of pregnancy
－ Viral infections,rubella,influenza,
enterovirus,paratitis
－ Physical and chemical factors,drugs,
radiation,alcohol,tobacco
－ Diseases,diabetes,connective tissue
problems,congenital defects,
hypertension syndrome
Pathogenesis
? Hypostasis（本质）：
－ Heart development is a very complex
process involving many coordinated
steps
－ Abnormal embryological development
of the heart leads to CHD
? Mechanism（机制）：
－ Genetic basis interacted with the
environmental factors play role possibly
in most cases
? 教学要求
1.掌握先天性心脏病的分类
2,熟悉心血管胚胎发育, 先心病病因及病理机制
3,熟悉小儿循环系统常用检查方法
4,了解胎儿循环特点及出生后变化
5,了解小儿循环系统疾病概况及先心病治疗方法
? 复习与思考题
1.先心病的分类及其代表性疾病
2,先心病的临床表现及常用的检查方法
3.心脏胚胎发育与先心病的关系
Classification of CHD
Based on Hemodynamics
? Left-to-right shunt lesions：
－ VSD,ASD,PDA
? Right-to-left shunt lesions：
－ TOF,D-TGA,TA
? Non-shunt lesions：
－ PS,AS,CoA
Left-to-right Lesions
Patent Ductus Arteriosus
Atrial Septal Defect
Ventricular Septal Defect
Right-to-left shunt lesions
? Tetralogy of
Fallot
? Transposition
of the Great
Arteries
? Tricuspid
Atresia
Non shunt lesions
? Pulmonary
Stenosis
? Aortic
Stenosis
? Coarctation
of the aorta
Diagnostic Evaluation of CHD
Approaches of evaluation：
－ History taking
－ Physical examination
－ Electrocardiogram（心电图）
－ Chest X－ ray（胸片）
－ Echocardiogram（超声心动图）
－ Magnetic resonance imaging（磁共振成像）
－ Cardiac catheterization（心导管检查）
－ Angiocardiography（心血管造影）
History Taking
? From mother and family:
－ History of mother during pregnancy
－ Abnormal history of previous pregnancy
－ Family history of CHD,chromosome diseases
? From the child
－ Recurrent pneumonia
－ Cyanosis
－ Heart murmur found before
－ Cardiac disfunction,feeding difficulty,tachypnea,
sweating,edema
－ Others,hoarseness when crying,skinny
Cardiac Examination
? Inspection：
－ Prominence of precordium（心前区隆起）
－ Increased cardiac activity（心脏搏动弥散）
－ Jugular venous distension（颈静脉怒张）
? Palpation：
－ Apex impulse (心尖搏动）
－ Heave of impulse（抬举样搏动）
－ Precordial thrill（震颤）
? Percussion：
－ Estimation of size and location of the heart
Cardiac Examination
Auscultation：
－ Rate and rhythm of heart beats
－ Normal heart sounds,
S1,S2 (A2 & P2),S3,S4
－ Abnormal heart sounds,
splitting,intensity,ejection clicks,S3,S4
－ Murmurs,
systolic,diastolic,continuous
－ Pericardial friction rub
Cardiac Examination
Heart Rate and Rhythm
Neonate 120～ 140 bpm
Infancy 110～ 130 bpm
Toddler’s age 100～ 120 bpm
Preschool age 80～ 100 bpm
School age 70 ～ 90 bpm
Cardiac Examination
Differential diagnosis of heart murmur
CHD Innocent
location 2～ 4LSB 2～ 4LSB,apex
phase SM,DM,CM SM,short
quality harsh soft
intensity >gradeII <gradeIII
radiation extensive localized
variation less more
Noncardiac Examination
? Arterial pulse,
－ rate,rhythm,quality,amplitude,
uniformity at limbs
? Arterial blood pressure:
? Extremities：
－ clubbing of fingers & toes,edema
? Abdomen:
－ hepatomegaly,spleenomegaly
Arterial Blood Pressure
? Upper limbs：
－ Systolic BP (mmHg) = age× ２ + 80
－ Diastolic BP (mmHg) = 2 / 3 × BPs
? Lower limbs：
－ BP is 20mmHg higher
? Abnormal BP：
－ 20mmHg higher or lower
Electrocardiography
EKG is essential in assessing hypertrophy of
atria or ventricles,arrhythmias and disorders of
conductive system
Enlargement of Atria
? Enlargement of left atrium：
－ P-wave ≥ 0.09 sec in duration
－ Double peaks of P-wave with
interval of 0.04 sec or more
? Enlargement of right atrium：
－ P II ≥ 0.25 mv in amplitude
－ PV1 ≥ 0.20 mv in amplitude
Enlargement of Left Ventricle
? Rv5 ≥ 3.0 mv in amplitude
? Sv1 ≥ 1.5 mv in amplitude
? Rv5+Sv1 ≥ 4.5mv in amplitude
? Sv1 ≥ 1.5 mv （ shown as QS）
Enlargement of Right Ventricle
? The following signs shown in lead V1:
－ qR complex
－ R > 0.7 mv
－ rsR’ complex with R’ > 1.5 mv
－ Rs complex with R/s ratio as follows：
? < 1yr >5
? 1-3yr >2.5
? 3-5yr >2
? 5-12yr >1.5
? > 12y >1
Roentgenography
? Chest X-ray is
essential in
the
evaluation of
heart size and
pulmonary
vascularity
Echocardiography
? The major
noninvasive
method for
diagnosis of CHD
? To define anatomy,
function,chamber
& vessel size,and
valve
abnormalities
Techniques of Echocardiography
? M-mode Echocardiography
? Two-dimensional Echocardiography
? Doppler Echochardiography
? Three-dimensional Echocardiography
? Transesophageal Echocardiography
? Fetal Echocardiography
? Others
M-mode Echocardiography
Two-dimensional Echocardiography
Doppler Echochardiography
? Christian Doppler:
－ Doppler shift（频移）
? Flow direction,to
detect shunting and
regurgitation
? Duration,systole or
diastole
? Flow character：
laminar or turbulent
? Pulsed Doppler
Echochardiography
? Continuous-wave
Doppler
Echochardiography
? Color Doppler
Echochardiography
Doppler Shift & Flow Character
Pulsed Doppler Echochardiography
Continuous-wave Doppler
Echochardiography
PG＝ 4 x Vmax2
Color Doppler Echochardiography
Color Doppler Echochardiography
Dynamic Three-dimensional
Echocardiography
3D view of ASD
Real-time Three-dimensional
Echocardiography
Magnetic Resonance Imaging
? Valuable tool in
the evaluation of
CHD
? Particularly in
the imaging of
vascular
structures of the
thorax
Cardiac Catheterization &
Angiocardiography
? Performed for the
need of further
anatomic or
physiologic
information
? Performed for the
purpose of
treatment in the
catheterization
laboratory
Cardioangiography
Principle of Management of CHD
? Physical activities properly
? Prevention and cure of infection
timely
? Protection of heart function
? Follow-up regularly
? Therapeutic methods：
－ Interventional catheterization
－ Surgical repair
Interventional Catheterization
?Bolloon Atrioseptostomy
( Rashkind,1966)
? Balloon Angioplasty & then Valvuloplasty
( Kan,1982)
? Device Occlusion of Defects
( Sideris,1990; Amplatzer,1998)
Surgical Repair of CHD
Thank you !
Guo-Ying Huang,M.D.
Professor of Pediatrics
Heart Center,Children’s Hospital
Fudan University
Tel,54524666 - 3065
Email,gyhuang@shmu.edu.cn
Department of Pediatrics
Fudan University Shanghai Medical School
Children’s Hospital of Fudan University
Cardiovascular Diseases
in Children
（ Conspectus ）
The Aim of This Class
? To understand the classification of CHD
? To know well the embryological development
of heart,causes of CHD and its pathogenesis
? To know well the approaches of evaluation of
CHD
? To know the characteristics of fetal
circulation & changes after birth
? To have a general idea about cardiovascular
diseases in children,and the principle of
treatment of CHD
Children’s Hospital
Founded in 1952 Professor CHEN Cuizhen
Cardiovascular Diseases in Children
? Congenital heart disease
? Viral myocarditis
? Rheumatic heart disease
? Cardiomyopathy
? Kawasaki Disease
? Arrhythmia
? Congestive heart failure
? Pericarditis
? Infective endocarditis
? Etc….
Embyological Development of Heart
? Formation of primitive heart tube
? Looping of primitive heart tube
? Formation of endocardial cushion
? Formation of interatrial septum
? Formation of interventricular septum
? Septation of aortic and pulmonary arteries
Formation and Looping of
Primitive Heart Tube
Formation of Endocardial Cushion
AV canal posterior right AV orifice
anterior left AV orifice
Formation of Endocardial Cushion
Septation of Atria and Ventricles
ostium primum
1＝ LA
胚胎第 3周 胚胎第 4周 胚胎第 5-6周 胚胎第 7-8周
2＝ LV 3＝ RA 4＝ RV 5＝ septum primum
6＝ septum secundum 7＝ endocardial cushion
ostium secundum foramen ovale
8,9＝ ventricular septa
Interventricular
foramen
Septation of Atria
Septation of Ventricles
IVS is composed of
three parts：
① muscular septum
② cushion component
③ A-P septum
component
Septation of Great Arteries
AO
PA
PA
AO
PA
PA AO
AO
PA AO
P
A
P
A PA
P
A
Malposition of Great Arteries
TOF TGA
DORV Taussig-Bing
Evolvement of Arterial Arches
obsolete
soon after birth
carotid arteries
subclavian A.
brachiocephalic A.
ductus arteriosus
branches of
pulmonary artery
1st pair
2nd pair
3rd pair
4th pair
5th pair
6th pair
Fetal Circulation
& Changes after Birth
after
birth
before
birth
Congenital Heart Diseases
? Definition：
Cardiovascular malformations present
during embryonic stages
（胚胎期发生的心血管畸形）
? Prevalence：
－ 7~8 / 1000 newborns（ reported data）
－ 6.87 / 1000 newborns in Shanghai
（ epidemiologic survey during 1989～ 1991）
Etiologic Considerations
Hereditary factors
? Trisomies of chromosome 21,18,13
? CATCH22 syndrome （ deletion of 22 q11 )
（ 22号染色体长臂 q11区域微小缺失）
? Gene defects：
－ Mutations of Fibrillin in William’s syndrome
－ Defects of Elastin in Marfan’s syndrome
－ Mutations of Cx43 in Hypoplastic left heart
Etiologic Considerations
Environmental factors
? Exposed to the following factors during
the first trimester of pregnancy
－ Viral infections,rubella,influenza,
enterovirus,paratitis
－ Physical and chemical factors,drugs,
radiation,alcohol,tobacco
－ Diseases,diabetes,connective tissue
problems,congenital defects,
hypertension syndrome
Pathogenesis
? Hypostasis（本质）：
－ Heart development is a very complex
process involving many coordinated
steps
－ Abnormal embryological development
of the heart leads to CHD
? Mechanism（机制）：
－ Genetic basis interacted with the
environmental factors play role possibly
in most cases
? 教学要求
1.掌握先天性心脏病的分类
2,熟悉心血管胚胎发育, 先心病病因及病理机制
3,熟悉小儿循环系统常用检查方法
4,了解胎儿循环特点及出生后变化
5,了解小儿循环系统疾病概况及先心病治疗方法
? 复习与思考题
1.先心病的分类及其代表性疾病
2,先心病的临床表现及常用的检查方法
3.心脏胚胎发育与先心病的关系
Classification of CHD
Based on Hemodynamics
? Left-to-right shunt lesions：
－ VSD,ASD,PDA
? Right-to-left shunt lesions：
－ TOF,D-TGA,TA
? Non-shunt lesions：
－ PS,AS,CoA
Left-to-right Lesions
Patent Ductus Arteriosus
Atrial Septal Defect
Ventricular Septal Defect
Right-to-left shunt lesions
? Tetralogy of
Fallot
? Transposition
of the Great
Arteries
? Tricuspid
Atresia
Non shunt lesions
? Pulmonary
Stenosis
? Aortic
Stenosis
? Coarctation
of the aorta
Diagnostic Evaluation of CHD
Approaches of evaluation：
－ History taking
－ Physical examination
－ Electrocardiogram（心电图）
－ Chest X－ ray（胸片）
－ Echocardiogram（超声心动图）
－ Magnetic resonance imaging（磁共振成像）
－ Cardiac catheterization（心导管检查）
－ Angiocardiography（心血管造影）
History Taking
? From mother and family:
－ History of mother during pregnancy
－ Abnormal history of previous pregnancy
－ Family history of CHD,chromosome diseases
? From the child
－ Recurrent pneumonia
－ Cyanosis
－ Heart murmur found before
－ Cardiac disfunction,feeding difficulty,tachypnea,
sweating,edema
－ Others,hoarseness when crying,skinny
Cardiac Examination
? Inspection：
－ Prominence of precordium（心前区隆起）
－ Increased cardiac activity（心脏搏动弥散）
－ Jugular venous distension（颈静脉怒张）
? Palpation：
－ Apex impulse (心尖搏动）
－ Heave of impulse（抬举样搏动）
－ Precordial thrill（震颤）
? Percussion：
－ Estimation of size and location of the heart
Cardiac Examination
Auscultation：
－ Rate and rhythm of heart beats
－ Normal heart sounds,
S1,S2 (A2 & P2),S3,S4
－ Abnormal heart sounds,
splitting,intensity,ejection clicks,S3,S4
－ Murmurs,
systolic,diastolic,continuous
－ Pericardial friction rub
Cardiac Examination
Heart Rate and Rhythm
Neonate 120～ 140 bpm
Infancy 110～ 130 bpm
Toddler’s age 100～ 120 bpm
Preschool age 80～ 100 bpm
School age 70 ～ 90 bpm
Cardiac Examination
Differential diagnosis of heart murmur
CHD Innocent
location 2～ 4LSB 2～ 4LSB,apex
phase SM,DM,CM SM,short
quality harsh soft
intensity >gradeII <gradeIII
radiation extensive localized
variation less more
Noncardiac Examination
? Arterial pulse,
－ rate,rhythm,quality,amplitude,
uniformity at limbs
? Arterial blood pressure:
? Extremities：
－ clubbing of fingers & toes,edema
? Abdomen:
－ hepatomegaly,spleenomegaly
Arterial Blood Pressure
? Upper limbs：
－ Systolic BP (mmHg) = age× ２ + 80
－ Diastolic BP (mmHg) = 2 / 3 × BPs
? Lower limbs：
－ BP is 20mmHg higher
? Abnormal BP：
－ 20mmHg higher or lower
Electrocardiography
EKG is essential in assessing hypertrophy of
atria or ventricles,arrhythmias and disorders of
conductive system
Enlargement of Atria
? Enlargement of left atrium：
－ P-wave ≥ 0.09 sec in duration
－ Double peaks of P-wave with
interval of 0.04 sec or more
? Enlargement of right atrium：
－ P II ≥ 0.25 mv in amplitude
－ PV1 ≥ 0.20 mv in amplitude
Enlargement of Left Ventricle
? Rv5 ≥ 3.0 mv in amplitude
? Sv1 ≥ 1.5 mv in amplitude
? Rv5+Sv1 ≥ 4.5mv in amplitude
? Sv1 ≥ 1.5 mv （ shown as QS）
Enlargement of Right Ventricle
? The following signs shown in lead V1:
－ qR complex
－ R > 0.7 mv
－ rsR’ complex with R’ > 1.5 mv
－ Rs complex with R/s ratio as follows：
? < 1yr >5
? 1-3yr >2.5
? 3-5yr >2
? 5-12yr >1.5
? > 12y >1
Roentgenography
? Chest X-ray is
essential in
the
evaluation of
heart size and
pulmonary
vascularity
Echocardiography
? The major
noninvasive
method for
diagnosis of CHD
? To define anatomy,
function,chamber
& vessel size,and
valve
abnormalities
Techniques of Echocardiography
? M-mode Echocardiography
? Two-dimensional Echocardiography
? Doppler Echochardiography
? Three-dimensional Echocardiography
? Transesophageal Echocardiography
? Fetal Echocardiography
? Others
M-mode Echocardiography
Two-dimensional Echocardiography
Doppler Echochardiography
? Christian Doppler:
－ Doppler shift（频移）
? Flow direction,to
detect shunting and
regurgitation
? Duration,systole or
diastole
? Flow character：
laminar or turbulent
? Pulsed Doppler
Echochardiography
? Continuous-wave
Doppler
Echochardiography
? Color Doppler
Echochardiography
Doppler Shift & Flow Character
Pulsed Doppler Echochardiography
Continuous-wave Doppler
Echochardiography
PG＝ 4 x Vmax2
Color Doppler Echochardiography
Color Doppler Echochardiography
Dynamic Three-dimensional
Echocardiography
3D view of ASD
Real-time Three-dimensional
Echocardiography
Magnetic Resonance Imaging
? Valuable tool in
the evaluation of
CHD
? Particularly in
the imaging of
vascular
structures of the
thorax
Cardiac Catheterization &
Angiocardiography
? Performed for the
need of further
anatomic or
physiologic
information
? Performed for the
purpose of
treatment in the
catheterization
laboratory
Cardioangiography
Principle of Management of CHD
? Physical activities properly
? Prevention and cure of infection
timely
? Protection of heart function
? Follow-up regularly
? Therapeutic methods：
－ Interventional catheterization
－ Surgical repair
Interventional Catheterization
?Bolloon Atrioseptostomy
( Rashkind,1966)
? Balloon Angioplasty & then Valvuloplasty
( Kan,1982)
? Device Occlusion of Defects
( Sideris,1990; Amplatzer,1998)
Surgical Repair of CHD
Thank you !
Guo-Ying Huang,M.D.
Professor of Pediatrics
Heart Center,Children’s Hospital
Fudan University
Tel,54524666 - 3065
Email,gyhuang@shmu.edu.cn
