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先天性心脏病先天性心脏病 Congenital Heart Disease Congenital Heart Disease
(CHD)(CHD)(二)
Department of PediatricsSoochow University Affiliated Children’s Hospital
Learning objectives
You should:• Know the signs , symptoms, diagnostic fe
atures and management of the common acyanotic congenital heart disease: PDA
PDA—concept 1
2. Ductus2. Ductus close in response to the rise in Po2 ,blood pH and prostacyclin after birth
3. If this mechanism fails or is reserved by prostaglandin E2, the resulting connection allows blood to flow under pressure from the aorta into the pulmonary arteries
1. Which is ductus Arteriosus?
PDA—concept 2
1.1. In a term infant In a term infant ,, ductus aterductus ateroisus closed spontaneously in oisus closed spontaneously in 3 months3 months in most infant cases. in most infant cases.
2.2. Ductus arteroisusDuctus arteroisus remained premained patent after atent after one one year old or moryear old or more– named e– named PDA PDA
PDA—concept 3
1.In a term infant , PDA is the result of a deficiency in the structural framework of the vessel wall.
2. In the preterm infant is the result of a delay in closure. Therefore, although 100% of premature babies born at 29weeks of gestation will have a PDA, in the vast majority this closes spontaneously.
3. In contrast, 6% of all term newborn have a persistent connection between the bifurcation of the pulmonary arteries and the aortic arch.
Patent Ductus Arteriosus(PDA)
1. L--R shunt CHD
2. 10% of CHD
3. Twice as common in females as in males
4. In preterm infant weighing less than 1500Kg,the frequency of PDA :20%-60%
5. Associated lesions CoA ,or VSD(sometimes)
Hemodynamics changes
The blood in lung field increased, Blood in systemic circulation decreasedPulmonary hypertension(PH) ,reversible -------- irreversible Eisenmenger syndrome Cardiac enlargement (LV,LA)Diameter of ascending aorta is large to normalA widened Pulse Pressure
LV AOPV LA
Pulmanory
circulation
RV RA
Systemic
circulation
SVC
IVCPA
PDA
Symptoms (depending on the shunt of PDA)
None (most common)
recurrent chest infections
Heart failure with large shunt
Signs (depending on the shunt of PDA)
1. None (most common)
2. Pink, normal or large volume, bounding /collapsing pulse
3. BP shows wide Pulse Pressure
4. Precordium is hyperdynamic with LV impulse at apex
5. Thrill at left infraclavicular area and second left intercostal space possible
6. Loud P2 with pulmonary hypertension
7. Third heart sound (S3) with CCF
8. Pulmonary crepitations and hepatomegaly with CCF
9. Continuous waterwheel/machinery murmur loudest at upper LSE, left infraclavicular area and back
Investigation
Chest X-ray (CXR)Electrocardiography(ECG)Echocardiography(2DE)Cardiac catheterization and
angiocardiography
Chest X-ray1. Pulmonary plethora2. The main pulmonary artery segment dilated3. Cardiomegaly (LV,LA)4. Diameter of ascending aorta is Large to norma
l
Normal PDA case
3
1
2
4
Echocardiography
The anatomic location
(the size and shunt of PDA) Color flow doppler
(the direction of the shunt) estimate the pressure
pulmonary pressure or hypertension
Course and prognosis
Closure spontaneously in infant in the vast majority
Adults with corrected defect have normal quality of life
Management Medical management
1.fliud restriction
2.indomathacin and prostacyclin
Interventional therapy
1.Implantation of various umbrella or coil device
2.The first choice of treatment
Surgery ligation in premature infant
Amplatzer occluder deviceDiameter of PDA>2.5mm.
Coiloccluder device ( 弹簧圈)
Diameter of PDA<2.5mm.
Device for PDA closure
Summary
1. PDA is a kind of L to R shunt CHD,
2. The symptom of PDA depends on the shunt.
3. The characteristic heart murmur and P2
4. Complication: (1)Respiratory infection (2)congestive heart failure (3)endocarditis
Summary
5. PDA can close spontaneously in infant
6. Enlarged chambers (LV,LA) can be observed by CXR , 2DE ,and ECG
7. Preventing PH is the key point during the management of PDA patients
Question
1. How to detect and estimate the PH in PDA patient in clinical experience? Why?
2. Important Concept:
① Pulmonary hypertension② differential cyanosis (Eisenmenger syndrome)
③ A widened Pulse Pressure
Learning objectives
You should;• Know the signs , symptoms, diagnostic
features and management of the commonest cyanotic congenital heart disease-TOF
Questions for TOF
1.The mechanism and clinical findings of h
ypercyanotic episode (spells)?
How to treat it?
2. The mechanism of squatting suddenly i
n TOF patient?
Anatomy of TOF
Bay( 隐凹 ) /Oligaemia ( 血量减少 )
1
2
3
(RVOTO)
4
Beneath the aortic outlet
Resulting from RVOTO
The aorta straddles both L and R ventricle
Boot-shaped heart
Hemodynamics changes1. The blood in lung field decreased (oligemia)
2. Cardiomegaly (RV,RA)
3. Diameter of ascending aorta is larger to normal.
LV AO systemicPV LA
SVCRAPApulmanory
VSD Over-riding
RVOTO
RVH
Symptoms depending on the degree of RVOT obstruction
1. Cyanosis (variable, progressive)
2. hypercyanotic episode /blue spells /
Hypoxemic spells 缺氧发作 aged 2years or lessaged 2years or less
3. Squat suddenly after exertion
to ward off hypercyanotic spellsto ward off hypercyanotic spells
4. Exercise tolerance poor
1.At birth the RVOT obstruction is usually not severe and cyanosis may not be obvious.
3.Progressive hypoxemia results in compensatory polycythaemia, including clubbing fingers and toes ( 杵状指、趾)
2.but this becomes evident with increasing activity, often when crawling commences around 10 months of age
Cyanosis (variable, progressive)
Symptoms depending on the degree of RVOT obstruction
1. Cyanosis (variable, progressive)
2. hypercyanotic episode /blue spells /
Hypoxemic spells 缺氧发作 aged 2years or lessaged 2years or less
3. Need to lie down/ Squat suddenly after exertion
to ward off hypercyanotic spellsto ward off hypercyanotic spells
4. Exercise tolerance poor
Squat after exertion
Need to lie down/ Squat suddenly after exertion to ward off hypercyanotic spellsto ward off hypercyanotic spells
Symptoms depending on the degree of RVOT obstruction
1. Cyanosis (variable, progressive)
2. hypercyanotic episode /blue spells /
Hypoxemic spells 缺氧发作 aged 2years or lessaged 2years or less
3. Squat suddenly after exertion
to ward off hypercyanotic spellsto ward off hypercyanotic spells Exercise tolerance poor Need to lie down/
Hypoxemic spells( 缺氧发作 )
Blue spells are characterised by 1.Increasing irritability 2.Prolonged crying
3. Rapid deep respiratory movement
4.A dramatic exacerbation of cyanosis
Paroxysmal hypercyanotic episodes arise in untreated young children aged less than 2 years,
Following defecation 排便, crying or feeding .
During blue spells, a significant increase in RVOT obstruction, blood flow through the outflow decrease ,and the systolic murmur disappears. (mechanism)
signs depending on the degree of RVOT obstruction
1. Central cyanosis 2. Plethoric appearance 3. Hyperdynamic precordium with RV heav
e at left sternal edge 4. Palpable systolic thrill at upper LSE in50
% patients5. S2 aortic and single ;(due to absent pul
monary component)6. Heart murmur: Grade -- / rough EⅡ Ⅳ Ⅵ
SM at upper LSE radiating to back
1. Loud ESM at the upper LSE due to turbulence caused by the infundibular stenosis
2. The large VSD little turblence and therefore does not produce a murmur.
e.g. Grade -- / ESM, Ⅱ Ⅳ ⅥP2 weaken or disappeared
ESM
Practice : typical murmur of TOF
Complications of TOF
1. Progressive cyanosis is associated with failure to thrive
2. Hypercyanotic spells may be associated with syncopal attacks
3. Cerebral ischaemia and thromboses usually occur in the first 2 years of life
4. Cerebral abscess develop in older children
5. Bacterial endocarditis and CCF are rare
Investigation1. Blood routine
Erythrocytosis , hyperglobulism and plasmahErythrocytosis , hyperglobulism and plasmah
yperviscositysyndrome yperviscositysyndrome
Avoiding dehydration such as diarrhea, vomitiAvoiding dehydration such as diarrhea, vomiting and sweatingng and sweating
2. Chest X-ray (CXR)3. Electrocardiography(ECG)4. Echocardiography(2DE)5. Cardiac catheterization and angiocardi
ography 红细胞增多
Chest X-ray
1. Pulmonary oligaemia
2. Small pulmonary conus, (concave)
3. Cardiomegaly (RV,RA)
4. Diameter of ascending aorta is larger
TOF: Boot-shaped heartNormal
2
4
3
1
Echocardiography
1. The anatomic location
2. Color flow doppler
the direction of the shuntthe direction of the shunt
3. estimate the pressure gradient
of RVOT
Medical management
Attempts to improve weight gain are essential
An adequate haemoglobin should be maintained ,especially i
n patients with severe cyanosis and those with hypercyanotic
spells
Emergent treatment for.hypercyanotic spells
1.placed knee to chest position (stimulated squatting)
2.Given oxygen
3.intravenous sodium bicarbonate (acidosis 酸中毒 )
4.Intravenous morphine (sedation, relief pain and RVOTO)
5. Regular oral Propranolol ( 心得安 ) until surgery
Management---Surgery
1.1. The palliative blalock-Taussig shuntThe palliative blalock-Taussig shunt
improves pulmonary blood flow ,It is employed in sever
ely cyanosed infants aged less than 6 months ,those wh
o are medically unfit for a major procedure, and those wi
th hypercyanotic spells
2.2. The definitive repairThe definitive repair
involves total reconstruction of the RV outflow tract and
closure of VSD, The operative mortality is less than 5%
Summary
The commonest cyanotic CHD,The commonest cyanotic CHD,
R to L shuntR to L shunt
The typical symptom :The typical symptom :
1. 1. Cyanosis after the neonatal periodCyanosis after the neonatal period
2. Hypercyanotic spells during infancy2. Hypercyanotic spells during infancy
3. Squatting suddenly after infancy3. Squatting suddenly after infancy
The characteristic heart murmur and PThe characteristic heart murmur and P2 decreased 2 decreased