congenital heart disease chd n.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
先天性心脏病 Congenital Heart Disease (CHD) PowerPoint Presentation
Download Presentation
先天性心脏病 Congenital Heart Disease (CHD)

Loading in 2 Seconds...

play fullscreen
1 / 49

先天性心脏病 Congenital Heart Disease (CHD) - PowerPoint PPT Presentation


  • 161 Views
  • Uploaded on

先天性心脏病 Congenital Heart Disease (CHD). (二). Department of Pediatrics Soochow University Affiliated Children’s Hospital. Patent Ductus Arteriosus (PDA) 动脉导管未闭. Learning objectives. You should:

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about '先天性心脏病 Congenital Heart Disease (CHD)' - benedict-estes


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
congenital heart disease chd

先天性心脏病Congenital Heart Disease (CHD)

(二)

Department of Pediatrics

Soochow University Affiliated Children’s Hospital

learning objectives
Learning objectives

You should:

  • Know the signs , symptoms, diagnostic features and management of the common acyanotic congenital heart disease: PDA
pda concept 1
PDA—concept 1
  • Which is ductus Arteriosus?

2. 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 pulmonaryarteries

pda concept 2
PDA—concept 2
  • In a term infant ,ductus ateroisus closed spontaneously in 3 months in most infant cases.
  • Ductus arteroisusremained patent after one year old or more– named PDA
pda concept 3
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 pda1
PatentDuctus Arteriosus(PDA)
  • L--R shunt CHD
  • 10% of CHD
  • Twice as common in females as in males
  • In preterm infant weighing less than 1500Kg,the frequency of PDA :20%-60%
  • Associated lesions CoA ,or VSD(sometimes)
types of pda
Types of PDA

funnel漏斗

tubiform管状

window窗型

slide9

PV

LA

LV

AO

Systemic

circulation

PDA

RA

Pulmanory

circulation

PA

RV

SVC

IVC

Hemodynamics changes

  • The blood in lung field increased, Blood in systemic circulation decreased
  • Pulmonary hypertension(PH) ,reversible -------- irreversible Eisenmenger syndrome
  • Cardiac enlargement (LV,LA)
  • Diameter of ascending aorta is large to normal
  • A widened Pulse Pressure
symptoms depending on the shunt of 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
Signs (depending on the shunt of PDA)
  • None (most common)
  • Pink, normal or large volume, bounding /collapsing pulse
  • BP shows wide Pulse Pressure
  • Precordium is hyperdynamic with LV impulse at apex
  • Thrill at left infraclavicular area and second left intercostal space possible
  • Loud P2 with pulmonary hypertension
  • Third heart sound (S3) with CCF
  • Pulmonary crepitations and hepatomegaly with CCF
  • Continuous waterwheel/machinery murmur loudest at upper LSE, left infraclavicular area and back
investigation
Investigation
  • Chest X-ray (CXR)
  • Electrocardiography(ECG)
  • Echocardiography(2DE)
  • Cardiac catheterization and angiocardiography
chest x ray
Chest X-ray

Pulmonary plethora

The main pulmonary artery segment dilated

Cardiomegaly (LV,LA)

Diameter of ascending aorta is Large to normal

4

2

1

3

PDA case

Normal

ecg typical pda
ECG (typical PDA)

Normal or left axis deviation

LA enlarged , LV hypertrophy

echocardiography
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
Course and prognosis
  • Closure spontaneously in infant in the vast majority
  • Adults with corrected defect have normal quality of life
management
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
slide21

Device for PDA closure

Amplatzer occluder device

Diameter of PDA>2.5mm.

Coiloccluder device

(弹簧圈)

Diameter of PDA<2.5mm.

summary
Summary
  • PDA is a kind of L to R shunt CHD,
  • The symptom of PDA depends on the shunt.
  • The characteristic heart murmur and P2
  • Complication: (1)Respiratory infection (2)congestive heart failure (3)endocarditis
summary1
Summary
  • PDA can close spontaneously in infant
  • Enlarged chambers (LV,LA) can be observed by CXR , 2DE ,and ECG
  • Preventing PH is the key point during the management of PDA patients
question
Question
  • How to detect and estimate the PH in PDA patient in clinical experience? Why?
  • Important Concept:
  • Pulmonary hypertension
  • differential cyanosis (Eisenmenger syndrome)
  • A widened Pulse Pressure
learning objectives1
Learning objectives

You should;

  • Know the signs , symptoms, diagnostic features and management of the commonest cyanotic congenital heart disease-TOF
questions for tof
Questions for TOF

1.The mechanism and clinical findings of hypercyanotic episode (spells)?

How to treat it?

2. The mechanism of squatting suddenly in TOF patient?

anatomy of tof
Anatomy of TOF

The aorta straddles both L and R ventricle

2

Boot-shaped heart

Beneath the aortic outlet

1

3

(RVOTO)

4

Resulting from RVOTO

Bay(隐凹) /Oligaemia (血量减少)

slide31

PV

LA

LV

AO

systemic

VSD

Over-riding

RVH

RA

pulmanory

PA

SVC

RVOTO

Hemodynamics changes

The blood in lung field decreased (oligemia)

Cardiomegaly (RV,RA)

Diameter of ascending aorta is larger to normal.

symptoms depending on the degree of rvot obstruction
Symptoms depending on the degree of RVOT obstruction
  • Cyanosis (variable, progressive)
  • hypercyanotic episode /blue spells /

Hypoxemic spells 缺氧发作

aged 2years or less

  • Squat suddenly after exertion

to ward off hypercyanotic spells

  • Exercise tolerance poor
slide33

Cyanosis (variable, progressive)

1.At birth the RVOT obstruction is usually not severe and cyanosis may not be obvious.

2.but this becomes evident with increasing activity, often when crawling commences around 10 months of age

3.Progressive hypoxemia results in compensatory polycythaemia, including clubbing fingers and toes (杵状指、趾)

symptoms depending on the degree of rvot obstruction1
Symptoms depending on the degree of RVOT obstruction
  • Cyanosis (variable, progressive)
  • hypercyanotic episode /blue spells /

Hypoxemic spells 缺氧发作

aged 2years or less

  • Need to lie down/ Squat suddenly after exertion

to ward off hypercyanotic spells

  • Exercise tolerance poor
slide35

Squat after exertion

Need to lie down/ Squat suddenly after exertion to ward off hypercyanotic spells

symptoms depending on the degree of rvot obstruction2
Symptoms depending on the degree of RVOT obstruction
  • Cyanosis (variable, progressive)
  • hypercyanotic episode /blue spells /

Hypoxemic spells 缺氧发作

aged 2years or less

  • Squat suddenly after exertion

to ward off hypercyanotic spells

  • Exercise tolerance poor Need to lie down/
hypoxemic spells
Hypoxemic spells(缺氧发作)
  • Paroxysmal hypercyanotic episodes arise in untreated young children aged less than 2 years,
  • Following defecation 排便,crying or feeding .
  • Blue spells are characterised by
  • 1.Increasing irritability
  • 2.Prolonged crying
  • 3. Rapid deep respiratory movement
  • 4.A dramatic exacerbation of cyanosis
  • 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
signs depending on the degree of RVOT obstruction
  • Central cyanosis
  • Plethoric appearance
  • Hyperdynamic precordium with RV heave at left sternal edge
  • Palpable systolic thrill at upper LSE in50% patients
  • S2 aortic and single ;(due to absent pulmonary component)
  • Heart murmur: GradeⅡ--Ⅳ /Ⅵ rough ESM at upper LSE radiating to back
slide39

Practice :typical murmur of TOF

e.g. Grade Ⅱ--Ⅳ /Ⅵ ESM,

P2 weaken or disappeared

  • Loud ESM at the upper LSE due to turbulence caused by the infundibular stenosis

ESM

  • The large VSD little turblence and therefore does not produce a murmur.
complications of tof
Complications of TOF
  • Progressive cyanosis is associated with failure to thrive
  • Hypercyanotic spells may be associated with syncopal attacks
  • Cerebral ischaemia and thromboses usually occur in the first 2 years of life
  • Cerebral abscess develop in older children
  • Bacterial endocarditis and CCF are rare
investigation1
Investigation
  • Blood routine

Erythrocytosis , hyperglobulism and plasmahyperviscositysyndrome

Avoiding dehydration such as diarrhea, vomiting and sweating

  • Chest X-ray (CXR)
  • Electrocardiography(ECG)
  • Echocardiography(2DE)
  • Cardiac catheterization and angiocardiography

红细胞增多

chest x ray1
Chest X-ray

4

Normal

TOF: Boot-shaped heart

2

3

1

Pulmonary oligaemia

Small pulmonary conus, (concave)

Cardiomegaly (RV,RA)

Diameter of ascending aorta is larger

ecg typical tof
ECG (typical TOF)

Right axis deviation

RV hypertrophy

echocardiography1
Echocardiography
  • The anatomic location
  • Color flow doppler

the direction of the shunt

  • estimate the pressure gradient

of RVOT

slide45

VSD

VSD

Over-riding ventricle septum

Over-riding ventricle septum

VSD

RV outflow obstruction

medical management
Medical management
  • Attempts to improve weight gain are essential
  • An adequate haemoglobin should be maintained ,especially in 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
Management---Surgery
  • The palliative blalock-Taussig shunt

improves pulmonary blood flow ,It is employed in severely cyanosed infants aged less than 6 months ,those who are medically unfit for a major procedure, and those with hypercyanotic spells

  • The definitive repair

involves total reconstruction of the RV outflow tract and closure of VSD, The operative mortality is less than 5%

summary2
Summary
  • The commonest cyanotic CHD,
  • R to L shunt
  • The typical symptom :

1. Cyanosis after the neonatal period

2. Hypercyanotic spells during infancy

3. Squatting suddenly after infancy

  • The characteristic heart murmur and P2 decreased