Congenital Cardiovascular Anomalies

7/28/2019 Congenital Cardiovascular Anomalies

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Congenital Cardiovascular

Anomalies

dr. Herlina Dimiati, SpA (K)

SMF / Bagian Kardiologi Pediatrik

RSU Dr. Zainal Abidin

Fakultas Kedokteran Universitas Syiah Kuala

Banda Aceh


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Classification of CHDs

1. Structural heart defects due to

abnormal development of the heart

during the first 2 months after conception

2. Functional heart defects

ex: congenital heart block

1. Positional heart defects ex: dextrocardia


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Dextrocardia

May occur: With Situs Inversus: carries

a slightly increased risk ofheart defects (~ 5 10%associated with other

CHDs)

Without Situs Inversus:carries a greatly increasedrisk of associated heartdefects (~95% associatedwith other CHDs)

Both conditions areEXTREMELY rare

Situs Inversus


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Classifications of

Structural Congenital Heart Defects

Increased

Pulmonary

Blood Flow

Decreased

Pulmonary

Blood Flow

Obstruction to

Systemic Blood

Flow

PDAASD

VSD

AV Canal

Total Anomalous

Pulmonary Venous

Return

Truncus Arteriosis

Tetralogy of FallotTransposition of the

Great Arteries

Pulmonary Stenosis

Pulmonary Atresia

Tricuspid Atresia

Coarctation of theAorta

Aortic Stenosis

Hypoplastic Left

Heart Syndrome

Mitral Stenosis


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Shunts

Right to Left vs. Left to Right

Right to left shunt: un-oxygenated blood is

shunted from the right side of the heart to the left

side, and then enters the systemic circulation.

Left to right shunt: a portion of the oxygenated

blood is shunted from the left side of the heart to

the right side and enters the pulmonarycirculation, increasing the work load for the right

heart


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Cyanotic vs. Acyanotic

Acyanotic (usually left to right shunts):

PDA, ASD, VSD

Cyanotic (right to left shunts):

TOF, Transposition of the Great Arteries, HypoplasticLeft Heart

O2 Sat less than 95%

Child may have chronic hypoxia

Caused by: Decreased pulmonary blood flowand/or-- Right-to-left shunting: de-oxygenated blood is shunted from the right side of the heart to the

left side without traveling though the pulmonary circulation, and blood ejected from the left

side of the heart to the systemic circulation is only partly oxygenated


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Most Common Congenital Heart

Defects

9%10%

12%

10%15%

44%

Atrioventricular Septal

Defect

Coarctation of theAorta

Tetralogy of Fallot

Transposition of the

Great Arteries

Ventricular Septal

Defects

All other congenital

heart defects

These account for 85% of all

CHDs:


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Some Statistics

Most common birth defect 30% of all

congenital birth defects

(36,000/yr in the United States)

Most common cause of infant death for children

dying as the result of a birth defect

In the US over 130,000 hospitalizations/year are

related to CHD


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Etiology of CHD

Unknown in most cases

Incidence of CHD in children is slightly

increased if a sibling or parent has CHD

Gender Factors

Environmental Factors

Genetic Factors


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Gender Factors

Occur equally among males and females,

but

More common in males:

aortic stenosis, coarctation of the aorta

More common in females:

PDAs, ASDs


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Environmental Factors

Maternal Infections: Rubella: PDA, pulmonary stenosis, VSD, ASD

Maternal Drugs:

Lithium: Tricuspid valve abnormalities, Ebsteins Anomaly

Thalidomide

Possibly related to CHDs: Dilantin & Cocaine Alcohol abuse: VSD

Maternal Disease:

Diabetes: transportation of the great vessels, VSD, situs inversus,single ventricle, hypoplastic left ventricle

SLE: Congenital heart block


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Genetic Factors

Trisomy 21 (Downs Syndrome):

A-V canal defects, VSD

XO (Turners Syndrome):coarctation of the aorta, aortic stenosis

Osteogenesis Imperfecta:

Aortic incompetence

Marfan Syndrome:

Aortic dilatation, aortic & mitral incompetence


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The good news is--

From 1991 2001 deaths related to CHD

declined 28% due to improvements in surgical

techniques and medical management


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Prevention of CHD

Not possible in most cases

But -- there are actions a woman can take to

reduce her risk of having a child with CHD:

Abstain from alcohol during pregnancy

Be immunized against rubella before conception

If diabetic, maintain tight control of blood sugars

Folic acid 400 mcg/daily before conception may help

to prevent CHD (unproven)

If there is a family history of CHD seek genetic

counseling prior to conception


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Signs/Symptoms of CHD

Murmurs

Cyanosisworsens with crying or other exertion

Respiratory distress

Signs of poor perfusion, such as slow capillary

refill, diminished peripheral pulses

Fatigue commonly observed during feedings in

newborns or during play in children Failure to thrive


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Embryonic Heart Development

The heart develops in the embryo duringpost-conception weeks 3 - 8


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Beginning Development

Early week 3 post-conception: heart begins as 2

endothelial tubes

Mid-week 3 : endothelial tubes fuse to form a

tubular structure 28 days following conception: the single-

chambered heart begins pumping blood


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Week 4

Heart has:

single outflow tract, the truncus arteriosus (divides to

form aorta & pulmonary veins)

Single inflow tract, the sinus venosus (divides to formthe superior and inferior vena cavae)

Single atrium

Single ventricle

AV canal begins to close


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Weeks 5 - 7

Week 5

AV canal closure

complete

Formation of atrial andventricular septums

Heart growing rapidly,

and folds back on itself to

form its completed

anatomic shape

Week 7

Ventricular septum fully

developed

Coronary Sinus forms Outflow tracts (aorta &

pulmonary truck) fully

separated


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8 Weeks After Conception

By the end of the 8th week after conception the

fetus has a fully developed 4-chambered heart


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Fetal Circulation

Before birth the placenta provides the

oxygen needed by the developing fetus

the lungs receive only enough blood to

perfuse the lung tissues due to highpulmonary vascular resistance & fetal

vascular shunts


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Fetal Circulation

Arterial blood in the fetus:

enters the fetal circulation via the umbilical vein:

passes through the ductus venosus and enters the

inferior vena cava flows into the right atrium and passes through the

foramen ovale into the left side of the heart

passes from the right side of the heart, through the

ductus arteriosus to enter the systemic circulation,bypassing the pulmonary circulation


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Fetal Circulation

Venous blood in the fetus:

returns to the placenta through the 2 umbilical

arteries


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After Birth

Lungs distend with air and pulmonary

vascular resistance falls. Pulmonary

blood flow increases

The foramen ovale and ductus venosususually close during the first day of life

The ductus arteriosus usually closes

during the first 24 72 hours of life


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Common Congenital Heart

Anomalies


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Patent Ductus Arteriosus (PDA)

Usually closes within 24 to 72 hours after birth

Closure of the ductus may be delayed, or not

occur at all in preterm infants

Patent PDA causes increased pulmonary bloodflow, pulmonary congestion, increases the

workload of the right ventricle; causes increased

pulmonary venous return and increases

workload of the right ventricle


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PDA


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Coarctation of the Aorta

Localized narrowing of

the aorta

More common in males

than females

Associated with Turners

Syndrome

Most common clinical

sign: weak pulses &

decreased blood

pressure in the lower

extremities


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VSD

Most common

congenital heart

defect

May occur alone, orwith other

abnormalities

About one-third of

small VSDs will close

spontaneously


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Truncus Arteriosus

Truncus fails to dividecompletely duringfetal life, leaving aconnection between

the aorta andpulmonary arteries

Mixed oxygenatedand de-oxygenatedblood exits the heartand enters thesystemic circulation


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Tetralogy of Fallot

TOF = Ventricular septal

defect

Aorta position is

shifted to the right andover-rides the VSD

Stenosis of thepulmonary outflow

tract, often involvingthe pulmonary valve

Hypertrophy of theright ventricle


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Transposition of the Great Vessels

The aorta originates from

the right ventricle; the

pulmonary artery

originates in the left

ventricle A PDA is necessary for

these infants to survive

until they can have

corrective surgery More common in infants

of diabetic mothers


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Hypoplastic Left Heart

Fatal without early

surgical intervention


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TAPVR

The pulmonary veins,

instead of being

connected to the left

ventricle, areconnected to the right

ventricle or superior

vena cava, and return

oxygenated blood tothe right side of the

heart.


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AV Canal

Includes:

ASD

VSD

Abnormalities of theMitral and/or Tricuspid

valves

Greater incidence inchildren with Downs

Syndrome

Congenital Cardiovascular Anomalies

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