CONGENITAL HEART DISEASE

DR SUSHEELA N CHOUDHARY PROF & HOD NSHMC

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 INTRODUCTION
 These are the abnormalities of heart present since birth.
 Incidence of such birth defect in children is about 0.5 to o.8 %


A N
Incidence of CHD is high in premature children.


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These defect occurs mostly at first 8 week of foetus.
AETIOLOGY
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

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Majority have no known cause.


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Congenital defect in heart is multifactorial


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factors are usually genetic and environmental

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Maternal factors –seizure disorder. intake of anti seizure medications


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 CONT….
 Intake of lithium for depression.
 Uncontrolled IDDM.
 Lupus

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German measles [rubella] in first trimester


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FAMILY HISTORY
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

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High risk if the parents having history of CHD


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if Mother has CHD then the risk is 2.5 to 18 %

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

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if the sibling born with CHD.

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 if one child has CHD then the risk in another child is 1.5 to 5%
 if two child is having CHD then the risk in another child is 5-10%

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 CHROMOSOMAL ABNORMALITY

 Chromosomal abnormality in baby with CHD is 5-10%.

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 trisomy 13,

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turner’s syndrom,


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cri du chat syndrome are having risk of CHD.

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 Down syndrome
• Trisomy 21
• Translocation Down syndrome
• Mosaic Down syndrome

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 Edward syndrome

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 Patau syndrome

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 Turner syndrome

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 Cri du chat syndrome

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 MALPOSITION OF THE HEART
 Normal anatomical –heart is located in chest between the lungs
behind the sternum and above the diaphragm apex slightly left


A N
Apex of the heart is points to the right side of the chest


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Dextrocardia with situs inversus- apex of the heart points to right

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side of the chest with all the organs of the body also transposed in
similar way .and the heart remain in normal position related to the
organs
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

. S H A
Isolated dextrocardia –major anomalies of the heart when apex is

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pointed towards the right without situs inversus so there is
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transpositions of great arteries or atria in relation to the ventricles.

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 SHUNTS
 CYANOTIC CHD
 A. L-R SHUNTS-[LEFT TO RIGHT SHUNTS][late cyanotic/acyanotic group of CHD]
 VSD [ventricular septal defect]

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 ASD [atrial septal defect]


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PDA[patent ductus arteriosus]

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B. R-L SHUNTS-[RIGHT TO LEFT SHUNTS][cyanotic or early cyanotic group of CHD]


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Tetralogy of Fallot


S H A
Transposition of great arteries

.
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Persistent truncus arteriosus



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Tricuspid atresia and stenosis

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 CONTINUING
 OBSTRUCTION
 Coarctation of Aorta
 Aortic stenosis & atresia

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

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Pulmonary stenosis and atresia.

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 VSD

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 VENTRICULAR SEPTAL DEFECT

 INTRODUCTION-most common congenital defect

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

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VSD is also associated with other CHD.

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Most of the VSD is spontaneously closed


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DEFINITION—it is a congenital anomaly where there is defect in

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ventricals.
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interventricular septum that allow shunting of blood between left and right

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 CLASSIFICATION ACCORDING TO LOCATION [TYPES OF
VSD]
 CONAL SEPTAL DEFECT-This type of defect is situated just below the
pulmonary valve .it is the rarest defect


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MEMBRANOUS SEPTAL DEFECT- this type of defect is situated near the
valve.and require surgical treatment because it does not heal spontaneously


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ATRIOVENTRICULAR CANAL TYPE [INLENT ]-VSD is located under the

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tricuspid and mitral valve.

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

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MUSCULAR VENTRICULAR DEFECT –It is the opening at lower portion of

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interventricular septum.heal spontaneously.

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 VENTRICULAR SEPTAL DEFECT

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 INTRODUCTION
 most common congenital defect
 3-5 infant per 1000 live birth having VSD
 It is 25-30% of all CHD

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

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VSD is also associated with other CHD.

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Most of the VSD is spontaneously closed
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DEFINITION—it is a congenital anomaly where there is defect in

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ventricals
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interventricular septum that allow shunting of blood between left and right

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CLASSIFICATION ACCORDING TO LOCATION [TYPES
OF VSD]
 CONAL SEPTAL DEFECT-This type of defect is situated just below
the pulmonary valve .it is the rarest defect


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MEMBRANOUS SEPTAL DEFECT- this type of defect is situated

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near the valve .and require surgical treatment because it does
E
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not heal spontaneously


S H
and mitralY
ATRIOVENTRICULAR CANAL TYPE [INLENT ]-VSD is located

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under the tricuspid
MUSCULARSVENTRICULAR A R valve.


R . D H DEFECT –It is the opening at lower

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portion of interventricular septum.heal spontaneously.

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 CLASSIFICATION ACCORDING TO SIZE [TYPES OF VSD]

 SMALL VSD- when the size is less than 0.5 mm it heals spontaneously.
 LARGE VSD—when the size of VSD is greater than 1cm .90% cases require
surgical management

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 ETIOLOGY
 Ventricular septum form before the 8th week of intrauterine life.
 it require downward growth from endocardial cushion and bulbar ridge
separating PA & AORTA.

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 PATHOPHYSIOLOGY

Pressure of left ventricle is higher than the right ventricle

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Abnormal opening allow the blood from left to right ventricle

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H E Mixing of blood in right ventricle

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S A
Blood enter into the pulmonary artery

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D OU Regurgitation of blood into right atrium

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 PATHOPHYSIOLOGY

 Hypertrophy of right heart

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Increased pulmonary vascular resistant

E L  L-R shunt reduces

H E
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
Y R-L shunt increased

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
S H A Decrease amount of blood for purification

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
D Deoxygenated blood supply to the body

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 CLINICAL FEATURES

 SMALL VSD—mostly asymptomatic. PAN systolic murmur

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usually due to regurgitation in cases such as mitral regurgitation, tricuspid

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regurgitation, or ventricular septal defect (VSD)


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MODERATE VSD-.breathing difficulty

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Slow to weight gain


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MID SYSTOLIC MURMUR-Mid-systolic ejection murmurs are due to blood flow through

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the semilunar valves. They occur at the start of blood ejection — which starts after

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S1 — and ends with the cessation of the blood flow — which is before S2. ... The
resultant configuration of this murmur is a crescendo-decrescendo murmur.



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Respiratory infections.
Sign of LV failure.

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 CLINICAL FEATURES CONTINUING
 LARGE VSD—hepatomegaly with oliguria
 SYSTOLIC MURMUR WITH THRILL- The palpable murmur is known as thrill,
 Failure to thrive

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Biventricular hypertrophy with ccf
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Other clinical features
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Difficult feeding
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Poor weight gain

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Failure to thrive

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 Palpitation ,dyspnoea, cyanosis.
 Increased sweating

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 MORPHOLOGICAL FEATURE
 DUE TO L-R shunting at the ventricular level –pulmonary flow increased and
increased volume of the left side of the heart.


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EFFECTS-volume hypertrophy of the right ventricle.

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 Enlargement and hemodynamic changes in the tricuspid and pulmonary
valves.

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 Endocardial hypertrophy of right ventricle.

S R Y
Pressure hypertrophy of the right atrium
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

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Volume hypertrophy of left atrium and left ventricle .

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Enlargement and hemodynamic changes in the mitral and aortic valves.


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 ATRIAL SEPTAL DEFECT

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 INCIDENCE OF ASD
 ASD is = 10-15% of all CHD
 1/1500 live birth
 M;F =3:1

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

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Condition remains unnoticed in infancy and childhood till pulmonary

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hypertension is induced resulting late cyanotic heart disease.

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 TYPES OF VSD
 FOSSA OVALIS TYPE OR OSTIUM SECUNDUM TYPE
 defect is commonly present in the region of fossa ovalis.


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This defect is commonly present at the upper part of septum.
 Fossa ovalis

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OSTIUM PRIMUM TYPE


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5% cases of ASD lies low in the interatrial septum adjacent to

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atrioventricular valve it affect the lower part of the septum.

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SINUSVENOSUS TYPE-it affect the upper part and may be associated with


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abnormalities of superior vena cava

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 INCIDENCE
 SEX –Female ; male=3:1
 1/5000 live birth
 Ostium secundum counts for 7% of CHD

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 100% ASD ≤3mm

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Most of the secundum are association with holt Oram ‘s syndrome


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Holt Oram syndrome is a disorder that affect bones in the arms and hands

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[the upper limbs] and may also cause heart problem

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 CLASSIFICATION OF ASD

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 PATHOPHYSIOLOGY OF ASD
 Increased left atrial pressure
 L R shunt [blood flow from left to
right
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

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burden on right atria

H Eincreased pulmonary pressure

R D increaded right atrial pressure

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 CONT…
 Most of the cases are asymptomatic
 Systolic murmur is heard on 2nd and 3rd intercoastal space


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Recurrent chest infection breathlessness
 Arrythmias

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Growth failure in children


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Hyperdynamic impulse of right ventricle at the lower left sternal border


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Late cyanosis
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

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S2 split

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 SURGICAL CLOSURE

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 PATENT DUCTUS ARTERIOSUS

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 PATENT DUCTUS ARTERIOSUS (PDA)
 THE DUCTUS ARTERIOSUS is a normal vascular connection between the
aorta and the bifurcation of the pulmonary artery.


life.
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Normally, the ductus closes functionally within the first or second day of

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If the ductus arteriosus remains open beyond 3 months of life in preterm

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infants and after 1 year of life in full-term infants, it is a persistent patent

H Y
ductus arteriosus (PDA). 5 – 10% of congenital heart defects in term infants

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are PDAs, but they are far more common in preterm neonates.


. S H A
The fetal heart starts functioning at around the fourth week of gestation and

R D
completes its formation by the sixth gestational week.

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 RISK & ETIOLOGY
 Prostaglandin-E2 is responsible for keeping PDAs open.
 Risk factors for persistent PDA include trisomy 21, Holt-Oram Syndrome,

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exposure to sodium valproate in-utero, and asphyxia during birth. Rubella
infections during pregnancy can also predispose to PDAs.


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We recognize a PDA by a continuous, machinery murmur over the upper left

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sternal border.

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Generally, they are asymptomatic, but large shunts can lead to recurrent


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lower respiratory tract infections, feeding difficulties, failure to thrive, and
even heart failure.


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We identify these via echocardiograms and can give indomethacin in preterm

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infants, or use surgical methods to close in-term, symptomatic infants.

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 FETAL HEART STRUCTURE
 the fetal heart has a number of different structures to direct blood flow:
 The umbilical vein delivers oxygenated blood from the placenta to the fetus,
providing oxygen and nutrients.

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 The umbilical arteries are used to transport deoxygenated blood away from

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the fetal tissue and back towards the placenta for re-oxygenation.

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 The ductus venosus allows blood from the placenta to bypass the relatively

S
inactive liver.

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The ductus arteriosus is the fusion of the primitive pulmonary artery to the


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aorta, therefore allowing blood to pass straight from the right ventricle into
the aorta and bypass the inactive lungs.

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 FETAL HEART STRUCTURE
 The foramen ovale creates a shunt between the right atrium and the left
atrium so oxygenated blood from the placenta can move to the left atrium.


A N
This allows for the oxygenated blood to pass through the left ventricle and
into the ascending aorta, oxygenating the brain.


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Approximately 50% of the highly oxygenated blood carried by the umbilical

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vein returning from the placenta passes into the ductus venosus (DV), which

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connects to the inferior vena cava (IVC).

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 The remainder of the blood carried by the umbilical vein enters the IVC after

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passing through the fetal liver.

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Blood flow through the DV is regulated by a sphincter mechanism close to
the umbilical vein.

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 PATHOLOGICAL EEFECT
 MORPHOLOGIC FEATURES. ………………. due to left-to-right shunt at the level
of ductus result……… increased pulmonary flow and increased volume in the

N
left heart.
These effects are as follows:
A


E L
i) Volume hypertrophy of the left atrium and left ventricle.


H E
ii) Enlargement and hemodynamics changes of the mitral and pulmonary
valves.

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S A
iii) Enlargement of the ascending aorta.


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 CLINICAL FEATURES OF PDA
 Rapid breathing.
 Shortness of breath (dyspnea).
 Sweating during feedings.

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Fatigue or tiredness.


E E
Feeding and eating problems.


SH Y
Poor weight gain or growth.


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Fast pulse or heart rate

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

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Chest X-ray.

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 Echocardiogram (heart ultrasound).
 Electrocardiogram (EKG).

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 CONT…
 Healthcare providers sometimes diagnose PDA in adults.
 If you had a small PDA as a baby, you may not have got treatment.
 Symptoms can include:

A N
 Heart murmur.

E L
E
Heart palpitations.


SH Y
Pulmonary hypertension.

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 SURGICAL TREATMENT
 Healthcare providers may treat PDA with surgical procedures
 including:


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CARDIAC CATHETERIZATION: During cardiac catheterization, experts insert a

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thin, flexible tube (catheter) into the groin and thread it up through a blood

E E
vessel to the heart. They insert a plug or coil into the heart through the
catheter to close the PDA and stop patent ductus arteriosus blood flow.


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NOTE…..Providers typically don’t perform cardiac catheterization on

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premature babies, though older babies and children can have this

S
. H
procedure.

R
D OU D
Patent ductus arteriosus surgery: Surgeons make an incision in the side of
the chest. They close the PDA with stitches (sutures) or a metal clip.

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