Congenital heart disease

Dr Ibraahim Abdullahi Guled MMED

Paediatrician and Neonatology
• Conginital heart defects can be divided into
three pathophysiologic groups

 Left to right shunts

 Right to left shunts
 Obstructive stenotic lesions
 Ventricular septal defect.
• VSDs is the most common form of CHD and accounts for 15%
to 20% of all congenital heart disease.

• The ventricular septum may be divided into a small

memmbaranous portion, and a large muscular portion.
• The VSD may be muscular , or perimemmbranous.

• The defect size may vary in size, ranging from tiny and a large
defect with accompanying CHF and pulmonary hypertension.

• The shunt through the defect is usually left to right, unless the
patient develop pulmonary hypertension.
 Clinical Manifestations:
• With small VSDs the patient is asympyomatic with
normal growth and development.
• With moderate VSDs to largeVSD, delayed growth
and development, decreased exercise
tolerance,repeated pulmonary infections and CHF
and a relatively common during infancy.
• With long standing pulmonary hypertension, a
history of cyanosis and a decreased level of activity.
 Physical Examination
• Infants with small VSD the patient is well developed
and acynotic . Before 2 or 3 month of age , infants
with a large heart disease have a poor weight gain
and congestive heart disease .
• If the patient developed pulmonary vascular
obstructive(PVOD) disease cyanosis and clubbing
occur.
• A systolic thrill may be found in the Lower left sternal
border, pericardial bulge and hyperactivity are
present with a large shuntVSD.
• The second heart disease may be loud in large
shunt and single and loud if patient develop a
PVOD.
• A regurgitant systolic murmur is present in the
lower sternal edge. An apical diastolic murmur
is presnt with a moderate to large shunt.
 Investigations :

• The ECG shows Left ventricular hypertrophy and the left

atrial hypertrophy may be seen with a moderate VSD.
• If pulmonary vascular obstructive diesese the ECG shows RVH
Only.
• The chest X-ray may show cardiomegaly, and increase in the
vascular markings.
• The echocardiography shows the position of the defect , the
effect on the chambers , the magnitude of the shunt and the
pulmonary pressure.
 Management:

• Medical
congestive heart disease is congested with duertics
and digoxin for 2 to 4 month. Frequent feeding with
high calorie formula.
• No exercise restriction if pulmonary hypertension
did not develop.
• Maintaince of good dental hygiene and antibiotic
therapy against infective endocarditis.
 Surgical

• Infants who are small and have no evidence of heart

failure or evidence of pulmonary hypertension are
not candidates for surgery.
• Surgery is usually restricted for patients with
moderate to large VSDs.
• The operation is usually done early before the
patients develop symptoms of pulmonary
hypertension.
 Atrial Septal Defect
• The atrial septal defect is found as an isolated
defected in 5 -10% of all cogenital heart
diseases. As a tpart of a complex disease in
30% to 50% of CHD.
• There are three types of ASDs disease
seccundum defect , primum defect and sinus
venosus.
 The Atrial Septal Defect
• The ostium seccundum is the most
common type of the ASD,making up to 50% to
70%.
• Ostuim primum defects occur in about 30% of
all the ASD.
• Sinus venouses occur in about 10% of all the
atrial septal defects.
 Clinical manifestation:
• Infants with ASDs are usually asymptomatic.
• Physical examination :
• A relatively slender body.
• A widely split S2 , fixed 2 and a grade2-3/6
ejection systolic murmur.
• A typical ascultatory findings may be absent in
infants.
 Investigations :
• Electriocardiography:
– Right ventricular hypertrophy.
• X-Ray studies:
– Cardiomegally with right atrial enlargmentand
right ventricular hypertrophy.
– Prominent main pulmonary artery and increased
pulmonary markings.
• Echocardiography:
– It can show postion ,size of the defect.
 Natural history

• Small defects between 3-8mm closes spontaneously..

• Most children remain asymptomatic, although congestive
heart failure occur.
• If untreated Congestive heart failure and pulmonary
hypertension develops in adulthood.
• With or wihout surgery atrial fibrillation develops in adults.
• Infective endocarditis does not occur in isolated ASD.
• CVA is a very rare complication.
 Medical mangment :
• Exercise restriction is unnecessary.
• Prophlaxis against infective endocarditis is not
uindicated,unless the patient has a mitral
valve prolapse.
• Surgical closure is delayed for 3 to 4 years for
possibility of spontaneous closure.
 Patent ductus arteriosus
• Patent ductus arteiosus occur in almost 5-10%
of all CHD. It is more common in females than
males. A common problem of the immature
babies.
• There is a presistant patency of the normal
fetal structure between the left pulmonary
artery and ascending aorta about 5 -10 mmm
distal to the origin of the left subclavin artery.
 Cinical manfestiation:
• Patients are asymptomatic is if the shunt is
small.
• A large PDA may cause lower respiratory tract
infection atelactasis and congestive heart
failure.
 Physical examination
• Tacycardia and exertional dypsnea may be
present in infants with a large shunt PDA.
• The percordium is hyperactive .
• Asystolic thrill may be presnt at the upper left
sternal border. Bounding pulses with wide
pulse pressure.
• Machinery murmur is best audible at the left
infraclavicular area.
 Electrocardiography :

• Normal or Left ventricular hypethrophy may

be seen.with moderate PDA. Large PDA
congestive heart failure.
 X-Ray studies:
• Normal in small shunts .
• Cardiomegally with an enlargement of left
atrium and left ventricle and ascending aorta.
Increased pulmonary markings.
• With PVOD the heart size is normal.
 ECHOCARDIOGRAPHY

• The PDA can be imaged in most of the

patients , the size can be assessed two
dimenshional ECHO.
 Natural history:
• Unlike PDA s in premature infants ,
spontaneous closure of a PDA does not usually
occur in term infants because it usually occurs
from structural abnormality of ductal smooth
muscle rather than decreased unresponsivnes
to the premature ductus to oxygen.
• Congestive heart failure or recurrent
pneumonia when the shunt is large.
 Natural history:
• Pulmonary vascular obstructive disease ,may
develop if a large PDA,with pulmonary
hypertension untreated.
• Subacute infective endocarditis may occur.
 Management:
• Indomethacin is ineffective interm infants with
PDA.
• No exercise restriction.
• Prophylaxis of endocarditis when necessary.
• Catheter closure using different sizes or
ligature.
 Cyanotic Congenital Heart Dısease .
• Most common cyanotic congenital heart
defects are five Ts:
 Tetralogy of fallot
 Transposion of great arteries
 Tricuspid atresia
 Truncus arteriosus
 Total anomalous pulmonary venous return
• Tetralogy of Fallot (TOF)
• Tetralogy of fallot occurs in 105 Of all CHD.
This is the most common cyanotic heart defect
seen in children beyond infancy.
 PATHOLOGY :
• The original description of TOF included the
following abnormalities :
1. A large VSD
2. RV outflow tract obstruction
3. RVH
4. Overriding of the aorta.
 Clinical manifestation:
• A heart murmur audible at birth,.
• Most patients are symptomatic with cyanosis
at birth. Dypsnea on exertion squatting or
hypoxic spells and later in mildly cyanotic
infants.
• Infants with acyanotic TOF may be
asymptomatic from a large left to right
ventricular shunt.
• Clinical findings
• Easy fatigability and dyspnea on exertion are common.
• When they begin to walk they frequently squat suddenly to
increase systemic vascular resistance to ward off cyanotic
spells.
• Hypoxemic spells, also called cyanotic spells or (Tet) Tetralogy
spells are one of the hallmarks of sever TOF.
• Signs and symptoms of Tet spells
• Sudden onset of cyanosis or deepening of cyanosis.
• Sudden onset of dysnoea.
• Alterations in consciousness, encompassing a spectrum from
irritability to syncope.
• Decrease in intensity and even disappearance of the systolic
murmur.
• Examination
• Child maybe cyanosed at rest.
• Finger and toe clubbing.
• Rt. Ventricular enlargement signs.
• S1 is normal, occasionally, an ejection click is evident at the apex that is
aortic in origin.
• S2 is predominantly aortic and single.
• Rough ejection systolic murmur at the left sternal border in the 3rd ICS
radiating to the back.
• Lab. findings
• Hb, haematocrit, RBC count are usually elevated depending on the degree
of arterial oxygen desaturation.
• Imaging
• CXR shows a heart of normal size.
• Boot shaped heart.
• Oligaemic lung fields..
• Rt. Aortic arch in 25 of cases.
 Physical EX:
• Varying degree of tachypnea, cyanosis and
clubbing.
• Right ventricular tap, systolic thrill.
• An ejection click .
• ECG:
Right atrial dilatation may be present, RVH.
• X_ray :
• Normal heart size decreased pulmonary
markings..
• Boot shaped heart
• Echo :
• shows theVSD size , the outflow tract
obstruction , pulmonary atresia, by showing
direction of the flow.
 Natural History:
• Patients are worsening as they get older due to
stenosis and polythycemia.
• Hyoxic spell(paroxysm of hyperapnea), irritability,
and prolonged crying and increasing cyanosis and
increasing intensity of heart murmur.usually
occurring the morning. A severe spell may cause an
CT scan.
• Growth retardation.
• Cerbrovascular accident and SBE May occur.
• Polythycemia secondary to cyanosis.
 Treatment :
• Treat the hypoxic spells patients are usually
given Oxygen during a spell,
• morphine sulphate is given subcutaneous .
• The infant held in the shoulder , or put in the
Knee –chest postion.
• Soduim bicarbonate for the the acidosis.
• Ketamine i.v.
• Propanalol i.v may reverse the spell.
• The parents should be educated to treat the spell.
• Oral propanalol to prevent the hypoxic spell.
• Dental hygiene and indications against SBE when
necessary.
• Iron deficiency anaemia should be treated.
• Patients should be protected from the dehydration,
anaemia , acidosis and treated if polycthemia with
Hct of more than 65%.
• Treatment
• Palliative treatment.
• (medical and/or surgical such as the modified Blalock
Taussig shunt where a Gore Tex shunt from the subclavian
artery to the ipsilateral pulmonary artery is made)
• Total correction.
• Age newborn to 2 years.
• Major limiting anatomical feature of total correction is the
size of the pulmonary arteries.
 Transposition of the great arteries

• Transposition of the great arteries

Is more common in males.
• Progressive cyanosis develops within the 1st few
hours or days of life not improving on 100% oxygen.
• Baby becomes increasingly blue and acidotic.
breathlessness and heart failure may follow.
• Examination
• Cyanosis.
• No heart murmur.
• 2nd heart sound is loud b/c the transposed aorta lies anteriorly, close to
the chest wall.
• Investigations
• Typical CXR is often typical, egg lying on its side.
• The heart is slightly enlarged.
• Plethoric lung fields.
• Diagnosis is made by ECCHO.
• Treatment
• Early corrective surgery is recommended for transposition.
• Arterial switch operation (ASO) is performed at age 4-7 days.
• Survival after the surgery is 95 in major centers
THANKS