4.

2 Cardio vascular system disorders

1.Congenital heart diseases

2.Acute Rheumatic Fever

08/01/2023 BY:Tamene F.
 Congenital heart diseases

♣ Objectives

♣ At the end of this presentation you will be able to ;

– Overview normal fetal circulation
– Define CHD
– Discuss the types of CHD
– Describe manifestations of d/t CHDs

08/01/2023 BY:Tamene F.
 Overview of Fetal Circulation

Brain storming questions:

? What is fetal circulation?

? How many blood vessels(veins &arteries) in

umbilical cord?
? How many shunts are there in F.circulation?
? What is CHD?

08/01/2023 BY:Tamene F.
 Fetal Circulation
Knowledge about fetal circulation is absolutely necessary
for proper understanding of congenital heart diseases.
Umbilical cord:

2 umbilical arteries: return non-oxygenated blood,

fetal waste, CO2 to placenta
1umbilical vein: brings oxygenated blood and
nutrients to the fetus(from placenta)
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 Fetal circulation cont …
Three shunts are present in fetal life:
 Ductus venosus: connects the umbilical vein to the
inferior vena cava
 Ductus arteriosus: connects the main pulmonary artery
to the aorta
 Foramen ovale: anatomic opening between the right and
left atrium.

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 Fetal circulation cont…
 Placenta is a site of gas exchange & excretion of fetal waste
 Intra cardiac & extra cardiac shunts are present

 Lungs take oxygen from blood rather than supplying it.

 Liver receives the highest percentage of oxygen &
nutrients.

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 Fetal circulation cont
 Fetus receives oxygenated blood from the placenta by umbilical
vein, which enters the fetus at the umbilicus.

 The umbilical vein carries blood to the liver & given off
branches to the left lobe to supply the oxygenated blood &
receives the deoxygenated blood from portal vein

 Most of the umbilical venous blood by passes the liver though

the ductus venosus & enters in the inferior vena cava (also
contains the deoxygenated blood from lower extremities), then
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the right atrium BY:Tamene F.
 Fetal circulation cont…
 From right atrium(RA),1/3rd of return blood enters the left
atrium(LA)though the foramen ovale & the rest 2/3rd flows to the
right ventricle(RV)

 In the LA, there is mixing of blood received from right atrium with
the small amount of venous blood returning from the lungs
through the pulmonary veins.

 From LA, blood flows to the LV which is then pumped in to

ascending aorta & arch of aorta to supply heart, head, neck &
upper extremities.
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 Fetal circulation cont
 The right ventricular blood is pumped in to the pulmonary trunk &
a small amount of it enters the pulmonary circulation.

 The major portion the blood by passes the non functioning lungs
through the ductus arteriosus in to the descending aorta &mixed
with the small amount of blood from aortic arch which then supply
to lower extremities & other structures below the diaphragm

 The deoxygenated blood leaves the blood by two umbilical

arteries(branches of internal iliac arteries)

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08/01/2023 BY:Tamene F.
 Blood Flow Changes at Birth

 Lungs expand with air

 Fetal lung fluid leaves air

sacs

 Fluid replaced by air in air

sacs

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 Blood Flow Changes at Birth

 Blood oxygen levels

rise

 Ductus arteriosus
begins to close

 Blood flows through

lungs to pick up
oxygen

08/01/2023 BY:Tamene F.
 CONGENITAL HEART DISEASES(CHD)
A problem in the structure of the heart or great vessels, present at birth.

Symptoms can vary from non to life-threatening.

Causes of CHD

Idiopathic in most of the cases

Chromosomal abnormality: Down syndrome

Adverse maternal conditions (environmental)

Congenital infections: Rubella (PDA)

Substance abuse: Alcohol (VSD)

Drugs – valproate

 Advance maternal age

08/01/2023 BY:Tamene F.
 Types of congenital heart disease
1. Acyanotic congenital heart disease
There is increased pulmonary blood flow due to left to right shunt
 Ventricular septal defect
 Atrial septal defect
 Patent ductus arteriosus
 Coarctation of aorta
2. Cyanotic congenital heart disease (4T’S)
There is decreased pulmonary blood flow due to right to left
shunt
 Tetralogy of fallot
 Truncus arteriosus.
 Transposition of great arteries
 Tricuspid atresia
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08/01/2023 BY:Tamene F.
 Acyanotic CHD

Ventricular septal defect

The commonest congenital heart defect

40% of all congenital heart diseases

Small up to large defects reported.

Small defects (< 5mm) close spontaneously

Blood shunts from left to right at ventricular level

with excess flow to the lung
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 VSD…

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 Cont…

Clinical Manifestation
 Small defects with trivial Lt to Rt Shunt

- Mostly asymptomatic
- Loud, harsh holosystolic M at LLSB

 Large defects
- Excessive pulmonary blood flow

- Pulmonary hypertension
- Dyspnea, feeding difficulties, poor growth, sweating, recurrent plum.
infections, heart failure
08/01/2023 BY:Tamene F.
 Diagnosis

- Clinical
- CXR - Cardiomegaly
- Increased/Plethoric lung
- ECG
- Echocardiography
Treatment
- Small defects - reassurance
- Prophylaxis against IE
- Large defects - Surgical repair between 6-12m

08/01/2023 BY:Tamene F.
 

Atrial Septal Defect

Defect occur in any portion of the atria septum

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 Diagnosis

 Clinical
 CXR - Right. V & A enlargement
- Large pulm. artery
- ↑ed pulm. vascularity
 ECG
 Echocardiography

Complications - pulm. Hypertension,Eismenger syndrome

Treatment
 Surgery-for all symptomatic

08/01/2023 BY:Tamene F.
 Patent ductus arteriosus(PDA)

 Defect range from few mm to large cm

 Left to right shunt at arterial level
 Excess blood flow to the lung
 Enlarged pulmonary artery and left atrium related to blood
volume
 Uncorrected defect leads to pulmonary vascular disease
and flow reversal

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 PDA…

08/01/2023 BY:Tamene F.
 Patent Ductus Arteriosus
• Symptoms:
– May be asymptomatic if small
– Loud machine murmur
– Dyspnea,tachypena,tachycardia
– Frequent respiratory infections
– Poor feeding , fatigue,
– No wt gain,
– Irritability
– If PDA is large size ,child may go for congestive heart
failure
08/01/2023 BY:Tamene F.
 Diagnosis

- Clinical
- Chest X-ray
- ECG
- Echocardiography
Prognosis
- Small PDA - normal life
- Large PDA - CHF
Treatment - Medical-indomethacine
- Surgical closure

08/01/2023 BY:Tamene F.
Coarctation Acyanotic
of the Aorta CHD…
Occur at any site from the arch of aorta to iliac
2.3 bifurcation
Coarctation of the Aorta
• Occur at any site from the arch of aorta to iliac
bifurcation

08/01/2023 BY:Tamene F.
 Cont …
 Classic signs
1- Disparty in pulse & BP
2 - Radio-femoral delay
3- Systolic M at LMSB & inter-scapular area
Treatment
- Medical - IV PGE1 in neonatal age
- Surgery

08/01/2023 BY:Tamene F.
 Cyanotic CHD (right – to left shunt)

 Develop symptoms early

 Cyanosis is the main feature

 Respiratory distress
 Signs of CHF if there is severe obstruction or
excess flow to the lung
 The presence of VSD and PDA is life saving by
mixing more oxygenated blood
08/01/2023 BY:Tamene F.
 Tetralogy of fallot
 Tetralogy of fallot comprises:
1.Ventricular hypertrophy,
2.Plumonary stenosis
3.VSD and
4. Dextroposition of aorta.
 Deoxygenated blood mixes
through VSD.
 The degree of severity is
determined by the size of
pulmonary stenosis.

08/01/2023 BY:Tamene F.
 Presenting Symptoms of TOF

 Diagnosed with first few weeks of life

 Loud murmur

 Cyanosis

 Respiratory distress

 “Tet Spells”
 Infant assume Squatting position(knee chest)

 CXR - Narrow base & uplifted apex

- A boot or wooden shoe heart

08/01/2023 BY:Tamene F.
 Tetralogy of fallot

Boot shaped heart due to concave PA and up lifted apex as a

result of RVH. There is also right side aortic arch. Up to date 210
08/01/2023 BY:Tamene F.
 Transposition of great arteries
 Aorta arise from RV and
pulmonary artery from LV.
 Deoxygenated blood from RV
circulates to the body
 While oxygenated blood goes to
the lungs.
 Patient dies soon unless there is
mixing of the two parallel
circulation via ASD, VSD or PDA.
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 Clinical Manifestations
 Tachypnea
 cyanosis at birth
 congestive heart failure
Treatment
- PGE1 - emergency
- Surgery

08/01/2023 BY:Tamene F.
 Truncus arteriosus

 Both aorta and pulmonary vessels arise from a

single trunk
 There is mixing of blood in the trunk

 Patients may manifest with CHF if the

pulmonary supply is excessive

08/01/2023 BY:Tamene F.
 Cont …

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 Cont…
Clinical Manifestation
- Cyanosis
- CHF
- Systolic ejection m

Treatment - surgery

08/01/2023 BY:Tamene F.
 Tricuspid atresia
 No outlet from Right atrium to right vent.

 Systemic venous return

Rt atrium

Lt atrium

Left ventricule

systemic Pulmonic
(VSD, PDA)
08/01/2023 BY:Tamene F.
08/01/2023 BY:Tamene F.
 Cont…
Clinical Manifestation
- Cyanosis at birth
- Easily fatiquability
- Exertional dyspnea
Treatment
- PGE1
- Surgery

08/01/2023 BY:Tamene F.
 Acute Rheumatic Fever
 Indirect (non-suppurative) complication of group A beta-
hemolytic streptococcal pharyngitis
 Delayed immune response

 Primarily affects the heart, CNS, joints and the skin

 Carditis is the only long-term complication
 All the others resolve
 Acute Rheumatic …
 Acute rheumatic fever is the most common cause of
acquired heart disease in children living in sub-Saharan
Africa and other 3rd world countries.
 Acute rheumatic fever is preventable
 Prompt and proper treatment of streptococcal pharyngitis
can eliminate the risk for acute rheumatic fever.
 Peak age for ARF is 5 – 15 years (rare before 3 years).
 Risk factors
Poverty, overcrowding and conditions facilitating
spread of gr.A streptococcal pharyngitis.
Magnitude of the immune response to the antecedent
streptococcal pharyngitis.

Rheumatogenicity of gr.A strept strains.

Previous attack of rheumatic fever

 Diagnosis of acute rheumatic fever
Modified Jones Criteria:
Major –
Carditis
Migratory polyarthritis
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules
 Modified Jones …
Minor criterias

Clinical findings

 Arthralgia

 Fever

Laboratory findings

 Elevated acute phase reactants (ESR, CRP).

 Prolonged PR interval on ECG.

 Modified Jones …
Supporting evidence for antecedent streptococcal
pharyngitis:
 Positive throat culture or rapid streptococcal antigen
test.
 Elevated or rising streptococcal antibody titer.
 Modified Jones …
Diagnosis made with:
2 major criteria or 1 major and 2 minor
+
Supporting evidence for antecedent

streptococcal pharyngitis (mandatory)

 Modified Jones …
Exceptions (strict adherence to Jones criteria
not needed):

1. Sydenham’s Chorea
2. Indolent Carditis

3. Rheumatic Fever recurrence

 Major manifestations
1. Carditis (in 50 – 60% of patients)
Pancarditis (myocardium, endocardium and pericardium).
The most specific manifestation of rheumatic fever.
Cardiac murmur – most important manifestation.

2. Migratory polyarthritis (in about 75%):

Most common major manifestation but least
specific.
Almost always asymmetrical and migratory.
 Major manifestations
Larger joints (knees, ankles, elbows, wrists).

Swelling, severe pain, redness, heat, limitation and

tenderness.
No permanent joint deformity.

Untreated – lasts 2 to 3weeks.

Dramatic response to salicylates - hallmark
 Major manifestations
3.Chorea (involvement of Basal ganglia & caudate
nucleus)
• In about 20% of patients with RF.

• Delayed manifestation – usually 3mo or longer.

• Purposeless and involuntary movements, muscle
incoordination, weakness and emotional liability.
• May disappear with sleep.
 Major manifestations
4. Erythema marginatum: In < 5% of cases.

Evanescent, erythematous, macular non pruritic rash

with pale centers and rounded or serpinginous
margins.

Mostly trunk and proximal extremities.

May be induced by application of heat.
 Major manifestations
5. Subcutaneous nodules
In less than 3% of patients with RF.
Firm, painless, freely movable nodules (0.5 – 2cm in size).

Most often seen in patients with carditis.

Usually located over the extensor surfaces of the joints
(elbows, knees and wrists), in the occipital portion of the scalp,
or over the spinous processes.
 Treatment of acute rheumatic fever
General
Place on bed rest and monitor closely for evidence of
carditis.
Antibiotic treatment for 10 days with oral penicillin or
erythromycin or a single IM dose of Benz. Penicillin.

Long-term antibiotic prophylaxis.

 Treatment of acute rheumatic fever
Anti – rheumatic therapy:
Withheld anti-inflammatory treatment till full blown
picture of RF appears.
Pain relief – achieved by acetaminophen.

Migratory polyarthritis and carditis with out

Cardiomegaly or CHF → ASA 100mg/kg/24hr divided
into 4 doses po for 3 – 5 days, then 75mg/kg/24hr
for 4weeks.
 Treatment of acute rheumatic fever
Carditis with cardiomegaly or CHF →
Prednisone 2mg/kg/24hr divided into 4 doses
po for 2 – 3weeks.

While tapering prednisone start ASA

75mg/kg/24hr in 4 divided doses for 6weeks.

Supportive treatment.
 Treatment of acute rheumatic fever

SYDENHAM CHOREA
 Sedatives may be helpful early in the course of chorea;
 phenobarbital (16-32 mg q 6-8 hr PO) is the drug of
choice.
 If phenobarbital is ineffective, then haloperidol (0.01-
0.03 mg/kg/ 24 hr divided bid PO) or chlorpromazine (0.5
mg/kg q 4-6 hr PO) should be initiated.
 Prevention
I. Primary Prevention (prompt and proper treatment of gr. A
streptococcal pharyngitis).
Benz. Penicillin

weight ≤ 27kg→ 600,000IU IM stat.

weight > 27kg→ 1,200,000IU IM stat.
II. Secondary prevention (prevention of recurrence).
Benz. Penicillin 1.2M IU IM every 3 – 4 weeks.
• Oral Penicillin V 250 mg Twice daily
• Sulfadiazine 500-1000 mg Once daily
• Erythromycin 250 mg Twice daily
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