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 to the right atrium
common cardio vascular system disorders in pediatrics
1. 4.2 Cardio vascular system disorders
1.Congenital heart diseases
2.Acute Rheumatic Fever
11/15/2019 BY:Tamene F.
2. 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
11/15/2019 BY:Tamene F.
3. 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?
11/15/2019 BY:Tamene F.
4. 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)
11/15/2019 BY:Tamene F.
5. 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.
11/15/2019 BY:Tamene F.
6. 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.
11/15/2019 BY:Tamene F.
7. 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 to the right atrium11/15/2019 BY:Tamene F.
8. 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.
11/15/2019 BY:Tamene F.
9. 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)
11/15/2019 BY:Tamene F.
11. Blood Flow Changes at Birth
Lungs expand with air
Fetal lung fluid leaves air
sacs
Fluid replaced by air in air
sacs
11/15/2019 BY:Tamene F.
12. Blood Flow Changes at Birth
Blood oxygen levels
rise
Ductus arteriosus
begins to close
Blood flows through
lungs to pick up
oxygen
11/15/2019 BY:Tamene F.
13. 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
11/15/2019 BY:Tamene F.
14. 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
11/15/2019 BY:Tamene F.
16. 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
11/15/2019 BY:Tamene F.
21. 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
11/15/2019 BY:Tamene F.
22. 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
11/15/2019 BY:Tamene F.
24. 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
11/15/2019 BY:Tamene F.
25. Diagnosis
- Clinical
- Chest X-ray
- ECG
- Echocardiography
Prognosis
- Small PDA - normal life
- Large PDA - CHF
Treatment - Medical-indomethacine
- Surgical closure
11/15/2019 BY:Tamene F.
26. Acyanotic CHD…
2.3 Coarctation of the Aorta
• Occur at any site from the arch of aorta to iliac
bifurcation
11/15/2019
Coarctation of the Aorta
Occur at any site from the arch of aorta to iliac
bifurcation
BY:Tamene F.
27. 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
11/15/2019 BY:Tamene F.
28. 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
11/15/2019 BY:Tamene F.
29. 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.
11/15/2019 BY:Tamene F.
30. 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
11/15/2019 BY:Tamene F.
31. 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
11/15/2019 BY:Tamene F.
32. 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.
11/15/2019 BY:Tamene F.
34. 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
11/15/2019 BY:Tamene F.
37. Tricuspid atresia
No outlet from Right atrium to right vent.
Systemic venous return
Rt atrium
Lt atrium
Left ventricule
systemic Pulmonic
(VSD, PDA)
11/15/2019 BY:Tamene F.
40. 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
41. 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).
42. 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
43. Diagnosis of acute rheumatic fever
Modified Jones Criteria:
Major –
Carditis
Migratory polyarthritis
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules
44. Modified Jones …
Minor criterias
Clinical findings
Arthralgia
Fever
Laboratory findings
Elevated acute phase reactants (ESR, CRP).
Prolonged PR interval on ECG.
45. Modified Jones …
Supporting evidence for antecedent streptococcal
pharyngitis:
Positive throat culture or rapid streptococcal antigen
test.
Elevated or rising streptococcal antibody titer.
46. Modified Jones …
Diagnosis made with:
2 major criteria or 1 major and 2 minor
+
Supporting evidence for antecedent
streptococcal pharyngitis (mandatory)
47. Modified Jones …
Exceptions (strict adherence to Jones criteria
not needed):
1. Sydenham’s Chorea
2. Indolent Carditis
3. Rheumatic Fever recurrence
48. 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.
49. 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
50. 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.
51. 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.
52. 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.
53.
54. 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.
55. 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.
56. 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.
57. 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.
58. 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
