CARDIOVASCULAR

DISEASES
IN PEDIATRICS

INTRODUCTION:
• The circulatory system, also called
the cardiovascular system or
the vascular system, is an organ
system that permits blood to
circulate and transport nutrients
(such as amino
acids and electrolytes), oxygen, car
bon dioxide, hormones, and blood
cells to and from the cells in the
body to provide nourishment and
help in fighting diseases, stabilize
temperature and pH, and
maintain homeostasis.
Fetal circulation
Flow direction of oxygenated blood (depicted in
pink) from the placenta → umbilical vein (UV) →
ductus venosus (DV) combined with flow from
the portal veins and hepatic veins and IVC → RA
→(mostly) through FO → LA → LV → AAO →
aortic arch branches and → (partly) into RV → PA
→ ductus arteriosus (DA) → descending aorta
(DAO) → umbilical arteries (UA) → lower part of
the body and the placenta Flow direction of less
oxygenated blood (depicted in blue) from the
SVC → (mostly) into RA → RV → PA → (mostly)
through ductus arteriosus → DAO and → (partly)
into both PA branches and lungs. Unrestricted
flow through a widely patent foramen ovale and
ductus arteriosus is essential for fetal survival
 congenital heart defect
• A congenital heart defect (CHD), also known as a congenital heart
anomaly and congenital heart disease, is a defect in the structure of
the heart or great vessels that is present at birth.
• congenital heart disease (CHD) is the most common congenital
abnormality in children, affecting nearly 1% of newborns each year
• One-quarter of these patients have critical CHD (CCHD), defined as CHD
that requires surgical or catheter intervention within the first month
after birth.
• Up to 30% of neonates with CCHD appear clinically normal and are
discharged with the defect undetected.
• Screening for CCHD before discharge from the birth hospitalization
therefore becomes an important aspect of newborn care.
ETIOLOGY AND EPIDEMIOLOGY
• GENETIC CAUSES:
• CHROMOSOMAL ABNORMALITY:
1. Trisomy 21 (Down's syndrome): associated with AV canal , VSD, ASD
2. Trisomy 18 : (Edwards syndrome)VSD, ASD.
3. Trisomy 13: (Patau syndrome) VSD, ASD,PDA
4. Turner syndrome : BICUSPID AORTIC VALVE, COA.

• GENE DEFECTS:
1. DiGeorge syndrome: VSD, conotruncal defect.
2. Noonan syndrome: pulmonary valve stenosis, HCM.

• ADVERSE MATERNAL CONDITION:

• 1. maternal infection: rubella during pregnancy my cause PDA, PS
• 2. maternal diserase: DM in mother may cause asymetric septal hyperatrophy.
• 3. drugs: phenytoin , carbamazipin may cause VSD,
• 4. fetal alcohol syndrome: VSD, ASD , COA
Pathophysiology:
• Congenital heart defects can be divided into three pathophysi-
ological groups:
1. Left-to-right shunts
2. Right-to-left shunts
3. Obstructive, stenotic lesions.
Acyanotic congenital heart disease includes left-to-right shunts
resulting in an increase in pulmonary blood flow (patent ductus
arteriosus, ventricular septal defect, atrial septal defect) and
obstructive lesions (aortic stenosis, pulmonary stenosis, coarctation of
the aorta), which usually have normal pulmonary blood flow.

• Cyanotic congenital heart disease:

Occurs when some of the systemic venous return crosses from the right
side of the heart to the left and returns to the body without going
through the lungs (right-to-left shunt).
Cyanosis occurs when approximately 5 g/100 mL of reduced hemoglobin
is present in systemic blood.
 Atrial Septal Defect

• An opening on the atrial septum, allowing blood flow from the LA into
RA initially.
• Atrial septal defect (ASD) can occur as an isolated lesion or in
combination with other CHDs.
• ASDs represent approximately 10% of all congenital heart defects.
• ASD occurs more often in females than in males (2:1).
• ASD occurs in 1 child per 1500 live births.
GENETICS:
• ASD occurs sporadically in most cases.
• ASD can be seen in genetic syndromes, particularly in Down syndrome
(Trisomy 21) and Holt-Oram syndrome.
• Familial ASD has been reported.
Types:
1. Secundum ASD: the most common
type of ASD
2. A primum ASD, located near the
endocardial cushions, may be part of
a complete atrioventricular canal
defect or may be present with an
intact ventricular septum.
3. The least common ASD is the sinus
venosus defect, which may be
associated with anomalous
pulmonary venous return.
Hemodynamics
• Initially, ASD allows an L–R
shunt (oxygenated blood
from pulmonary veins and LA
enters RA)
→ causing RA, RV, and PA
dilatation
→ eventually pulmonary
hypertension and pulmonary
vascular obstruction
(Eisenmenger reaction) → R–L
shunt→ systemic desaturation
and cyanosis
→ complications of
Eisenmenger reaction
eventually occur

Symptoms:
• Symptoms may not be seen during childhood.
• Frequent symptoms in patients with a large ASD: fatigue, frequent URI, failure to
thrive, and dyspnea on exertion.
• Arrhythmias, particularly those of the atrial type, typically atrial fibrillation, occur
frequently in old patients with untreated ASD.
• Mitral prolapse with regurgitation and tricuspid regurgitation can occur.
• When Eisenmenger reaction occurs, the patient may develop cyanosis, fatigue,
and exercise intolerance.
Physical Signs:
• RV lift (or heave) may be palpable over the precordium.
• Wide and fixed S2 splitting.
• Systolic ejection murmur (SEM) in patients with ASD is loudest at the second
intercostal space along the left upper sternal border and radiates to both lungs.
• When ASD is large (Qp /Qs ratio > 2), diastolic rumble at the tricuspid area
(tricuspid rumble) can be audible.
• No visible cyanosis unless severe Eisenmenger reaction has developed.
Chest Radiography:
• Cardiomegaly, increased pulmonary vascular markings, RA enlargement, RV
dilatation, and prominent MPA conus.
Electrocardiography:
• Right-axis deviation, RA enlargement, RVH, and long PR interval.
Echocardiography:
• Location and size of the ASD
• RA, RV, and PA dilatation
Cardiac Catheterization and Angiography:
• Necessary for device closure of ASD.
• Necessary for measuring PA pressure.
CT and MRI:
Necessary to evaluate pulmonary veins or other structures when their
connections and morphology are not clearly delineated by echocardiography
only.

Natural Course:
1. Spontaneous closure of ASD can occur in young patients, most
frequently before 1 year of age. The possibility of spontaneous closure is
higher in younger patients with smaller defects (<7–8 mm).
2. When a large defect remains unrepaired for a long time, the following
complications can occur.
a) Eisenmenger reaction: – Occurs mainly after 20 years of age. More
frequent in female than male patients, Approximately 5–10% of patients
with a large ASD develop Eisenmenger reaction.
b) Atrial arrhythmia: atrial fibrillation is much more common than atrial
flutter.
c) Mitral valve prolapse and mitral regurgitation.
d) Right ventricular dysfunction and right heart failure.
e) Cerebrovascular complications due to paradoxical embolization.
Treatment
• All ASDs need to be closed by device or surgery before school age.
However, as spontaneous closure can occur in small defects, waiting until 4
years of age is advised when the defect is small with no pulmonary
hypertension or volume overload of the RV.
• ASD can be closed by catheter intervention with a device delivered
percutaneously from the femoral vein.
• Surgery is necessary for large ASDs or their associated defects such as
PAPVC.

Ventricular Septal Defect

(VSD)
 Definition
• An opening on the interventricular septum, allowing blood flow from the LV
to the RV initially.
• Ventral septal defect (VSD) can occur as an isolated lesion or in
combination with other CHDs.
• VSD, the most common congenital heart defect, accounts for 25% of all
congenital heart disease. Perimembranous VSDs are the most common of
all VSDs (67%).

Types:
• Perimembranous VSD: most
common type of VSD accounting
for (67%) of all VSD.
• Subarterial VSD: (subpulmonary
VSD): the upper boundaries of
the defect are the pulmonary and
aortic valves. This type is more
common in the oriental
population.
• Inlet type(AV canal type)
• Muscular VSD: defect
surrounded entirely by the
muscular rim.
Hemodynamics:
• Initially VSD allows LV–RV
shunt
→ causing PA, LA, and LV
dilatations → pulmonary
hypertension and
pulmonary vascular
obstruction (Eisenmenger
reaction)
→ R–L shunt → systemic
desaturation, cyanosis →
complications of
Eisenmenger reaction
eventually occur.

Symptoms:
• Small VSD does not cause any symptoms.
• Large VSD causes symptoms of congestive heart failure, such as poor or
small frequent feeding, failure to thrive, tachypnea, frequent respiratory
infection, fatigue, and dyspnea on exertion.
• As Right ventricular pressure increases with time in patients with large VSD,
the symptoms of heart failure gradually improve. However, as Eisenmenger
reaction eventually occurs, patients develop symptoms and complications of
Eisenmenger reaction.
Physical Signs:
• The typical physical finding with a VSD is a pansystolic murmur, usually
heard best at the lower left sternal border. There may be a thrill.
• Large shunts increase flow across the mitral valve causing a mid-diastolic
murmur at the apex.
• The splitting of S2 and intensity of P2 depend on the pulmonary artery
pressure.
• No visible cyanosis unless severe Eisenmenger reaction has developed.

Chest Radiography:
• Cardiomegaly, increased pulmonary vascular (arterial) markings, LA and
LV enlargement, and prominent MPA conus.
 Normal CXR CXR in VSD

Electrocardiography:
• Small VSD with small L–R shunt: normal ECG
• Large VSD with large L–R shunt: LVH (“still operable state”)
• Large VSD with very large L–R shunt and some degree of pulmonary
hypertension → biventricular hypertrophy (“still operable state”)
• Eisenmenger reaction → RVH and right-axis deviation (probably
“inoperable state”)
Echocardiography:
• Location and size of the VSD
• LA and LV dilatation
• Estimate PA pressure .
• Evaluate valve regurgitation, particularly AR and MR
• Associated defects, particularly anomalies of the aortic arch and
pulmonary veins
 Cardiac Catheterization and Angiography:
• Rarely done for diagnostic purpose only
• Necessary when Eisenmenger reaction is suspected
• Necessary for the device closure of VSD

TREATMENT
Approximately one third of all VSDs close spontaneously.
Small VSDs usually close spontaneously and, if they do not close, surgical
closure may not be required.
Initial treatment for moderate to large VSDs includes diuretics with some time
digoxin and/or afterload reduction.
Continued poor growth or pulmonary hypertention despite therapy require
closure of the defect.
Most VSDs are closed surgically, but some VSDs, especially muscular defects,
can be closed with devices placed at cardiac catheterization