Download Congential heart disease

Approach to patients with
congenital heart disease
Dr.Mazen Naghawi – Pediatric Cardiologist
Albasheer hospital
Pediatric department
Congenital heart disease
congenital cardiac malformations resulting
from defective embryonic development
without obvious cause.
Between 3-8 weeks “gestation” all the fetal
heart structures are formed “organogenesis”
Incidence
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8-10/1000 live births in Jordan
approximately 2000/ year
Incidence is more in :a-Premature
b-abortions
c-still births
Incidence increased for siblings.
2-6%,10-12%,25%
Etiology
Multifactorial inheritance pattern “mostly”

Chromosomal abnormality (5-10%).
-Trisomy 21 (50%) > A-V canal,VSD,ASD, others.
-Trisomy 18 (80%)> VSD,ASD,others.
-Trisomy 13 (40%)> VSD,ASD,PDA,others.
-Turner syndrome (xo)>Bicuspid aortic valve and co-ao
-others.
 Adverse maternal conditions (enviromential)
-Maternal infections >Rubella:PDA,PS
-Maternal diseases> PKU-VSD,ASD
DM:left septal hypertrophy
-Drugs>fetal hydntoin syndrome- VSD
Valproate effect-co ao left heart hypoplasia
-Fetal alcohol syndrome> VSD,ASD,CO-AO.
-Advance maternal age.
 Syndrome complexes
-VACTREL syndrome
Vertebral,Anorectal,Cardiac(VSD,TOF and
others),tracheal,Renal,Oesophageal and Limb
abnormalities.
-CHARGE syndrome
“Coloboma,Heart(VSD,TOF,A-V canal),
Atresia
choanal,Retardation,Gential,Ear abnormalties.
-Kartagener syndrome
-TAR syndrome
Dextrocardia
Majority of cases of the
congenital heart diseases
are unknown cause
Classification
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C.H.D with little or no cyanosis “acyanotic”
a-With Lt. ventricular enlargement
ex.- PDA=8% ,CO-Ao= 5%
- aortic stenosis =7%
b-With Rt. Ventricular enlargement
ex.-ASD=10% ,PS=6%,MS
c-With both ventricular enlargement
ex.- VSD= 25%
Common acyanotic lesions
Ventricular septal defects
 Atrial septal defects
 Atrio-ventricular septal defects
 Patent ductus arteriosus
 Truncus arteriosus
 Aortic stenosis
 Mitral stenosis/incompetence
 Coarctation of aorta
 Tricuspid regurgitation
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C.H.D with cyanosis “mainly Rt. Lt. shunt”
a-With decrease pulmonary vascularity
ex.-TOF=5% , Tricuspid Artesia=1-3%
- PS with or without VSD=5%
b-With increase pulmonary vascularity
ex.-TGA =5% ,Truncus arteriosus=2%
-TAPVR=1%
Common Cyanotic Lesions
Decreased flow
1. Tetralogy of Fallot
2. Tricuspid Atresia
3. Severe Pulmonic Stenosis
4. Ebstein’s anamoly
Increased Flow
5. Transposition of great vessles
6. VSD with pulmonary atresia
Cont-Common Lesions
producing cyanosis
7. Truncus Arteriosus
8. Hypoplastic left heart
9. Single ventricle
10. TAPVR with infradiaphragmatic
obstruction
Prevalence
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Cyanotic: 22%
Acyanotic: 68%
–
–
–
–
–
–
VSD
ASD
PDA
TOF
PS
AS
25%
6%
6%
5%
5%
5%
Ceylon Med J 2001 Sep; 46 (3): 96-8; Indian J Pediatr. 2001 Aug;68 (8):757-7
Nelson’s Textbook of pediatrics; 17 ed.
Diagnosis
Early diagnosis of C.H.D mean better
results.
40% of C.H.D diagnosed at 1st w of life.
50-60 % diagnosed at 1st two months .
Others are usually later during routine
medical examination.
Diagnosis depend on good clinical history +
good medical examination and
investigations.
History
Age of the patient.
Ask for
-Feeding difficulties
D.D
Sepsis
Neonate
Metabolic disorders
-Vomiting
Hypothermia
-Lethargy
-Increased perspiration
IVH
-Rapid respiration
Infant
Others
-Hypoactivity
-F.T.T
All may be manifestation of congestive heart failure.
Older child
-History of dysnea on exertion.
-Shortness of breathing.
-Orthopnea.
-Lower limbs swelling
-Palpitation.
-Convulsion.
-All may be manifest of congestive heart failure.
Physical examination
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Inspection look for:
-Cyanosis
-Digital clubbing
-Tachypnea
-Prominence of the precordium (cardiomegaly,Rt.heart enlargement )
-Jugular veins engorgement “older children”
-Any associated defects or findings (down syndrome, Digorge syndrome…etc)
- Focal neurological lesion.
Palpation
-Pulses (rate, rythem,volume,peripherial pulses ,brachio-femoral delay)
-Cardiac impulses.
-Thrill.
-Hyper dynamic precordium.
-Hepato-splenomegaly
-Sacral edema(neonate, and infancy) ,Lower limb edema in older children
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Auscultation
a-First heart sound (A-V valves closure)
“Best heard at the Lt. lower sternal border or apex”
b-Second heart sound (semilunar valve closure)
“Best heard on the 1st and 2nd I.C.S” , normally there
is normal splitting of the 2nd heart sound ,
-Single
Aortic atresia,Pulmonary Artesia
-Fixed splitting
ASD,PS,Rt.B.B.B
c-Murmurs
Systolic
Diastolic
Continous
Blood pressure
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Methods
sphingnonaometer(different cuffs)
-Flush method
-Palpation method
-Doppler method
Wide pulse pressure
-Aortic insufficiency
-A-V communication
-PDA
Low blood pressure(H.F, pericardial
tamponade,cardiomyopathy).
Difference in BP between upper and lower extremities
Co-ao.
If we suspect C.H.D
Investigation
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CBC---- polycythemia, anemia….etc
CXR----heart size and shape
ECG---HR,axis www.heartaxis.com ,rythm
LVH,RVH,BVH,BBB.
Echocardiography
MRI
Cardiac catheterization
Cyanosis: is it a cardiac
cause or lung cause

Hyperoxia test
– Neonates with cyanotic congenital heart
disease usually do not have significantly
raised arterial Pao2 during administration
of 100% oxygen.
Ventricular Defect

Small VSD
– Asymptomatic
– A loud, harsh, or
blowing holosystolic
murmur.
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Large VSD
– dyspnea, feeding
difficulties, poor
growth, profuse
perspiration, recurrent
pulmonary infections,
and cardiac failure in
early infancy.
80%
Ventricular Septal Defect
(VSD)
Small VSDs, the chest radiograph is usually normal
Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields
(pulmonary hypertension or an associated pulmonic stenosis), gross
cardiomegaly with prominence of both ventricles, the left atrium.
Ventricular Septal defects
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30–50% of small defects close spontaneously, most
frequently during the 1st 2 yr of life.
Small muscular VSDs are more likely to close (up to
80%) than membranous VSDs are (up to 35%).
infants with large defects have repeated episodes of
respiratory infection and heart failure despite optimal
medical management.
Surgical repair prior to development of an irreversible
increase in pulmonary vascular resistance (usually prior
to the patient's second birthday).
Atrial Septal Defects:
secundum
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Most common form of
ASD (fossa ovalis)
In large defects, a
considerable shunt of
oxygenated blood
flows from the left to
the right atrium.
Mostly asymptomatic
The 2nd heart sound is
characteristically widely
split and fixed.
Secundum
Atrial Septal Defects:primum
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Situated in the lower portion of
the atrial septum and overlies
the mitral and tricuspid valves.
In most instances, a cleft in the
anterior leaflet of the mitral
valve is also noted.
Combination of a left-to-right
shunt across the atrial defect
and mitral insufficiency
C/F similar to that of an ostium
secundum ASD
Atrial Septal Defect
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Enlargement of the
right ventricle
Enlargement of
atrium
Large pulmonary
artery
increased pulmonary
vascularity is.
Atrial Septal Defects
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Secundum ASDs are well tolerated during
childhood.
Antibiotic prophylaxis for isolated secundum
ASDs is not recommended.
Surgery or transcatheter device closure is
advised for all symptomatic patients and also for
asymptomatic patients with a Qp:Qs ratio of at
least 2:1.
Ostium primum defects are approached
surgically
Patent Ductus Arteriosus
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Small defect no
symptoms.
Large defect:
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Wide pulse pressure
Enlarged heart
Thrill in L second IS
Continuous murmur
X-ray: prominent
pulmonary artery
with increased
vascular markings.
Primary Pulmonary
Hypertension
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Prominent
pulmonary artery.
Prominent right
ventricle
Prominent
vascularity in the
hilar areas
Decreased vascualr
marking in the
periphery.
No treatment
Cardiac disease with
normal/decreased vasculature
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Tetralogy of Fallot
Pulmonary atresia
Tricuspid atresia
Endocardial fibroelastosis
Aberrant left coronary artery
Cystic medial necrosis
IODM ‘septal hypertrophy”
Tetralogy of Fallot
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Ventricular septal
defect
Pulmonic stenosis
Overriding aorta
Right ventricular
hypertrophy
Cyanotic
Cardiac disease with increased
vasculature
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Atrioventricular septal defects
Congestive cardiac failure
Transposition of great arteries with VSD
Total anomalous pulmonary venous
drainage
Truncus arteriosus
Single ventricle without pulmonary
stenosis
Hypoplastic left heart syndrome
Congestive Cardiac Failure
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Enlarged heart
Plethoric lung
fields specially at
bases
Boot shaped heart in pt. with
TOF
Snow man or figure8 in pt. with TAPVR
Cardiomegaly
Cardiomegaly with pulmonary
edema
Ribs notching in pt. with Co-Ao
Situs inverses
Acrocyanosis:Bluish discoloration of the hands and feet
commonly seen in newborns
Suffused face due to cold around
neck and not central cyanosis
2D echo “Transthoraic echocardiography
3D Echocardiography
Transoseophageal echocardiography
Fetal echocardiography
Treatment of C.H.D
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This is depend on the type of the C.H.D.
No treatment (observation+reassurance)
Medical treatment(antifailure,antiarythmaic..etc).
Surgical treatment (palliative or curative).
Cardiac transplant or lung heart transplant.
1-General measures
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Special positions. (semisiting ,knee chest position (
O2 (most patients need O2 and other need little O2).
IVF(again depend on type of CHD , some need IVF as PDA and PS ,
OTHERS need IVF when ever there are pulmonary congestion or
volume overload).
Salt restriction.
Exercise restriction.
Rx of anemia.
Rx of polycythemia. PCV>65
Avoidances of dehydration mainly polycythemic patients.
Avoidances of high altitude.
Avoidance of contraceptive “thrombosis+hypertension”.
Correction of acidosis.
Correction of electrolyte disturbances .
Careful monitoring during surgery.
2-Rx of congestive heart
failure
– Digoxin
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Digitalization “0.04mg/kg”
Maintenance “0.01mg/kg”
Loop diuretics “frusemide 1-2 mg/kg/day”.
Potassium sparing diuretics “spironlactone”
After load reducing agents
eg. Captopril 0.5-6mg /kg/24 hours.
Positive intropic agents .”dopamine and
dobutamine”
3-Rx of cardiac arrhythmias
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Digoxin (be aware of toxicity –nausia ,vomiting,AV block, K, ca).
Quinidine( hemolytic anemia.SLE,perodic paralysis)
Procainamide(agranulocytosis,+ve coombs hemolytic anemia)
Lidocaine (confusion, convulsion,respiratory failure,AV –block).
Propranolol(bradycardia,bronchspasm,hypotension…etc).
Verapamil(bradycardia,PR interval prolongation,CHF).
Adenosine(chest pain,flushing,bronchospasm,bradycardia)
4-Rx of the cyanotic
spells
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Try to calm the patient .
Knee chest position,
O2
Propranolol IV.
Morphine s.c
NaHCO3 iv
Increase IV fluid.
5-Prophylaxis against infective
endocarditis
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By oral or parental antibiotics.
Given in case of dental procedures,
urinary and GIT .
Instrumental
“proctoscopy,cystescopy”.
Now a days lot of controversy about
who should take the prophylactic
antibiotics.
New guidelines regarding antibiotics to
prevent infective endocarditis
The American Heart Association recently updated its guidelines
regarding which patients should take a precautionary
antibiotic to prevent infective endocarditis (IE) before a trip to
the dentist.
The guidelines, published in Circulation: Journal of the American
Heart Association ,are based on a growing body of scientific
evidence that shows that, for most people, the risks of taking
prophylaxis antibiotics for certain procedures outweigh the
benefits. These guidelines represent a major change in
philosophy.
The new guidelines show taking preventive antibiotics is not
necessary for most people and, in fact, might create more
harm than good. Unnecessary use of antibiotics could cause
allergic reactions and dangerous antibiotic resistance.
Only the people at greatest risk of bad outcomes from infective
endocarditis — an infection of the heart's inner lining or the
heart valves — should receive short-term preventive
antibiotics before common, routine dental and medical
procedures.
http://www.americanheart.org/presenter.jhtml?identifier=3047051
Patients at the greatest danger of bad outcomes from IE
and for whom preventive antibiotics are worth the risks
include those with:
1.
artificial heart valves
2.
a history of having had IE
3.
certain specific, serious congenital (present from birth) heart
conditions, including:
- unrepaired or incompletely repaired cyanotic congenital heart
disease, including those with palliative shunts and conduits
-a completely repaired congenital heart defect with prosthetic
material or device, whether placed by surgery or by catheter
interventions, during the first six months after the procedure
-any repaired congenital heart defect with residual defect at the site
or adjacent to the site of a prosthetic patch or prosthetic
device
4.
a cardiac transplant which develops a problem in a heart valve
http://www.americanheart.org/presenter.jhtml?identifier=3047051
Don’t forget prostglandine
PGE2, AND indomethacine
for selective cases of CHD
Preventive measures
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Avoid pregnancy above age of 38 year.
Avoid smoking.
Avoid alcohol.
Avoid exposure to radiation.
Avoid drugs intake before medical
consultation.
Vaccination of all childbearing women for
rubella.
Innocent murmur
It is functional,normal,insignificent.
 Patient is asymptomatic.
 Murmur heard on routine cardiac
examination without pathological causes.
 Main age between 3-7 years.
 Incidence : up to 30% especially child
with fever,infections,anxiety,and with
cardiac output, the incidence is decreased
with age.
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Characteristics of the
innocent murmur
Mild murmur usually grade one.
 Brief in duration.
 Changing with position.
 No radiation.
 It is systolic,musical,shortand
ejectional.
 Best heard over Lt. lower sternal and
midsternal border.
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Thanks