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Approach to patients with congenital heart disease

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Approach to patients with congenital heart disease. Dr.MazenNaghawi Pediatric Cardiologist. Albasheer hospital. Pediatric department – PowerPoint PPT presentation

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Title: Approach to patients with congenital heart disease


1
Approach to patients with congenital heart disease
  • Dr.Mazen Naghawi Pediatric Cardiologist
  • Albasheer hospital
  • Pediatric department

2
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

3
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4
Incidence
  • 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

5
Etiology
  • Multifactorial inheritance pattern mostly
  • Chromosomal abnormality (5-10).
  • -Trisomy 21 (50) gt A-V canal,VSD,ASD,
    others.
  • -Trisomy 18 (80)gt VSD,ASD,others.
  • -Trisomy 13 (40)gt VSD,ASD,PDA,others.
  • -Turner syndrome (xo)gtBicuspid aortic valve
    and co-ao
  • -others.

6
Adverse maternal conditions (enviromential)
  • -Maternal infections gtRubellaPDA,PS
  • -Maternal diseasesgt PKU-VSD,ASD
  • DMleft septal
    hypertrophy
  • -Drugsgtfetal hydntoin syndrome- VSD
  • Valproate effect-co ao left
    heart hypoplasia
  • -Fetal alcohol syndromegt
    VSD,ASD,CO-AO.
  • -Advance maternal age.

7
Syndrome complexes
  • -VACTREL syndrome Vertebral,Anorectal,Ca
    rdiac(VSD,TOF and others),tracheal,Renal,Oesoph
    ageal and Limb abnormalities.
  • -CHARGE syndrome
  • Coloboma,Heart(VSD,TOF,A-V canal), Atresia
    choanal,Retardation,Gential,Ear abnormalties.
  • -Kartagener syndrome Dextrocardia
  • -TAR syndrome

8
Majority of cases of the congenital heart
diseases are unknown cause
9
Classification
  • C.H.D with little or no cyanosis acyanotic
  • a-With Lt. ventricular enlargement
  • ex.- PDA8 ,CO-Ao 5
  • - aortic stenosis 7
  • b-With Rt. Ventricular enlargement
  • ex.-ASD10 ,PS6,MS
  • c-With both ventricular enlargement
  • ex.- VSD 25

10
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

11
  • C.H.D with cyanosis mainly Rt. Lt. shunt
  • a-With decrease pulmonary vascularity
  • ex.-TOF5 , Tricuspid
    Artesia1-3
  • - PS with or without VSD5
  • b-With increase pulmonary vascularity
  • ex.-TGA 5 ,Truncus
    arteriosus2
  • -TAPVR1

12
Common Cyanotic Lesions
  • Decreased flow
  • 1. Tetralogy of Fallot
  • 2. Tricuspid Atresia
  • 3. Severe Pulmonic Stenosis
  • 4. Ebsteins anamoly
  • Increased Flow
  • 5. Transposition of great vessles
  • 6. VSD with pulmonary atresia

13
Cont-Common Lesions producing cyanosis
  • 7. Truncus Arteriosus
  • 8. Hypoplastic left heart
  • 9. Single ventricle
  • 10. TAPVR with infradiaphragmatic obstruction

14
Prevalence
  • Cyanotic 22
  • Acyanotic 68
  • VSD 25
  • ASD 6
  • PDA 6
  • TOF 5
  • PS 5
  • AS 5

Ceylon Med J 2001 Sep 46 (3) 96-8 Indian J
Pediatr. 2001 Aug68 (8)757-7 Nelsons Textbook
of pediatrics 17 ed.
15
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.

16
History
  • Age of the patient.
  • Ask for
  • -Feeding difficulties
  • -Vomiting
  • -Lethargy
  • -Increased perspiration
  • -Rapid respiration
  • -Hypoactivity
  • -F.T.T
  • All may be manifestation of congestive heart
    failure.

D.D
Sepsis Metabolic disorders Hypothermia
IVH Others
Neonate
Infant
17
Older child
  • -History of dysnea on exertion.
  • -Shortness of breathing.
  • -Orthopnea.
  • -Lower limbs swelling
  • -Palpitation.
  • -Convulsion.
  • -All may be manifest of congestive heart
    failure.

18
Physical examination
  • 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 syndromeetc)
  • - 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

19
  • 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
20
Blood pressure
  • 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.

21
If we suspect C.H.D Investigation
  • CBC---- polycythemia, anemia.etc
  • CXR----heart size and shape
  • ECG---HR,axis www.heartaxis.com ,rythm
  • LVH,RVH,BVH,BBB.
  • Echocardiography
  • MRI
  • Cardiac catheterization

22
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.

23
Ventricular Defect
  • Small VSD
  • Asymptomatic
  • A loud, harsh, or blowing holosystolic murmur.
  • Large VSD
  • dyspnea, feeding difficulties, poor growth,
    profuse perspiration, recurrent pulmonary
    infections, and cardiac failure in early infancy.

80
24
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.
25
Ventricular Septal defects
  • 3050 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).

26
Atrial Septal Defects secundum
  • 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
27
Atrial Septal Defectsprimum
  • 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

28
Atrial Septal Defect
  • Enlargement of the right ventricle
  • Enlargement of atrium
  • Large pulmonary artery
  • increased pulmonary vascularity is.

29
Atrial Septal Defects
  • 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 QpQs ratio of at
    least 21.
  • Ostium primum defects are approached surgically

30
Patent Ductus Arteriosus
  • Small defect no symptoms.
  • Large defect
  • Wide pulse pressure
  • Enlarged heart
  • Thrill in L second IS
  • Continuous murmur
  • X-ray prominent pulmonary artery with increased
    vascular markings.

31
Primary Pulmonary Hypertension
  • Prominent pulmonary artery.
  • Prominent right ventricle
  • Prominent vascularity in the hilar areas
  • Decreased vascualr marking in the periphery.
  • No treatment

32
Cardiac disease with normal/decreased vasculature
  • Tetralogy of Fallot
  • Pulmonary atresia
  • Tricuspid atresia
  • Endocardial fibroelastosis
  • Aberrant left coronary artery
  • Cystic medial necrosis
  • IODM septal hypertrophy

33
Tetralogy of Fallot
  • Ventricular septal defect
  • Pulmonic stenosis
  • Overriding aorta
  • Right ventricular hypertrophy

Cyanotic
34
Cardiac disease with increased vasculature
  • 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

35
Congestive Cardiac Failure
  • Enlarged heart
  • Plethoric lung fields specially at bases

36
Boot shaped heart in pt. with TOF
37
Snow man or figure8 in pt. with TAPVR
38
Cardiomegaly
39
Cardiomegaly with pulmonary edema
40
Ribs notching in pt. with Co-Ao
41
Situs inverses
42
AcrocyanosisBluish discoloration of the hands
and feet commonly seen in newborns
43
Suffused face due to cold around neck and not
central cyanosis
44
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45
2D echo Transthoraic echocardiography
46
3D Echocardiography
47
Transoseophageal echocardiography
48
Fetal echocardiography
49
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50
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51
Treatment of C.H.D
  • This is depend on the type of the C.H.D.
  • No treatment (observationreassurance)
  • Medical treatment(antifailure,antiarythmaic..etc).
  • Surgical treatment (palliative or curative).
  • Cardiac transplant or lung heart transplant.

52
1-General measures
  • 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. PCVgt65
  • Avoidances of dehydration mainly polycythemic
    patients.
  • Avoidances of high altitude.
  • Avoidance of contraceptive thrombosishypertensio
    n.
  • Correction of acidosis.
  • Correction of electrolyte disturbances .
  • Careful monitoring during surgery.

53
2-Rx of congestive heart failure
  • Digoxin 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

54
3-Rx of cardiac arrhythmias
  • 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,hypotensionet
    c).
  • Verapamil(bradycardia,PR interval
    prolongation,CHF).
  • Adenosine(chest pain,flushing,bronchospasm,bradyca
    rdia)

55
4-Rx of the cyanotic spells
  • Try to calm the patient .
  • Knee chest position,
  • O2
  • Propranolol IV.
  • Morphine s.c
  • NaHCO3 iv
  • Increase IV fluid.

56
5-Prophylaxis against infective endocarditis
  • 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.

57
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?ident
ifier3047051
58
  • Patients at the greatest danger of bad outcomes
    from IE and for whom preventive antibiotics are
    worth the risks include those with
  • artificial heart valves
  • a history of having had IE
  • 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
  • a cardiac transplant which develops a problem in
    a heart valve

http//www.americanheart.org/presenter.jhtml?ident
ifier3047051
59
Dont forget prostglandine PGE2, AND
indomethacine for selective cases of CHD
60
Preventive measures
  • 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.

61
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.

62
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.

63
  • Thanks
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