Approach to Pediatric cardiovascular diseases: Congenital heart diseases ASD VSD PDA Coarctation of the Aorta PS AS TOF TGA Tricuspid atresia Truncus arteriosus Total Pulmonary venous return Hypoplastic left Heart Syndrome Acute rheumatic fever (ARF) Chronic Rheumatic Disease Infective endocarditis (IE)

2. CVS SYSTEM
Dr. Raheel Ahmed Shaikh
MBBS,FCPS
Children Hospital Chandka Medical College, Larkana

4. Approach to CHD

5. History
 Maternal history of medication, drugs,
alcohol abuse, excessive smoking.
 Prenatal history: infection
 Family history of CHD, hereditary,
chromosomal

6. Symptoms
Difficult Feeding
Sweating during
feeding
Poor growth
Poor weight gain
Difficult
Breathing
Chest indrawing
Fast breathing
Frequent
respiratory
infections
Syncope
Exercise intolerence
Easy fatigability
Seizure
Focal neurological
lesion

7. Chest Pain Stridor
Palpitations Syndromic
appearance
Cynosis
Bluish spells

8. Physical Examination
• Appearance : Pale, Dusky, Polycythemic, Syndromic
• Presence of Cyanosis, Clubbing,Edema
• Tachypnea, Respiratory distress
• Weight, Height for physical development
• Skeletal abnormalities: Polydactyly, others
• Pulse : Tachycardia, Arrhythmia, Volume, Palpability
• BP : All 4 limb BP in complex CHD
• JVP : - Elevated in Tricuspid Atresia, Eisenmenger
physiology
- Normal in TOF
• Abd: - Sidedness of liver/spleen + palpation of Apical
Impulse
to rule out Dextrocardia
- Hepatomegaly

9. Blood pressure
 Methods sphingnonaometer (different cuffs)
-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.

10. What is cyanosis?
Cyanosis is a bluish discoloration of skin
and mucus membrane that results when
the absolute level of reduced hemoglobin
in the capillary bed exceeds 3 g/dL.

11. 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.

12. Physical examination
 Inspection
-Prominence of the precordium (cardiomegaly,Rt.heart enlargement )
-Jugular veins engorgement “older children”
-Any associated defects or findings (down syndrome, Digorge syndrome…etc)
Palpation
-Pulses (rate, rythem,volume,peripherial pulses ,radio-femoral delay)
-Cardiac impulses.
-Thrill.
-Hyper dynamic precordium/heave.
-Hepato-splenomegaly

13.  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

15. innocent or functional
murmurs
 most common
 heard in up to 30% of patients
◦ Asymptomatic
◦ Soft blowing
◦ Systolic
◦ Left sternal edge
 Also
◦ Normal heart sound
◦ No thrill
◦ No radiation
 Heard during
◦ Febrile illness or anemia
◦ Inc cardiac output

16. If we suspect C.H.D
Investigation
 CBC---- polycythemia, anemia….etc
 CXR----heart size and shape
 ECG---HR,axis ,rythm
LVH,RVH,BVH,BBB.
 Echocardiography
 MRI
 Cardiac catheterization

17. Prevalence
 Congenital 8/1000
 The incidence is higher in
◦ stillborns (3-4%),
◦ spontaneous abortuses
(10-25%),
◦ premature infants
 Acyanotic: 68%
 Cyanotic: 22%
Congenital Heart Disease

18. 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.
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.
Majority of cases of the congenital heart diseases are unknown

21. Acyanotic Cyanotic
Outflow &
Arterial
obstructions
Congenital Heart Disease

22. Common Acynotic CHD
Left to right lesions
1. Ventricular septal
defects
2. Atrial septal defects
3. Atrio-ventricular
septal defects
4. Patent ductus
arteriosus
5. Aortopulmonary
window defect
6. Coronary artery
fistula
Obstructed lesion
1. Pulmonary stenosis
2. Aortic stenosis
3. Coarctation of aorta
4. Congenital Mitral
stenosis
5. Pulmonary venous
HTN
Regurgitate lesions
1. Congenital Mitral
incompetence
2. Mitral valve prolapse
3. Tricuspid
regurgitation
4. Pulmonary valve
insufficiency

23. Ventricular Septal Defect
 Subtype
◦ Small (<0.5cm2)
◦ Moderate (0.5-1 cm2)
◦ Large (>1cm2)
◦ Perimembraneous
◦ Muscular
◦ Supracristal (superior to
crista supraventricularis)
80%

24. Ventricular Septal Defect
 Small VSD (<0.5cm2)
◦ Asymptomatic
◦ A loud, harsh, or blowing
holosystolic murmur at LSE
 Large VSD(>1cm2)
◦ dyspnea, feeding
difficulties, poor growth,
profuse perspiration,
recurrent pulmonary
infections, and cardiac
failure in early infancy.
◦ Apical thrust, systolic thrill
at LSE
◦ Pansystolic murmur(less

25. Ventricular Septal Defect
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.
Small VSDs, the chest radiograph is usually normal

26. Ventricular Septal defects:
management
 30–50% of small defects close spontaneously, most
frequently during the 1st 2 yr of life. Vast majority will
close up to 4yaers.
 Small muscular VSDs are more likely to close (up to
80%) than membranous VSDs are (up to 35%).
 Medical management: treat heart failure if present.
 Surgical repair prior to development of an irreversible
increase in pulmonary vascular resistance (usually prior
to the patient's second birthday), chronic volume
overload and heart failure.

27. Indications of surgery
 Failure to controle CCF
 Failure to thrive
 Supracristal VSD
 Associated Pulmonary stenosis
 Development of AR
 6-12 month child with rising P.HTN
 >2years child PBF twice of Systemic
BF

28. Atrial Septal Defects
 Subtypes
◦ Sinus venosus (high)
◦ Ostium secundum (mid
portion) most common
◦ Ostium primum (low )

29. Atrial Septal Defects: secundum
 Most common form of ASD
 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.
 Soft systolic murmur at
upper LSE
Secundum

30. Atrial Septal Defects:primum
 Situated in the lower portion of the
atrial septum
 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
 Apical pansystolic murmur(AVr)
 The 2nd heart sound is split and
fixed

31. Atrial Septal Defect
X-ray
 Enlargement of the
right ventricle
 Enlargement of
atrium
 Large pulmonary
artery
 increased pulmonary
vascularity
ECG :supeior axis in
primum, RVH, partial
RBBB

32. Atrial Septal Defects
 Secundum ASDs are well tolerated during
childhood.
 Antibiotic prophylaxis for isolated secundum ASDs
is not recommended.(except if MR present)
 Surgery or transcatheter device closure is advised
for all symptomatic patients and also for
asymptomatic patients with a shunt ratio of at least
2:1. or those with RVH
 Intervention : after 1 year before school entery.
 Ostium primum defects are approached surgically

33. Prognosis
 Small to moderate-sized ASDs detected in
term infants may grow smaller or close
spontaneously
 Pulmonary hypertension, atrial dysrhythmias,
tricuspid or mitral insufficiency, and heart
failure are late manifestations
 The results after surgical or device closure in
children with moderate-size to large shunts
are excellent

34. Patent Ductus Arteriosus
 Connects pulmonary
artery and descending
aorta.
 Normally closed shortly
after birth.
 Common in VLBW with
pulmonary diseases and
in congenital rubella.
 F:M 2:1
 Spontaneously close in
premature
 PDA persisting in term
beyond 1st few weeks will
rarely close
spontaneously

35. Patent Ductus Arteriosus
 Small defect:
◦ no symptoms.
◦ Pulses are normal
 Large defect:
◦ Breathlessness while
feeding
◦ Slow growth
◦ Repeated Lower RTI
◦ Wide pulse pressure
◦ Enlarged heart
◦ Apical heaving
◦ Thrill in L second IS
◦ Continuous murmur
(machinary)in 2nd LICS
◦ X-ray: prominent pulmonary
artery with increased
vascular markings, Left heart

36. Patent Ductus Arteriosus
 Medical Management
◦ Medical closure in preterm tried with
indomethacin (0.2mg/kg/dose iv for 3 doses 8-
12hr apart) or brufen (ibuprofen).
◦ Start therapy after echo only.
 Surgical Management
◦ Ligation and division of ductus is treatment
of choice
◦ In asymptomic patiants before 1year
◦ P.HTN is not contraindication.

37. Coarctation of the Aorta
 In boys more than in girls
 Almost always juxtaductal in
position.
 Weak femoral pulses, Radio
femoral delay, hypotention in
lower parts of body.
 High BP in upper body part in
the arm 20mmhg more than
leg, headache, vertigo,
epistaxis.
 Murmur at left interscapular
area in back.
 Treatment
Digoxin & diuretic PGE1

38. Pulmonary Stenosis
 Narrowing in pulmonary valve
 RT side heart failure
Ejection Systolic murmur at Left 2nd
ICS radiate to back, S2 widely split
 ECG …Echo RT side hypertrophy
 Treatment
Balloon valvuloplasty
Surgical repair

39. Aortic stenosis
 Increased pressure in the LF side of the
heart (LV hypertrophy)
 Easy fatigability, exertional chest pain,
syncope
 Ejection systolic murmur at right 2nd ICS
radiate to neck, high HR.
 Treatment..
- Beta-blocker or ca channel blocker to
decreased hypertrophy
- Balloon valvoplasty
- Surgical repair

40. Cyanotic Congenital Heart Disease
- Cardinal Clinical features -
 Cynosis
 Clubbing
 Polycythemia

41. Common Cynotic Lesions
Decreased Pulmonary blood flow
1. Tetralogy of Fallot
2. Tricuspid Atresia
3. Severe Pulmonic Stenosis
4. Pulmonary atresia
5. Ebstein’s anamoly
6. Double Outlet Right ventricle
Increased Pulmonary blood Flow
1. Transposition of great vessles
2. VSD with pulmonary atresia.
3. Truncus Arteriosus
4. Hypoplastic left heart
5. Single ventricle
6. TAPVR

42. DECREASED PBF
(eg. TOF)
INCREASED PBF
(eg. TGA, TAPVC)
Presentation at Any age Neonate / Infant
Appearance Comfortable Sick, Lethargic, Irritable
Cyanosis Mild - Severe Mild
(except TGA with intact IVS)
Squatting /Cyanotic spells Common Uncommon
Feeding difficulty / Sweating Absent Present
Failure to thrive Absent Present
Weight Gain Normal Suboptimal
Recurrent LRI No Yes
Tachypnea Absent Present
CHF, Tachycardia, S3, S4 Absent Present
Thrill Maybe Present Absent
CLINICAL DIFFERENCES BETWEEN CYNOTIC HD WITH  AND  PBF

43. ONSET OF
CYNOSIS
 Pul. Atresia
 Tricuspid atresia
 TGA
1st week
 TOF
 TGA
 TAPVC
After 1 month
period
 TGA
 Tricuspid atresia
 TAPVC
 Truncus Arteriosus
 TOF (severe type)
 Pulmonary Atresia
with Hypoplastic RV
 Hypoplastic RV

44. Cynosis : Differential
Normally related GV +
Severe PAH + Rt.  Lt. Shunt thro PDA
FINGERS : RED
TOES : BLUE
Normally connected GV
Severe PHT
Rt to Lt shunt thro PDA
FINGERS : BLUE
TOES : RED
D-Transposition of GV
Severe PHT
Rt to Lt shunt thro PDA
Cynosis : Reversed Differential
D-TGA +
Severe PAH + Rt.  Lt. Shunt thro
PDA

45. Cyanotic
Tetralogy of Fallot
 Most common cynotic
◦ Ventricular septal defect
◦ Pulmonic stenosis
◦ Overriding aorta
◦ Right ventricular
hypertrophy

46. Risk factors
Environmental factors
 maternal diabetes [threefold increased
risk],
 retinoic acids,
 maternal phenylketonuria (PKU), and
 trimethadione
Genetic cause : heterogeneous

47. Clinical Presentation
 Clinical presentation is directly related to the
degree of pulmonary stenosis.
 Severe stenosis results in immediate cyanosis
following birth.
 Mild stenosis will not present until later.
◦ Growth is retarded – insufficient oxygen and nutrients
◦ SOB on exertion → rest
◦ Digital clubbing
◦ Paroxysmal hypercynotic attacks (tet spells)
◦ Left parasternal heave
◦ Systolic thrill (50%) at left PSE 3 and 4 ICS
◦ S2 is often single
◦ Harsh ejection systolic murmur at left PSE 3rd ICS

48. “Tet Spell”
 “Tet spells” at 2-3yo, child
becomes cyanotic,
restless, gasping
respiration,may experience
syncope
 More frequently in morning
upon awakening or after
vigorous cry.
 Children assume squatting
position
 During spell, dec in
intensity or disappearance
of systolic murmur

49. Exams and Tests
 CBC
-  hematocrit
 ECG
-RVH, RAD
 CXR
-boot shaped
heart, right sided
aortic arch
 Echocardiogram
-VSD, PS, RVH

50. Tetralogy of Fallot
Apex is lifted, concavity in pumonary segment, oligemic lung field.
Boot shaped Heart

51. Treatment
 Severe TOF with
worsening cynosis in
early neonatal period
require prostaglandins E
infusion; and surgery
(modified Blalock-
taussing shunt)
 Corrective surgery
carried out from 3
months to 2year
depending upon
expertise availablity
Blalock-
Taussig shunt

52. POTTS SHUNT WATERSTON
SHUNT

53. Treatment of the cyanotic spells
 Try to calm the patient .
 Knee chest position,
 O2
 Propranolol(0.1-0.2mg/kg slow IV).
 Morphine s.c
 NaHCO3 iv
 Increase IV fluid.
 Prevented by oral Propranolol (1mg/kg
every 4hr)

55. Transposition Of great
arteries
 Most serious cynotic lesion,
 Seen in newborn period (5%)
 More common in infants of diabetic
mothers and in males.
 Survive when ASD, PDA, or VSD

56.  TGA with intact ventricular septum
◦ Cynosis and tachypnea within 1st hours of life.
◦ Single S2, no murmur
◦ PGE1 (o.o5-o.2ug/kg/min infusion)is immediately
started to maintain patency of DA.
◦ CCF is less common
 TGA with VSD
◦ Mild cynosis recognised 1st month of life.
◦ Murmur is pansystolic
◦ Many Neonates are large 4kg at birth then
growth retardation occurs.
◦ They need anti-CCF measures
Clinical Features

57. Slight cardiomegaly, narrow base
Egg shaped Heart
Transposition Of great
arteries

58. Management
 Corrective surgery by
age of 2 weeks
 Procedures:
◦ Rashkind or ballon atrial
septoplasty
◦ Mastured procedure
◦ Total repair: Arterial swich
technique.

59. Tricuspid Atresia

60. Clinical Features
 Progressive Cynosis
 Poor feeding
 Tachypnea over the first 2 weeks
 Holosystolic murmur due to VSD
 Single S2
 Left axis Deviation and left VH on ECG
characteristics.
 Normal heart size

64. Clinical Findings

65. Treatment
 Medical management:
◦ Anti-CCF measure
 Surgical Repair:
◦ VSD closure and
placement of conduit
between right ventricle
and Pulmonary artries.

66. Total Pulmonary venous
return
2%
Pulmonary vein return to the right
atrium or the superior vena cava
instead of the left atrium
Atrial level communication is
required.
Without obstruction: Hyperactive
RV impulse with wide split S2
Systolic ejection murmur at Left
USB
Mid diastolic murmur at left LSB
With obstruction: signs of right
sided heart failure, no murmur, no
change in S2
* Treatment
Give PGE, cath, and surgical
treatment

67. Hypoplastic left Heart
Syndrome
Most common cause of death
from cardiac side in 1st month
Failure of development of
MV,Av,or arch.
Infants may appear healthy at
birth, but signs of HLHS soon
become apparent after the
ductus arteriosus closes. :
Cyanosis minimal, weak pulses,
Cold extremities, greyish
colour(low cardiac output)
S2 single and loud no heart
murmur
Right ventricle Hypertrophy
* Treatment

68. Treatment of C.H.D
 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.

69. 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 ).
 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.

70. 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”

71. Acute rheumatic fever (ARF)
 in response to infection with group A
B-haemolytic streptococcus
 aged 5–15yrs
 incidence is highest in those from
socially and economically
disadvantaged areas

72. Clinical features
 • There is a latent
period of 2–6wks
between onset of
symptoms and
previous streptococcal
infection (e.g.
pharyngitis).
 Symptoms are non-
specific.
 The grouping together
of clinical features
makes the diagnosis
more likely (Jones
criteria)

75. Management
 In the acute phase treatment will include: • bed rest;
• anti-infl ammatory drugs (e.g. aspirin);
• corticosteroids (2–3wks);
• diuretics/ACE inhibitors if in heart failure;
• antibiotics (e.g. penicillin V for 10 days).
 Sedatives may be helpful early in the course of chorea;
◦ phenobarbital (16-32 mg every 6-8 hr PO) drug of
choice.
◦ If ineffective, then haloperidol (0.01-0.03 mg/kg/24hr divided
bid PO) or
◦ chlorpromazine (0.5 mg/kg every 4-6 hr PO) should be
initiated
 Long-term therapy
◦ prevention of further attacks of acute rheumatic
◦ development of chronic rheumatic heart disease.

77. Chronic Rheumatic Disease

78. Infective endocarditis
 Children at risk are those
◦ with turbulent blood flow through the heart or
◦ where prosthetic material has been inserted
following surgery: e.g.
• PDA or VSD;
• coarctation of aorta;
• previous rheumatic fever
 special risk groups have emerged,
including
intravenous drug users;
patients taking immunosuppressant medications;

79. Most common pathogens
• Streptococcus viridans
(50% cases): often after
dental procedures.
• Staphylococcus aureus:
often related to central
venous catheters.
• Group D streptococcus
(enterococcus): often
after lower GI surgery

85. Treatment
 Antibiotic therapy:.
Empirical therapy: vancomycin plus gentamicin
◦ in patients without a prosthetic valve
◦ when there is a high risk of S. aureus, enterococcus, or
viridans streptococci
◦ 4-6 wk of treatment is usually recommended.
 Fubgal infection: amphotericin B and 5-fluorocytosine
 signs of heart failure
◦ diuretics,
◦ afterload reducing agents,
◦ digitalis, in some cases
 Bed rest is recommended and heart failure should be
treated.
 Surgery

88. Surgery indications
◦ severe aortic,
◦ mitral or prosthetic valve involvement
with intractable heart failure
◦ failure to sterilize the blood despite
adequate antibiotic levels in 7-10 days in
the absence of extracardiac infection,
◦ myocardial abscess,
◦ recurrent emboli,
◦ Increasing size of vegetations while
receiving therapy

90. Prognosis
 mortality may be as high as 20– 25%
 complications (50–60%) include
◦ heart failure.
◦ Myocardial abscesses and toxic myocarditis
◦ arrhythmias
◦ Systemic emboli from left-sided vegetations
(>10-15 mm) may result in
 brain abscess
 stroke.
 Fungal endocarditis is difficult to manage
and has a poorer prognosis