Dr. Nagendra Prasad provides an overview of acyanotic congenital heart diseases. He discusses that they present with signs of congestive heart failure and/or heart murmurs during infancy or early childhood. The most common presentations are feeding difficulties, tachypnea, sweating, subcostal recession, and recurrent respiratory infections. Echocardiography, ECG, chest x-ray and cardiac catheterization are used in evaluation. The most frequent acyanotic heart defects are ventricular septal defect, atrial septal defect, patent ductus arteriosus, coarctation of aorta, and pulmonary valve stenosis.

1. Dr.k. Nagendra
prasad
APPROACH TO ACYANOTIC
CONGENITAL HEART DISEASES

2.  Congenital cardiovascular defects, also known as congenital heart
defects, are structural problems that arise from abnormal formation
of the heart or major blood vessels. Defects range in severity from
tiny pinholes between chambers that may resolve spontaneously to
major malformations that can require multiple surgical procedures
before school age and may result in death in utero, in infancy, or in
childhood.
AMERICAN HEART ASSOCIATION
INTRODUCTION

3. Congenital heart disease is a problem
with the heart's structure and function
that is present at birth.
DEFINITION

4.  Approximately 5 to 8 per 1000 live births .
 About 2 or 3 in 1000 infants will be symptomatic
during the first year of life with significant heart
diseases that will require treatment.
 CHD is the major cause of death in the first year of life.
 There are more than 35 well recognized cardiac defects
the most common heart anomaly is VSD.
INCIDENCE

5. MULTIFACTORIAL
GENETIC - 90%
ENVIRONMENTAL - 10%
PREDISPOSING FACTORS
- Drugs / abortificient
! Infection
! Radiation
! Systemic disease (maternal)
Etiology

6. The systematic approach to CHD
includes:-
 1.Detailed antenatal and postnatal history.
 2.Systematic physical examination: Which includes general
examination, followed by detailed examination of the
cardiovascular system by inspection, palpation, percussion
and auscultation. The examination of the respiratory system
and abdomen should be done in a methodical manner.
 3. Electrocardiography (ECG)
 4. Chest X-ray
 5. Echocardiography
 6.Computed tomography (CT) angiogram and cardiac
magnetic resonance imaging (MRI) in selected cases
 7.Invasive procedures like cardiac catheterization and
angiocardiography, if needed.

7. Approach to CHD
 Is the Heart Disease Congenital?
CONGENITAL
1. Recognition early in age
2. Murmurs more
parasternally
3. Presence of central
cyanosis
4. Presence of extra-cardiac
anomalies
ACQUIRED
1. H/o. joint pains,
fever
2. Apical murmurs

8. Does the child have Heart Disease?
NADAS Criteria
 MAJOR
1. Systolic murmur Gr.
III or > intensity
2. Diastolic murmur
3. Cyanosis
4. CHF
 MINOR
1. Systolic murmur Gr.
II intensity
2. Abnormal 2nd Sound
3. Abnormal ECG
4. Abnormal Chest X
Ray
5. Abnormal Blood
Pressure

9. ACYANOTIC vs CYANOTIC
 Normal saturations are >94%.
 Between 93 and 85 human eye cannot detect
desaturation.
 Below 85% we can visually diagnose cyanosis.

10.  Central cyanosis is characterized by blueness of the
tongue and oral mucosa.
 Central cyanosis most likely is related to cardiac or
respiratory disease.
 If the source of cyanosis is cardiac, it is constant.
 Children in shock also may appear cyanotic owing
to venous stasis, right-to-left intrapulmonary
shunting, and increased peripheral oxygen
extraction.

11. Classification
CHD
Acyanotic Cyanotic
Left-to-right
shunts
Outflow
obstruction
- Ventricular Septal
Defect (VSD)
-Patent Ductus
Arteriosus (PDA)
-Atrial Septal Defect
(ASD
- Pulmonary
Stenosis
- Aortic Stenosis
-Coarctation of
aorta
• Teralogy of fallot
• Tricuspid atresia
• Transposition of
great vessels
• Truncus arteriosus
• Total anomalous
pulmonary venous
return (TAPVR)
• Ebstein’s anomaly

12. Lesions % of all Lesions
- Ventricular septal defect 25-30
- Atrial septal defect (Secundum) 10
- Patent ductus arteriosus 10
- Coarctation of aorta 7
- Tetralogy of Fallot 6
- Pulmonary Valve Stenosis 5-7
- Aortic Valve Stenosis 4-7
- Transposition of great arteries 4
- 0thers 20

14.  Acyanotic congenital heart diseases - present
with signs of congestive heart failure and/or heart
murmurs that are heard during physical
examination and can manifest any time during
infancy or early childhood.
 Most of these patients present during the first 6
months of life.

15. PRESENTING COMPLAINTS
 1.Feeding difficulties
 2. Tachypnea
 3. Sweating
 4. Subcostal recession
 5. Recurrent respiratory infections
 6. Growth impairment in the infant
 7. Exercise intolerance
 8. Easy fatigability or murmur in the older child
 The magnitude of the shunt or the severity of the
obstruction determines the clinical presentation and
symptoms.

16. FEEDING DIFFICULTIES
 It is a common symptom in significant acyanotic
CHDs.
 In infants, feeding itself is a form of exercise or effort.
 An infant with heart failure has the inability to
complete feeds within 15 to 20 minutes, sucks less
volume of milk (< 3.5 ounces per feeding), gets tired
easily and takes frequent feeds (less than 2 hours).
 As the feeds are inadequate they become irritable
and cry excessively. This suck-rest-suck cycle
continues round the clock.
 As pulmonary vascular resistance declines, irritability
and fussiness with feeding may indicate angina and
ischemia in a child with an anomalous left coronary
artery from the pulmonary artery.

17. RESPIRATORY DISTRESS
 It is the most prominent sign of heart failure caused by
significant lefttoright shunting in infancy.
 Symptoms of respiratory distress include tachypnea or
rapid breathing (respiratory rate > 60/min in newborn and
> 50/min in infants) and intercostal and subcostal chest
retractions.
 In very sick infants, grunting and nasal flaring can also
be present.
 Unlabored tachypnea often accompanies cyanotic heart
disease, whereas grunting and dyspnea are associated
with left-sided obstructive lesions or respiratory illness.
 Leftsided heart failure produces tachypnea with or
without dyspnea.

18. Easy Fatigability
 In an infant, it is seen as poor ability to suck and
feed.
 In older children, heart failure may be manifested
as exercise intolerance; difficulty in keeping up
with peers during sports or need for a nap after
coming home from school and poor growth.

19. Repeated Lower Respiratory
infections
 Recurrent pneumonia - two or more episodes of
RTI in one year or more than three in any time
frame requiring hospitalization or IV antibiotics.
 Frequent upper respiratory tract infection are not
related to CHD.

20. Growth Retardation or Failure to
Thrive
3rd
 Failure to thrive is defined as weight <
percentile for age.
 Infants should gain about 20 g/day.
 A normal infant can grow while receiving 100
calories/kg/day; infants whose growth is impaired
by heart disease typically require 130 to 140
calories/kg/day
 Acyanotic patients - weight is more affected due
to high catabolic rate & poor feeding due to
dyspnea ,fatigue.
 Cyanotic patients – disturbances in both height
and weight.

21. CHEST PAIN
 Chest pain or angina is rare, but not unknown in infants and
children.
 Pain associated with palpitations, dizziness and panic attacks
- mitral valve prolapse (Barlow syndrome).
 LVOT obstruction – stenosis of the aortic valve, subaortic
valve area, supra-aortic valve area or coarctation of the aorta,
may present with chest pain and associated with dizziness and
fatigue - due to demand-supply mismatch resulting in
myocardial ischemia.
 Chest pain in association with exercise intolerance and fatigue
- hypertrophic or dilated cardiomyopathy.
 Chest pain due to myocardial ischemia - abnormal coronary
artery anatomy, including congenital anomalies of the
coronary artery, coronary artery fistulas and stenosis or
atresia of the coronary artery ostium and have been
recognized in infants with an aberrant left coronary artery.

22. Syncope
 History of syncope on mild to moderate exertion
indicates severe
cardiomyopathy
aortic
(HOCM),
stenosis,
severe
hypertrophic
pulmonary
hypertension or complete transposition of the
great vessels (CTGV).
 In CTGV , syncope is due to significant bradycardia.
 long QT syndrome – A uncommon cause of
syncope.

23. Peripheral Edema
 Pretibial and presacral edema are late developments
in the child with congestive circulatory failure,
apparently due to the difference in tissue turgor.
 When peripheral edema due to heart failure does
develop in an infant, it first appears periorbitally, and
is usually preceded by other manifestations
such as tachypnea, tachycardia, dyspnea and liver
enlargement.

24.  Sweat on forehead – low cardiac output –
increased sympathetic activity- seen in CHF.

27. Family History
 If one parent has a congenital heart anomaly, the
risk of the child having one – 10 percent.
 When a first cousin has a congenital heart anomaly,
the risk of a sibling having one is approximately 2
percent.
 With no family history of CHD, if the firstborn has a
congenital heart lesion, the risk of a second child
having a congenital heart lesion is 2 to 3 percent.

28. Maternal History

29. BIRTH WEIGHT
 HIGH BIRTH WEIGHT – TGA
 LOW BIRTH WEIGHT – Intra uterine
infections,drugs( alcohol)

30. CYANOSIS
 onset –at birth / several days after birth
 Severity of cyanosis
 Permanent /paroxysmal nature
 Parts of body that is cyanotic
 Whether cyanosis becomes worse after feeding
 Cyanotic spells – time of appearance , duration of
spell, frequency of spell
 Should be differentiate from breath holding spell
 History of squatting.

31. HEART MURMUR
 Heard within few hours after birth – stenotic lesion /
AV valve regurgitation, small left to right shunt
lesions as their flow characteristics is independent of
PVR changes.
 Murmurs of large left to right shunts are delyed due
to slow regression of pulmonary vascular resistence.
 Murmur that is first noticed in a routine examination
of a healthy boy – innocent

32. How to assess PBF
 SYMPTOMS –
 Increased respiratory rate, retractions ,
 Increased respiratory infections,
 Sweating while feeding
 Failure to thrive
 Harrison sulcus
 Infants with lesions associated with shunting at
the ventricular or great vessel level are generally
more symptomatic than those with only atrial-level
shunting..

33. GENERAL APPEARANCE
 A wide spectrum of
extracardiac
malformations occur
in 15-45% of cases
with CHD.
 Extracardiac
malformations can
give a clue towards
certain CHD.

36. PULSE
 Character of the pulse –
1. Bounding pulses – PDA , BAV with severe AR , large systemic
AV Fistulas , persistent truncus arteriosus.
2. Pulsus parvus et tardus- severe AS.
3. Weak ,thready pulse – cardiac failure /circulatory shock
,myocarditis, cardiomyopathy, legs in patients with CO A, arm
pulses on the side of classic BT shunt / subclavian flap
angiplasty.
 Difference between upper and lower limb pulses – CO A/
interrupted aortic arch.
 Discrepancy in both radial pulses - supravalvular aortic
stenosis, aortic isthmus stenosis or pre-ductal CoA with the left
subclavian arising below the coarctation & the aortoarteritis
with obstruction to left subclavian artery.

37. BLOOD PRESSURE
 Upper extremity hypertension – first sign of CoA.
 A narrow pulse pressure - low cardiac output or
severe aortic stenosis.
 Pulse pressure widens in conditions with an
elevated cardiac output (anemia and anxiety) or with
abnormal runoff of blood from the aorta during
diastole (PDA or aortic insufficiency).

39. Jugular Venous Pressure
 The JVPis difficult to interpret in newborns and
infants (due to short neck and tachycardia).
 ASD- V ≥ A – large shunt
A> V – small shunt , associated PHT , LV
failure
increased mean pressure – LV dysfunction.
 VSD- JVP elevated with incresed A and V in patients
with moderately restrictive ,non restrictive VSD eith
CHF.
EISENMENGER – normal/ small dominant A
wave.
 PDA – same
 AS – increase of Awave amplitude in the absence of
PHT.

40.  PS – prominent A wave and a presystolic liver pulse ,
With the advent of right ventricular failure and
tricuspid regurgitation, the ʻvʼ wave increases, the Y
descent becomes brisk and the liver manifests
presystolic and systolic pulsations.

41. INSPECTION
 Precordial bulge – suggests chronic cardiac enlargement
 Pectus excavatum – pumonary systolic murmur and
cardiomegaly in PAview
 Harrisons groove – large left to right shunts leading to HF
> 2 months .
 left precordial bulge - left atrial enlargement in post-
tricuspid shunts.
 The upper sternum may bulge in children with a large left-
to-right shunt and pulmonary hypertension or with
elevated pulmonary venous pressure. Seen after 3
months of age.
 In older patients with right ventricular(RV)
hypertrophy secondary to pulmonary hypertension there

42.  Hyper active precordium – heart diseases with
volume overload – large left to right shunts , AV valve
regurgitation.
 Left parasternal pulsations and lift can be seen in
large atrial septal defects and in cases of RV
hypertrophy.
 In patients with PDA, aortopulmonary window, aortic
insufficiency, aortic stenosis and CoA,
suprasternal pulsations can be visible.

43. GENERAL APPEARANCE -
SCAR
 Right thoracotomy - right Blalock Thomas Taussig
shunt, atrial septal defect repair, or mitral valve
surgery.
 Midline sternotomy - most heart operations,
especially if cardiopulmonary bypass is used.
 A left thoracotomy - patent ductus ligation,
coarctation repair, and placement of a left Blalock
Thomas Taussig shunt.

44. Apex Beat
 In infants and children below 4 years, the apex beat is
located in the fourth intercostal space (ICS).
 Between 4 and 7 years it can be either in the fourth or
fifth ICS and thereafter in the fifth ICS.
 The displacement of the apex beat laterally or inferiorly
indicates cardiac enlargement.
 A hyperdynamic apical impulse is seen in volume
overload conditions like post-tricuspid shunts and
 A sustained heaving apical impulse is seen in pressure
overload conditions like in LVOTO.

45. Thrill
 Thrills at the lower sternal border are more likely
to be associated with VSDs than mitral or
tricuspid regurgitation.
 Thrills at the right upper sternal border (RUSB) or
suprasternal notch are most likely to be due to
severe aortic stenosis.
 Other less common cause of thrill in the suprasternal
notch is pulmonary stenosis.
 Diastolic thrills are less common

46. Auscultation
 loud S1 can occur with increased flow across the
AV valves from large lefttoright shunts, such as
ASD,VSD,PDA.
 Normal splitting: - small VSD, mild aortic or
pulmonic stenosis
 Wide splitting-
 Volume overload (e.g., ASD, PAPVR)
 Pressure overload (e.g., PS)
 Electrical delay (e.g., RBBB)
 Early aortic closure (e.g., MR)
 Occasional normal child.

47.  Paradoxical splitting or reversed splitting – PDA,
severe aortic stenosis, complete left bundle branch
block, WPW syndrome Type 2.
 Single S2
 Pulmonary hypertension
 One semilunar valve (e.g., pulmonary atresia, aortic
atresia, persistent truncus arteriosus)
 P2 not audible (e.g., TGA, TOF, severe PS)
 Severe AS
 Occasional normal child
 Narrowly Split S2
 Pulmonary hypertension
 AS

48. Third Heart Sound
 The S3 - low-frequency sound in early diastole and
is related to rapid filling of the ventricle .
 It is best heard at the apex or lower left sternal
border. It is commonly heard in normal children and
young adults.
 A loud S3 is abnormal and is audible in conditions
with dilated ventricles and decreased ventricular
compliance (e.g., large-shunt VSD or CHF).
 When tachycardia is present, it forms a
“Kentucky” gallop.

49. Fourth Heart Sound or Atrial
Sound
 The S4 is a relatively low-frequency sound of late
diastole (i.e., presystole) and is rare in infants and
children.
 When present, it is always pathologic and is seen
in conditions with decreased ventricular
compliance or CHF.
 With tachycardia, it forms a “Tennessee” gallop.

50. SYSTOLIC and DIASTOLIC
SOUNDS
 An ejection click (or ejection sound) follows the S1
very closely and occurs at the time of the ventricular
ejection's onset. It is usually audible at the base
(either side of the upper sternal border), whereas the
split S1 is usually audible at the lower left sternal
border.
1. Stenosis of semilunar valves (e.g., PS or
AS).
2. Dilated great arteries, which are seen in
systemic or pulmonary hypertension, idiopathic
dilatation of the PA, TOF (in which the aorta is
dilated), and persistent truncus arteriosus.
 Midsystolic click with or without a late systolic murmur
is heard at the apex in mitral valve prolapse(MVP)

51. SYSTOLIC MURMURS
 Mid-systolic murmurs- crescendo-decrescendo or
diamond-shaped murmurs-
 Bicuspid aortic valve - murmur is brief with an aortic
ejection sound. The murmur is best heard over the right
second interspace with little or no radiation.
 valvular aortic stenosis- the maximum intensity is
appreciated over the right second interspace; murmur is
harsh and rough and a thrill may be present. The murmur
radiates up into the neck and over both carotid arteries. In
patients with aortic stenosis, the longer and later peaking
murmur is usually associated with hemodynamically
significant obstruction; The intensity of the murmur is
variable and may not correlate with the severity of
stenosis.

52.  In supravalvular aortic stenosis, the murmur may
be loudest at a slightly higher location than in
valvular aortic stenosis.
 In subvalvular left ventricular outflow obstruction
(hypertrophic cardiomyopathy), the maximum
intensity of the murmur is usually located along the
lower left sternal border or over the cardiac apex. It
radiates poorly to the base and neck.

53. FIXED LVOTO DYNAMIC LVOTO
PULSE PARVUS AT TARDUS Sharp upstroke with
normal volume
with STANDING -
MURMUR
DECREASE INCREASE
STRAIN phase of
VALSAVA
MURMUR intensity INCREASE DECREASE
CAROTID UNCHANGED or
decrease
DECREASE

54.  The murmur of valvular pulmonary stenosis is harsh
and best heard over the left second interspace and
radiates to the left side of the neck and is frequently
accompanied by a palpable thrill.
 A pulmonary ejection sound at the onset of the
murmur may be heard and S2 is widely split with a
decreased intensity of P2.
 The murmur duration correlates reasonably well
with the severity.

56. Holosystolic (pansystolic)
murmurs
 This type of murmur is typically heard in AV valve
regurgitation.
 Mitral and tricuspid regurgitation murmurs are
high pitched, with variable intensity and blowing
quality.

58. Diastolic Murmurs
abnormal and may be
aortic and pulmonary
 Diastolic murmurs are usually
early, mid or late diastolic.
 Early diastolic murmurs -
regurgitation.
 Mid-late diastolic murmurs occur due to stenosis or
increased flow across the mitral or the tricuspid valves.
These murmurs can occur in mitral stenosis, tricuspid
stenosis, or as flow murmurs of ASD, VSD and PDA.
 Late diastolic (presystolic) murmurs - pathological
narrowing of the AV valves.
 The pulmonary regurgitation murmur in patients with
normal pulmonary artery pressure, is low-pitched and
early diastolic because of the low-pressure gradient.
 In patients with pulmonary hypertension, the murmur,
known as the Graham-Steell murmur is heard, which is
also early diastolic but is high pitched and decrescendo,
because of the high pressure gradient between the
pulmonary artery and the right ventricle in diastole.

60.  The continuous murmur from aortopulmonary
communications is loudest around the S2.
 The arterial continuous murmur is characterized by
a more pronounced systolic component while a
venous continuous murmur is characterized by a
more pronounced diastolic component.

61.  Abdominal Examination-
 Normally liver is palpable (2–3 cm below costal margin)
at midclavicular line up to 4 to 5 years of the age, after
that it remains palpable up to 1 cm till late childhood. If
liver is palpable further downwards and the infant is
irritable it probably indicates presence of congestive
heart failure.
 If the liver is in midline and palpable symmetrically, it
indicates cardiac malpositions and underlying complex
heart disease.
 The site of the gastric fundus is detected by percussion,
which determines visceral situs.
 Spleen is not normally palpable; if palpable it indicates
possibility of infective endocarditis.
 Respiratory System Examination
 Unlike adults, where crepitations is commonly basal, in
neonates and infants it is more diffusely heard and often

62. CXR
 Feature of increased PBF –
1. Visibility of pulmonary vascularity in lateral 1/3rd
2. Vascularity extending below diaphragm.
3. End on views – 3 on oneside / 5 on both sides
4. Trachea /right MPA >1.5 in children <12 yrs
5. Hilar haze in lateral views.
6.Ratio between The end on vessel diameter to that of
accompanying Bronchus Normally it is 1.2:1 and in plethora it
is ≥1.5:1.
7.The size of the RDPA in nomally < 14 mm and if it increased
(> 16 mm in male and >14 mm in female), it indicates a shunt
lesion.
 In normal children diameter of RDPA is less than that of
trachea. If the ratio of RDPA to trachea is more than 1 it
indicates significant left to right shunt.

63.  Plethora occurs in left to right shunts, admixture
lesions and transposition of the great arteries (d-
TGA) without PS.
 Increased pulmonary blood flow in an acyanotic
child - ASD, VSD, PDA, ECD, partial anomalous
pulmonary venous return (PAPVR).

64.  Prominent Main Pulmonary Artery Segment-
 1. Poststenotic dilatation (e.g., pulmonary valve
stenosis)
 2. Increased blood flow through the PA(e.g., atrial
septal defect [ASD], ventricular septal defect
[VSD])
 3. Increased pressure in the PA(e.g., pulmonary
hypertension)
 4. Occasional normal finding in adolescents,
especially girls
 Dilatation of the Aorta- patent ductus arteriosus
(PDA), coarctation of the aorta (COA), Marfan

65.  PULMONARY VENOUS CONGESTION
 Pulmonary venous congestion is characterized by
a hazy and indistinct margin of the pulmonary
vasculature.
 This is caused by pulmonary venous
hypertension secondary to left ventricular failure
or obstruction to pulmonary venous drainage
(e.g., mitral stenosis, TAPVR, cor triatriatum).
 NORMAL PULMONARY VASCULATURE
 Pulmonary vascularity is normal in patients with
obstructive lesions such as pulmonary stenosis or
aortic stenosis.
 Patients with small left-to-right shunt lesions also
show normal pulmonary vascular markings.

66.  Associated anomalies -hypoplasia of the right
lung and right pulmonary artery, sequestration of
right lung tissue receiving arterial supply from the
aorta, and other congenital heart diseases such
as VSD, PDA, COA, or TOF.

67. ASD - CXR
 1. increased PBF
 2. dilated MPA, both PA
 3. right atrail enlargement
 LAE – rare , seen in older adults with AF , associated
MS
 RVH – acute angle at apex
 LVH – if MR is present in OP- ASD.
 scimitar syndrome- A vertical vascular shadow along
the lower right cardiac border - PAPVR from the lower
lobe ,pulmonary veins drain in to IVC.
 SV –ASD – localized dilatation of SVC at junction with
RA.

68. SCIMITAR SIGN
• The scimitar sign is produced by
an anomalous pulmonary vein
that drains any or all of the lobes
of the right lung.

69. VSD
 SMALL VSD - N , mild dilatation of pulmonary
trunk
 MOD VSD - cardiomegaly , enlarged LA –
SPLAYING OF CARINA., increased PBF features
 LARGE VSD – cradiomegaly , globular silhoutte-
LA,LV,RV ,occ RA dilatation
 With increasing PVR , left to right shunt
decresese , cardiac size decrease , but
enlargement of pulmonary trunk persists.
 VSD with AR – LVH is disproportionate to shunt
with dilated aortic root.
 VSD Is a part of many congenital defects. So
Xray feature is suggestive of the original defect
that VSD.

71. PATENT DUCTUS
ARTERIOSUS
 Enlargement of the left heart
chambers.
 Enlargement of the ascending
aorta or aortic arch.
 Pulmonary plethora with enlarged
central and peripheral artery.
 Filling up of the angle between
the aortic arch and the pulmonary
artery : Most specific sign.
• Possible PDA calcification in
adults.

72. CO A
 In a heavily exposed film, the
precoarctation and
postcoarctation dilatation of
the aorta may be seen as a
“figure of 3.”
 This may be confirmed by a
barium esophagogram with
E-shaped indentation.
 Rib notching is a specific
finding of COA in an older
child (usually older than 5
years) and is usually found
between the fourth and
eighth ribs.

73. obtained with barium
esophagography
 Unilateral rib notching :
◦ left rib:anamolous origin of right subclavian artery
distal to coarctation
◦ Right rib:if it involves origin of left subclavian artery

74. DDS FOR RIB NOTCHING
 Chronic superior venacava obstruction
 Chronic inferior venacava obstruction
 Aortoarteritis
 Intercostal AV malformations
 Post BT shunt

75. PRE TRICUSPID
SHUNT
POST TRICUSPID
SHUNT
CARDIOMEGALY MORE( RA, RV ) LESS (LA, LV)
With development of
PAH
PERSIST REGRESS
PULMONARY
VASCULARITY
MORE - PERIPHERAL
PRUNNING +
MPA DILATATION MORE LESS
AORTA DILATATION ABSENT PDA & VSD with AR

76. ECG

77. ASD

78. Severity of shunt
Size of Shunt Mild Moderate Severe
Qp/Qs <1.4 1.4 – 2 >2
RAE No Yes Yes
RV volume
overload
No Yes Yes
N rsr’ rsR’/evidence of
PAH
Crochetage No No Yes
Evidence of PAH No No Yes
R’ > 7mm in V1

79. CROCHETAGE
 In inferior leads, a notch near the apex of the R
wave,coined “crochetage,” has been correlated with ASD
 Seen in 73.1%, specificity - 92% (all 3 inf
leads),86.1%(atleast 1 inf lead)

80. ASSOCIATED CONDITIONS
 Lutembacher’s syndrome – LAE,RVH more
common,Atrial arrhythmias more common

81.  TAPVR-resembles OS ASD,PR prolonged, AF in
older persons
 PFO – no hemodynamic changes
ECG is normal or shows crochetage
 Common Atrium –
Absent atrial septum
ECG shows features of combination of
OS, OP, Sinus venosus ASD

82. 2. VSD
 Small shunt –Normal / mild LVH
 Moderate shunt - LVH, LAH (±)
 Large shunt - BVH, LAH
 Katzwatchal phenomenon- R+S in mid precordial leads >60 /
>50 in single lead
 Inlet VSD – LAD , counter clock wise loop
 PVOD (Eisenmenger's syndrome) – RVH, RAE, R in v1, RAD
>120.
SIZE OF THE SHUNT –
1.restrictive – N / Rsr in v1
2. mod restrictive – volume overloaded LV with BVH ,LAE
3. large non restrictive – BVH ,LAE , PAH
 Number of VSDs- multiple VSD – left axis deviation with
clockwise loop.
 GERBODE defect – RAE with LV volume overload.
 VSD with AR – deep Q ,tall R , deep inverted T ,coved ST
segment in lateral precordial leads.

84. SITE OF VSD
Axis Loop
Inlet LAD Counterclockwise
Multiple muscular LAD Clockwise
L TGA with VSD LAD Clockwise
DORV with VSD LAD Counter clockwise

85. SUMMARY
VSD
LEFTAXIS
Clockwise loop-
L-TGA
MULTIPLE
MUSCULAR
Counter clockwise-
DORV
INLET VSD
TRICUSPIDATRESIA
RAD
Severe PAH

86. VSD
LVH
MODERATELY
RESTRICTIVE
WITH RAE-
GERBODES
BVH
NONRESTRICTIVE

87. BVH
Q IN
LATERAL
LEADS
PRESENT-
simple VSD
ABSENT-
LTGA

88. AV SEPTAL DEFECTS
 Marked left to right
shunt
 Early development of
PAH
 Associated MR
determines outcomes

89. AV SEPTAL DEFECTS
 Characteristic finding :
 - left axis deviation
 - QRS vector counter clockwise loop
Mech : due to posterior and inferior displacement of
the AV node and the left bundle
 S waves in leads II, III, and aVF have a
characteristically
notched upstroke
 QRS pattern in right leads resembles OS-ASD-Due
to longer than normal Rt bundle

90. PDA
 Diagnosis by -
 LV Volume overload
 LAE
 +/- RV pressure overload
 QRS axis – normal

91. SHUNT SEVERITY
 Small –normal
 Moderate – LAE + LV Vol overload
q & Tall R in V5-6,deep S in V1-2,
T remain upright
 Large – Biatrial enlargement + BV hypertrophy
 Eisenmenger – RAE + RVH

92. COMPLICATIONS
 PAH
 BUT Q IN V5-V6 PERSIST EVENAFTER
DEVELOPMENT OF PAH
 PR interval prolongation -10 – 20%
 AF – in older persons with large shunt

93. ASSOCIATED CONDITIONS
 RAD – Neonates ,
LAD- Rubella, sometimes superior axis
 CoA – LVH with secondary ST- T changes

94. Pulmonary stenosis
 Obstruction to RVOT increases afterload on RV – RV
systolic overload
 RVH With or without RAE
 Good correlation with hemodynamics and ECG

95.  Normal ht of R in V1 =<8 mm in infancy,
<10 mm in children
 Persistent upright T waves in V1 & V2 after 4
days age indicates RVH

96. SEVERITY OF PS
MILD MODERATE SEVERE
P Wave Sharp Sharp Sharp
pointed/peaked P pointed/peaked P pointed/peaked P
waves <2.5 mm in II waves >2.5 mm in II waves >5 mm in II
& V1 & V1 & V1
QRS Axis 40 to 60(N) 100 to 120 120 to 150
QRS voltage Tall R in V1/V2,but Tall R in V1/V2,but Tall R /qR in
R/S <1 R/S >4:1,<20mm or V1/V2,but R/S
<10 – 15mm rsR’/Notched R’ >>4,with ht >20mm
Shift of transition Shift of transition
zone to left zone to left
QR in V1
T wave Inverted in V1/V2 Inverted upto V4-V5, Deep symmetric
only In 50% cases inversions,upto V6
Upright T in V1 –V2 upright in right leads T inversions
after 4 days may be II,III,AVF
only sign

97. RVSP MEASUREMENTS
• Ht of R in V1/V4R x 5,between 2 – 20 yrs age, if
pure R wave is present.
• Ht of R in V1 + 27
• Ht of R in V1 x 3 + 47
• Pressure gradient across pulmonary valve=
10.5(ISM)+2.6 (S1)+S2 Score+T score
ISM=intensity of systolic murmur(gr 1-6)
S1=S in lead I in mm
S2 score= -10 if P2 is normal,+2 if P2 is audible but diminished,+15 if
P2 is inaudible
T score= +15 if T in V1 is diphasic with R in V1 >10mm,otherwiseT
score = 0

99. TYPES OF PS
• Valvular PS – As described
• Sub/Supra valvular –same
• Sub infindibular PS/DCRV –Degree of RVH in V1-3
less than anticipated,
Upright T in V3R may be the only finding in 40%
cases
• Dysplastic Pulmonary valvular stenosis as a part of
Noonan syndrome –Extreme axis deviation, QRS
prolonged and splintered, QS in inferior and left
precordial leads

100. Pure PS
 RAD,But not marked
 Tall R in Rt precordial
leads
 Deeply inverted
symmetric T waves
TOF(VSD + PS)
 Marked RAD
 Tall R only in V1,with sudden
transition
 Less deeply inverted T
waves/upright T in V1 & V2
 EXCEPTION-
 Pentology –LA or LV
enlargement
 Triology –marked increase in
RVSP ~PS + LAE
 Pulmonary atresia –marked
increase in RVSP

101. 4. AS
 Mild to moderate- Normal or LVH
 Severe - LVH with or without “strain”
5. COA
 Infants younger than 6 mo- RBBB or RVH
 Older children- LVH, normal, or RBBB
6. Anomalous origin of the left coronary artery
from the PA - Myocardial infarction, anterolateral

102. COR TRIATRIATUM
 RAE, RVH,RAD are common
 LAE may be seen due to prolonged conduction in
proximal accessory chamber

103. REGURGITANT LESIONS
 CONGENITAL PR
 Normal in mild-mod cases
 RV volume overload in severe cases
 AF unlikely
 CONGENITALAR
 LV volume overload with LV q waves

104. APPROACH
Acyanotic congenital heart disease>
Step -1-see which chamber is enlarged
Step -2-suppose it is RV
Step-3-is it volume overload(rsr’/rsR’)or
pressure overload(monophasic R/qR)
Step-4-
• Volume overload-ASD
• Pressure overload-PS, Infantile
coarctation,cor triatriatum & congenital MS-

105. Step-2-suppose it is LV
Step-3-is it LVH alone/BVH
Step-4-LVH alone-
Step-5-volume/pressure
Step-6-
• LV Volume overload-Moderately restrictive VSD,
PDA
• LV Pressure overload-coarctation of
aorta/congenital AS/interrupted aortic
arch/critical PS of infancy

106. Step-4-BVH-nonrestrictive VSD,
large PDA, AP window ,L-TGA
Step -5- q in lateral leads/v1
Step -6- q in v1,absent in lateral
leads-L TGA
q in lateral leads-simple VSD,PDA

107. Introduction- ASD
 Incidence is 1 child per 1,500 live births
 More frequent in females than males by about 2:1
Samanek M et al. Pediatr Cardiol1999;20:411–7
 Most ASDs occur sporadically; however, a few families
have the defect as a genetic abnormality.
 Mutation in-TBX5, NKX2.5 , GATA4, Myosin heavy chain 6

108.  Small ASD –ASYMPTOMATIC
 MODERATE ASD- symptoms starts at 2nd decade
 Symptoms after 40 yrs –
1.Decrese in LV distensibility -> increased left to right
shunt
2.Age related increase in atrial arhhythmias –
precipitates CHF
3. Development of PAH .
 By 4th decade 50% will became symptomatic, 5th
decade – 75% will become symptomatic.
 Depending on ASD size and PBF – 7% will develop
Eisenmengerization in 1st decade and 8% in 2nd
decade.
 Infective endocarditis does not occur in patients
with isolated ASDs.

109.  SURGICAL CLOSURE -
1. Primum septal defects, sinus venosus and coronary sinus
defects and defects with associated anomalous pulmonary
venous drainage.
2. Secundum atrial defects that are larger than 38 mm in
diameter or defects that have insufficient rims (< 5 mm) are
also not suitable for transcatheter device closure.
 DEVICE CLOSURE-
 Devices available for clinical use have included the Sideris
buttoned device, the Angel Wings device, the CardioSEAL
device, and the Amplatzer ASD Occlusion Device.
 There must be enough rim (4 mm) of septal tissue around
the defect for appropriate placement of the device.
 The patients are administered aspirin 81 mg/day for 6
months.

110. VSD
 The incidence of spontaneous closure in
perimembranous and muscular VSDs is high, while
it is low in outlet defects and inlet defects do not
close.
 60 percent close before 3 years and 90 percent by 8
years of age.
 Surgery for Ventricular Septal Defect-

111.  RECOMMENDATIONS FOR DEVICE CLOSURE
OF MUSCULAR VSDS-
 Class IIa - Class IIA is reasonable for infants who
weigh ≥ 5 kg, children and adolescents with
hemodynamically significant muscular ventricular
septal defect (MVSD) to undergo percutaneous
VSD device closure (level of evidence: B).

112.  Exclusion criteria - Weight less than 3.0 kg
(unless the hybrid perventricular approach is
used); distance of less than 4 mm between the
VSD and the aortic, pulmonic, mitral or tricuspid
valves.
 Except the subpulmonic and inlet VSDs, most of
the other VSDs can be closed without surgery.

113. PDA
INDICATIONS FOR PDA CLOSURE –
Premature Infants
 PDA closure is indicated in all patients less than 10 days
of age with symptomatic PDA, who had no response to
indomethacin and for those who are more than 10 days
old.
 Symptomatic PDA is defined as respiratory rate of more
than 70 per minute, heart rate more than 160 per minute,
liver enlargement more than 3 cm below costal margin
and cardiomegaly more than 60 percent on chest X-ray.
Children and Adults
 Symptomatic and even asymptomatic PDA should be
closed to avoid CHF, pulmonary vascular disease, IE
and aneurysm formation.

114.  Gianturco coils- The coil diameter should be at least two
times the minimum PDA diameter and yet fit within the ampulla
of the ductus.
 The immediate success rate using single or multiple coils
ranges from 67 to 95 percent, almost invariably related to
ductus size.
 Although ducts as large as 7 mm have been closed with coils,
a larger ductus diameter is an unfavorable factor.
 Sideris Buttoned Device-
 Gianturco-Grifka Vascular Occlusion Device- ideal for long
tubular PDA
 Duct-occlud and Nit-occlud Device- for <2 mm and < 6 mm
PDA s
 Amplatzer duct occluder (ADO)- a device is chosen so that
the diameter of the pulmonary arterial end of the device is 2 to
3 mm larger than the narrowest diameter of the ductus
(usually the pulmonary end of the ductus)

115.  Indications for Surgery –
1.Failure of catheter based closure or anatomy of
ductus not suitable (Type C).
2.Preterm infants with contraindications or failure
of pharmacotherapy.
3.Calcific PDA, aneurysmal ductus, hypertensive
ductus, inflamed ductus.
4. Recurrent ductus, giant ductus.
5. PDA with complex CHD.

116. Intervention
 For infants and older children
1. Left ventricular dysfunction: Immediate intervention by
balloon dilatation, irrespective of gradients (Class I).
2. Normal left ventricular function: Balloon dilatation if any of
these present:
 i. Gradient greater than 80 mm Hg peak and 50 mm Hg
mean by echo-Doppler (Class I).
 ii. ST-T changes in ECG with peak gradient of greater than
50 mm Hg (Class I).
 iii. Symptoms due to AS with peak gradient of greater than
50 mm Hg (Class IIa). In case of doubt about
severity/symptoms, an exercise test may be done for older
children (Class IIb).
 For neonates: Balloon dilatation if symptomatic or if
there is evidence of left ventricular dysfunction/mild left
ventricular hypoplasia (Class I), or if Doppler gradient
(peak) greater than 75 mm Hg (Class IIa).

117.  The balloon size used should not exceed the aortic
annular size.
 A successful valvotomy is defined as fall in gradients
of more that 50 percent of predilatation values.
 surgical Intervention-
 In children valve replacement is always difficult as
adequate size of prosthesis may not be available.
 Ross procedure remains the choice for small infants
but there might be gross aortic and pulmonary
annulus mismatch and hence it may not be very
successful.
 Aortic valve repair is still not a very preferred mode of
treatment due to the poor outcome in long run.

118. PS
NATURAL HISTORY
 The severity of stenosis is usually not progressive in mild PS.
 over 95% of patients who had an initial Doppler gradient less
than 25 mm Hg were free of cardiac operation over a 25-year
period. The severity tends to progress with age in moderateor
severe PS.
 Indications for the Procedure- Currently pulmonary balloon
valvuloplasty is the first line of treatment for pulmonary valve
stenosis at any age.
1. Any symptomatic patients , asymptomatic patients with severe
obstruction &
2. Patients with moderate obstruction should undergo elective
valvuloplasty, if the right ventricular pressure is ≥ 50 percent of
systemic or higher,
3. Patient with echocardiographic peak instantaneous gradient of >
40 mm Hg
4. Clinically significant pulmonary valvar obstruction in the
presence of RV dysfunction

119.  A balloon is chosen that is 20 to 40 percent larger
than the angiographically measured pulmonary
valve annulus and it is positioned over a
guidewire with the valve at its midpoint
 Surgical Technique - Indications
1.Dysplastic pulmonary valve with valve ring
hypoplasia.
2.Fixed infundibular and supravalvar stenosis with
pulmonary valvar stenosis.

121. Natural history- CO A
 Bimodal presentation.
 produces significant symptoms in early infancy and after
age 20 to 30 years.
 Neonates with severe coarctation become acutely
symptomatic when the ductus closes.
 Most who survive the hazards of infancy reach adulthood
 25% die by age 20 years,
 50% die by age 30 years
 75% die by age 50 years.
 Survival has been reported at age 74 years and 76 years.
natural history of

122. CO A
 Indications for treatment-
1. In infants with hemodynamic compromise .
2. In older patients a consistent systolic blood pressure gradient between
the arms and legs of more than 20 mm Hg
3. Systemic hypertension at rest or following provocation with exercise
greater than the 97th percentile for age along with evidence of
important luminal stenosis.
Surgical treatments-
1. End-to-End Anastomosis Including Extended Repair
2. Subclavian Flap Repair
3. Interposition Grafts, Bypass Tube, Extra-anatomic Bypass Grafts
4. Patch Aortoplasty
Interventional treatments-
 1. Balloon Angioplasty-sick hemodynamically compromised infantas a
bridge to definitive surgical repair , children weighing less than 25 kg
when faced with significant residual or early recurrence of gradient
 2. Stent Placement- Covered stents – preferred where possible.

123. RSOV
 M:F- 4:1
 More in asians
 Presentation
 Acute tearing chest pain/interactable sob
 Small shunt –asymptomatic – 20%
 Small shunt – incidious onset sob – 45%
 Large shunt – acute hf – 35%
natural history of

124. RSOV
natural history of acynoticchd
 Involvement of
 RCC – 67%
 NCC - 25%
 LCC – 8%
 Rupture in to
 RV - 70-75%
 RA – 15-20%
 LA – 5-10%
 LV - <5%
ASSOCIATED WITH-
 Vsd – 30-40% - Sub
pulmonic
 AR – 39%
 Prolapse of cusps
due to vsd
 BAV
 Rvot obs -10 %
 BAV - 15-20%

126.  Device closure should not be attempted in -
1.Patients with an aneurysm which has ruptured into the
pulmonary artery and left ventricle,
2.Presence of associated lesions such as as VSD and
aortic regurgitation,
3. Aneurysmal opening within 5mm of coronary ostia,
4.Right-to-left shunting across the defect with systemic
saturation less than 94 percent,
5.Patients with pulmonary vascular resistance greater
than 7 Wood units and
6.Significant right ventricle/left ventricle dysfunction
with left ventricular ejection fraction less than 30
percent

135. THANK
YOU