congenital heart disease, PDA, TOF, VSD, ASD , Management, PAPVD, Ebstains anomaly,

1. Congenital Heart Disease
ffor Post graduates
Dr. Md.Toufiqur Rahman
MBBS, FCPS, MD, FACC, FESC, FRCPE, FSCAI,
FAPSC, FAPSIC, FAHA, FCCP, FRCPG
Associate Professor of Cardiology
National Institute of Cardiovascular Diseases(NICVD),
Sher-e-Bangla Nagar, Dhaka-1207
Consultant, Medinova, Malibagh branch
Honorary Consultant, Apollo Hospitals, Dhaka and
STS Life Care Centre, Dhanmondi
drtoufiq19711@yahoo.com
CRT 2014
Washingt
on DC, USA

2.  USA - 5-8 Per 10,000 live births
 Also a major cause of Abortion and Still birth
 About 1500-2000 (stipulated) children born with
congenital Heart disease every year in Bangladesh.
Incidence

3. Distribution of CHD
CYANOTIC
21%
OTHERS
8%
ACYANOTIC
71%
ACYANOTIC
CYANOTIC
OTHERS

4. Relative Frequency of CHD
0
5
10
15
20
25
30
35V
SD
PD
A
PS
A
SD
C
O
A
O
A
S
A
V
-C
A
N
A
L
ACYANOTIC

5. Relative Frequency of CHD
0
1
2
3
4
5
6
TOF SV/TA TAPVD
CYANOTIC

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

7. EMBRYOLOGY
ORIGIN
Mesodermal cells Ectodermal/ Neural crest cells
Inter atrial Septum Endocordial cushion
Interventricular septum Dysmorphogenesis
Spiral septum
Abnormal Apoptosis

8. CLASSIFICATION
ACYANOTIC
(a) 3 holes (L-R) shunt
Ventricular Septal Defect(VSD)
Atrial septal Defect(ASD)
Persistent Ductus Arteriosus(PDA)
(b) 3 tube Defect (Increase Pressure load)
Aortic Stenosis(AS)
Pulmonary Stenosis(PS)
Coarctation of Aorta(CoAo)
( c) Others

9. CYANOTIC
DECREASED PBF INCREASED PBF
TOF TGA
Tricuspid Atresia TAPVD
Pulmonary atresia TRUNCUS ARTERIOSUS(TA)
DORV SINGLE VENTRICLE(SV)
Ebstein’s Anomaly(E.A) HYPOPLASTIC LEFT HEART SYNDROME
OTHERS :-
Abnormal position of heart and heterotaxy syndrome

10. DIAGNOSIS OF CHD
S MAJOR COMPONENTS
 History & Physical Examination
 X-ray chest
 E.C.G
 Echo cardiography
 Cardiac cathetarization

11. CLUES TO CHD
 Feeding difficulties
 Respiratory distress/Tachypnea
 Cyanosis/clubbing/Cynotic spell
 Apneic spell
 Persistent tachycardia
 Excessive sweating
 Frequent RTI
 Exercise intolerance/orthopnea
 Syncope/seizure/Headache/ vomiting
 Failure to thrive/Growth Retardation
 Developmental Delay
CHD usually identified during routine clinical exm. Or with a emergency
e.g. CCF or with complains of exertional dyspnea, or with FTT.

12. PHYSICAL EXAMINATION
Appearance
Anaemia, Cyanosis, clabbing, oedema
Pulse/Heart rate - in quiet state, RR Apex beat, heart sound,
Murmur - innocent/organic(Thrill), Timing - systolic/diastolic,
location- where more intense ?
Hepatomegaly, bilateral basal creps
But always be sure before leveling a case of CHD

13. X-Ray Chest
Lungs - Normal Acyanotic - VSD,PDA,ASD.AS & CoAo(if CCF)
Plethoric - Cyanotic - TGA, TAPVD,TA,HLHS,
Oligaemic Acyanotic - PS
Cyanotic - TOF. Tricuspid Atresia, Pul. Atresia
DORV WITH PS. EA.
Heart - Normal/
Cardiomegaly - if CCF (VSD,PDA,AS CoAo, TGA, TA.
Characteristic cardiac shadow
* Boot shape - TOF
* EGG on side - TGA
* Box shape - E.A.
* Square shape - Tricuspid Atresia
* Snowman - TAPVD (Supracardiac)
Bony skeleton - Rib notching - CoAo.

14. ECG
* QRS axis - Lead - I Lead - II
Normal Positive Positive
LAD Positive Negative
RAD Negative -----------
* Ventricular Hypertrophy
LVH S in V1 + R in V5/V6 > 40 mm ( > 1 yr)
RVH R>S in V1 > 30 mm (< 1 yr)
BVH R + S in V3 / V4 > 50 mm any age

15. VSD
An opening in the interventricular septum.
Type - Perimembranous ©, Out let, inlet, Muscular.
L-R Shunt - size, small/restrictive, Large / Non restrictive
Magnitude on QP : QS.
Symptomatic - at 4 - 6 wks

17. Clinical Presentation
* Small (<0.5 cm) - asymptomatic,develops normally.
* Moderate to Large VSD with Large L-R shunt-
Symotomatic in early infancy, Dyspnea at rest or
during feeding, sweating, C.C.F. failure to thrive frequent
RTI.
* Large VSD with PHT-With above features, cyanosis
usually absent but intermittent with crying / exertion
(if R-L shunt)

18. On Examination
Normal pulse, S1 masked by murmur, S2 Accentuated if PH,
murmur - Pansystolic in LLSE, Apical mid-diastolic (if large
shunt), ESM in ULSE
On Investigation
X-ray - normal/Cardiomegly, Pul. Plethra
ECG - normal/LVH or RVH & LVH
ECHO - diagnostic, anatomical defect size location pressure
gradient & assoc. problems.
CATH - Diagnostic, determine PVR, QP : QS.

19. ASD
ASD

20. An opening in inter atrial septum.
Type - O.Secundum (c), O.Primum, S.Venosus.
C/F - Secundum type- Usually asymptomatic. With large L-R
shunt- recurent RTI, Easy fatigue, PHT in older.
Primum type- asymptomatic but with large L-R shunt-
Dyspnea, RTI & C.C.F may occur.
O/E - Normal pulse, S2 wide and fixed splitting. ESM in
ULSE, Apical mid-diastolic if large shunt.
On investigation- X-Ray chest normal/Cardiomegaly, Pul.Plethora.
ECG-rsR’in V1.ECHO-Diagnostic. Cath-QP:QS,
PVR.
ASD

22. PDA
* Persistent communication by ductus between PA &
AO. Neonate - pre-term/term. Infant & children.
* Small-no symptom, large -Growth retardation, easy fatigue
exertional dyspnea, heart failure.
* Collapsing pulse, S1 S2 masked by continuos machinery
murmur in ULSE to left clavicle.
* X-Ray chest - Normal/cardiomegaly. Pul. Plethora.
* ECG - Normal or LVH.
* ECHO - Diagnostic.
* CATH - Diagnostic & Therapeutic.
* Complication - Infective endarteritis, PAHT.

23. * Type - Valvar, Infravalvar, Supravalvar.
* C/F - Mild to Modarate - No symtom. Severe stenosis-
Exertional Dyspnea & C.C.F. Critical stenosis - Neonatal
cyanosis with C.C.F.
* O/E - Normal pulse, Click if pliable valve, ESM in ULSE.
* Chest X-Ray - Normal/cardiomegaly.
* ECG - RVH with strain pattern.
* ECHO - Diagnostic, determine peak systolic gradient across
pulmonary valve & indicate the requirement of intervention.
* CATH - Diagnostic and therapeutic for valvuloplasty.
PUlMONARY STENOSISPUlMONARY STENOSIS

24. AORTIC STENOSIS

25. * Type - Valvar, Supravalvar, Infravalvar, IHSS.
* C/F - Mild from-asyptomatic. Moderate- dizziness, easy
fatigue, sweating, exertional dyspnea, syncope,
angina. Severe- C.C.F. sudden death, FTT.
* O/E - Pulse reduced in volume, click if pliable valve, ESM in
URSE, O/I
* X-Ray Chest - Normal/Cardiomegali.
* ECG - Normal / LVH with strain pattern.
* ECHO - Diagnostic. Thickend/Doomed AV. Peak systolic
gradient classify the degree of stenosis.
AORTIC STENOSIS
Narrowing at LVOT

26. COARCTATION OF AORTA
Discrete narrowing of Aorta from Arch to Iliac bifurcation
Type - Infantile & Adult. Preductal/Postductal.
C/F - Severe from - Headache, dyspnea on exertion, hypertension, C.C.F.
differential cyanosis & differential blood presser. Less severe from -
well during infancy but hypertension in later life. Pulse -Decreased or
delayed femorals. Click if bicuspid aortic valve. Mid-systolic murmur in
Apex and back.
O/I
X-Ray chest - Normal/ cardiomegaly. Figure of “3” sign. Notching of 4-8 ribs.
ECG - Normal or LVH.
ECHO - Diagnostic. Pressure gradient classify the degree of
coarctation.
CATH - Diagnostic and Therapeutic or Balloon angioplasty.

27. TGA

28. D-TGA
AO arises from RV and PA arises from LV with or without shunt (ASD, VSD,
PDA)
C/F - Without shunt: Early Neonatal severe cyanosis, Hypoxemia, acidosis,
Heart failure, Sudden Death if not urgently treated. With shunt: Late
neonatal Presentation of cyanosis, acidosis, Failure to thrive & CCF
O/E
Heart sound - S2 Loud
Murmur - Absent or systolic (if shunt)
O/I
Chest radiology - “Egg on side” Heart Shadow with narrow base,
pulmonary plethora.
E.C.G - RAD RVH, RVH+LVH, may be normal
Echo - Aorta arises from RV & PA from LV also confirms other associate.
Cath - Diagnostic & Therapeutic for atrial septostomy

31. TOF
1.RVOT obstruction, 2. Large VSD, 3. Overriding of AO to RV,
4. RV hypertrophy. Degree of cyanosis depends on Severity
of RVOT obstruction and systemic versus pul. resistance.
C/F - Neonatal cyanosis (if severe)
Infancy - Hypercyanotic spell, clubbing, Failure to thrive,
convulsion, Hemiplegia Cerebral infarction
(<2yrs.) or cerebral Abscess (>2yrs.)
O/E - Heart Sound - S2 single , Murmur- Ejection
Systolic in left sternal edge.
Less the intensity of murmur more severe the

32. TOF
Chest radiology - Boot shaped heart with pulmonary
concavity & apex tilted up, Oligaemic lungs.
E.C.G - RAD, RVH
Echo - Aorta Large with overriding to RV, RVOT-
Narrow PA/PV- Variable in morphology,
RV
Hypertrophied, Large Nonrestrictive VSD
with
bi-directional shunt.

33. TAPVD
All four pul. veins open to a common sac which opens to
other than LA
Type - Supracardiac (C), Cardiac, infracardiac, Mixed.
C/F - With obstruction: (With/Without ASD) Early/neonatal
cyanosis, Acidosis, Hypoxemia, Heart failure (with
small
heart in radiology).
Without obstruction:Late cyanosis / Clubbing, RTI,
failure to thrive.

34. TAPVD
Heart sound - Normal , or S1-loud S2- single accentuated
Murmur - Absent or Ejection Systolic in pul. area
radiating to lungs.
Chest radiology - Wide supracardiac shadow as figure of ‘8’
(supra cardiac form), pul. plethora.
E.C.G - Normal or RVH, RAD, Tall-P
Echo - LA, LV smaller, Rt sided volume overload,
Identify anomalous pul. venous
connections.

36. Tricuspid Atresia
TV atretic, with ASD and VSD with variable RV and PA
C/F - Early central cyanosis & clubbing later on failure to
thrive, exertional dyspnea and C.C.F.
Heart sound - S1-Normal, S2-Single accentuated
Murmur - Soft systolic in LLSE

37. Tricuspid Atresia
Chest radiology - Small cardiac shadow Often square
shape, oligaemic lungs.
E.C.G - LAD , RAH, LVH
Echo - Thick atretic TV with ASD may be with
TGA/ without TGA, with variable size of
VSD or PDA.

39. TRUNCUS ATERIOUSUS.
Ao & PA arises from single great vessel overriding a VSD
without right venticular infundibulum
Type - 1,2,3,4 according to position of origin of PA
C/F - At birth-minimal cyanosis, at 2-3 wks-collapsing
pulse, breathlessness, RTI, failure to thrive & finally
C.C.F.
Heart Sound - S2 loud & single
Murmur - Click, Systolic murmur
Chest radiology - Cardiomegaly, pul. plethora.

40. TRUNCUS ATERIOUSUS.
E.C.G - Normal in early case, RVH or (RVH
+LVH) in older case.
Echo - Diagnostic. Characteristic overriding of a
single great artery and determine the
position and flow across PA from
truncus and bi-directional shunt across
VSD.
Cath - Diagnostic

42. EBSTEIN’S ANOMALY
Downward displacement of TV into the RV with a large RA
due to atrialized portion of RV which is reduced.
C/F - In Severe from - Neonatal cyanosis, C.C.F.
Less servere -Late presentation with palpitation,
dyspnea, chest deformity, failure to Thrive,
Arrhythmia like RBBB/AF.
Heart Sound - Gallop rhythm or irregular Heart beat. (if
CCF/AF)
Murmur - Systolic & Diastolic Murmur with/without
click.

43. EBSTEIN’S ANOMALY
Chest radiology - Cardiomegaly with Box-shaped heart
with Oligaemic Lungs.
E.C.G - Tall-P increased RP interval, RBBB or
AF
Echo - Downward displacement of TV with
atrialzed RV and also any associated
lesion.
Cath - Confirms Diagnosis.

44. PULMONARY ARTESIA
Absence of PV with no or very little communication between
RV and MPA usually with ASD, VSD or PDA.
Type I - With a small tricuspid and small RV
TypeII - With tricuspid vale insufficiency and a large
RV
C/F - Without shunt -Central cyanosis at birth.
Hypoxia acidosis & Right Heart failure.
With shunt - Variable cyanosis, Clubbing,
FTT, CCF

45. PULMONARY ARTESIA
Heart Sound - S2 Single
Murmur - Absent or may be systolic (If shunt)
Chest radioloy - Mild cardiomegaly, Oligaemic Lungs.
E.C.G - Normal or Tall-P (RAH)
Echo - PV not visualized or very thick with a little or
no blood flow. Small RV, variable TV

46. Congenital Heart Disease (CHD)
Look for
central cyanosis
Acyanotic Cyanotic
Auscultate Murmur
PSM in LLSE ESM
in ULSE
ESM
in URSE
Continuous
Machinery
in ULSE & LICLA
BSM in Left
Axilla & Radio
-femoral delay
CoAoPDA
As
RSCLA
PS/ASD
VSD
S2 Split & wide S2 Absent
ASD PS Contd...

47. Cyanotic
Look for Age
Early (<6M)
TGA, TAPVD (obs)
Sevre TOF, Tr. Ar.
Tricuspid Atresia (TA)
with small shunt
pulmonary atresia (PA)
(without shunt)
Late (>6M)
TOF
TAPVD (without obs)
TA (with large shunt
Ebstein’s Anomaly (EA)
S.V, A-V canal defect
Chest Radiography
Plethoric Lungs
TGA, TAPVD
Tr. Ar.
TOF, PA,
EA, TA
Oligaemic Lungs
Charactprostoc
Cardiac shadow
Egg shape Figure of 8 Square shape Boot shape Box shape Large RA
cardiomegaly
TGA TAPVD Tr, Ar TOF EA
PA/TA

48. Thank Youdrtoufiq19711@yahoo.com
Asia Pacific Congress of
Hypertension, 2014, February
Cebu city, Phillipines
Seminar on
Management of
Hypertension,
Gulshan, Dhaka