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1. “CYANOTIC &
ACYANOTIC HEART
DISEASE”
S. GRACELET MELITA
2ND YEAR M.SC(N)

2. EMBRYOLOGY:
 Development of the heart
 Development of the heart begins in the third week with the formation of two
endothelial strands called the angioblastic cords. These cords canalize forming
two heart tubes, which fuse into single heart tube by the end of the third week
due to lateral embryonic folding. By the fourth week, the developing heart
receives blood from three pairs of veins: the vitelline veins, umbilical veins, and
common cardinal veins. The vitelline veins carry poorly oxygenated blood from
the yolk sac, and enter the sinus venosus; the umbilical veins carry oxygenated
blood from the chorion, the primordial placenta; and the common cardinal veins
carry poorly oxygenated blood from the rest of the embryo.
2

3.  As the primordial liver develops in close association with the
septum transversum, the hepatic cords join and surround
epithelial-lined spaces, forming the primordial hepatic
sinusoids. These primordial sinusoids become connected to the
vitelline veins. Vitelline veins pass through the septum
transversum and enter sinus venosus, also called as venous end
of the heart. Left vitelline veins regress while right vitelline veins
form the hepatic veins, and a network of vitelline veins around
the duodenum form the portal vein.
3

4.  As the development of liver progresses, umbilical veins lose
connection with heart and empty into liver. The right umbilical
vein and cranial part of the left umbilical vein degenerate
during seventh week of gestation, leaving only the caudal part
of the left umbilical vein. The caudal part of the left umbilical
vein carries oxygenated blood to the embryo from the
placenta. The umbilical vein is connected to the inferior vena
cava (IVC) via the ductus venosus, a venous shunt that
develops in the liver. This bypass directs most of the blood
directly to the heart from placenta without passing through
liver.
4

5. INCIDENCE
It affects 8 – 12 of every
1000 neonates
Right sided lesions are
common in females and
left sided lesions are more
common in males
5

6. ETIOLOGY
 The heart begins as a single cell and develops into 4 chambered
pumping system during the 3rd to 8th week of gestation.
 the exact etiology of heart defects is unknown in 90% cases
 Factors associated with congenital heart defects include-
- fetal or maternal infections like rubella during 1st
trimester of pregnancy
- chromosomal abnormalities like trisomy 13, 18 and
21
- maternal insulin dependent diabetes
- teratogenic effects of drugs and alcohol
6

7. Syndromes that include congenital
heart defects are;
 MARFAN’S SYNDROME( mitral valve prolapse and dilated aortic root)
 TURNER’S SYNDROME( aortic valve stenosis and coarctation of aorta)
 NOONAN’S SYNDROME( dysplastic pulmonary valve)
 WILLIAM’S SYNDROME( supravalvular pulmonary stenosis)
 DI GEORGE SYNDROME( interrupted aortic arch. Truncus arteriosus,
transposition of great arteries and tetralogy of fallot)
 DOWN’S SYNDROME( atrioventricular defect and VSD)
7

8. FETAL CIRCULATION: 8

9. CONGENITAL HEART DISEASE:
 Congenital heart defects are one of the most
common congenital anomalies that may
involve chambers, valves and great vessels
arising from the heart. In most of the cases,
cause is unknown.
9

10. CLASSIFICATION 10

11. PATENT DUCTUS ARTERIOSIS:
 The ductus arteriosus is a normal pathway in the fetal circulatory
system, connecting the left pulmonary artery with descending aorta
 During the fetal life , blood flow is shunted away from lungs through
ductus arteriosus directly into systemic circulation
 Functional closure of ductus arteiosus usually occurs simultaneously
during 1st 10- 15 hours after birth
 Permanent closure occurs within 5-7 days in most newborns
 If it is not occur even after 2- 3 weeks o age it is known as PDA
11

12. INCIDENCE:
 Twice more common in females as compared
to males
 Common in infants who weigh less than 1500g
and accounts for 5-10% of all congenital heart
disease
12

13. ANATOMY AND PHYSIOLOGY OF HEART
13

14. CLINICAL FEATURES
Usually asymptomatic may have,
Growth retardation
fatigue
14

15. DIAGNOSTIC EVALUATION:
 CARDIAC EXAMINATION( Systolic or continuous
murmer, diastolic rumble, gallop, bounding pulse)
 ECG( left ventricular hypertrophy and left atrial
dilatation)
 CHECT RADIOGRAPH( increased pulmonary
vascularity with normal heart size)
 ECHOCARDIOGRAM
15

16. THERAPEUTIC MANAGEMENT:
 MEDICAL MANAGEMENT:
- Indomethacin, a prostaglandin
inhibitor( 0.2mg/kg)
 SURGICAL MANAGEMENT:
- at 6 months lateral thoracotomy
16

17. VENTRICULAR SEPTAL DEFECT:
It is an abnormal communication
between the right and left ventricle.
17

18. INCIDENCE:
 20% of all congenital heart disease
 TYPES:
 MEMBRANOUS VSD: they lie beneath the aortic valve
 SUBPULMONIC VSD: they lie beneath the pulmonary valve and
account for about for approximately 5- 7% of VSD
 ATRIOVENTRICULAR CANAL TYPE VSD OR POSTERIOR DEFECTS: 8%
 MUSCULAR VSD: they are frequently multiple and represent 5- 20%
of VSD
18

19. PATHOPHSIOLOGY
19

20. 20

21. CLINICAL FEATURES:
 Failure to thrive and congestive heart failure
 Dyspnea, tachypnea, slow physical
development development , feeding
difficulties and frequent pulmonary infections
21

22. DIAGNOSTIC EVALUATION
 CARDIAC EXAMINATION: ( systolic murmur,
increased regurgitation of blood across mitral valve
produces a diastolic low rumble)
 ECG( normal, may be with RV hypertrophy)
 CHEST RADIOGRAPH( large size VSD)
 ECHOCARDIOGRAM( the degree of left to right
shunting can be assessed)
22

23. THERAPEUTIC MANAGEMENT:
 75 – 85% usually close during the 1st 2 years of
life
 Small VSD- no surgery, only antibiotics to
prevent endocarditis
 Digoxin and diuretics
 Open heart surgery
 <7 kg- repaired in deep hypothermia causing
circulatory arrest
 Cardiopulmonary bypass
23

24. ATRIAL SEPTAL DEFECT
ASD are abnormal openings in the
wall separating the right and left atria
24

25. INCIDENCE AND TYPES
 17%
 Girls> boys
TYPES:
a) OCTIUM SECUNDUM:
Abnormal opening is present in the middle of the atrial
septum
b) OSTIUM PRIMUM:
the defect is located in the septum just above the tricuspid
valve and is associated with a cleft in the mitral valve and defects in the AV
septum
c) SINUS VENOSUS:
abnormal opening at the top of the atrial septum( SVC)
25

26. PATHOPHYSIOLOGY 26

27. 27

28. CLINICAL FEATURES:
Usually asymptomatic
Rarely with CHF, usually after 3rd or 4th decade of life
Decreased exercise tolerance and dyspnea
28

29. DIAGNOSTIC EVALUATION:
 CARDIAC EXAMINTAION( systolic ejection murmur in
the upper left sternal border)
 ECG( RV volume overload
 CHEST RADIOGRAPH( enlargement of heart and
increased pulmonary vascular markings)
 ECHO( define the location of ASD and dilatation of
the atria)
29

30. THERAPEUTIC MANAGEMENT:
 Small ASD closes automatically
 2- 4 years of age is recommended for surgery
 Median sternotomy and cardiopulmonary
bypass
 Purse string closure
 Knitted dacron patch is sewn over the defect
30

31. POST -OP COMPLICATIONS
Cardiac enlargement
Dysrhythmias
Infective endocarditis
31

32. COARCTATION OF AORTA
 Disorder with decreased pulmonary blood flow
 It is a discrete narrowing of aortic arch, usually
in the region of ductus arteriosus and left
subclavian artery)
32

33. INCIDENCE AND TYPES:
 7%
TYPES:
1) INFANTILE OR PREDUCTAL TYPE:
constriction of aorta between left
subclavian artery and ductus arteriosus
2) POST DUCTAL TYPE:
constriction at or distal to DA
33

34. PATHOPHYSIOLOGY: 34

35. CLINICAL FEATURES
 Increased blood pressure in the upper part of body ,
resulting in headache , dizziness, dizziness, fainting,
epistaxis and later CVA
 Low BP in the lower extremities, resulting in absent or
diminished femoral and pedal pulse
 Weakness or pain in their legs on exercise, their legs
may be cooler than arms
 Respiratory distress, poor weight gain, feeding
problems, irritability and tachycardia.
 Mottling in lower extremities
35

36. DIAGNOSTIC EVALUATION
 CARDIAC EXAMINATION( systolic murmur along the
left mid to upper sternal border that radiates to the
back)
 ECG( left or right ventricular hypertrophy)
 ECHO( the presence of coarctation and degree of
narrowing as well as the presence of other cardiac
defects ca be assessed)
 MRI AND CARDIAC CATHETERIZATION( to define the
area and extent of narrowing)
36

37. THERAPEUTIC MANAGEMENT
END TO END ANASTOMOSIS
SUBCLAVIAN FLAP AORTOPLATY
PATCH AORTOPLASTY
BALLON AORTOPLASTY
Antibiotic prophylaxis
 follow up for 1 – 2 years is
recommended
37

38. CYANOTIC HEART DEFECTS
 Bluish discoloration of skin, nail beds and mucous membrane caused due
to hypoxia
 The presence of cyanosis correlates with an arterial oxygen saturation of
75- 85%
 Peripheral cyanosis
 Central cyanosis
 Pulmonary origin
 Cardiac origin
38

39. TETRALOGY OF FALLOT
 It is the most complex congenital heart defect with Decreased pulmonary
blood flow.
 It is the combination of 4 defects
- VSD
- OVERRIDING OF AORTA
- PULMONARY STENOSIS
- RIGHT VENTRICULAR HYPERTROPHY
INCIDENCE:
6-10%
39

40. 40

41. CLINICAL FEATURES:
 CYANOSIS
 Skin – dusky or bluish in color
 Clubbing of finger and toe nails occur by 1 – 2 years of age
 Dyspnea
 Paroxysmal dyspneic attacks
 Poor nutritional status
41

42. DIAGNOSTIC EVALUATION:
 CARDIAC EXAMINATION
 ECG
 CHEST RADIOGRAPH
 ECHOCARDIOGRAPHY
 CARDIAC CATHETERIZATION
42

43. THERAPEUTIC MANAGEMENT
MEDICAL:
 KNEE CHECT POSITION
 PROPANOLOL
 IV prostaglandin
SURGICAL:
 Blalock Taussig shunt
 POTT’S PROCEDURE
 Waterston shunt
 Brock’s procedure
43

44. POST OP COMPLICATIONS
 Conduction abnormalities
 Residual VSD
 Residual PS
 Pulmonary valve regurgitation
44

45. TRICUSPID ATRESIA:
 In TA, the tricuspid valve fails to develop and no communication exits
between the right atrium and right ventricle
INCIDENCE:
2- 3 %
45

46. PATHOPHSIOLOGY 46

47. 47

48. CLINICAL FEATURES
 Profound cyanosis
 Hypoxic spells
 Tachypnea
 Delayed growth
 Acidosis
 Clubbing of nails
 CHF
48

49. DIAGNOSTIC EVALUATION
CARDIAC EXAMINATION
ECG
CHEST RADIOGRAPH
ECHOCARDIOGRAPHY
49

50. THERAPEUTIC MANAGEMENT
MEDICAL:
 PGE1
SURGICAL:
 Blalock Taussig shunt
 Balloon Atrial Septostomy
 Fontan procedure
50

51. POST OP COMPLICATION
 CHF
 RF
 RESIDUAL VSD
 Conduit obstruction
 Dysrhythmias
 Infective endocarditis
51

52. TGA
 Cyanotic disorder with mixed circulation
 TGA is a cyanotic defect in which the aorta arises from the RV and
pulmonary artery arises from the left ventricle resulting in 2 separate and
parallel circulations
INCIDENCE:
 9%
 MALES> FEMALES
 TERM INFANTS
52

53. PATHOPHYSIOLOGY 53

54. CLINICAL MANIFESTATIONS
 Cyanosis
 Hypoxic spells
 clubbing
54

55. DIAGNOSTIC EVALUATION
 CARDIAC EXAMINATION
 ECG
 CHEST RADIOGRAPH
 ECHOCARDIOGRAPHY
55

56. MANAGEMENT
MEDICAL:
 Prostaglandin infusion
 O2 therapy
SURGICAL:
 Rashkind procedure
 Blalock hamlon procedure
 Arterial switch procedure
 Mustard procedure
 Senning procedure
56

57. 57

58.  Anne Casey is an English nurse who developed a nursing theory
known as Casey’s Model of Nursing. The model was developed in
while she was working in pediatric oncology at the Great Ormond
Street Hospital in London.
 Casey’s Model of Nursing focuses on the nurse working in partnership
with the child and his or her family. It was one of the earliest attempts
to develop a nursing model designed specifically for child health
nursing.
 The five aspects of this nursing theory are child, family, health,
environment, and the nurse.
 The philosophy of Casey’s model is that the best people to care for
child are the members of the family, with health care professionals
assisting. This necessitates a relationship between the parent(s) and
nurse.
58

59. NURSING MANAGEMENT:
 Obtain a thorough history of the infant from parents
Perform a head to toe physical assessment with focus on following:
- vital signs
- skin color
- extremities are checked for peripheral pulse , edema, color and
temperature
- presence of clubbing
- heart sounds to determine rate and rhythm and identify any murmur
- Signs of respiratory distress
- Childs level of activity tolerance
- Childs growth and developmental level
59

60. NURSING DIAGNOSIS
 IMPAIRED GAS EXCHANGE RELATED TO ALTERD PULMONARY BLOOD FLOW
OR PULMONARY HYPERTENTION
 DECREASED CARDIAC OUTPUT RELATED TO REDUCED MYOCARDIAL
FUNCTIONING
 IMPAIRED PHYSICAL MOBILITY AND FATIGUE RELATED TO ACTIVITY
INTOLERANCE SECONDARY TO PULMONARY CONGESTION AND HYPOXIA
 ALTERED NUTRITION LESS THAN BODY REQUIREMENT REALTED TO
ANOREXIA AND DECREASED ENERGY AVAILABLE FOR SUCKING AND
CHEWING
 HIGH RISK FOR IMPAIRED GROWTH AND DEVELOPMENT RELATED TO
INADEQUATE TISSUE PERFUSION
 RISK FOR INFECTION RELATED TO HOSPITALIZATION
60

61. JOURNAL INFORMATION
 Spectrum of CHD in a tertiary care center if eastern India
 Frequency of CHD in Indian children with Down syndrome
61

62. 62

63. REFERENCE
 BOOKS:
 “Susan woods,( 2010), cardiac nursing, 6th edition, wolter Kluwer publication , page- 742- 745”
 “Barbara riegel, (2008), cardiac nursing, Elsevier publication, page- 1085- 1106”
 “cloherty and stark’s manual of neonatal care, (2017), south Asian edition of 8th edition, wolter Kluwer
publication”
 “Kyle terri, Carmen susan,essential of paediatric nursing, 3rd edition, wolters kluwer publication”
 “Swarna rekha bhat, (2009), Achars textbook of paediatrics, 4th edition, university pren india pvt limited,
”
 “Ball jane, bindler ruth, principles of paediatric nursing, 5th edition, pearson publication”
 “Singh meharban, essential paediatric for nurses, 3rd edition, CBS publishers, page-”
 “Scott Julius, scott’s paedia tricks, (2011), 3rd edition, paras medical books publishers”
 “Sharma rimple, essential of paediatric nursing, (2017), 2nd edition, jaypee publication”
 “Paul k vinod, baggar aravind, essential paediatrics, 2013, 8th edition, CBS publishers”
 “piyush gupa, (2017), essential paediatric nursing, 4th edition, CBS publishers, page-”
 “Wong’s, nursing care of infants and children, 10th edition, Elsevier publication, page-”
 “ Datta Parul, (2009), padiatric nursing, 2nd edition, jaypee Brothers medical publishers”
63

64.  WEB:
 www.uptodate.com
 www.stanfordchildrens.org
 www.healthline.com
 www.mayoclinic.org
 JOURNALS:
 “Anuspandana Mahapatra, 2017, spectrum of congenital heart disease in a tertiary
care centre of eastern India, International Journal of contemporary paediatrics,
4(20: 314-316”
 “ Ambreen Asim, 2016, frequency of congenital heart defects in Indian children
down syndrome, Austin journal of genetics and genomic research”
64

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