this slide share contain congenital heart diseases

1. CONGENITAL HEART DISEASE
(CHD)
MODERATOR
Dr. Mukesh chandra
Associate professor
AIIMS Jodhpur
PRESENTER
Mr. Maneesh
Msc 2nd year
Paediatric Nursing

3. INTRODUCTION
• CHD encompasses a broad and diverse range of
conditions that manifest from prenatal period to late
adulthood.
• In common usage, CHD refers to structural heart
defects that are present at birth.
• History, physical examination, chest X-ray, ECG and
echocardiography help in identifying the presence of
CHD, except perhaps in the early newborn period
where the diagnosis can be challenging.
• Palliative or corrective surgery is feasible for most
patients with CHD, provided if undertaken in a timely
fashion

4. INCIDENCE
• One third of all major congenital anomalies
• The prevalence of CHD in infancy is estimated at 6-
8/1000 live births in which 25% life threatening and
require early intervention
• A proportion of patients with CHD have an
identifiable genetic basis

5. ETIOLOGY
• Exact cause of CHD is unknown in about 90% cases.
• Heredity and consanguineous marriage are important
etiological factors
• Genetic and chromosomal aberrations are also known
to predispose CHD
Prenatal exposure that increase risk of congenital
heart disease
• Gestational diabetes (transposition, atrioventricular
septal defects, hypo plastic left heart,
cardiomyopathy, PDA)

6. Cont..
• Febrile illness in first trimester (increased risk)
• Rubella (PDA, peripheral pulmonary stenosis,
VSD)
• Lupus (complete heart block)
• Phenylketonuria (VSD, TOF, PDA, single ventricle)
• Vitamin deficiency (increased risk of heart disease)
• Teratogens, (first trimester) e.g. anticonvulsants,
NSAIDs, cotrimoxazole, thalidomide, retinoic acid
• Exposure to organic solvents, herbicides, pesticides,
ionizing radiation

7. Type of CHD
Acynotic Cyanotic obstructive
•Ventricular septal
defect (VSD)
•Atrial septal defect
(ASD)
•Patent ductus
arteriosus (PDA)
•Tetralogy of Fallot
•Tricuspid atresia
•Transposition of
great arteries
•Coarctation of aorta
•Congenital aortic
stenosis
•Anomalous origin of
coronary arteries
•Congenital mitral
stenosis
•Congenital mitral
incompetence
•Dextrocardia

8. ACYNOTIC HEART DISEASE
• In this type of disorder left to right shunting
occurs
• The blood flow in systemic circulation is
oxygenated
• In acynotic disorder the systemic circulation is
decrease and the pulmonary circulation is
increase

9. ASD

10. ATRIAL SEPTAL DEFECT
ASD is abnormal opening in the septum between
the atrium is called ASD. ASD account for as an
isolated anomaly 5-10% of all CHD.
Type of ASD
1. Ostium Primum (ASD-I)
2. Ostium Secondum/fossa ovalis
3. Sinus venosus ASD- These defects are
associated with anomalous drainage of one or
more right pulmonary veins

11. Pathophysiology
D.E.F
Defect between left and right atrium
Blood from left atrium to right atrium
Left to right shunting increase burden in right side of heart
Increase pulmonary blood flow
Pulmonary congestion and right ventricular enlargement

12. CLINICAL MENIFESTATION
– Hypotension
– Tachycardia
– Tachypnea
– Murmur like heart sound
– Pulmonary edema
– Chance of repeated chest infection
Diagnostic evaluation
– Echocardiography
– Cardiac catheterization

13. Management
• Small ASD may occasionally close spontaneously, not
require any intervention
• Surgical management is available abnormal opening
should be closed by surgery, ASD is recommend
between 2-4 year of age
• Surgical repair is most often done through a median
sternotomy and require cardiopulmonary bypass
• If the defect is small, purse string closure is done by
stitching around the opening and pulling it closed
• If the defect is large a knitted Dacron patch is sewn
over the defect

14. Post operative complication
• Cardiac enlargement
• Dysrthymias
• Infective endocarditis

15. VSD

16. VENTRICULAR SEPTAL DEFECT (VSD)
Assessment finding
– Hypotension
– Tachycardia
– Tachypnea
– Murmur like heart sound
– Pulmonary edema
– Chance of repeated chest infection

17. Pathophysiology
D.E.F
Blood from left ventricle to right ventricle
Left to right shunting increase, burden in right side of heart
Increase pulmonary blood flow and pulmonary hypertension
Pulmonary congestion and right ventricular enlargement

18. Diagnostic evaluation
• Echocardiography
• Cardiac catheterization
Management
• Only surgical management is available
• Abnormal opening should be closed by
surgery

19. Complication
• FTT
• Recurrent pneumonia
• Infective endocarditis
• Repeated episodes of CCF
• Pulmonary hypertension and its complications
(Eisenmenger syndrome)
• Pulmonary stenosis (Gasul’s VSD)
• Aortic regurgitation

20. PDA

21. PDA (PATENT DUCTS ARTERIOSUS)
• Failure of ductus arteriosus to became closed
within 7dyas after birth is called PDA
• The abnormal blood flow will occur from
descending aorta to pulmonary artery and this
condition will cause increased pulmonary
blood flow and decrease systemic circulation

22. Assessment finding
• Hypotension
• Tachycardia
• Tachypnea
• Murmur like heart sound
• Pulmonary edema
• Chance of repeated chest infection
• Water hammer pulse (Corrigan pulse)- sudden
bounding and sudden collapsing pulse

23. Pathophysiology
D.E.F
Failure to closer of ductus arteriosus
Blood flow into pulmonary artery from left ventricle (half or more of
ventricular output)
Increase pressure in pulmonary artery
Pulmonary hypertension
Pulmonary congestion
Pulmonary vascular disease (if left untreated)

24. Cont…
Diagnostic evaluation
• Angiography
• Cardiac catheterization
Management
• Asymptomatic PDA should be treated by ligation or
division of the ductus, preferably between 3 to 10
years of age
• Medical management – Indomethacin is DOC, this
drug is a NSAID drug and it will inhibit the synthesis
of prostaglandin
• Surgical management- ligation of ductus arteriosus
should be done by surgery

25. Complications
• CCF
• Infective endocarditis
• Rarely, aneurismal dilatation of pulmonary artery
and/or ductus, calcification of ductus,
thromboembolism, rheumatic heart disease and
Eisenmenger syndrome

27. TETRALOGY OF FALLOT (TOF)
• It is a cyanotic heart disease in this condition
abnormal blood flow occurs and right to left shunting
is present
• In this condition the blood in systemic circulation is
deoxygenating and it can cause cyanosis
Four disorder are present in this condition
1. Ventricular septal defect
2. Pulmonary stenosis
3. Right ventricular hypertrophy
4. Overriding of aorta

28. Pulmonary stenosis and Overriding of aorta

29. Assessment finding
• the blood flow is from right ventricular to left
ventricle
• the severity of symptoms depend on the level of
pulmonary stenosis
• new born has cyanosis
• poor feeding
• Tet spell (blue spell)- it is the sudden bluish
discoloration of the infant during any physical
activity like feeding and crying

30. Cont..
• In older child
– Growth retardation
– Muscle wasting
– Chronic hypoxia - symptoms are clubbing of
fingers occurs and nails beds has drum stick
appearance

31. Pathophysiology
D.E.F
Pulmonary artery stenosis
Increase pressure in right ventricle
Right ventricular hypertrophy
Right to left shunting due to VSD

32. Cont.
Blood flow from L-R increase pressure in left ventricle
Overriding of aorta
Deoxygenated blood supply to systemic
circulation due to right to left shunting
Polycythemia, cyanosis and poor pulmonary
vascularity

33. DIAGNOSIS
• Blood studies show Polycythemia and high
Hematocrete

34. Cont…
• 2-dimensional echocardiography shows the
anterior-superior displacement of the outflow
ventricular septum, causing stenosis of the
subpulmonic right ventricular outflow

35. Cont….
• X-ray chest , a small boot-shaped heart

36. • ECG shows right axis deviation, RVH with tall
and peaked P waves

37. Cont..
• Cardiac catheterization and selective
angiocardiography are of great value to elucidate
anatomic anomalies in tract and associated anterior
ventricular septal defect in doubtful cases
• Cardiac catheterization shows remarkable fall in
systolic pressure in the right ventricle as the catheter
enters the pulmonary artery.
• Ventriculography shows the anatomy of TOF at its
best

38. TREATMENT
• General measures include correction of iron-
deficiency anemia, and dehydration and an
appropriate antibiotic for bacterial infection
• Management of spells Mild sedative like
promethazine reduces the frequency of spells,
provided it is given regularly. Oral propranolol
therapy is of value in prevention. The dose is 0.5 to
1.0 mg/kg/6hourly. Iron-deficiency anemia, if
coexisting, should also be treated

39. Cont..
Selective treatment of acute hyper cyanotic spell is outlined
following
1. Comfort the child and place in knee-chest position
2. Administer humidified O2 by face mask
3. Give morphine, 0.1-0.2 mg/kg. (IV)
4. Begin IV fluid replacement and volume expansion (if child is
anemic, administer blood)
5. Treat acidosis
6. Administer propranolol, 0.1 to 0.2 mg/kg (IV)
7.Increase systemic vascular resistance by IV administration of
vaso-pressors like methoxamine or phenylephrine

40. Surgical management
I. Palliative Surgery
• Modified Blalock-Taussig Operation It consists in
anastomosing the subclavian and the pulmonary
arteries. This is the most popular systemic-to
pulmonary artery shunt today. It can be performed
successfully even in a preterm neonate
• Potts’ Operation Here, a side-to-side anastomosis of
pulmonary artery with aorta is created
• Waterson operation It consists in constructing a
shunt between the ascending aorta and the right
pulmonary artery.

41. Cont...

42. Cont..
II. Total Correction
• Direct-vision open heart surgery for repair of VSD and
pulmonary stenosis later in childhood is the procedure of
choice under ideal circumstances.
• Total corrections carry a mortality of 15%.
• Those who survive operation show complete disappearance of
cyanosis and clubbing and improvement in growth and
development.
• Risk of “sudden death” due to arrhythmias as also “exercise
disability” remains high, however. Infrequently, a permanent
complete heart block may occur following surgery.
• It is an indication for placement of a permanently implanted
pacemaker.

43. TRANSPOSITION OF GREAT ARTERIES (TGA)
• In this condition interchange of two major artery occur
• In this condition aorta is started from right ventricle and
pulmonary artery from left ventricle
• TGA occurs predominantly in males (4 times more than in
females). Incidence of diabetes in their grandparents is
significantly high. Also, these babies are of relatively large
birth weight
• Due to this interchange two separate circulation are developed
• Separate systemic circulation by aorta
• Separate pulmonary circulation by pulmonary artery

44. TRANSPOSITION OF GREAT ARTERIES

45. Assessment finding
• The patient of this condition has sever cyanosis and
the patient may became dead within few hour after
birth if no any other defect present like ASD,VSD
and PDA
• Severe cyanosis
• Dyspnea, CCF and growth failure occur
• Clubbing also develops in few months
• Heart is always enlarged

46. Diagnosis
• Radiology shows enlarged heart

47. Cont...
• Echocardiography confirms the diagnosis. It
shows equal peak systolic pressure in both
ventricles aorta and pulmonary artery

48. Cont..
• ECG reveals RVH, right axis deviation and
often P-pulmonale

49. Cont..
• Cardiac catheterization and selective
angiocardiography help in confirming the diagnosis.

50. Treatment
• Medical treatment with IV prostaglandin E1 (PG-E1)
digoxin, diuretics, iron, etc. should be given as and
when indicated
• Beffe’s operation- the location of this two artery is
corrected by surgery

51. TRICUSPID ATRESIA
• The term denotes congenital absence of tricuspid
valve, resulting in absence of any outlet from the
right atrium to right ventricle. The entire systemic
venous return, therefore, enters the left heart by
means of the foramen ovale or an ASD

52. Tricuspid atresia

53. Management
• Palliative treatment is the placement of
a shunt (pulmonary–to–systemic artery
anastomosis) to increase blood flow to
the lungs.
• If the ASD is small, an atrial
septostomy is performed during
cardiac catheterization.
• Some children have increased
pulmonary blood flow and require
pulmonary artery banding to lessen the
volume of blood to the lungs.
• A bidirectional Glenn shunt
(cavopulmonary anastomosis) may be
performed at 4 to 9 months as a
second stage.

55. COARCTATION OF AORTA

56. COARCTATION OF AORTA
• In this condition some part of descending aorta became
coarctated and it will cause narrowing of descending aorta and
obstruction will occur in normal blood circulation.
Assessment finding
• Upper extremities has hypertension and lower extremities has
hypotension
• Upper extremities has bounding pulse and lower is
weak/thread pulse
• Upper extremities are warm and lower are cool
• Headache, Epistaxis
• Dyspnea on running
• Dysfunction of left ventricle and heart failure

57. Diagnosis
• X-ray chest findings include some left ventricular
enlargement, notching of the ribs caused by
intercostals collaterals and “E” sign on barium
swallow

58. • ECG
• Echocardiography (real-time 2-dimensional)
may visualize the coarctation directly
• Cardiac catheterization and
angiocardiography

59. Treatment
• Medical management consists in tackling CCF and
hypertension, dilatation of the associated PDA with a
constant infusion of prostaglandin E1 in critical
situations, and restriction of strenuous exercise.
Antibiotic prophylaxis against bacterial endocarditis
is desirable.
• Surgical management The best age for surgery is 3
to 5 years, provided that significant systemic
hypertension has not developed

60. Course and Complications
• Congestive failure in infancy. If congestive failure
does not occur in infancy, it is unlikely to occur
throughout the pediatric age group unless
complicated by infective endocarditis or anemia

61. Aortic stenosis

62. AORTIC STENOSIS
• Depending on the site of obstruction to the outflow of
blood from the left ventricle in relation to the aortic
valve, congenital aortic stenosis may be divided into:
• valvular, sub valvular (sub aortic), and supra valvular.

63. Cont..
• Valvular stenosis accounts for 75% of the
cases of aortic stenosis.
• Subvalvular stenosis is of three types: discrete
membranous, fibro muscular and idiopathic
hypertrophic.

64. Diagnosis
• X-ray chest shows somewhat prominent left ventricle though
heart size is usually within normal limits. Dilatation of aorta
suggests valvular and, sometimes, discrete membranous sub
valvular stenosis
• ECG
• Echocardiography
• Serial catheterization may well be the only dependable guide
to the progression of the disease

65. Treatment
• Patient should be discouraged from overexertion, i.e.
competitive sports, athletics and strenuous exercise
• Surgery in the form of aortic valvotomy and aortic valve
replacement is indicated in the presence of significant
manifestations or a large resting gradient of 60 to 80 mm Hg
• Unfortunately, surgery in the form of valvotomy may be
complicated by aortic regurgitation which is worse than the
stenosis.
• The patient who gets valve replacement has got to be on
anticoagulants.
• Secondly, neither the prosthetic nor the homograft valve lasts
indefinitely.

66. CLINICAL MANIFESTATION OF CHD
• Cyanosis
• Difficult feeding and poor growth
• Difficult breathing
• Frequent respiratory infections
• Specific syndromes
• Impaired myocardial function
• Pulmonary congestion

67. Cont....
Impaired Myocardial
Function
Pulmonary
Congestion
Systemic Venous
Congestion
•Tachycardia
•Sweating (inappropriate)
•Decreased urinary output
•Fatigue
•Weakness
•Restlessness
•Anorexia
•Pale, cool extremities
•Weak peripheral pulses
•Decreased blood pressure
•Cardiomegaly
•Tachypnea
•Dyspnea
•Retractions (infants)
•Flaring nares
•Exercise intolerance
•Orthopnea
•Cough, hoarseness
•Cyanosis
•Wheezing
•Grunting
•Weight gain
•Hepatomegaly
•Peripheral edema,
especially periorbital
•Ascites
•Neck vein distention
(children)

68. NADAS' CRITERIA
• The assessment for presence of heart disease can be
done using the Nadas' criteria
Major Minor
Systolic murmur grade III
or more
Diastolic murmur
Cyanosis
Congestive cardiac failure
Systolic murmur grade I or II
Abnormal second sound
Abnormal electrocardiogram
Abnormal X-ray
Abnormal blood pressure

69. DIAGNOSTIC TEST
• History collection
• Physical examination and systemic examination
• Diagnostic Implications of the Second Heart
Sound
• Echocardiography
• Cardiac magnetic resonance
• Computed tomography/ MRI
• Diagnostic cardiac catheterization
• Chest X-ray
• Angiography

70. MANAGEMENT
Surgery
• Surgery is the best option for definitive treatment or palliations
• Open heart (requiring use of cardiopulmonary bypass CPB)
• Closed heart (not requiring use of cardiopulmonary bypass
CPB).
• These procedures are more significant and more expensive
undertaking because of use CPB circuit and a substantially
large number of disposable items

71. Corrective operation
• Corrective surgeries possible for VSD and
ASD, with no significant long-term concern, if
the repair of TOF does not result in pulmonary
valve incompetence, long term concerns are
minimal

72. Cont..
Surgery for single ventricle physiology
The surgical management of single ventricle physiology is
performed in stages
• 1st stage involves early pulmonary arterial band (usually under
the age of 3 months) for patients who have increased pulmonary
blood flow and the modified Blalock-Taussig shunt for those
who have reduced pulmonary blood flow with cyanosis
• 2nd operation is the bidirectional Glen shunt. The superior vena
cava is anastomosed to the right pulmonary artery. This
operation allows effective palliation until the age of 4-6 yr.
• The Fontan operation is finally required for elimination of
cyanosis. All the systemic venous return is routed to the
pulmonary artery

73. Cont..
• For ASD-
Surgical patch closure (pericardial patch or Dacron
patch) is done for moderate to large defect
• For VSD-
Small defects are repaired with sutures. Large defects
usually require that a knitted Dacron patch be sewn
over the opening
• For PDA- Indomethacin and ligation of PDA

74. Cont..
• For TOF- Palliative modified blalock Taussig
shunt, Potts’ Operation, Waterson and
complete repair requires a medial sternotomy
and use cardiopulmonary bypass

75. Cont…
• Tricuspid atresia- The first stage, also called
a Bidirectional Glenn procedure or Hemi-Fontan The
second stage, also called Fontan completion, involves
redirecting the blood from the IVC to the lungs

76. Cont..
• For TPG - Prostaglandin E1 (PG-E1) digoxin,
diuretics, iron, etc and Beffe’s operation
• For Aortic Stenosis- balloon dilation and
valvotomy konno procedure – replace the
valve

77. Cont..
• Catheter Interventions
Many simple defects such as Secondum ASD, PDA
and selected muscular VSD can now be closed in the
catherization laboratory. Additionally, balloon
valvotomy is now the first line of treatment for
congenital stenosis of the pulmonary and aortic
valves. Catheter-based interventions are far less
traumatic than surgery, accomplished with ease and
allow rapid recovery

78. COMPLICATION of CHD
• Pulmonary arterial hypertensions (PAH)
• Infective endocarditis
• Growth and nutrition
• Myocardial dysfunction
• Neurologic problem
• Polycythemia
• Rhythm disorders and sudden death Chronic
• Cyanotic spells
• Eisenmenger syndrome- condition that affects blood flow from
the heart to the lungs in some people who were born with
structural problems of the heart

79. PREVENTION
• Education of lay public on the risks associated with
consanguinity, drugs and Teratogens in 1st trimester
of pregnancy and widespread immunization against
rubella has limited role in preventing CHD
• Fetal echocardiography is emerging as a modality for
early diagnosis of CHD
• Once a serious CHD is identified, it is vital to counsel
the families about postnatal manifestations, natural
history, surgical options and their long term outlook

80. • Results of fetal echocardiography enable delivery at a
center with comprehensive pediatric heart program.
While echocardiography is recommended for future
pregnancies after diagnosis of serious CHD in a child,
this practice has low yield because only 2-8% CHD
recur.
• The highest chance of recurrence is with obstructive
lesions of the left heart

81. Research update
Development of feeding information for infants with CHD
Luise V. Marino , Mark J. Johnson, Natalie J. Davies , Catherine Kidd
DOI: https://doi.org/10.1017/S1047951119001665
Published online by Cambridge University Press: 05 August 2019
Results:
• A total of 23 unique articles were identified of which 5 studies
were included. From the grey literature, four web pages were
reviewed. A total of 22 parents and 25 health-care
professionals were interviewed. All parents/health-care
professionals felt that the feeding information developed
provided sufficient information; however, many wanted
information on how to introduce complementary food,
particularly if weaning was delayed.

82. Conclusions:
This study describes the development of feeding
information for infants with CHD. From parent
interviews, gaps identified focused on the
introduction of complementary foods and uncertainty
regarding the feeding journey beyond surgery.

83. 2. Evaluation of children with cardiac murmur
using Nadas criteria
Mary James, Poornima K. N., Praveen Jacob Ninan
Results: 3070 children were screened. 150 children
were detected to have murmur of grade2 and more.66
children were detected to have a structural heart
disease by an echo study.74% of the study population
in the age group <1 year had a heart disease.8 out of
78 children with a Nadas score of 1 and 10 out of 24
with a score of 2 had a heart disease. All with a score
3 and more had congenital heart disease (CHD).
Conclusions: Nadas criteria can be used as screening
test with a sensitivity of 87% and specificity of 83%.

84. Conclusion and summary