congenital heart defects- Acyanotic heart defects.

2. Acyanotic Heart
Diseases

3. CONGENITAL HEART DISEASES
DEFINITION:
• Congenital heart disease is
defined as the structural,
functional or positional
defect of the heart in
isolation or in combination
present at birth but may
manifest at anytime after
birth or may not manifest at
all.

4. CONGENITAL HEART DISEASES
DEFINITION:
• Congenital heart disease is
defined as malformation of
heart, aorta or other large
vessels that is present at
birth.

5. DEFINITION OF CYANOTIC HEART DISEASE
• A condition where the blood
is pumped around the body
contains less than normal
level of oxygen. It causes the
skin to appear bluish in color
i.e, cyanosis

6. DEFINITION OF ACYANOTIC HEART DISEASE
• Is a congenital heart defect
where the blood contains
enough oxygen but it is
pumped abnormally around
the body.

7. EPIDEMIOLOGY OF ACYANOTIC HEART DISEASE
• Females: males ratio is 3:1
• VSD: 25% of total CHD
• PDA: 9%
• PS :10%
• AS :5%
• COA : 4%
• ASD :10%

10. CAUSES OF ACYANOTIC HEART DISEASE
• Exact cause is unknown
• Abnormal embryonic development.
• Possible causes are:
Fetal and maternal infection
Maternal disease like:
→ German measles, cytomegalovirus infection
→ Teratogenic effects of drugs &alcohol e.g) Lithium,thalidamide.
→Maternal dietary deficiencies e.g) Poor nutritional status
→ Hereditary &consanguineous marriage.
→ Maternal age greater than 40
→Radiation exposure
→ Maternal insulin dependent diabetes

11. CAUSES OF ACYANOTIC HEART DISEASE
FETAL CAUSES
• Fetal hypoxia, birth asphyxia
• Genetic disorder & Chromosomal
abnormality example:
• Down syndrome –VSD
• Turner syndrome – COA
• Trisomy 13,18-VSD,ASD PDA.

12. REASON FOR NO CYANOSIS
• No abnormal communication
between pulmonary &systemic
circulation
• The peripheral blood is therefore
oxygenated as in normal infant
and cyanosis doesn’t result.

13. TYPES OF ACYANOTIC HEART DISEASE
ATRIAL SEPTAL DEFECT
Definition: ASD is a defect in the
septum between the atria that
allows shunting of blood from the
left to right atrium.
Incidence:
Females >Males (3:1)
Occurs 10% of total CHD

14. Types of atrial septal defect
• Ostium primum:
• Opening at lower end of septum may be associated with mitral valve
abnormalities. It accounts about 20%
• Ostium Secundum:
• Opening near center of septum. It accounts about 70%.
• Sinus venous defect:
• Opening near junction of superior venacava & RA may be associated with
partial anomalous pulmonary venous connection. It accounts about 5-10%

16. Anatomy
• A patent foramen ovale is an
opening between the right and
left atria that is normally present.
It typically closes shortly after
birth, but if at all it doesn't closes,
it is said to be atrial septal defect.

17. Haemodynamics:
Patent foramen ovale (Fails to close)
Blood shunted from LA to RA
Increase burden on the right side of the Heart
Increase pulmonary blood flow
Increase pressure in RV
Right sided heart failure

18. Clinical manifestation:
• Congestive heart failure.
• Murmur
• Atrial dysrhythmias
• ASD child will appear
-Thin
-Undernourished
• Marfan’s syndrome:
- High arched palate
-Laxity of ligaments
-Hypermobility of joints

19. Clinical manifestation
• Lutembacher’s syndrome:Diastolic murmur at
the apex with or with out mitral stenosis.
• Protrusion of left chest along with a slender
build.
• Frequent episodes of pulmonary inflammatory
disease.
• Ostium primum: Systolic murmur will be loud
,harsh &long, high pitch, loudest at the apex.

20. Investigation
• Health history
• Physical examination
• X-ray :Shows heart enlargement, PA enlargement
• Electrocardiogram: Right ventricular hypertrophy.
• Cardiac catheterization :Denotes the left to right shunt.
• Angiocardiography:Reveals opacification of both the atria
• Echocardiography: Right ventricular over load.

21. Management
• Medical management treats only
symptomatically.
• Surgical approach is the exact
management.
CARDIAC CATHETERIZATION
OPEN HEART SURGERY

22. Prognosis
• Very low operative modality less than 1 %.
• COMPLICATION:
• Pulmonary hypertension
• Congenital mitral valvulitis
• Death due to pulmonary infection

23. VENTRICULAR SEPTAL DEFECT
• Definition:
VSD is the abnormal opening between the right &left
ventricle.
Incidence:
1.20 -25% of all cardiac lesions
2.More common in premature babies.
3.Equal Male :Female ratio.
4.VSD is most common CHD in
Edward syndrome , Down syndrome

24. Types of VSD
1.PERIMEMRANOUS VSD:
• Defect in the membranous septum is called ashigh or memraneous VSD
• It accounts for 70 -80% of all VSD
• It frequently occurs with other defects like COA,PDA
2. MUSCULAR VSD:
• The defect present at interventricular septum of the muscle portion.
• It is called as low or muscular VSD.
• Accounts about 10%
• It can be single or multiple.
• Occasionally entire ventricular septum may be absent resulting single
ventricle.

25. Types of VSD cont.
3.Inlet( Inflow) VSD:
• It is called canal VSD.
• Because it may form a part of AV canal.
• It is found in 5 to 8% of all VSD.
4.Outlet VSD:
• It is called subarterial VSD
• More common in south east Asian population-Japan
• Accounts about 5 to 8%
• Aortic valve can prolapse into this VSD, causing aortic regurgitation.

27. According to size of the VSD it is classified into
3:
• Small VSD: When defect is about < 5mm
• Moderate VSD: 5 to 10 mm
• Large VSD : > 10mm

28. VSD Description
• Many VSD 20-60% are thought to
close spontaneously.
• Left to right shunt develops in
VSD.

29. Pathophysiology:
Congenital causes
Abnormal opening between the RV &LV
Pressure in the LV is higher than RV
Blood is shunted from left to right ventricle
Increase blood flow to the PA
Increase blood flow to the lungs.
Increase pulmonary vascular resistance- Increase pressure in right
ventricle.
Right ventricular hypertrophy
Eisenmenger syndrome

30. Haemodynamics of VSD-depend upon the size
of the defect:
• Small defect:
Found in muscular portion
Effect is slight
Small amount of O2 passes from LV to RV.
Large defect:
Found in membranous portion of the septum
Pulmonary hypertension
Greater amount of oxygenated blood passes from RV to LV

31. • Extreme defect:
In extreme defect there may be only one
ventricle.

32. Clinical manifestation:
Large defects:
• Harsh,loud,pansystolic murmur
• Breathlessness
• Difficult in feeding
• Failure to thrive.
• Congestive cardiac failure
• Peripheral pulse is small because of poor systemic blood flow
• Tachypnea
• Slow physical development
• Frequent pulmonary infection.
• Cardiac enlargement

33. Clinical manifestation:
Small defects:
• Mostly asymptomatic
• Pansystolic murmur
• Bacterial endocarditis
• Pulmonary vascular obstructive disease

34. Investigation
• Physical examination: murmur with a thrill
• ECG: Large ventricular defect shows left ventricular hypertrophy
• Chest X ray: left atrial enlargement, pulmonary edema
• ECHO: VSD
• Cardiac catheterization: detects the number, size, location of defect.

35. Management
• MEDICAL MANAGEMENT:
• Treat iron deficiency if present
• Infective endocarditis prophylaxis
• Treat chest infection promptly
• Treatment of CCF

36. Management
• SURGICAL MANAGEMENT
Pulmonary artery banding
Corrective repair- Open heart
surgery/ cardiac catheterization

37. Prognosis
• Membranous defect : Low mortality < 5%
• Multiple muscular defects: High mortality >20%
COMPLICATION:
• CHF.
• PAH
• Eisenmengers syndrome
• Infective endocarditis
• Growth failure
• Reoccurring pneumonia

38. PATENT DUCTUS ARTERIOSUS
• Definition:
Failure of the fetal ductus
arteriorus to close within first
week of life. The continued
patency of this vessel allows
flowing of blood from high
pressure aorta to low
pulmonary artery causing left
to right shunt.

39. Incidence
• Most common in premature infants
• Occurs with other cardiac lesions
• Female : male (2: 1)
DESCRIPTION
DA - Artery connecting the aorta & PA
Continued patency of this vessel allows blood flow from the higher
pressure aorta to lower pressure PA.

40. Types of PDA
SMALL PDA:
• The opening usually less than 4 mm size at aortic end
• Usually asymptomatic
Moderate:
• It can be symptomatic
• Mild growth failure
• The defect size is more than 4mm

41. PATHOPHYSIOLOGY
Due to etiological factors
PDA does not closes
Left to Right shunt
Blood flow from aorta to PA through PDA
Recirculation of oxygenated blood
Increase burden on left side of the heart
Increase pulmonary congestion
Left arterial, ventricular enlargement
Left ventricular hypertrophy.

42. Clinical manifestation
• Signs of CHF
• Murmur
• Widen pulse &bounding pulse
• Bacterial endocarditis
• Dyspnoea
• Physical underdevelopment
• Increased respiratory infections
• Gallop Rhythm (Due to rapid filling of the ventricle)
• Hepatosplenomegaly

43. Investigation:
• X-ray : Left & Right ventricular enlargement
• Electrocardiograph: Left ventricular hypertrophy
• Echocardiography: Size of PDA
• Cardiac catheterization : Reveals increase pressure in RV

44. MANAGEMENT
Medical:
• Prostaglandin synthase inhibitors
• Transcatheter closure
• Ligation of ductus arteriosus

45. Complication
• Sub acute bacterial endocarditis.
• CCF
• Bronchitis
• Aneurysm
• Reversal of shunt

46. COARCTATION OF THE AORTA
• Coarctation of aorta is defined
as localized narrowing near the
insertion of ductus arteriosus
resulting in increased pressure
proximal to the defect, and
decreased pressure distal to
obstruction.

47. Incidence
• Accounts about 5% of CHD
• Male>Females(2:1)
TYPES:
On the basis of their anatomical presentation COA is classified into 2
types:
• INFANTILE PREDUCTAL TYPE
• POSTDUCTAL TYPE

48. TYPES
INFANTILE PREDUCTAL TYPE:
• There is a constriction between the
subclavian artery &the arteriosus. It is
more common and located at near the
region of the aortic isthmus.
POSTUCTAL TYPE:
• Constriction at on distal to the ductus
arteriosus.

49. Pathophysiology
Congenital causes
Narrowing within Aorta
Decrease pressure to the distal part of the defect-Increase pressure to
the proximal part of the defect
Increase left ventricular workload
The flow of blood to the trunk &extremities through collateral arteries.
Collateral arteries bypass the coarctation

50. connect arteries to the branches of the subclavian artery on to the
arteries which arise from Aorta below coarcation
Suzman’s sign(Dilatation of collateral arteries are often seen over the
scapular regions of the back)

52. HAEMODYNAMICS:
• Preductal type:
The lower half of the body supplied by
Right ventricle through the ductus arteriosus
• Postductal:
RV cannot maintain blood flow to the descending Aorta
Collateral arteries develops
It maintain flow from ascending to the descending Aorta.

53. Clinical manifestation
• High BP (Upper part of the body)
• Bounding pulses in arms, weak femoral pulse
• cool lower extremities with lower BP
• Signs of CHF
• Increase pressure it resulting in headache.
• Dizziness
• Epistaxis
• Cerebrovascular accidents
• Muscle cramps in the leg while exercise due to hypoxia

54. INVESTIGATION
• Physical examination
• X- ray: Shows Dock’s sign
• ECG : Left ventricular hypertrophy
• Retrograde aortography: It passes via brachial
artery may demonstrate the presence & extent
of coarcted area & state of collateral circulation.
• Angiography:It shows COA
• Barium swallow : E Sign

55. MANAGEMENT:
Medical management:
• Administer prostaglandin E1 (Ductal
patency)
• Treatment of Hypertension
Surgical management:
• End To End Anastomosis
• Grafting
• Percutaneous balloon angioplasty

56. Prognosis
• Mortality < 5%
• Preductal is poor. Postductal is better.
• Increase risk in infants with other complex cardiac defects
COMPLICATION
• Cardiac failure
• Subacute bacterial endocarditis
• Intracranial hemorrhage.
• Rupture of the aorta

57. PULMONARY STENOSIS
Definition:
• Ps is an obstructive lesion that
interferes with blood flow from
the right ventricle to pulmonary
artery.

58. Types of pulmonary stenosis
• On the basis of their anatomical presentation 2 types of pulmonary
stenosis:
VALVULAR STENOSIS:
• Occurs more than 90% of cases
• Cups of the pulmonary valves are fused
• Cause dome like stenotic valve &Right ventricular hypertrophy.
INFUNDIBULAR STENOSIS:
• Less common
• Pulmonary valve is normal but outflow of right ventricle is narrow.
• Converting the narrowed region into an infundibular channel.

60. Pathophysiology
Due to Etiology
Pulmonary valve is obstructed by fusion of cups
Resistance to blood flow from RV to PA
Increased pressure in the RV
Increased pulmonary stenosis
Blood is backed up into the RA
Reopening of the foraman ovale
Shunting of unoxygenated blood to the LA
Systemic cyanosis occur only if severe

61. Clinical manifestation
• Asymptomatic
Mild PS produce:
• Exertional fatigue
• Chest pain with exercise
• Systolic murmur
In severe PS produce:
• Dyspnoea
• Cardiac failure
• Cyanosis
• Child may squat to relief dyspnoea

62. Investigation
• Physical examination
• Chest X Ray: right ventricle dilated
• ECG: right ventricular hypertrophy
• ECHO: Measures pressure gradient

63. Management
Medical management:
• Prophylaxis- Bacterial endocarditis
Surgical management:
• Pulmonary valvotomy Brock procedure
• Balloon angioplasty

64. Complication
• CCF
• Bacterial endocarditis
• Primary tuberculosis
• Anoxic spells
Prognosis:
• Mortality-2 to3%
• Good for children with mild PS

65. AORTIC STENOSIS
• Definition:
• AS is narrowing or stricture of the aortic valve, causing resistance to
blood flow in the LV, decreased cardiac output, left ventricular
hypertrophy & pulmonary vascular congestion.
Incidence:
• Males >Females
• 80% of CHD is AS
Types of aortic stenosis:
• 1.Valvular stenosis 2.Subvalvular stenosis 3.Supravalvular stenosis

67. TYPES
• VALVULAR STENOSIS: It is a most common type is
usually caused by malformed cups resulting in a
bicuspid rather than tricuspid valve or fusion of
the cups. It accounts about
75%.Male>Female(2:1)
• SUBVALVULAR STENOSIS:It is a stricture caused
by a fibrous ring below a normal valve. Accounts
about 20% of cases. Fibrous muscular obstruction
forms ring 5-10 mm the aortic valve.
• SUPRAVALVULAR STENOSIS: Stenosis occurs just
above the coronary arteries. It occurs
infrequently.

68. PATHOPHYSIOLOGY
Etiology
Narrowing of the aortic valve
Resistance to blood outflow from the left ventricle to the aorta Extra
workload in the LV.
Left ventricle hypertrophy
Blood backs up in the left atrium
Increased pressure in the left ventricle
Increased pressure in the pulmonary veins
Pulmonary vascular congestion
Pulmonary edema due to AS

69. Clinical manifestation
• Hypotension
• Decreased cardiac output with faint pulse.
• Tachycardia
• Poor feeding
• Exercise intolerance
• Chest pain
• Dizziness when standing for long period
• Murmur

70. INVESTIGATION
• Physical examination: systolic ejection
murmur
• ECG: left ventricular hypertrophy
• Chest X ray: prominent aortic knob
• ECHO: to visualize anatomy
• Cardiac catheterization: pressure gradient
Mild: <40mmHg
Moderate : 40-75 mm Hg
Severe : >75mm Hg

71. Management
MEDICAL MANAGEMENT:
• Treatment of CCF
• Treatment of bacterial endocarditis
SURGICAL TREATMENT
VALVULAR AS:
• Aortic valvotomy

72. SUBAORTIC:
• Konno procedure
• Ross procedure
COMPLICATION:
• Sudden death
• Endocardial fibroelastosis
• Associated malformation like
ASD,VSD,PS,COA.

73. NURSING DIAGNOSIS
• Ineffective breathing pattern related to decreased PBF
Nursing intervention:
• Assess the general condition.
• Check breathing pattern
• Poisoning & Head elevation
• Avoid any constructing clothing
• Administer humidified O2
• Assess the respiratory rate
• Assess O2 saturation
• Provide calm & warm place
• Provide comfort bed.

74. Decreased cardiac output related to
structural defect.
Nursing intervention:
• Assess cardiac function
• Provide quite environment
• Provide tender loving care
• Provide appropriate play to reduce anxiety
• Administer Digoxin as order
• Observe signs of hypokalemia
• Observe for signs of hypotension
• Monitor electrolyte level
• Observe cardiac monitoring carefully

75. Activity intolerance related to imbalance
between O2 supply & demand
• Assess the child’s response to activity
• Maintain neutral thermal environment
• Respond promptly ti crying
• Provide calm &comfortable environment
• Feed small volume at frequent intervals
• Provide divertional activity
• Change the position of the child every 2 hours
• Teach the parents ,about child’s activity

76. Imbalance nutritional status less than body
requirement related to less food intake.
• Assess the child's nutrional status
• Assess the child’s Nausea,vomiting,inability to eat
• Check the weight daily
• Provide small amount of formula &food frequently
• Feed slowly &Buddle to prevent distention of stomach
• Provide low fat diet
• Provide fruits &fiber rich diet

77. RESEARCH
• A case-control study was carried out in Uttar Pradesh, India, from February
2014 to August 2015 to determine the prevalence and spectrum and identify
risk factors associated with the development of heart defects.
• Out of 400, Acyanotic heart defects were 290 (72.50%), cyanotic heart
defects was 110 (27.50%). Out of all CHDs, ventricular septal defect was
the most common lesion with contribution of 152 (38%) cases
• paternal age (odds ratio, OR, 2.01), bad obstetric history (OR, 2.65),
antenatal febrile illness (OR, 4.12), and advanced maternal age (OR, 3.28)
were found to increase the risk of CHD whereas intake of multivitamin
(OR, 3.02) was found to be protective.
• There is a need to prioritize antenatal care and counseling to pregnant
mothers along with good maternal nutrition and folic acid supplementation.