3. Objectives:
At the end of active learning discussion, the
group will be able to:
1. Define acyanotic heart defects
2. Know the incidence of acynotic disease
3. Explain different types of acynotic congenital
defects
4. Describe the etiology, clinical manifestation ,
diagnosis and management of acynotic
congenital defects.
5. Formulate nursing diagnoses and
appropriate nursing care
4. Congenital Heart Disease
• Congenital heart defect (CHD), also known as a congenital
heart anomaly or congenital heart disease, is a problem in
the structure of the heart that is present at birth.
• Signs and symptoms depend on the specific type of
problem.
• Symptoms can vary from none to life-threatening. When
present they may include rapid breathing, bluish skin, poor
weight gain, and feeling tired. It does not cause chest pain.
• Most congenital heart problems do not occur with other
diseases. Complications that can result from heart defects
include heart failure.
6. Defined:
Acyanotic Heart Defects
A congenital disorder manifested with left to right
shunting and obstructive lesions. Clinical signs are not
always apparent at birth, they manifest anytime
during infancy or early childhood.
7. Defects:
a. Left to right shunting lesions, increased pulmonary blood
flow
• The blood is shunted through an abnormal opening from the
left side of the heart to the right side of the heart
• Pulmonary blood flow increases because of the extra volume
in the right side. Physiologic effects include increased
pulmonary blood flow, increased cardiac workload (including
ventricular strain, dilation, and hypertrophy).
Examples: Atrial Septal Defect (ASD), Ventricular
Septal Defect (VSD), Patent Ductus Arteriosus
(PDA), and Atrioventricular Septal Defect (AVSD).
8. b. Obstructive or stenotic lesions – stenosis of an opening can
occur in a valve or vessel constricting or obstructing blood
flow through the area. Pressure rises in the area behind the
obstruction; blood flow distal to the obstruction may be
decreased or absent. Physiologic effects of obstructive or
stenotic lesions include increased cardiac workload and
ventricular strain, clinical consequence of CHF, decreased CO
and pump failure.
Example: Pulmonary stenosis, aortic stenosis,
Coarctation of Aorta, and interrupted aortic
archs.
11. Atrial Septal Defect (ASD)
Incidence and Pathophysiology:
● ASD accounts for approximately 10% of all
CHDs. It is seen more frequently in females
than males.
● The lesion consists of an abnormal opening
between the atria
Types of Lesions:
1. Ostium Secundum – located at the middle
of the atrial septum (fossa ovalis), the most
common type.
2. Ostium Primum – located low in the atrial
septum, results from a defect in endocardial
tissue formation and is often associated with
a left mitral valve malformation.
3. Sinus Venosus – which is located high in the
septum close to the SVC
12.
13. Atrial Septal Defect (ASD)
Altered Hemodynamic:
● Lower right ventricular compliance which is the
ease of ventricular diastolic filling, compared with
left ventricular compliance leads to left to right
shunting at the atrial level through the ASD. This
increased blood flow through the ASD leads to an
enlarge RA and RV and increased pulmonary
blood flow.
14. Manifestations:
● Most infants and children are asymptomatic but over years to decades may
experience:
1. Fatigue and SOB
2. Palpitations or atrial dysrythmias – result of atrial enlargement
3. Recurrent respiratory infections can occur when there is a large amount of
pulmonary blood flow
4. Systolic murmur is produced by increased blood flow across the pulmonary
valve.
5. Diastolic murmur is present with large shunts
6. Stroke or major organ damage can occur because of embolization of
thrombus, air or other materials – PARADOXIMAL EMBOLISM
15. Auscultation in ASD
•Increased flow across the
pulmonary valve produces a systolic
ejection murmur and fixed splitting
of the second heart sound
•Increased flow across the TV
produces a diastolic rumble at the
mid to lower right sternal border.
17. Physical examination
• Shown in this video mid/late
DIASTOLIC murmur (unlike
VSD which is heard in the
same place but is a
holoSYSTOLIC murmur) heard
best over the lower left
sternal border
(LLSB). afterwards, there
will be a pulmonary flow
murmur (systolic murmur)
due to increased flow
through the pulmonic valve
after the blood goes through
the tricuspid valve. This is
heard over the 2nd left
intercostal space,
18. Investigations:
• CXR:
– enlarged heart
– Enlarged PA
– increased pulmonary vascular markings,Central plethora
• ECG:
– Right axis in secundum defect
– hallmark of primum defect is extreme Left axis deviation
– RVH,RBBB
• ECHO:RVH,valve anatomy,flow direction.
• Treatment:(indicated if symptoms+,RV overload)
– Device closure during cardiac cathetrization
– surgical closure.
20. Chest X-ray
• Plain radiograph
• can be normal in early stages +/-
when the ASD is small
• signs of increased pulmonary
flow (pulmonary plethora or
shunt vascularity)
– enlarged pulmonary vessels
– upper zone vascular prominence
– vessels visible to the periphery of
the film
– eventual signs of pulmonary
arterial hypertension
• chamber enlargement
– right atrium
– right ventricle
– note: left atrium is normal in size
unlike VSD or PDA
– note: aortic arch is small to
normal
There is bilateral increased pulmonary
vascularity and prominence of the atrial
appendage. The heart size is within normal
limits. No rib or soft tissue abnormality. The
radiological features are compatible with the
known diagnosis of ASD.
21. • chest X-ray of
a patient with
atrial septal
defect
showing an
enlarged right
pulmonary
artery (RPA),
main
pulmonary
artery (MPA)
and right
atrium (RA).
End-on view
of an enlarged
pulmonary
vessel is also
seen.
22. ECG
1. The most frequent ECG
abnormalities in ASD
are right bundle branch
block (RBBB)
pattern or
2. rsR' pattern in lead
C1 . Complete RBBB is
observed less
frequently.
3. In patients with large
ASD, presence of tall R
or R' in lead C1 suggests
development
of pulmonary
hypertension .
4. Size of defect
determines the shunt
flow which in turn
results in most of the
ECG abnormalities.
5. If the ASD is very small,
the ECG may be
normal.
23.
24.
25. Atrial Septal Defect
Therapeutic Management:
1. Asymptomatic child is followed by cardiologist. Spontaneous closure can occur
in the first years of life for smaller size secundum ASDs.
2. Elective surgical repair is performed around 2-5 years of age
3. Surgical repair is recommended for all sinus venosus and ostium primum
defects.
Medical Management:
1. Asymptomatic patients with moderate size secundum ASDs are monitored for
spontaneous closure in the first years of life with medication.
2. Symptomatic infants and children are treated with diuretics and digoxin as
indicated
3. Atrial dysrythmias are treated with appropriate antidysrythmics
Surgical Management:
● Surgical closure using either sutures or a pericardial prosthetic patch is
performed on an elective basis early in childhood. This is an open heart
procedure, through a sternal incision.
● Mortality rate is <2%, with most centers near 0%. For the young adult with
ventricular dysfunction or pulmonary, the risk can be significantly higher.
● Complications include sinus node and atrial dysrythmias
29. ASD: Therapy
• Percutaneous Closure
– only for secundum (contra
in others)
– adequate superior/inferior
rim around ASD
– no R-L shunting
• Surgical Closure
– Good prognosis:
• closure age < 25, PA
pressure <40
• If >25 or PA>40, decreased
survival due to CHF, stroke,
and afib
31. Ventricular Septal Defect (VSD)
Incidence and Pathophysiology:
● VSDs account for approximately 25% of all CHDs.
● VSD is the most common congenital cardiac
lesion and is often accompanied by other cardiac
defects.
● The lesion consists of an abnormal opening
between the right and left ventricles which may
vary in size from a miniscule hole to complete
absence of the septum, resulting in a common
ventricle.
32. Altered Hemodynamics:
● The degree of left to right shunting through the VSD
depends on the size of the defect and the pulmonary
vascular resistance compared with the systemic
vascular resistance. The pulmonary vascular system
is high in the newborn. Over the first few weeks of
life, the resistance decreases. As this occurs, an
increased amount of blood shunts left to right of the
VSD level. The pulmonary vascular circulation
receives increased pulmonary blood flow. With large
defects the pulmonary arteries are exposed to
systemic pressures, causing pulmonary hypertension,
and over time, progressive pulmonary vascular
disease.
Ventricular Septal Defect (VSD)
33.
34. Ventricular Septal Defect (VSD)
Manifestations:
Signs and symptoms vary with the size
of the defect and the presence of
associated cardiac lesions. Clinical
symptoms are usually not seen at birth
because of continued high pulmonary
vascular resistance in the newborn.
Infants with moderate to large defects
will become symptomatic within the
first few weeks of life.
● Children with small defects will remain
asymptomatic.
35. Clinical Manisfestations
• Tachypnea, dyspnea
• Poor growth
• Palpable thrills
• Systolic murmur at left lower sternal border
• Shortness of breath
• Failure to gain weight
• Fast heart rate
• Pounding heart
• Frequent respiratory infections
36. Investigations
• History
• Physical Examination:
– parasternal thrill
– pansystolic murmur at lower left sternal edge(Loud if small defect,if large VSD
increase flow across pulmonary valve ejection systolic murmur
– loud P.(Pulmonary HT)
• CXR:
– cardiomegaly,enlarged LA&LV.
– Enlarged PA,increased pulmonary vascular markings
– Pulmonary oedema
• ECG:
– extreme leftt axis is charecteristic,biventricular hypertrophy.
• ECHO:chamber size & pressures.
• Cardiac catheter:O2 content,PA pressure,size & no of defects.
41. ECG
• signs of right ventricular
hypertrophy are observed
more frequently.
Signs of left ventricular
hypertrophy may also
accompany.
The rhythm is generally
sinus.
Right atrial abnormality may
be seen.
42.
43. Ventricular Septal Defect (VSD)
Therapeutic Management:
● From 20%-80% of all VSDs closed spontaneously.
1. Many small lesions do not require surgical intervention.
2. If there is aortic valve regurgitation related to VSD position near the valve
and even if the defect is small, surgery is indicated to reduce the
progression of valve insufficiency.
3. Antibiotic prophylaxis is indicated for all VSDs.
Medical Management:
1. Infants who develop CHF- digoxin diuretics, ACE inhibitors to reduce
afterload.
2. Nutritional supplements are added to infant formula to increase caloric
intake.NGT feeding or gastrostomy tube feeding for infants who are
unable to obtain adequate calories orally
3. Avoid exposure to respiratory infections.
44. Ventricular Septal Defect (VSD)
Surgical Management:
1. Pulmonary artery banding for children with multiple muscular VSDs. In this palliative
procedure, a band is placed around the main pulmonary artery, decreasing blood flow,
reducing the severity of CHF and decreasing the risk of pulmonary vascular disease.
● The current trend is to perform corrective surgery earlier in life, and consequently,
pulmonary artery banding is performed less frequently than in the past.
3. Total correction is accomplished by placing sutures to close small defects or by placing a
pericardial or prosthetic patch over moderate to large defects.
● The surgical approach is usually through the RA to avoid a right ventricular incision which
could impair the contractility of the ventricle.
● VSDs just below the pulmonary valve are closed through an incision in the main
pulmonary artery. Mortality is 5%-8%, depending on the age and type of VSD.
● Complications include residual VSDs, pulmonary hypertension in the postoperative period,
heart block that may require a pacemaker and an abnormal rhythm called junctional
ectopic tachycardia.
●CO can be significantly decreased if dysrythmias are persistent. Post pericardiotomy
syndrome can also occur.
45.
46. Complications:
• Congestive heart failure.
• Growth failure, especially in infancy.
• Bacterial endocarditis
• Irregular heartbeat or rhythm
• Pulmonary artery hypertension
47. Eisenmenger’s Syndrome
• Final common pathway for all significant LR
shunting in which unrestricted pulmonary
blood flow leads to pulmonary vaso-occlusive
disease (PVOD); RL shunting/cyanosis
devleops
• Generally need Qp:Qs >2:1
50. DEFINITION
Patent ductus arteriosus (PDA) the vessel does
not close and remains "patent" resulting in
irregular transmission of blood between two
of the most important arteries close to the
heart, the aorta and the pulmonary artery.”
51. INCIDENCE
• Patent ductus arteriosus is a common
finding in premature infants, occurring in as
many as 80% of infants born before 28 wk
gestation.
• There is a progressive decrease in frequency
of persistence of the ductus with increasing
gestational age. Persistent patency of the
ductus in term infants occurs in about 1 in
2000 live births.
52. CLINICAL MANIFESTATION
PDA is usually diagnosed after age 6 to 8 wk on the basis of a
continuous murmur at the upper left sternal border. The
peripheral pulses are full, with a widened pulse pressure.
While some cases of PDA are asymptomatic, common
symptoms include:
• tachycardia
• shortness of breath
• Cardiomegaly
• Left subclavicular thrill
• Bounding pulse
• Widened pulse pressure
53. • If the ductus is moderate or large, widened pulse
pressure and bounding peripheral pulses are
frequently present.
• Prominent suprasternal and carotid pulsations may
be noted secondary to increased left ventricular
stroke volume.
• Poor growth
• Frequent respiratory infections
54.
55. Investigations
• History
• Physical examination
• CXR:cardiomegaly,increased pul vascularity.
• ECG:Lt or biventricular hypertrophy.
• ECHO:2D visualises PDA,doppler shows turbulance.
• Cardiac catheter:PA pressures & O2 sats.
56. Physical Examination
• If the pulmonary circulation is markedly overloaded there will be
tachycardia, tachypnoea and a wide pulse pressure.
• The precordium is hyperactive and a systolic thrill may be present
at the upper left sternal border.
• The first heart sound is normal but the second is often obscured by
the murmur.
• A grade 1 to 4/6 continuous ('machinery') murmur is best audible
at the left infraclavicular area or upper left sternal border.
• In the case of a large PDA shunt, a diastolic mitral rumble may be
heard because of the high flow rate across the mitral valve.
• Patients with a small PDA do not have the above-mentioned
findings.
• Peripheral pulses are bounding as the run-off into the pulmonary
circulation drops the diastolic pressure and causes a wide pulse
pressure
58. Echocardiography
Two-dimensional echocardiogram (suprasternal view). This
image shows a large patent ductus arteriosus (arrow) that
runs above the left atrium (LA) between the aorta (Ao) and
the pulmonary artery (PA).
Frontal chest radiograph in a patient with patent ductus
arteriosus. This image shows filling in of the
aortopulmonary window (arrow).
59. ECG changes in PDA
•
Left atrial abnormality.
Left ventricular hypertrophy.
If PDA is large or if there is
aorticopulmonary window, then
pulmonary hypertension may
develop
and result in biventricular
hypertrophy pattern.
Prolongation of PR interval (rarely
seen in adults).
Deep S wave in C1 and/or tall R
waves in C5 and C6.
60. Chest X ray
• Pulmanory plethora
Enlarged Left Atrium
and Left Ventricle
Dilated aorta and
pulmonary artery
61. Mangement
• Medical Mangement
– Endocardial prophylaxis as long as patent
– Indomethacin:a prostaglandin E1 inhibitor may close a PDA.
– Administration of indomethacin(prostaglandin inhibitor) has
proved successful in closing a PDA in preterm infants and some
newborns before the age of 10 days.
Orally dose 0.2mg /kg tds at an interwal of 12 to 24 hours.
• Surgical Management :
ligation /coil/clipping/division
62.
63.
64. 4.Coarctation of Aorta
• Narrowing in proximal descending aorta usually just beyond the
origin of Left subclavian artery.
• Blood flow to the lower body maintained through collateral
vessels
• 98% of all coarctations at segment of aorta adjacent to ductus arteriosus.
• Natural hx:
– poor prognosis if unrepaired
– High BP in UA & Low BP in LA
– Systemic HypertensionLVF,Aortic Aneurysm/dissection,ICH
– murmur (continuous or systolic murmur heard in back or SEM/ejection
click of bicuspid AV)
– weak/delayed LL pulses
– Rib notching on CXR is pathognomonic
• Associated with
– Turner’s syndrome
– Subarachinoid haemorrhage
65.
66. Auscultation of Heart Sounds
• Auscultation on the
posterior thorax just
medial to the right
scapula reveals a
systolic ejection
murmur. Ec
67. Plain Radiograph
• figure of 3 sign: contour
abnormality of the aorta
• inferior rib
notching: Roesler sign
– secondary to dilated
intercostal collateral
vessels which form as a
way to bypass the
coarctation and supply the
descending aorta
– the dilated and tortuous
vessels erode the inferior
margins of the ribs,
resulting in notching
– the descending thoracic
aorta
69. MANGEMENT:Coarctation Repair
• Surgical correction
1) Patch aortoplasty
with removal of
segment and end
to end anastomosis
or subclavian flap
repair
2) bypass tube
grafting around
segment
71. Pulmonary Stenosis
• No symptoms in mild or moderately severe
lesions.
• Cyanosis and RVH, right-sided heart failure in
patients with severe lesions.
• High pitched systolic ejection murmur maximal
in second left interspace.
• Ejection click often present.
• Oligaemic lung fields(Reduced pulmonary
vascular marking)
72. Pulmonary Stenosis
• Usually associated with ASD, symptoms d/t dominant defect.
Valvular dysplasia with Noonan.
• Hypertrophy of Rt ventricle with increased RV pressure
• Arterial oxygen saturation remain normal even in severe
stenosis unless Rt to Lt shunt through ASD/VSD/PDA exists.
• Mild to moderate PS is usually asymptomatic.
• Critical pulmonary stenosis: Severe PS in neonate leading to Rt
to Lt shunting through PFO- cyanosis and CHF.
• Treatment with balloon valvuloplasty
74. • Right axis devition
• If pulmonary stenosis is
severe, the R wave
amplitude in C1 may be >20
mm (>2mV).
Right atrial
abnormality: Pulmonary
stenosis is one of the few
diseases that increase P
wave
• Right ventricular
hypertrophy pattern in
adults.
75. Bicuspid Aortic Valve/AS
• Mostly an incidental finding
• Calcification of bicuspid valve in later life
leads to stenosis/insufficiency or aortic root dilatation.
• Usually systolic murmur with opening snap
• Treatment:
- Stenosis: valvuloplasty, surgery
- Insufficiency: surgery and vasodilators
- Dilatation: ACE inhibitors
• Associated with Turner’s, Williams, CoA
76. Atrioventricular (AV) septal defect
• Antrioventricular septal
defects is characterised
by a deficiency of the
atrioventricular septum
of the heart.
77. Continued…
• Atrioventricular septal defect
accounts for about 5% of
congenital heart anomalies. An AV
septal defect may be
• Complete, with a large
(nonrestrictive) inlet ventricular
septal defect
• Transitional, with a small or
moderate-sized (restrictive)
ventricular septal defect
• Partial, with no ventricular septal
defect
• The majority of patients with the
complete form have Down
syndrome. AV septal defect is also
common among patients with
asplenia or polysplenia
(heterotaxy) syndromes.
78.
79. Symptoms and Sign
• atrioventricular septal defect
with a large left-to-right shunt
causes signs of heart
failure (HF—eg, tachypnea,
dyspnea during feeding, poor
weight gain, diaphoresis) by
age 4 to 6 wk. Pulmonary
vascular obstructive disease
(Eisenmenger syndrome) is
usually a late complication but
may occur earlier, especially in
children with Down syndrome.
81. • Chest X-ray
cardiomegaly with right atrial enlargement, biventricular
enlargement, a prominent main pulmonary artery segment,
and increased pulmonary vascular markings.
echocardiography
• Two-dimensional with color flow and Doppler studies
establishes the diagnosis and can provide important
anatomic and hemodynamic information. Cardiac
catheterization is not usually necessary unless
hemodynamics must be further characterized before
surgical repair (for example, to assess pulmonary vascular
resistance in a patient presenting at an older age).
83. ECG
• ECG shows a
superiorly directed
QRS axis (eg, left
axis deviation or
northwest axis),
frequent 1st-degree
AV block, left or
right ventricular
hypertrophy or
both, and
occasional right
atrial enlargement
and right bundle
branch block.
86. Mangement
• Surgical repair
• For heart failure,
medical therapy (eg,
diuretics, digoxin,
ACE inhibitors)
before surgery
87. Continued …
• Pulmonary artery banding
may be used, particularly
in premature infants or
those with associated
abnormalities that make
complete repair higher
risk.
• For patients with large
shunts and heart failure,
diuretics, digoxin, and
ACE inhibitors may help
to manage symptoms
before surgery.
88. Nursing management
Nursing Assessment
• Family history
• history of pregnancy
• Assessment of manifestation of CHD
• Observe and record Child’s Level of exercise tolerance
• Observe and record Child’s skin and mucus membrane
for color and temperature change
• Observe for clubbing of fingers, especially the thumb
nails with thickening and shininess of the terminal
phalanges may occur in cyanotic children by2-3 months
of age
89. Continued…
• Observe for Chest deformities /Observe for
Respiratory pattern Dyspnea on activity.
• Fatigue
• Palpate the child’s pulses in all extremities
• Auscultate the child’s heart
• Record the vital signs
• Complications or consequences of hypoxemia.
• Construction of a weak body
90. Nursing Dx
• Risk for decreased cardiac output r / t defect
structure.
• Altered Growth and Development r / t
inadequate oxygen and nutrients to the tissues.
• Risk for infection r / t poor physical status.
• Altered family processes r / t have children with
heart disease.
• Risk for injury (complications) r / t the heart
condition and therapy.
91. Slide 91
Impaired gas exchange
• Monitor intake and output
• Limit fluids as ordered
• Administer diuretics as ordered
• Position changes every 2 hours or as ordered
92. Slide 92
)
Risk for impaired growth and development
• Treat child as normally as possible
• Teach parents that children are more comfortable
when they know what to expect
• Promote age-appropriate activities as condition
allows
93. Slide 93
)Altered nutrition: less than body requirements
• Offer small, frequent feedings
• Use soft nipple for infant to ease the stress
of sucking
• NG Feedings
• Organize care to allow for rest
94. Slide 94
Continued..
Risk for infection
• Limit exposure to individuals with infections
• Promote good pulmonary hygiene
• Prophylactic antibiotics when undergoing
surgical or dental treatment to prevent
subacute bacterial endocarditis