PDA,AP WINDOW, TA,nursing, cardiovascular nursing

1. COLLEGE OF NURSING
MADRAS MEDICAL COLLEGE,
CHENNAI-03
MEDICAL SURGICAL NURSING-II
BY
EDWIN JOSE.L
MSC(N) II YEAR,
COLLEGE OF NURSING,
MADRAS MEDICAL COLLEGE,
CHENNAI

2. introduction
 Congenital heart disease is a general term for a range of birth defects that affect the normal
way the heart works.
 The term "congenital" means the condition is present from birth.
 According to the American Heart Association, about 9 of every 1,000 babies born in the
U.S. have a congenital heart defect.
 This is a problem that occurs as the baby's heart is developing during pregnancy, before the
baby is born. Congenital heart defects are the most common birth defects.
 Patent ductus arteriosus (PDA) is a congenital heart defect – a structural heart problem that
is present at birth.
 Patent ductus arteriosus is an abnormal connection between the aorta and the pulmonary
artery in the heart.
 The pulmonary artery carries blood from the heart’s right lower ventricle to the lungs, where
it is loaded up with oxygen.

3. Definition –Patent Ductus Arteriosus
Patent ductus arteriosus (PDA) is a vascular structure that connects the
proximal descending aorta to the roof of the main pulmonary artery near the
origin of the left branch pulmonary artery which is persistent after birth.
-American Heart Association
 Ductus arteriosus is essential fetal structure normally closes spontaneously
after birth.
 After the first few weeks of life, persistence of ductal patency is abnormal.
 The physiological impact and clinical significance of the PDA depend largely
on its size and the underlying cardiovascular status of the patient.
 The PDA may be “silent” (not evident clinically but diagnosed incidentally
by echocardiography done for a different reason), small, moderate, or large.

8. Embryology
 In normal cardiovascular development, the
proximal portions of the sixth pair of
embryonic aortic arches persist as the proximal
branch pulmonary arteries, and the distal
portion of the left sixth arch persists as the
ductus arteriosus, connecting the left
pulmonary artery with the left dorsal aorta.
 Normally, the distal right sixth aortic arch loses
its connection to the dorsal aorta and
degenerates.
 This transformation is completed by 8 weeks
of fetal life.

10. DUCTUS ARTERIOSUS IN FETAL LIFE
 In the normal fetal heart, blood from the right ventricle traverses the DA and
provides most of the blood flow to the lower body via the descending aorta.
 This diverts blood to the relatively low-resistance placenta and away from
the lungs, with their inherent increased vascular resistance.
 Ductal patency is maintained by the presence of a certain neurohormonal
milieu in the fetus.
 Continuous production of prostaglandin E2 within the vessel wall help
maintain patency of the DA.
 Nitric oxide also plays a role in maintaining ductal patency.
 It is also maintained both by inhibition of intracellular release of calcium
ions and by a level of insensitivity of the vascular smooth muscle cells to
calcium ions.

11. NORMAL POSTNATAL CLOSURE OF THE DUCTUS ARTERIOSUS
There are two processes involved in the postnatal closure of
the DA
functional closure
anatomic closure
Functional closure is normally initiated by the constriction of spiral
muscles within the medial muscle layer.
During functional closure, there is shortening and thickening of the
ductal wall along with disruption of the intimal layer which results
in the formation of intimal cushion.
A subsequent process of migration of smooth muscle into the
subintimal layer results in hemorrhage and necrosis.

12. Cont……
Initial closure occurs at the pulmonary end and extends toward the
aortic end.
Patency of the conical aortic end may persist for weeks after
closure.
Functionally complete closure occurs within 24–48 hours after birth
in the full-term
Anatomical closure - During the next 2–4 weeks, involution of the
endothelium as well as changes in the subintimal layer (that is,
structural/anatomic closure) occurs that is a fibrous band called the
ligamentum arteriosum.

13. Incidence of Pda
 Patent ductus arteriosus (PDA) occurs in approximately 20-50% of neonates
born before 32 weeks gestation and in up to 60% of neonates born before 29
weeks gestation
 1 in every 2,500 to 5000 infants
 The incidence of an isolated PDA in term infants ranges from 0.03 to 0.08
percent
 There is a 2:1 female to male predominance
 Increased incidence in infants born at high altitude compared to sea level
 Increased incidence in infants with congenital rubella
 Having a sibling with a PDA increases the chance of having a PDA by 2-4%

14. TYPES OF PDA
There are five types of patent ductus arteriosus determined by angiographic
appearance (Krichenko classification):
1. Type A (conical): prominent aortic ampulla with a constricted pulmonary
end
2. Type B (window): large width with a very short length
3. Type C (tubular): long and without any evidence of constriction
4. Type D (complex): complicated course with potentially multiple areas of
constriction
5. Type E (elongated): extended length with a more remote constriction

16. Risk factors

17. Causes of pda
Prematurity
 PDA is most prevalent in premature neonates, probably as a result of
abnormalities in oxygenation.
Prostaglandin E
 The relaxant action of prostaglandin E prevents ductal spasm and contracture
necessary for closure.
Other congenital defects
 PDA commonly accompanies rubella syndrome and may be associated with
other congenital defects, such as coarctation of the aorta, ventricular septal
defect, and pulmonary and aortic stenoses.

18. PATHOPHYSIOLOGY OF PATENT DUCTUS ARTERIOSUS
PRETERM
IMMATURE AD
Immature expression
of o2 in kv channels
Activity of cAMP PDEs Activity of cAMP PDEs
Sensitivity of oxygen
O2 induced
contration of smooth
muscle
Degradation of cAMP
SENSITIVITY OF PGEs
Degradation of cAMP
Relaxation of vascular
smooth muscle
Sensitivity of NO
PDA

19. CLINICAL MANIFESTATIONS
 Degree of left to right shunt, which depends on the size and length
of the PDA
 The difference between pulmonary and systemic vascular resistance
CLINICAL MANIFESTATIONS:
 MURMUR - medium pitched high-grade continuous murmur heard
best at the pulmonic position, with a harsh machine like quality that often
radiates to the left clavicle.
 The first heart sound is normal but the second heart sound is obscured by
a continuous crescendo-decrescendo murmur, which runs from the start
of systole to the end of diastole.

20. CONT…..
Respiratory distress
Heart failure
Low immune system
Slow motor development
Physical underdevelopment
Bounding peripheral pulses
Widened pulse pressure

21. DIAGNOSTIC EVALUATION
 History collection – family history, maternal infections, Prematurity,
perinatal distress, and hypoxia
 Physical examinations:
 Patients usually appear well and have normal respirations and heart rates
 A widened pulse pressure
 Suprasternal or carotid pulsations may be prominent
 tachypnea
 Tachycardia
 The apical impulse is laterally displaced; a thrill may be present in the
suprasternal notch or in the left infraclavicular region

22.  The first heart sound (S1) is typically normal, and the second heart sound
(S2) is often obscured by the murmur
 paradoxical splitting of S2 related to premature closure of the pulmonary
valve and a prolonged ejection period across the aortic valve
 The murmur may be only a systolic ejection murmur, or it may be a
crescendo/decrescendo systolic murmur that extends into diastole
 Occasionally, auscultation of the patent ductus arteriosus (PDA) reveals
numerous clicks or noises resembling shaking dice or a bag of rocks

23. CONT…..
 Pulse oximetry/ABG
 usually demonstrate normal saturation because of pulmonary over circulation.
 A large ductus arteriosus could cause hypercarbia and hypoxemia from congestive
heart failure (CHF) and air space disease (atelectasis or intra-alveolar fluid/pulmonary
edema).
 CHEST X –RAY:
 range from normal to those consistent for congestive heart failure (CHF).
 Cardiomegaly may be present with or without CHF.
 the pulmonary arteries, pulmonary veins, left atrium, and left ventricle are enlarged on
chest films.
 the ascending aorta may be prominent.
 peripheral pulmonary vascular markings and increased pulmonary venous markings
may be noted

24. Marked cardiomegaly with dilatation of the main pulmonary artery. Bilateral pulmonary
plethora.

25.  Electrocardiography
 With a small patent ductus arteriosus (PDA), the electrocardiographic
(ECG) findings are typically normal.
 Left ventricular hypertrophy may be present with a larger PDA.
 Left atrial enlargement may also be present with large shunts.
 Doppler Echocardiography
 High velocity jets of turbulent flow in the pulmonary artery

26. Complications of pda
Pulmonary hypertension
Congestive cardiac failure
Infective endarteritis
Aneurismal dilatation of the pulmonary Artery
Calcification of the ductus
Non infective thrombosis
Pulmonary or systemic emboli
Paradoxical emboli

27. MANAGEMENT
Pharmacological therapy:
 Prostaglandin analogs. The ductus arteriosus can be induced to remain open
by administering prostaglandin analogs such as alprostadil (a prostaglandin
E1 analog).
 Indomethacin. Indomethacin is a prostaglandin inhibitor that’s an alternative
to surgery in premature neonates and induces ductus spasm and closure.
 Ibuprofen - Prophylactic ibuprofen is also widely used. The dose used for
ibuprofen is 10 mg/kg bolus followed by 5 mg/kg/d for 2 additional days.
 Antibiotics – because of the risk of bacterial endocarditis associated with
the open structure.
 Diuretic agents
 digoxin

28. Surgical management
Cardiac Catheterization
The use of the percutaneous route to close the patent ductus arteriosus
(PDA) is becoming more common. Transcatheter occlusion is an effective alternative
to surgical intervention and is becoming the treatment of choice for most cases of
patent ductus arteriosus (PDA) in children and adults.
 Gianturco spring occluding coils
 Amplatzer duct occluder
 Rashkind ductus occlusion device

30. SURGICAL LIGATION
SURGICAL CLIPPING LIGATION

31. AORTOPULMONARY WINDOW

32. INTRODUCTION
 Aortopulmonary window (APW) is a rare congenital heart defect.
 It occurs as an isolated cardiac lesion or in association with other cardiac
anomalies and rarely with abnormal coronary arteries.
 Aortopulmonary window (APW) was first described in the third decade of
last century by Elliotson .
 It accounts for (0.2% - 0.6%) of all congenital heart lesions

33. DEFINITION- AP WINDOW
Aortopulmonary window is a rare congenital heart disease
characterized by a communication between the ascending aorta and
the pulmonary artery in the presence of two separate semilunar
valves arising from separate sub arterial ventricular outflow tracts.
-American thoracic society

37. EMBRYOLOGY
The aortopulmonary septum develops as a wedge of tissue (capped
by neural crest cells) that grows ventrally from the dorsal wall of
the aortic sac between the origins of the fourth and sixth aortic
arches.
This structure fuses with the distal margins of the embryonic
outflow cushions (also capped by neural crest cells) to close the
embryonic aortopulmonary foramen.
Failure to close this foramen by fusion of the neural crest cells
results in an aortopulmonary window.

38. Classification of ap window
Type I
Type II
Type III

39. Type I
 It is also called as proximal defects which occur in the proximal part of the
aortopulmonary septum
 This defect is more proximally located between the origin of the main pulmonary artery
and the ascending aorta immediately above the sinus of Valsalva with little inferior rim
separating the AP window from the semilunar valves.
 These defects tend to be large, round or oval shaped, and are more often seen.

40. TYPE II
 It is also called as distal defects which occur in the distal part of the
aortopulmonary septum adjacent to the right pulmonary artery
 This defect is more distal, between the ascending aorta and the origin of the
right pulmonary artery
 These defects are more rare and tend to be smaller in size.

41. Type III
 It is a combination of types I and II.
 Consists of extension of the defect into the right pulmonary artery with
anomalous origin of the right pulmonary artery from the ascending aorta
with a well-formed inferior rim but little superior rim and involves the
majority of the ascending aorta.
 This type is more frequently linked with other cardiac anomalies

42. INCIDENCE
 Aortopulmonary window is a very rare defect accounting for 0.2% to 0.6% of
all congenital malformation
 There is a male preponderance.
 No specific genetic abnormalities have correlations with AP window, although
Berry syndrome, a combination of AP window, interrupted aortic arch, and
right pulmonary artery originating from the aorta is a defined entity.

43. ASSOCIATED CONDITIONS
This condition can develop by itself or in conjunction with other cardiac
conditions such as
1. Tetralogy of Fallot
2. Pulmonary atresia
3. Truncus arteriosus
4. Atrial septal defect
5. Patent ductus arteriosus
6. Interrupted aortic arch

44. pathophysiology

45. CLINICAL MANIFESTATIONS
The clinical features of APW are not specific, but majority of
patients have the manifestations of a large left to right shunt.
Patients with small defects may be asymptomatic.
Patients with large APW usually have symptoms of pulmonary
hypertension and congestive heart failure (tachypnea, diaphoresis,
failure to thrive, and recurrent respiratory difficulty) in the first
weeks of life.
Severe pulmonary vascular hypertension can occur in the first
months of life.

46. Cont….
 Tachypnea
 Diaphoresis
 Poor feeding
 Poor growth
 Delayed growth
 Rapid heartbeat
 Respiratory infections
 Minimal cyanosis present
 Heart failure symptoms generally emerge in early childhood,
 The cardiac murmur is usually systolic with a mid-diastolic rumble as a result of increased
blood flow over the mitral valve.

47. Diagnostic evaluvation
 Echocardiography – left or biventricular hypertropy
 X-Rays – prominence of pulmonary artery and intrapulmonary
vasculature
 Computerized tomography
 electrocardiogram - left and right ventricular hypertrophy

49. management
Initial management:
Medical therapy is focused on preoperative stabilization.
It will retrogress spontaneously during follow up in majority of
cases.
The only effective therapy for aortopulmonary window is surgical
repair (APW).
 However there are reports of transcatheter occlusion of simple
APW.

50. First line treatment
 Intravenous prostaglandins (e.g., alprostadil) may be required in persons with
an interrupted aortic arch to maintain the ductus arteriosus open and enable
blood flow to the lower half of the body.
 The increased pulmonary blood flow may be exacerbated by the
accompanying pulmonary arterial vasodilation.
 Digoxin and furosemide are widely used to treat heart failure and volume
overload associated by this lesion.
 Inotropic drugs (for example, dopamine and dobutamine) may also be an
effective therapy for babies with severe heart failure and low cardiac output
due to myocardial dysfunction.

51. Surgical management
 Aortopulmonary window is generally treated with surgery.
 Surgery should be done as soon as feasible after initial stabilization and
correction of acidosis.
 Surgery is performed with the use of cardiopulmonary bypass.
 The aortopulmonary window, the major pulmonary artery, or the anterior
portion of the aorta can all be incised.
 Associated lesions are generally treated at the same time as the primary lesion.
 In patients with associated lesions, more complicated repairs and myocardial
protection methods are necessary, increasing the morbidity and mortality
associated with the surgery
 Transverse aortotomy – an autologous pericardial patch was used

52. Truncus arteriosus

53. introduction
 Truncus arteriosus (TA) is a rare form of congenital heart disease occurring in
1-3% of patients with congenital heart disease.
 During fetal development, the embryonic truncus arteriosus gives rise to the
aorta and the pulmonary trunk.
 Persistent truncus arteriosus results from incomplete or failed septation.
 It is characterized by a single great artery arising from the heart with a single
semilunar valve that overrides the right and left ventricles.
 The common trunk gives rise to the pulmonary arteries, providing systemic,
pulmonary and coronary perfusion.

54. definition
Truncus arteriosus (TA) is an uncommon congenital
cardiovascular anomaly that is characterized by a single arterial trunk
arising from the normally formed ventricles by means of a single
semilunar valve .
Truncus arteriosus (TA) is a rare, congenital, cyanotic heart
defect characterized by a ventricular septal defect (VSD), a single
truncal valve, and a common ventricular outflow tract (OT).

55. EMBRYOLOGY
During fetal development, persistent truncus arteriosus represents an
arrest in the normal embryological separation of the anterior
pulmonary artery and posterior aorta, resulting in a single great
artery.
Because these neural crest cells are known to play a role in the
normal formation of the pulmonary artery and aorta, experimental
ablation of these cells results in the specific congenital heart defect
of persistent truncus arteriosus.
In addition, since neural crest cells contribute to thymus and
parathyroid development, there is an association between truncus
arteriosus and DiGeorge syndrome

56. CLASSIFICATION
The Collett and Edwards system is the earliest form of classification,
developed in 1949. It is based on where pulmonary arteries arise from the
common trunk.
 Type I: The main pulmonary is present and bifurcates into the left and right
pulmonary arteries
 Type II: the right and left branches arise adjacent to each other from the
posterolateral segment of the common trunk
 Type III: The right and left branches originate separately from the right and
left lateral segments of the common trunk.
 Type IV: Neither of the branches arise from the common trunk, but are
perfused by aortopulmonary collaterals. This type is now categorized as a
form of pulmonary atresia with a ventricular septal defect rather than TA.

58. The Van Praagh classification system is based on where the branch pulmonary
arteries arise from the trunk as well as the development of the aortic arch and the
presence of a patent ductus arteriosus (PDA). Each type may include a modifier
“A” (with VSD) of “B” (without VSD).
 Type A1: The main pulmonary is present and bifurcates into the left and right
pulmonary arteries (same as Collette and Edwards classification).
 Type A2: The right and left branch pulmonary arteries arise from the common
trunk.
 Type A3: One branch pulmonary artery (typically the right) arises from the
common trunk and the other arises from a PDA or the aorta.
 Type A4: This type is defined by presence of aortic arch hypoplasia,
coarctation or interrupted aortic arch and a large PDA.

60. ASSOCIATED CONDITIONS
Ventricular Septal Defect- A VSD is present in the vast majority of
cases. It is usually large and cono ventricular.
Aortic arch anomalies- Approximately 30% of patients with TA have
a right aortic arch and 12% have aortic arch hypoplasia or an
interrupted aortic arch.
Truncal valve anomalies- The leaflets are usually thickened. The
valve is most commonly tricuspid, but may also be bicuspid, quadri
cuspid or penta cuspid (rare) valve.
Coronary artery anomalies- atypical origin, single coronary, or
narrowed ostia resulting in coronary stenosis

61. ETIOLOGY
 Unknown cause
 chromosomal and genetic abnormalities- including duplication of
chromosome arm 8q and mutation of the NKX2.6 and GATA6 genes.
 Genes -including Tbx20, ALK2, Cited2, and Semaphorin 3c,
 children of mothers with significant diabetes mellitus during pregnancy had
an increased incidence of truncus arteriosus
 teratogens - eg, retinoic acid, bis-diamine

62. PATHOPHYSIOLOGY
 The VSD allows oxygenated and deoxygenated blood to mix before it is
ejected through a common truncal valve to a single great artery,
subsequently supplying the coronary, pulmonary and systemic circulations.
 The common semilunar valve may have 1 to 4 cusps with tricuspid most
frequently seen.
 The presence of a single arterial trunk can be associated with several
cardiac, aortic, and pulmonary abnormalities.
 These abnormalities include right-sided, interrupted, or hypoplastic aortic
arches, abnormal origins of the coronary arteries, pulmonary artery stenosis,
and patent ductus arteriosus.

63. CLINICAL MANIFESTATION
 rapid breathing (tachypnea)
 lethargy
 Cyanosis
 poor feeding,
 difficulty breathing (dyspnea)
 broadening of the fingertips (clubbing).
 abnormal accumulations of fluid in the face, arms, and/or legs (edema),
 an abnormally rapid heartbeat,
 slow weight gain,
 failure to thrive,
 recurrent respiratory infections,
 poor physical development,
 growth delays.

64. Diagnostic evaluation
History collection
Physical examination
Arterial blood gas analysis- to determine the degree of
acidosis
Serum electrolytes
ECG- Biventricular hypertrophy, A normal sinus rhythm,
normal intervals, and either a normal QRS axis or minimal right-
axis deviation are generally observed. Biventricular hypertrophy
is a characteristic finding.

65. X ray chest- show moderate cardiomegaly with pulmonary
plethora (mainly as a result of collateral formation) and widened
mediastinum.
Echocradiography- Allows direct visualization of a single trunk.
Outflow tract views are the most useful. Color Doppler may
additionally show flow across both ways through an associated
VSD
Magnatic resonance imaging- Allows direct display of anomalous
anatomy. SSFP cine sequences can offer an additional functional
assessment.

66. management
Medical management:
 Intravenous prostaglandin
 Ionotropic agents e.g. Dopamine - Stimulates adrenergic and dopaminergic
receptors
 Diuretic agents e.g. frusemide - Increases excretion of water by interfering
with chloride-binding cotransport system
 Cardiac glycoside, antiarrhythmic e.g. digoxin - Acts directly on cardiac
muscle, increasing myocardial systolic contractions.
 ACE inhibitor e.g. captopril - Inhibits activity of the angiotensin-converting
enzyme, preventing conversion of angiotensin I to angiotensin II, which is a
potent vasoconstrictor.

67. Surgical management
 The Rastelli procedure involves creating a “baffle” to close the ventricular
septal defect (VSD), separating the right & left ventricles.
 The baffle directs blood flow from the left ventricle to the aorta.
 During this surgery, a right ventricle to pulmonary artery (RV-PA) conduit is
also placed to supply blood flow to the lungs
 During surgery, a median sternotomy (incision through the middle of the
chest) is done. The patient is placed on cardiopulmonary bypass (heart–lung
machine).

68. Cont…..
 Depending on the patient’s anatomy and surgical plan, an incision is made on
either on the right atrium or right ventricle to view the VSD.
 A Dacron patch is cut to the appropriate size.
 The patch is then sewn over the VSD to “baffle” blood flow from the left
ventricle to the aorta.
 Once this is completed, if not already done, incisions are made on the
pulmonary artery and right ventricle.
 An appropriate sized right ventricle-to -pulmonary artery conduit is selected.
 One end of the conduit is sewn onto the incision on the pulmonary artery and
the other end is sewn onto the incision on the right ventricle.

70. Nursing management
Nursing Assessment
Assessment should focus on:
 Activity and rest. The nurse should assess for weakness, fatigue, dizziness,
a sense of pulsing, and even sleep disorders.
 Circulation. Circulatory assessment should include history trigger
conditions, history of heart murmurs and palpitations, BP, and pulse
pressure.
 Food and fluids. The nurse should assess for dysphagia and changes in
body weight

71. Nursing Diagnosis
 Decreased Cardiac Output related to Structural factors of congenital heart defect
 Compromised Family Coping related to Situational and developmental crises of family
and child
 Risk for Injury related to Cardiac function compromised by congenital defects
and medication administration
 Risk for Infection related to Chronic illness
 Activity intolerance related to imbalance between oxygen consumption of the body and
supply of oxygen to the cells.
 Anxiety related to hospital care or lack of support system.
 Deficient knowledge related to the condition and treatment needs

72. Nursing Care Planning & Goals:
 Maintain adequate cardiac output.
 Reduce the increase in pulmonary vascular resistance.
 Maintain adequate levels of activity.
 Provide support for growth and development.
 Maintain appropriate weight and height development.

73. Nursing Interventions
Signs and symptoms
Monitoring
Adverse effects of indomethacin
Preoperative instructions
Postoperative procedures

74. Discharge and home care instruction
Instructions. Review instructions with parents about activity
restrictions based on the child’s tolerance and energy levels.
Activities. Advise the parents not to be overprotective as the
child’s tolerance for physical activity increases.
Follow-up checkups. Stress the need for regular follow-up
examinations.
History. Advise parents to inform any practitioner who treats his
child about his history of surgery for PDA-even if the child is
treated for an unrelated medical problem.

75. conclusion
 Congenital Heart Defects are very common in our setup and
early detection of CHD is increasing. Overall burden of CHD is
also increasing therefore a proper population based study on a
large scale is needed to estimate the prevalence accurately.