Congenital heart diseases are serious, common birth defects that affect approximately 8 in 1,000 live births in the United States. The most common types seen in children are ventricular septal defects, atrial septal defects, and patent ductus arteriosus. Treatment options depend on the type and severity of the defect, ranging from observation for mild defects to surgical repair or replacement of heart valves. Prognosis has improved due to advances in surgical techniques and post-operative care.

1. Neonatal heart diseases
DRISYA.V.R.
2nd year MSc Nursing

4. CONGENITAL HEART DISEASES
Incidence
• Congenital heart defects are serious and common
conditions that have significant impact on morbidity,
mortality, and healthcare costs in children and adults.
• The most commonly reported incidence of congenital heart
defects in the United States is between 4 and 10 per 1,000,
clustering around 8 per 1,000 live births.

5. • Continental variations in birth prevalence have been
reported, from 6.9 per 1000 births in Europe to 9.3 per
1000 in Asia.
• An estimated minimum of 32,000 infants are expected
to be affected each year in the United States. Of these,
an approximate 25%, or 2.4 per 1,000 live births,
require invasive treatment in the first year of life.

6. Prevalence
• It is estimated that the total number of adults living
with congenital heart disease in the United States in
2000 was 800,000.
• In the United States, 1 in 150 adults are expected to
have some form of congenital heart disease.
• The most common types of defects in children are
(at a minimum) ventricular septal defects,620,000
people; ASD, 235,000 people; valvular pulmonary
stenosis, 185,000 people; and patent ductus
arteriosus, 173,000 people. The most common
lesions seen in adults are ASD and TOF.

7. Mortality
• Mortality related to congenital cardiovascular defects
in 2011 was 3,189. Any-mention mortality related to
congenital cardiovascular defects in 2009 was 5,051.
• In 2011, congenital cardiovascular defects were the
most common cause of infant death resulting from
birth defects; 26.6% of infants who died of a birth
defect had a heart defect.
• The mortality rate attributable to congenital heart
defects in the United States has continued to decline
from 1979 to 1997 and from 1999 to 2006.

8. • The 2011 death rate attributable to congenital
cardiovascular defects was 1.0. Death rates
were 1.1 for white males, 1.4 for black males,
0.9 for white females, and 1.2 for black
females. Infant mortality rates (<1 year of age)
were 31.4 for white infants and 42.2 for black
infants.

9. CAUSES
• Genetic
• Environmental
• Maternal obesity

10. CLASSIFICATION
• Based on lesion severity.
 Severe
 Moderate
 Mild

11. CLINICAL CLASSIFICATION
Congenital
Heart Diseases
Acyanotic
congenital
heart disease
Cyanotic
congenital
heart disease

12. Acyanotic
congenital heart
disease
Left-to-right
shunts
Left-sided
obstructive
lesions
Cyanotic
congenital heart
disease
Right-to-left
shunts
Complete
mixing
lesions

13. Left-to-
right
shunts
Atrial septal
defects
Ventricular
septal defects
Atrioventricu
lar septal
defects
Aortopulmo
nary
window
Patent ductus
arteriosus

14. Left-sided
obstructive
lesions
Coarctation
of aorta
Congenital
aortic
stenosis
Interrupted
aortic arch
Mitral
stenosis

15. ATRIAL SEPTAL DEFECT
An atrial septal defect (ASD) is a communication
or opening between the atria that result in
shunting of blood between the two chambers.

16. TYPES
• Ostium Secundum ( 75% )
• Ostium Primum ( 15 – 20% )
• Sinus venosus ( 5 – 10% )
• Coronary sinus ASDs

18. Signs and symptoms
• Fatigue (tiredness)
• Tiring easily during physical activity
• Shortness of breath
• A buildup of blood and fluid in the lungs
• A buildup of fluid in the feet, ankles, and legs
• Sweating
• Rapid breathing
• Poor growth

19. Physical Exam Findings
• Wide, fixed splitting of S2 (delayed closure of
pulmonic valve with reduced respiratory variation)
• Midsystolic pulmonary flow or ejection murmur
– Usually over 2nd intercostal space
– Peaks in early-to-mid systole, ends before S2
• Palpable RV heave
• Mid-diastolic murmurs are the result of augmented
tricuspid flow.

20. Pregnancy and ASD
• Despite the gestational increase in cardiac output and
stroke volume, young gravida with an atrial septal
defect generally endure pregnancy, even multiple
pregnancies, without tangible ill effects.
• However, brisk hemorrhage during delivery provokes a
rise in systemic vascular resistance and a fall in
systemic venous return, a combination that augments
the left-to-right shunt, sometimes appreciably.
• There is also a peripartum risk of paradoxical
embolization from leg veins or pelvic veins because
emboli carried by the inferior vena cava traverse the
atrial septal defect and enter the systemic circulation.

26. Treatment
• No medical treatment
• Surgical closure

27. Closure of ASD
Indication
• American and European practice guidelines86, 87
state that an atrial septal defect can be closed if
the pulmonary vascular resistance is lower than
two-thirds of the systemic vascular resistance (at
baseline or after pulmonary vasodilator acute
challenge or targeted pretreatment course) and
there is evidence of a pulmonary-to-systemic
flow ratio greater than 1·5 (class IIB, level of
evidence C)

28. • 1.) Symptoms
– Exercise intolerance, fatigue, dyspnea, heart failure
– Atrial tachyarrhythmias
• Occur in 20% and often the presenting symptom
• Not an indication by itself (incidence may not be
reduced after surgery).

29. • 2.) Defect Size and Qp/Qs
– Larger ASDs impose a greater hemodynamic burden
on the RV.
– In the absence of pulmonary hypertension, Qp/Qs is
closely correlated with the size of the ASD.
– Qp/Qs > 2:1 is a well-established indication, though
many authors advocate 1.7:1 or even 1.5:1.
– AHA recommends a threshold Qp/Qs ≥ 1.5:1, but
these guidelines exclude patients > 21 years of age.
– Canadian Cardiac Society recommends Qp/Qs >2:1, or
>1.5:1 in the presence of reversible pulmonary
hypertension.

30. Contraindication
• American and European practice guidelines:
PVR> 8 Woods units precludes closure and
Eisenmenger syndrome
• Pop-off valve: COPD with cor pulmonale
• Balloon occlusion :rise in PAP

31. Timing of surgery
• Ideally: 3-5 years
• Usually before 25 years
• Elderly: Improves morbidity and mortality

32. Median sternotomy with direct closure of small
to moderate defect
- Larger defects closed with autologous
pericardium or syntethic patches like
polyester polymer
( Dacron )or polytetrafluoroethylene ( PTFE )

33. • Minimally invasive techniques with hemisternotomy
and limited thoracotomy is to improve cosmetic
outcome
• Percutaneous Transcatheter Closure
- via femoral vein
- success is as good as 96% in good hands

34. Percutaneous Closure
• Amplatzer Occlusion Device
• Consists of two round disks made of Nitinol
(nickel + titanium) wire mesh linked together
by a short connecting waist.

36. VENTRICULAR SEPTAL DEFECT
• A ventricular septal defect (VSD) is a
communication (or multiple communications)
between the right and left ventricles.

37. Types
Based on anatomic types:
• Perimembraneous
• Sub pulmonary
• Muscular
• Atrioventricular canal

38. Based on Lesion Size
• Restrictive VSD
– < 0.5 cm2 (Smaller than Ao valve orifice area)
– Small Left to Right shunt
– Normal RV output
– 75% spontaneously close < 2yrs
• Non-restrictive VSD
– > 1.0 cm2 (Equal to or greater than to Ao valve orifice area)
– Equal RV and LV pressures
– Large hemodynamically significant L to R shunt
– Rarely close spontaneously

39. Based on size
1) Large
2) Moderate
3) Small

44. CHEST X-RAY
• Cardiomegaly : proportional to the volume overload.
• Mainly LV, LA and RV enlargement.
• Increased pulmonary blood flow, PAH.
• Unless LA is significantly enlarged its difficult to
differentiate from ASD.
• RV may not be as enlarged as anticipated as it
receives the shunt into its outflow tract.

45. Management
• Observation & follow up
Small VSDs
• Medical management
Medium sized VSD
CCF- treat with diuretics & digitalis, ACEI
failure precipitated by LRTI- Treat both
2-3 months follow up
RV & PA pressures assessed
Failure to thrive
• Surgical
Large VSD

46. • drugs
digoxin 10-20mcg/kg per day
furosemide 1–3 mg/kg per day
captopril 0.5–2 mg/kg per day
enalapril 0.1mg/kg per day

47. Indications of surgical intervention
• Large VSD with pulmonary hypertension
• VSD with aortic regurgitation
• VSD with associated defects
• Failure of congestive cardiac failure to
respond to medications

49. AORTO PULMONARY WINDOW
• Aorto-Pulmonary window is an opening
between the ascending aorta and the main
pulmonary artery. There must be two distinct
and separate semilunar valves before this
diagnosis can be made.

50. Types
• Type I
• Type II
• Type III

54. PATENT DUCTUS ARTERIOSUS
• The ductus arteriosus is a communication
between the pulmonary artery and the aortic
arch distal to the left subclavian artery. Patent
ductus arteriosus (PDA) is the failure of the
fetal ductus arteriosus to close after birth.
• In normal term infants it closes spontaneously
at 3-5days.

65. COARCTATION OF AORTA
• Coarctation of the aorta is a narrowing in the aortic arch.
• A congenital narrowing of upper descending thoracic aorta
adjacent to the site of attachment of ductus arteriosus
A congenital narrowing of upper descending thoracic
aorta
adjacent to the site of attachment of ductus arteriosus

67. CLINICAL FEATURES
INFANT
• Depends on patency of PDA
• Shock and HF
• Metabolic disturbances
• Hypothermia
• Hypoglycemia
• Hypo perfusion
• Renal failure

68. Child
• Upper extremity HTN
• Widened pulse pressure
• Variability in right and left arm pressures
• Murmurs
•

69. Others
–Intermittent claudication (due to a
temporary inadequate supply of
oxygen to the muscles of the leg)
–Pain and weakness of legs and
–Dyspnea on running

70. INVESTIGATION
• X RAY – rib notching ,3 sign , cardiomegaly

71. ECHO
• High parasternal, suprasternal long axis
• Shelf within lumen of thoracic aorta
• Color and pulse wave doppler to locate area
• Continuous wave doppler to detect maximum
flow velocity

72. • ECG
• MRI
• BARIUM SWALLOW
• CARDIAC CATHETERISATION

75. Complete mixing
lesions
Transposition of the great vessels
Double-outlet right ventricle (DORV)
Total anomalous pulmonary venous
connection
Truncus arteriosus
Hypoplastic left heart syndrome

77. AORTIC STENOSIS
• Aortic Stenosis (AS) is a narrowing that
obstructs blood flow from the left ventricle,
leading to left ventricular hypertrophy and/or
aortic insufficiency.

81. Types
• Valvar
• Subaortic
• Supravalvar

84. INTERRUPTED AORTIC ARCH
• Interrupted Aortic Arch (IAA) refers to the
congenital absence of a portion of the aortic
arch. There are three types of IAA, and they
are labeled according to the site of the
interruption.

85. Types
• Type A
• Type B
• Type C

86. Right-to-leftshunts
Tetralogy of Fallot
Pulmonary
stenosis
Pulmonary atresia
Tricuspid atresia
Ebstein’s anomaly

88. MANAGEMENT
• Prostaglandin
• Surgical repair

91. TETROLOGY OF FALLOT
• Tetrology of Fallot (TOF) is a congenital heart
defect characterized by the association of four
cardiac abnormalities; VSD, pulmonary
stenosis, overriding of aorta and right
ventricular hypertrophy.

111. PULMONARY STENOSIS
• Pulmonary stenosis (PS) is a narrowing that
obstructs blood flow from the right ventricle.
It may be subvalvular, valvular, supravalvular
or in the pulmonary arteries

114. CLINICAL FEATURES
• Usually asymptomatic and in normal health.
• Heart murmur
• Cyanosis
• In an older child, severe pulmonary valve
stenosis may cause easy fatigue or shortness
of breath with physical exertion. Severe
pulmonary valve stenosis rarely results in right
ventricular failure or sudden death.

115. DIAGNOSIS
• Presence of heart murmur.
• The heart murmur of pulmonary stenosis is a turbulent noise
caused by ejection of blood through the obstructed valve.
• There is often an associated click sound when the thickened
valve snaps to its open position. These sounds can be
detected through careful examination of the heart by a
physician well-trained in cardiac diagnosis.
• The electrocardiogram is typically normal in the presence of
mild pulmonary stenosis.
• The echocardiogram is the most important non-invasive test
to detect and evaluate pulmonary valve stenosis.
• Cardiac catheterization is an invasive technique that enables
physicians to accurately measure the degree of pulmonary
stenosis

116. TREATMENT
MILD PULMONARY STENOSIS: Requires no
surgical intervention. These infants and children
are examined by cardiologists at regular
intervals for signs of progression of the stenosis.

117. MODERATE TO SEVERE PULMONARY STENOSIS
• Pulmonary Balloon Valvuloplasty
• Surgical Valvotomy

118. PULMONARY ATRESIA
• Pulmonary Atresia with an Intact Ventricular
Septum (PA with IVS) includes a spectrum of
defects that can include disorders of the
tricuspid valve, right ventricle and coronary
circulation. The term ‘atresia’ indicates failure
of the pulmonary valve or pulmonary trunk
vessel to develop; therefore there is no
connection between the right ventricle and
pulmonary artery.

122. Management
• Prostaglandin infusion
• Oxygen
• maintenance of acid-base balance

123. Surgery
• Palliative
• Corrective

124. TRICUSPID ATRESIA
• Tricuspid atresia is the failure of development
of the tricuspid valve, resulting in a lack of
direct communication between the right
atrium and the right ventricle.
• Incidence : 0.06 per 1000 live births

126. TYPES
• MUSCULAR
• MEMBRANEOUS
• VALVAR

127. Surgery
• Palliative
• Reconstructive

128. EBSTEIN’S ANOMALY
• Ebstein’s anomaly is a rare congenital defect
of the tricuspid valve. The tricuspid valve
leaflets do not attach normally to the tricuspid
valve annulus. The leaflets are dysplastic and
the septal and posterior leaflets are
downwardly displaced, adhering to the right
ventricular septum.

130. PATHOPHYSIOLOGY
• ATRESIA OF TRICUSPID VALVE
• No communication between RA AND RV
• RV id underdeveloped.
• Systemic venous blood received by RA
• Enters LA through PFO or ASD

131. • Mixing of systemic and pulmonary blood
• Enters LV
• Blood enters RV through VSD
• From RV blood enters Pulm trunk

132. • Blood enters pulm trunk via PDA
• Increased pulmonary blood flow
• LA and LV hypertrophy
CHF

133. CLINICAL FEATURES
DECREASED PULM FLOW 90%
• severe cyanosis, hypoxemia, and acidosis
• LV apical impulse
• Waves in jugular venous pulse
• pulmonary oligemia
• may have central cyanosis,
• tachypnea or hyperpnea

134. INCREASED PULM FLOW
• may not appear cyanotic but may present with
signs of heart failure later in infancy
• pulmonary plethora present with symptoms of
dyspnea, fatigue, difficulty feeding, and
perspiration, which are suggestive of
congestive heart failure.
• Cyanosis is minimal

135. Other features
• holosystolic type of murmur at the lower
sternal border, suggestive of VSD,
• Problems related to chronic cyanosis, such as
• clubbing,
• polycythemia, relative anemia,
• stroke, brain abscess,
• coagulation abnormalities,

136. INVESTIGATIONS
• History and PE
• Pulse oximetry
• ABG
• Hb and hematocrit

137. MANAGEMENT
• an intravenous infusion of PGE1
• 0.03-0.1 mcg/kg/min to open the ductus
arteriosus
• anticongestive therapy with digoxin, diuretics

138. Surgery
• Palliative
• Tricuspid Valvuoplasty

139. TRANSPOSITION OF GREAT ARTERIES
•

151. Surgery
• Rashkind Procedure (Palliative)
• Arterial Switch Procedure (Corrective)

155. DOUBLE OUTLET RIGHT VENTRICLE
• Double Outlet Right Ventricle (DORV) spans a
wide spectrum of physiology from Tetrology of
Fallot to Transposition of the Great Arteries.
DORV is a complex cardiac defect where both
great vessels (aorta and pulmonary artery),
either completely or nearly completely arise
from the right ventricle

158. Types
1. a. DORV with subaortic VSD and without PS
1. b. DORV with subaortic VSD and pulmonary
stenosis
2. DORV with subpulmonic VSD (Taussig-Bing
anomaly)
3. DORV with doubly committed VSD
4. DORV with remote VSD

159. TREATMENT

161. TOTAL ANOMALOUS PULMONARY
VENOUS RETURN
• Total Anomalous Pulmonary Venous Return (T-
APVR) results from the failure of the
pulmonary veins to join normally to the left
atrium during fetal cardiopulmonary
development.

162. Incidence
• TAPVC occurs in approximately 4-6 per
100,000 live births.
• This anomaly accounts for 1-3 percent of the
cases of congenital heart disease

163. Types
• Supracardiac
• Cardiac
• Infradiaphramatic
• Mixed

168. INVESTIGATIONS
• Chest X-ray –
• Figure of 8 or snow-man appearance
• Ground-glass appearance

169. • ECG
• Echo
• Cardiac catheterization

170. MANAGEMENT
Emergency medical management
• Immediate endotracheal intubation and
hyperventilation with 100% oxygen to a PaCO2 of ˂ 30
mm Hg and correction of pH.
• Induced respiratory alkalosis decreases pulmonary
vascular resistance and improves oxygenation.
• Metabolic acidosis should be treated with NaHCO3 or
Tromethamine (THAM) infusions.

171. • Isoproterenol has special merit for inotropic
support in obstructed TAPVC because it has
pulmonary vasodilatory properties (0.1
microgm/kg/min for 24-48 hrs).
• PGE1 infusion given to maintain patency of
ductus venosus to decompress the pulmonary
veins in obstructed TAPVC.

173. Surgery
various approaches for surgery-
1. Posterior approach
2. Right atrial approach
3. Superior approach

178. TRUNCUS ARTERIOSUS
• Truncus arteriosus is a rare congenital heart
defect in which a single great vessel arises
from the heart, giving rise to the coronary,
systemic and pulmonary arteries.

179. Types
• Type I
• Type II
• Type III
• Type IV

185. HYPOLPLASTIC LEFT HEART
SYNDROME
• Hypoplastic Left Heart Syndrome (HLHS) is
identified as a small, underdeveloped left
ventricle usually with aortic and/or mitral
valve atresia or stenosis and hypoplasia of the
ascending aorta.

189. Surgery
• Stage I Norwood
• Bi-directional Glenn
• Fontan

191. CONGESTIVE CARDIAC FAILURE
• Congestive heart failure (CHF) is a term as the
heart does not pump enough blood out to the
rest of the body to meet the body's demand
for energy.

192. Treatment
• A diuretic like furosemide (Lasix), which helps the kidneys to eliminate
extra fluid in the lungs, is often the first medicine given both in babies
and older children.
• Sometimes medicines to lower the blood pressure like an ACE inhibitor
(Captopril), or more recently, beta blockers (Propranolol) are used.
Theoretically, lowering the blood pressure will decrease the workload
of the heart by decreasing the amount of pressure against which it has
to pump.
• Sometimes a medication called Digoxin is used to help make the heart
squeeze better, and help pump blood more efficiently. Since weight
gain is a major challenge for infants with congestive heart failure, giving
babies high calorie formula or fortified breast milk can help give the
extra nutrition they require.
• Sometimes babies will need to have extra nutrition given to them via a
tube that goes directly from

193. • The nose to the stomach, a nasogastric feeding tube. This is
good for babies who work hard or get very tired from
feeding in order to prevent them from using up all the extra
calories needed for growth. Older children with significant
heart failure can also benefit from nasogastric feeding to
give them more calories and energy to do their usual
activities.
• Oxygen can worsen blood flow to the lungs in babies with
large ventricular septal defects but may be helpful as a
buffer to children with weak hearts.
• Some kids with cardiomyopathy may also need restriction
of certain kinds of exercise and competitive sports,
although they may benefit from light activity like
swimming.

194. PERSISTENT PULMONARY
HYPERTENSION
• Persistent pulmonary hypertension (PPHN)
has also been referred to as persistent fetal
circulation, persistent pulmonary vascular
obstruction, pulmonary vasospasm, neonatal
pulmonary ischemia and persistent
transitional circulation.

195. Types
• Persistent pulmonary hypertension
associated with pulmonary parenchymal
disease
• Persistent pulmonary hypertension with
radiographically normal lungs
• Persistent pulmonary hypertension
associated with hypoplasia of the lungs

196. Causes
• Repeated intrauterine closure of the ductus with
redirection of blood flow into the high-resistance fetal
pulmonary circulation.
• High dose aspirin ingestion by the mother and the
administration of prostaglandin inhibitors have been cited
by research to cause persistent pulmonary hypertension.
• Abnormal responsiveness of the pulmonary vasculature to
hypoxia with an inability to relax after the stimulus for
vasoconstriction is removed as in birth asphyxia.
• Repeated intrauterine hypoxia, which causes hypertrophy
of pulmonary arteriole smooth muscle, which enables
these vessels to extremely constrict for a long period of
time.

197. • Regional alveolar hypoxia due to poor ventilation that
cannot be detected with a chest radiograph.
• In cases such as congenital diaphragmatic hernia or
other causes of pulmonary hypoplasia, undergrowth of
the pulmonary vascular structures.
• Alteration in vasoactive mediator levels may cause
pulmonary vasoconstriction. These include nitric oxide,
eicosanoids, endothelin, leukotrienes, platelet
activating factor and tumor necrosis factor.
• Micro thrombi and released mediators have been
reported in infants with persistent pulmonary
hypertension.

198. Clinical conditions frequently
associated with persistent pulmonary
hypertension of the newborn
• Meconium Aspiration Syndrome (MAS)
• Asphyxia
• Sepsis/infection
• Pneumonia (bacterial)
• Congenial Diaphragmatic Hernia (pulmonary
hypoplasia)
• Transient Tachypnea of the Newborn (TTNB)
• Respiratory Distress Syndrome (RDS/HMD)

199. • Polycythemia/ hyper viscosity
• Metabolic disturbances (hypoglycemia,
hypocalcemia, hypomagnesemia)
• Hypothermia
• Systemic hypotension
• Prenatal fetal hypertension
• Congenital heart disease
• Hypoventilation resulting from asphyxia or other
neurological conditions

200. Clinical picture
• May be entirely normal
• Term or post term infant (because the pulmonary arterial
musculature does not develop until late gestation)
• Dysmaturity may exist
• Meconium staining
• Cyanosis (if this is the main sign/symptom, the clinician
should rule out congenital heart and severe pulmonary
parenchymal disease)
• Respiratory distress with tachypnea, but minimal
retractions on day one.
• Right-to-left shunting

201. • Breath sounds may be normal (If pneumonia
or meconium staining exists, crackles or
wheezes may be present)
• Heart sounds may be normal or a murmur
may be heard
• Systemic hypotension later in course following
heart failure and persistent hypoxemia.
• Sensitivity to handling
• Hyperdynamic precordium

202. Diagnosis
• Chest X-ray
• CBC
• Serum electrolytes
• Blood gases

203. TREATMENT
• Administer oxygen
• High frequency ventilation
• Surfactant administration
• Continuous monitoring of pre and post-ductal
PaO2
• Alkalinizing agents
• Dopamine and Dobutamine

204. • Prostaglandin I2
• Magnesium sulfate, perflourocarbon liquid
ventilation and arginine infusion
• ECMO

206. CONGENITAL COMPLETE HEART
BLOCK
• Congenital complete heart block is the most
common cardiac cause of fetal and neonatal
bradycardia. It is a disorder of conduction due
to abnormality in the AV node with total
dissociation between atrial and ventricular
contractions.

207. Management
• Isoproterenol – to increase ventricular rate
• Transventricular cardiac pacing
• Caesarean section if FHR < 50 bpm or hydrops
fetalis
• Close monitoring
• Digitalis
• Diuretics
• Intraventricular pacing if HR <50 bpm during
waking hours