The document discusses various congenital cardiac lesions seen in newborns. It describes the three shunts present in the fetal circulation (ductus arteriosus, ductus venosus, foramen ovale) and how they normally close after birth. It then examines cyanotic lesions involving right-to-left shunts like Tetralogy of Fallot, Transposition of the Great Arteries, and Tricuspid Atresia. Acyanotic lesions with left-to-right shunts such as VSD, ASD, and PDA are also reviewed. Surgical/catheter interventions for some of these conditions are mentioned.

1. Congenital Cardiac Lesions

2. Fetal Circulation
Ductus Venosus
Ductus Arteriosus
Foramen Ovale

3. Three Shunts of Fetal
Circulation
• Ductus Arteriosus
– Protects lungs against circulatory overload
– Allows RV to strengthen
– High pulmonary vascular resistance, low pulmonary
blood flow
– Carries moderately saturated blood
• Ductus Venosus
– Connects umbilical vein to IVC
– Flow regulated via sphincter
– Conducts highly oxygenated blood
• Foramen Ovale
– Shunts highly oxygenated blood from RA to LA

4. Circulatory Changes at Birth
• Aeration of Lungs at Birth
– Increase in pulmonary blood flow- raising LA
pressure to higher than that of the IVC
– Thinning of walls of PA secondary to stretch
as lungs increase in size with first few
breaths

5. Changes Associated with
First Breath
• Alveoli open
• Pressure in R. heart decreases
• Pressure in the L. heart increases as
blood returns from highly vascularized
pulmonary tissue to the LA

6. Fate of the shunts
• Foramen Ovale:
– Closes at birth due to decreased flow from placenta and IVC
– Pulmonary venous return causes pressure in LA to be higher
than that in RA
• Ductus Arteriosus:
– Due to decreased pulmonary vascular resistance, PA pressure
falls below systemic pressure and blood flow through DA is
diminished
– Closure mediated by bradykinin
– Prostaglandin E2 may reopen DA
• Umbilical Vessels
– Constrict at birth and are then tied and cut

7. The Normal Heart

8. Cyanosis in the Newborn
• Arterial oxygen saturation less than
90%
• Common Causes:
– Intrinsic pulmonary disease
– Congenital heart lesions
– Central nervous system depression with
hypoventilation

9. Cyanotic Heart Lesions
• The 5 Ts
– Tetralogy of Fallot
– Transposition of the Great Arteries
– Truncus Arteriosus
– Tricuspid Atresia
– Total Anomalous Pulmonary Venous
Return

10. Tetralogy of Fallot
• 4 lesions
– Overriding aorta
– Right ventricular hypertrophy
– Ventricular septal defect
– Right ventricular outflow tract
obstruction
• Cyanosis varies with the degree of
outflow tract obstruction and size
of VSD
• Characterized by hypercyanotic
episodes- Tet Spells
• Cyanosis is caused by
Right Left shunting through
the VSD

11. • Correction
– Early repair, unless
patient is premature
or low birth weight
– VSD is closed with
patch
– Obstructing RV muscle
is removed
– All other outflow tract
abnormalities are
addressed
Tetralogy of Fallot

12. Transposition of the Great
Arteries
• Most common
cyanotic condition
that requires
hospitalization in
first 2 weeks of
life
• Aorta arises from
RV and carries
oxygenated blood
to the lungs

13. Transposition of the Great
Arteries
• Survival depends on allowing bloodflow
from the 2 outflow tracts to mix.
– Prostaglandins augment ductal flow
– Inter-atrial septum may be opened with
balloon septostomy
– Surgical correction requires removal of aorta
and pulmonary artery from their origins and
re-attached to the correct ventricles

14. Truncus Arteriosus
• Single artery arises from
the heart, supplying both
aorta and pulmonary
artery.
• VSD below the truncal
valve allows mixing of
right and left ventricular
blood
• Degree of cyanosis is
variable
• Presents with progressive
heart failure

15. Truncus Arteriosus
• Medical Management
– Digoxin and Diuretics
• Surgical Repair
– Usually required by 2-3 months of age
– VSD is closed
– PA trunk is separated from truncus
– Conduit created between RV and PA using a valved
graft
– May require further procedures if conduit becomes
obstructed, if graft calcifies, or if patient outgrows
repair

16. Tricuspid Atresia
• Tricuspid valve fails
to develop, therefore
leaving no connection
between RA and RV
(which is hypoplastic)
• Desaturated blood
from RA must cross
through PFO to LA and
LV

17. Tricuspid Atresia
• Repair allows venous return to flow passively to
the lungs without a pumping chamber.
• Pulmonary blood flow is dependent on low
pulmonary vascular resistance and elevated
CVP
• Repair not usually performed in neonatal
period- but rather over a series of procedures
– Systemic to PA shunt
– SVC to PA shunt (followed by ligation of first shunt)–
Glenn Shunt
– IVC to PA shunt– completion Fontan

18. Right-Sided Heart Lesions
– Other right-sided cardiac abnormalities that
may present with or without cyanosis
include:
• Pulmonary Valve and Infundibular Stenosis
• Pulmonary Regurgitation
• Absence of the pulmonary valve
• Pulmonary Artery Stenosis
• Tricuspid Stenosis
• Double-chambered right ventricle
• Ebstiens anomaly

19. Acyanotic Heart Lesions
• Acyanotic congenital heart disease: a
group of cardiac diseases with a Left to
Right shunt or left heart abnormality
• Acyanotic lesions make up about one
third of congenital heart disease.

20. Acyanotic Congenital Heart
Lesions
• Acyanotic heart lesions with left-to-
right shunts include:
– ASD
– VSD
– PDA
– AV Canal Defects

21. Atrial Septal Defects
• Most commonly occurs in
the ostium secundum-
covering the central
portion of the inter-atrial
septum.
• Generally asymptomatic
for the first 3 decades of
life.
• Symptoms may include
exercise intolerance,
dyspnea on exertion, and
fatigue cause by right
heart failure and
pulmonary hypertension
• Stroke can result from
paradoxical emboli.

22. Atrial Septal Defects
• The decision to close and ASD is based on
the size of the shunt and the presence or
absence of symptoms.
• Closure is indicated in patients who are
symptomatic or who have systemic
embolization.
• Smaller defects may be closed using
trans-catheter techniques, thus avoiding
sternotomy and bypass

23. Ventricular Septal Defect
• May be anywhere in
intra-ventricular
septum- clinical course
depends on the shunt
size and involvement of
pulmonary vascular bed.
• Approx ½ of all VSDs are
small, and more than ½
close spontaneously.
– Highest closure rates in
the first decade of life.

24. Ventricular Septal Defect

25. Ventricular Septal Defect
• Eisenmenger’s complex:
– Left Right shunt causes pulmonary hypertension with
resulting reversal of the direction of the shunt.
– Ultimately the resistance in the lungs may exceed the
resistance in the arteries of the rest of the body, which
leads to a reversal of flow from Left Right to
Right Left shunt.
– Reversal of the shunt leads to cyanosis, dyspnea,
hemoptysis, reduced exercise tolerance, syncope,
palpitations, and atrial fibrillation
– Brain events such paradoxical embolus, thrombosis, and
hemorrhage may occur.
– Heart failure suggests a poor prognosis, and sudden
death is possible.

26. Patent Ductus Arteriosus
• Ductus Arteriosus connects
the descending aorta to the
main pulmonary trunk near
the origin of the left
subclavian
• Normal postnatal closure
results in fibrosis- which
becomes the ligamentum
arteriosum.
• Small PDA does not
increase risk for heart
failure- but does carry a
risk for bacterial
endocarditis.

27. Patent Ductus Arteriosus
• Without ligation- there is an ongoing risk for
bacterial endocarditis, heart failure, and
development of Eisenmenger’s complex.
• Ligation of PDA
• May be closed surgically or via transcatheter
procedure
• Endocarditis prophylaxis is not required after
ligation
• Cardiac function generally returns to normal

28. Acyanotic Heart Lesions
Acyanotic heart lesions with left heart
abnormalities include:
– Aortic Stenosis
– Aortic Regurgitation
– Coarctation of the Aorta
– Anomalous Right Subclavian or Innominate
arteries
– Aortic Atresia
– Mitral Atresia, Mitral Stenosis, Mitral
Regurgitation, Mitral Prolapse

29. Coarctation of the Aorta
• Coarctation usually
occurs just distal to the
left subclavian artery at
the ligamentum
arteriosum
– May also occur just
proximal to the left
subclavian.
• Causes systemic
hypertension and
secondary LVH with
heart failure.

30. Coarctation of the Aorta

31. Coarctation of the Aorta
• Diagnosis:
– Young adults may be aysmptomatic except for
hypertension and decreased lower extremity pulses.
– May be seen on CXR- characterized by the “3 sign”-
coarct segment between dilated left subclavian
above and convexity of descending aorta below.
• Treatment:
– Considered for patients with gradients greater than
30 mm Hg on cardiac cath.
– Balloon angioplasty is the treatment of choice

32. Review
• Cyanotic Heart Lesions; Right Left Shunts and right
sided lesions:
– tetralogy of Fallot
– Transposition of the Great Arteries
– Truncus Arteriosus
– Tricuspid Atresia
– Total Anomalous Pulmonary Venous Return
• Acyanotic Lesions; Left Right Shunts and left sided
lesions:
– ASD
– VSD
– PDA
– Coarctation of Aorta