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Congenital Cardiac Lesions
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Fetal Circulation
Ductus Venosus
Ductus Arteriosus
Foramen Ovale
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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
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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
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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
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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
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The Normal Heart
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Cyanosis in the Newborn• Arterial oxygen saturation less than
90%• Common Causes:
– Intrinsic pulmonary disease– Congenital heart lesions– Central nervous system depression with
hypoventilation
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Cyanotic Heart Lesions• The 5 Ts
– Tetralogy of Fallot– Transposition of the Great Arteries– Truncus Arteriosus– Tricuspid Atresia– Total Anomalous Pulmonary Venous
Return
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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
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• 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
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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
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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
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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
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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
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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
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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
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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
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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.
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Acyanotic Congenital Heart Lesions
• Acyanotic heart lesions with left-to-right shunts include:– ASD– VSD– PDA– AV Canal Defects
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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.
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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
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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.
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Ventricular Septal Defect
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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.
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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.
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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
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Acyanotic Heart LesionsAcyanotic 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
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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.
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Coarctation of the Aorta
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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
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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