cyanoticheartlesions

8/13/2019 CyanoticHeartLesions

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Cyanotic Heart Lesions


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Cyanosis

Arterial saturation less than 90% and a PO2less than60 torr

In all cyanotic heart lesions the amount of cyanosis

seen is dependent on the amount of pulm bloodflowo Decreased PBF- increased cyanosis

o Increased PBF- minimal cyanosis but CHF may develop

With 100% oxygeno PO2 >250 is not congenital heart disease

o PO2


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5 Ts Most common cyanotic lesions of the newborn

o Tetralogy of Fallot

o Transposition of the Great Arteries

o Truncus Arteriosus

o Total Anomalous Venous Return

o Tricuspid Atresia


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General Sources of Arterial

SaturationDecreased PulmonaryBlood Flow

Admixture Lesions Increased PulmonaryBlood Flow

Tetralogy of Fallot Transposition of GreatVessels

Truncious Arteriosus

Tricuspid Atresia Anomalous pulmonaryvenous return

Pulmonary Atresia


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PGE For cyanotic heart lesions with reduced blood flow

RE-opens PDA and prevents it from closing

Allows partially desaturated systemic arterial bloodto enter the pulmonary artery and be oxygenated

Initial dose 0.1mg/kg/min

Side effects- apnea, fever, hypotension


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Complete Transpositionof the Great Arteries

5% of all CHD

Boys 3:1

Most common cyanotic condition that requireshospitalization in the first two weeks of life


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Complete Transposition of

the Great Arteries Aorta arises from the right ventricle

Pulmonary artery arises from the left ventricle


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the Great Arteries Complete

separation of the2 circuits

o Hypoxemic bloodcirculating in thebody

o Hyperoxemicblood circulating

in the pulmonarycircuit


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the Great Arteries Defect to permit

mixing of 2circulations- ASD, VSD,

PDA.o VSD is present in 40% of cases

Necessary for survival


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Clinical Symptoms Depend on anatomy present

No mixing lesion and restrictive PFOo Profound hypoxia

o Rapid deterioration

o Death in first hours of life

o Absent respiratory symptoms or limited to tachypnea

o Single second heart sound, no murmurs


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Clinical Symptoms Mixing lesion present (VSD or large PDA)

o Large vigorous infant

o Cyanotic

o Little to no resp distress

o Most likely to develop CHF in first 3-4 months of life

excessive sweating (a cold, clammy sweat often noted during

feeding); poor feeding, slow weight gain, irritability or lethargy,

and/or rapid breathing


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CXR

Egg shapedcardiac silhouette

Narrow superiormediastinum
http://www.radswiki.net/main/index.php?title=File:Transposition-of-great-vessels-101.jpg


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Management

Prostaglandin to establishpatency of the ductusarteriosus

o Increases shunting fromaorta into the pulmonaryartery

o Increases pulmonary

venous return distendingthe left atrium

o Facilitates shunting fromthe left to the right atriumof fully saturated bloodacross the foramen ovale.


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Management

Therapeutic balloon atrial septostomy (RashkindProcedure) if surgery is not going to be performedimmediately

Improves mixing and pulmonary venous return atthe atrial level


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Treatment

Surgery consists of switching the right and left sided

structures at the atrial level, at the ventricular level,or at the great artery level.


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Tetraology of Fallot

1. VSD

2. RVOT Obstruction

3. RVH

4. Overriding aorta

T I t t


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Two ImportantAbnormalities

NonRestrictive VSDo Large enough to equalize pressures in both ventricles

Degree of RVOT


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Clinical Presentation of

Cyanotic TOF Cyanosis, clubbing, dyspnea on exertion, squatting,hypoxic spells.

Loud systolic ejection murmur, systolic thrill atmiddle LSB

Soft murmurs are associated with less blood flowand more hypoxia


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CXR- boot shaped

ll


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Hypoxic Spell(TET Spell)

Peak incidence of 2-4 months

Characterized by:o Hyperapnea (Rapid and deep respirations)

o Irritability and prolonged crying

o Inc cyanosis

o Decreased heart murmur


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Pathophysiology

o Lower SVR or inc

resistance of RVOT

can increase the R-

L shunt

Stimulates therespiratory center toproducehyperapnea

Results in anincrease in systemicvenous return

In turn, increases R-Lshunt through VSD


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TET Spell Treatment1. Hold infant in knee-chest position

2. Morphine

3. Sodium bicarbonate to treat acidosis- decreases

resp stimulating effect of acidosis4. Vasoconstrictor (phenylephrine)

5. Propranolol


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Treatment Early surgical repair depending on pts weight

VSD is closed and obstructing ventricular muscle isremoved


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Total Anomalous PulmonaryVenous Return

The pulmonary veins drain into the RA or its venoustributaries rather than the LA

A interatrial communication (ASD or PFO) isnecessary for survival

Pulmonary venous return reaches the RAo Systemic and pulmonary venous blood are completely mixed


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4 Types1. Supracardiac

Common pulmonary vein drains into the SVC via theleft SVC and left innominate vein.


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2. Cardiac The common PV drains into the coronary sinus


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3. Infracardiac The common PV drains into the portal vein, ductous

venosus, hepatic vein, or IVC.

Infracardiac-type TAPVC. Pulmonaryvenous blood draining through the liver toreach the IVC and right atrium.


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4. Mixed A combination of the other types


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Clinical Signs for

Unobstructed Veins Mild cyanosis, signs of CHF in infancy, history ofpneumonia

Widely split S2, Grade 2-3/6 systolic murmur heard

at the ULSB CXR- marked cardiomegaly

Cli i l Si f


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Clinical Signs forObstructed Veins

Profound desaturation

Acidosis

PGE1administration does not improve oxygenationbecause elevated pulmonary pressures in the rightside of the heart (due to obstructed pulmonaryoutflow) will result in right to left shunting across an

open ductus further decreasing arterial saturation.


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Treatment

Digitalis and diuretics to treat heart failure

Intubation and inc PEEP for those with severe pulm

over load Corrective surgery


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Tricuspid Atresia Tricuspid valve is absent RV and PA are hypoplastic

Associated defects- ASD, VSD, or PDA (necessary

for survival) Dilation of LA and LV

Essentially single

ventricle physiology


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Clinical Signs Severe cyanosis, poor feeding, tachypnea

Single S2, grade 3/6 systolic murmur at LLSB if VSD ispresent

CXR- boot shaped heart


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Treatment PGE IV infusion

Blalock-Taussig shunt in infancyo systemic to pulmonary arterial shunt

o Provide stable blood flow to the lungs

o A gortex tube is sewen between the subclavian artery and the right

pulmonary artery


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Bidirectional Glenn

Superior vena cava is connected to the pulmonaryarteries

IVC continues to be connected to the heart


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Fontan Procedure Redirects IVC to lungs


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Truncus Arteriosus A single trunk leaves the heart

o Gives rise to pulm, systemic, and coronarycirculations

o Large VSD is always present


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Clinical Signs Cyanosis immediately after birth

Early signs of CHF

2-4/6 systolic murmur at LSB suggestive of VSD


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Treatment

Anticongestive medications (diuretics and digitalis)

Corrective surgeryo VSD is closedo Pulmonary artery is separated from the truncus

o Continuity is then established between the right ventricle and the

pulmonary artery utilizing a valved homograft conduit


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