[LEC_OLESON] CHD

8/7/2019 [LEC_OLESON] CHD

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CONGENITAL HEART DISEASE


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Definition Definition

Congenital Cardiovascular malformationsgenerally are the result of aberrantembryonic development of normal structureor failure of such a structure to progressbeyond an early stage of embryonic or fetaldevelopment.Malformations are due to complex

multifactorial genetic and environmentalfactors.


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Causes Congenital Heart Disease

In the majority of people, the cause of congenital heart disease isunknown.However, there are some factors that are associated with an

increased chance of getting congenital heart disease.

These risk factors include:Genetic or chromosomal abnormalities in the child such as

Down syndrome.Taking certain medications or alcohol or drug abuse during

pregnancy.Maternal viral infection, such as rubella (German measles) in the

first trimester of pregnancy.

The risk of having a child with congenital heart diseaseis higher if a parent or a sibling has a congenital heartdefect -- the risk increases from eight in 1000 to 16 in1000.


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Causes Congenital Heart Disease

Associated with embryogenesis

1st heart of embryo: 1 chamber ( sac )

Atrium and ventricle

2 atriums and 2 ventricles

Disorder of embryogenesis:Viral infectionduring 1st trimester

Unknown disorders affecting vesselsand heart structure


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Congenital heart disease is often divided into two types:

cyanotic (blue discoloration caused by a relative lack of oxygen) and

non-cyanotic.The following lists cover the most common of the congenital heartdiseases:

Cyanotic:

Tetralogy of FallotTransposition of the great vesselsTricuspid atresiaTotal anomalous pulmonary venous returnTruncus arteriosusHypoplastic left heartHypoplastic right heartSome forms of total anomalous pulmonary venous

returnEbstein's anomaly and Eisenmeigers syndrome


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N on-cyanotic:

Ventricular septal defect (VSD)Atrial septal defect (ASD)Patent ductus arteriosus (PDA)Aortic stenosisPulmonic stenosisCoarctation of the aortaAtrioventricular canal (endocardial cushion

defect)These problems may occur alone or together.


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V entricular septal defect Ventricular septal defect is the

most common chd in infants andchildren. It occurs with similar frequency in boys and girls. 25 to40%of such defects closespontaneously when the child is2 years.90% of those that

eventually close when the childis 10Anatomically, 70% are located inthe membranous portion of theinterventricular septum, 20% inthe muscular portion of the

septum, 5 %t just below theaortic valve (therebyundermining the valve annulusand causing regurgitation), and5% near the junction of themitral and tricuspid valves (so-

called atrioventricular canaldefects


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Ventricular septal defect L ocalised in central part, ie fibrosisof part of septumThere is flow of blood from LV to RV

Therefore increase volume of bloodto pulmonary artery and thus volumeassociated lung hypertensiondevelops

Gradient between LV and RV sia bout100mmHG, in middle of systole it is120mmHg ( as in aorta )

Pressure in RV is about 50mmHg

During diastole this gradient isequal.

This big gradient produce turbulentflow through osteum ( defect )

Thus patient develops intensivesystolic murmur localized under

sternum


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VSD Classification

Involving the crista supraventricularis, termedinfundibular (outlet) VSD - 30%

P ars atrioventriculare of septum membranaceum,atrioventricular septal defect (inlet VSD) - 2%

P ars interventriculare of septum membranaceum,termed membranous VSD - 15%

S mooth posterior septum or smooth VSD - 49%

T rabeculated posterior septum, termed trabecularVSD - 4%


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S ymptoms:

S hortness of breathFast breathing

Hard breathingPalenessFailure to gain weightFast heart ratePounding heartS weating while feedingFrequent respiratory infections

Exams and TestsListening with a stethoscope usually reveals a heart murmur (the sound of the bloodcrossing the hole). The loudness of the murmur is related to the size of the defectand amount of blood crossing the defect.ECG: shows signs of an enlarged left ventricleparadoxical situationQR S complex not changes if the defect is not along the branch as the same volumeflows out of LV as RV nowX-ray: heart size to left and right side ( due to equivalent load )Echocardiogram -- used to make a definite diagnosis

Catherization:Venous blood seen in RA oxygenated blood in RV(rarely needed, unless there are concerns of high blood pressure in the lungs, inwhich case surgery to close the defect is generally not recommended) S ymptoms


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E CG & CXRECG: P atients with small defects may have normal ECG s. NormalQR S axis is the rule in most patients with V SD .

Atrial arrhythmias (uncommon)Left ventricular hypertrophyMarked left axis deviation (endocardial cushion defect)

Chest roentgenographic findingsLeft ventricular prominenceP rominent pulmonary vasculature (shunt vessels)E nlarged main pulmonary arteriesLeft atrial enlargementS mall aortic knob

P atients with marked pulmonary hypertension or infundibular stenosis

Right ventricular hypertrophy by ECG

Right ventricular enlargement by chest x-ray


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E CG in VSD


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E CHOD efective S eptum easilyvisualized


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This is an ultrasound showing a ventricular septal defect pattern of the fetal heartbeat.S ome ultrasound machines have the ability to focus on different areas of the heartand evaluate the heartbeat. This is useful in the early diagnosis of congenital heartabnormalities.


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Treated surgicalIndication is of right chamber 2 times( if 3 times is it associated with pressure in right

side )S urgery is done when there is increase 2 timesbecause in persons this defect can close byitself during some years especially during puberty.

If symptoms continue despite medication, surgery toclose the defect with a Gore-tex patch is needed.S ome V SD s can be closed with a special deviceduring a catheterization. Treating a V SD that doesnot have symptoms is controversial, and should becarefully discussed with your health care provider.


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Possible Complications

Congestive heart failureInfective endocarditis (bacterial infection of the heart)

Aortic insufficiency (leaking of the valve that separates the left ventricle from the aorta)D amage to the electrical conduction system of the heart during surgery (causing

arrhythmias)D elayed growth and development (failure to thrive in infancy)Pulmonary hypertension (high blood pressure in the lungs) leading tofailure of the right side of the heart

Outlook (Prognosis)Many small defects will close on their own.For those defects that do not spontaneously close,the outcome is good with surgical repair.

Complications may result if a large defect is not treated.


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A trial septal defect Atrial septal defect accounts 1/3 of thecases of congenital heart disease detectedin adults. It occurs in women 2 to3 times asoften as in men.

Anatomically, it may take the form of ostiumsecundum, in the region of the fossa ovalis;ostium primum, in the lower part of the atrialseptum; or sinus venosus, in the upper atrial

septum. Ostium secundum defects make up75% of all atrial septal defects, ostiumprimum defects make up 15 %,&sinusvenosus defects make up 10 %. Additionalcardiac abnormalities may occur with eachtype of defect; these include mitral-valveprolapse (with ostium secundum defects),4mitral regurgitation (due to a cleft in theanterior mitral-valve leaflet, which occurs

with ostium primum defects), and partialanomalous drainage of the pulmonary veinsinto the right atrium or venae cavae (withsinus venosus defects).5 Although mostatrial septal defects result from spontaneousgenetic mutations, some are inherited


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An atrial septal defect is a congenital heart defectwhere the wall between the right and left atria does not close properly,leaving a hole between the two atria.


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Increased flow across the pulmonaryvalve produces a systolic ejectionmurmurand fixed splitting of the secondheart sound.

T he hallmark of patients with A SD is the Fixedsplitting of S2 which may in part

be due todelayed right bundle conduction.

Increased flow across the T ricuspid

Valve produces a diastolic rumble at themid tolower right sternal border.

O lder pts lose pulm ejection

murmur as shunt becomes bidirectional

signs of pulm H T N/ C HF may predominate

ASD - AUSCULTATION


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Intraatrial septum defect

Non closing of foramen Ovale or secondary defect in another place of this septum

Pressure in LA: 10mmHgPressure in RA: 0mmgHg

Therefore blood moves from the LA to the RA

Thus summary of blood volume increases in RA which goes to RV, and increased loadhere.

Hypertrophy of the RV develops, because supply of bigger volume in Pulmonary artery.S pasm of arterioles begin ( Kitief reflex ) Arterial hypertension beginsClinical symptoms develop

S ymptoms

Frequent respiratory infections in childrenD ifficulty breathing (dyspnea)S hortness of breath with activityS ensation of feeling the heart beat (palpitations) in adults

Note: People with small-to-moderate-sized defects may show no symptoms, or not untilmiddle age or later.


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Exams and TestsRarely, there may be a palpable pulsation of the pulmonary artery in the chest.Examination with a stethoscope (auscultation) of the heart usually

reveals abnormal heart sounds.There may be a murmur caused by the increased blood flow across the pulmonic valve,and the second heart sound is widely split and fixed.

S igns of heart failure can occur in adults.

If the shunt is large, increased blood flow across the tricuspid valve(between the right atrium and ventricle) may be responsiblefor an additional murmur when the heart relaxes between beats.

Tests that may be performed in the diagnosis of A SD include:

Chest x-rayEchocardiography (ultrasound of the heart)D oppler study of the heartTransesophageal echocardiography (TEE)

Cardiac catheterizationCoronary angiography (for patients over 35 years old)MRI of heartECG - may show atrial fibrillation in adults, right atrial and ventricular enlargement,or a pattern of delayed electrical conduction in the heart


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E CG


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CXR and ECHOCARDIOGRAM


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ECG: S evere deviation of electrical axis toright,Hypertrophyof RV or right bundlebreak

X-ray:Increase heart size to right,Increaseof LV,Increase of pulmonary artery, S lightmitral stenosis ( see venous vessels toborderline of chest )

Echocardiography: D oesnt give a lot of Information because interseptal is very thin

D oppler effect: If there is movement theangle is not equal

Therefore since pressure is very slow,

pathology is very slow and difficult to find

contrast of RA:We see contrast in RA

Catherization of right part of heart: D onethrough the subclavian vein,to the Venacava inferior and to the RA, and we seeoxygenated blood is more in the Vena cavainferior


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Treatment

ASD may not require treatment if there are few or no symptoms, or if the defect is small.S urgical closure of the defect is recommended if the atrial septal defect is large,

the heart is enlarged, or symptoms occur.

A relatively new procedure has been developed to close the defect without surgery.The procedure involves the introduction of an A SD closure device (such as the Amplatzer

device)into the heart through catheters. A tiny incision is made in the groin to introduce the catheters.They are then advanced into the heart, where the closure devise is placed across the A SD

and the defect is closed.

All atrial septal defect patients may not be eligible for this procedure. Prophylactic (preventive)antibiotics should be given prior to dental procedures to reduce the risk of developing infectiveendocarditis immediately after surgery for the A SD , but they are not required later on.

Outlook (Prognosis)

With a small-to-moderate atrial septal defect,

a person may live a normal life span without symptoms.Larger defects may cause disability by middle age because of increased blood flow andshunting of blood back into the pulmonary circulation.

Possible ComplicationsPulmonary hypertension

Arrhythmias, particularly atrial fibrillationHeart failure


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P atent ductus arteriosus

DefinitionPatent ductus arteriosus(PDA) is a condition inwhich a blood vessel called

the ductus arteriosus failsto close normally in aninfant soon after birth.(The word "patent" means

open.)

The condition leads toabnormal blood flowbetween the aorta andpulmonary artery, two major blood vessels surroundingthe heart.


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Causes

Before birth, the ductus arteriosus allows blood to bypass the baby's lungs byconnecting the pulmonary arteries (which supply blood to the lungs) with the aorta

(which supplies blood to the body).S

oon after the infant is born and the lungs fill with air,this blood vessel is no longer needed. It will usually close within a couple of days.If the ductus arteriosus does not close, there will be abnormal blood circulationbetween the heart and lungs.

P D A is rare. It affects girls more often than boys. The condition is more common inpremature infants and those with neonatal respiratory distress syndrome. Infants withgenetic disorders, such as D own syndrome, and whose mothers had German measles

(rubella) during pregnancy are at higher risk for P D A.P D A is common in babies with congenital heart problems,such as hypoplastic left heart syndrome, transposition of the great vessels,and pulmonary stenosisS ymptoms

A small P D A may not cause any symptoms.However, most infants do not tolerate a

P D A and may have symptoms such as:

Bounding pulseFast breathingPoor feeding habitsS hortness of breathS weating while feeding

Tiring very easilyPoor growth


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Opened D uctus arteriosus

In systole pressure in aorta is 120mmHgIn diastole pressure in aorta is 80mmHg

In opened ductus arteriosus the pressure is 25mmHg

Therefore there is pathological flow all the time

Murmur is known as mechanical murmur

This flow goes to pulmonary artery, thus load on RA, not by volume but by pressure ( ie pressure load )an dthus there is and increases in size that is not acute, but over a period of time

( ie hypertrophy is gradual ) since the heart tries to compensate this pathology

Pulse is quickBlood after going to aorta goes quickly to the pulmonary artery.

ECG: D eviation of electrical axis to the rightHypertrophy of LV

X-ray: S igns of arterial lung hypertension size to the right

Calcification of vessels ( ductus arteriosus has venous load, but from aorta blood goeswith increased pressure thus destruction of wall chronically and thus calcification develops )

Echocardiography: Cant recognize this defect

Catherization: A lot of information about this pathology is foundOxygenated blood is seen in the pulmonary artery


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AN GIOGR AP HY AN D COLOR DO PP L E R

T he ductus can be well visualized from the left parasternal area (A) with low velocity flow back intothe pulmonary artery from the aorta (B). After therapy with indomethacin the PD A significantly

decreases in size (C

) with aliasing color D

oppler flow in a smaller jet (D

), and a high velocity,restrictive spectral D oppler pattern ( E ). (M P A = main pulmonary artery, R P A = right pulmonary


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TreatmentS urgical treatment immediately after diagnosisCan use embolization by endocardiovascular methods to close the duct

NB: oedema of legs is due to pressure in venous systems unlike in vulvular diseases.

Eventual Compensation

Izamiyo syndrome, if you close defects you stop the circulation

Oedema

viscosity of blood

defects with lung circulation

chronic problems with blood oxygenation

secondary erythrocytosis

Necessary haem transfusion


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The type and timing of surgicalrepair depends onthe child's conditionand the type and severity of heartdefects.

In general, symptoms that indicatethat surgery

is needed are:

difficulty breathing becausethe lungs are wet,congested, or

fluid-filled (congestive heart failure)problems with heart rate or rhythm (arrhythmias)

excessive work load on heart thatinterferes withbreathing, feeding, or sleeping


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Outlook (Prognosis)

If a small P D A remains open, heart symptoms may or may not eventually develop.Persons with a moderate or large P D A will usually develop heart problems sooner

or later unless the P D A is closed.

Closure with medications can work very well in some situations, with few side effects.Early treatment with medications is more likely to be successful.

S urgery carries its own significant risks. It may eliminate some of the problems of a P D A,but it can also introduce a new set of problems. The potential benefits and risks shouldbe weighed carefully before choosing surgery.

Possible ComplicationsIf the patent ductus is not closed, the infant has a risk of developing heart failure,bleeding in the lungs, problems with lung development, or infective endocarditis --

an infection of the inner lining of the heart


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Most children need to stay in the IntensiveCare Unitfor 3 to 7 days and stay in the

hospital for 5 to 14 days. By the time the childistransferred out of the intensive care unit,most of thetubes and wires have been removed and heisencouragedto resume many of his daily activities. At the

time of discharge,the parents are instructed on activity, how to

care for the incision and how to give medications

their childmay need to take such as D igoxin, Lasix,

Aldactoneand Coumadin. The child needs at least

several moreweeks at home to recover.


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Aortic stenosis

Aortic stenosis is a heart valve disorder that narrows or obstructs the aortic valve opening.Narrowing of the aortic valve prevents the valve from opening properly and obstructs theflow of blood from the left ventricle to the aorta. This can reduce the amount of bloodthat flows forward to the body.


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The most common pathological finding in patients with symptomatic aortic stenosiswho are younger than 65 years of age is a bicuspid aortic valve, which is found

in 2 to 3 percent of the population.44 It is four times as common in men and boysas in women and girls. Twenty percent of patients with bicuspid aortic valve have

an associated cardiovascular abnormality,45 such as patent ductus arteriosus or aortic coarctation. In patients with bicuspid aortic valve, the bicuspid valve has asingle fused commissure and an eccentrically oriented orifice. Although the deformedvalve is not stenotic at birth, it is subjected to abnormal hemodynamic stress,which may lead to thickening and calcification of the leaflets, with resultant immobility.In many patients, there is a coexisting abnormality of the medial layer of the

aorta above the valve, which predisposes patients to have dilatation of the aortic root.

The area of the aortic orifice in a normal adult is 3.0 to 4.0 cm2. Aortic stenosis does not become hemodynamically important unless the valve areais reduced to approximately 1.0 cm2.


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The following tests may be performed:EchocardiogramD oppler ultrasonographytransthoracic echocardiography with D oppler flow permits an accurate assessment of the

severity of the stenosisand of left ventricular systolic function.

Transesophageal echocardiogram (TEE)Chest x-ray :Unless the left ventricle dilates, the chest x-ray film demonstrates a normal

cardiothoracic silhouetteECG :Left ventricular hypertrophy results from gradually worsening aortic stenosis and is

usually evident on electrocardiography.MRI of the heartExercise stress testing

Aortic angiographyLeft cardiac catheterizationis performed to determine the severity of aortic stenosis in cases in which it cannot beassessed noninvasively and to determine whether concomitant coronary artery disease is

present.


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TreatmentIf there are no symptoms or symptoms are mild, you may only need to be monitored.If symptoms are moderate to severe, you may need to stay in the hospital.Infants and children may need immediate surgery.

Medications can include diuretics, digoxin, and other medications to control heart failure.S ymptomatic people may be advised to avoid strenuous physical activity.

People with symptoms of aortic stenosis (difficulty breathing, chest pain, fainting episodes)should have a physical exam every 6 to 12 months and an ECG performed every 1 to 3 years.

S urgery to repair or replace the valve is the preferred treatment for adults or childrenwho have symptoms. Even if symptoms are not very bad, the doctor may recommend surgery.S ome high-risk patients are poor candidates for heart valve surgery.

A less invasive procedure called balloon valvuloplasty may be done in adults or children instead.This is a procedure in which a balloon is placed into an artery in the groin, advanced to the heart,placed across the valve, and inflated. This may relieve the obstruction caused by the narrowed

valve.Infants and children may have various forms of surgery. If the diagnosis is isolated aortic stenosis,the pulmonary valve may be used to replace the aortic valve.


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Outlook (Prognosis) Aortic stenosis can be cured with surgery, although there may be a continued risk for irregular heart rhythms, which can sometimes cause sudden death. The person may be symptom-free

until complications develop. Without surgery, a patient who has signs of angina or heart failuremay do poorly.

Persons with aortic stenosis, particularly moderate and severe forms, should not participate instrenuous activities, such as competitive sports.


Left ventricular hypertrophy (enlargement) caused by the extra work of pushing blood throughthe narrowed valveLeft-sided heart failure

ArrhythmiasEndocarditis


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P ulmonary valve stenosis

D efinition

Pulmonary valve stenosis is a condition, usually present at birth (congenital),in which outflow of blood from the right ventricle (lower chamber) of theheart is obstructed at the level of the pulmonic valve (the valve whichseparates the heart from the pulmonary artery).

Pulmonary stenosis constitutes10 to 12% of chd in adults.Obstruction of right ventricular outflow is valvular in 90 %

of patients, and in the remainder it is supravalvular or subvalvular.

S upravalvular pulmonary stenosisresults from the narrowing of the pulmonary trunk, its

bifurcation,or its peripheral branches;

it often coexists with other congenital cardiac abnormalities

(valvular pulmonary stenosis,atrial septal defect, ventricular septal defect,patent ductus arteriosus,

or tetralogy of Fallot).It is a common feature of Williams syndrome


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Causes

Pulmonary valve stenosis is most often caused by a malformation during fetal development.The cause is unknown. A narrowing may occur in the pulmonary valve or below thepulmonary valve at the pulmonary artery.

The defect may occur alone, but is relatively common in connection with other heartdefects.The condition can be mild or severe. It occurs in approximately 10% of patients with

congenital heart disease.

Pulmonary stenosis can also occur later in life as a result of conditions that cause damage

S ymptomsS hortness of breathFatigueBluish coloration to the skin (cyanosis)Chest painFaintingPoor weight gain or failure to thrive in infantsS udden deathNote: There may be no symptoms until the disorder is severe. S ymptoms,when present, may get worse with exercise or activityor scarring of the heart valves. These include rheumatic fever, endocarditis,

and other disorders


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Exams and Tests

A heart murmur may be heard by stethoscope:The first heart sound is normal, and the second heart sound is widely

split but moves normally with respiration; its pulmonary component is softand delayed. A harsh crescendodecrescendo systolic murmur that increasesin intensity with inspiration is audible along the left sternal border.

If the valve is pliable, an ejection click often precedes the murmur;typically, the click softens or disappears with inspiration.

As the stenosis becomes more severe, the systolic murmur peaks later in systole and the ejection click moves closer to the first heart sound

, eventually becoming virtually superimposed on it.

Tests used in the diagnosis of pulmonary stenosis may include:Chest x-ray :Post-stenotic dilatation of the main pulmonary artery and diminished pulmonary

vascular markings are evident on radiography. The cardiac silhouette is usually normal in size. An enlarged cardiac silhouette may be seen if the patient has right ventricular

failure or tricuspid regurgitation

ECG :In cases of moderate or severe pulmonary stenosis, the electrocardiogramshows right-axis deviation and right ventricular hypertrophy.Echocardiogram :

right ventricular hypertrophy and paradoxical septal motion during systole are evident.The site of obstruction can be visualized in most patients. With the use of D oppler flow studies,the severity of stenosis can usually be assessed,Cardiac catheterization:unnecessary


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TreatmentIn some cases, treatment may not be required.

Percutaneous balloon pulmonary dilation (valvuloplasty) has recentlybeen found quite successful as treatment for the form of pulmonary

valve stenosis that occurs without the presence of other heart defects.

S urgical repair of the defect (heart valve surgery) is usually performedwhen the child has reached preschool age. Oxygen may be required prior to surgery if symptoms are severe.

Medications used before surgery may include prostaglandins (PGE) tomaintain pulmonary blood flow, water pills to remove the excess fluid,anti-arrhythmics to improve the heart function, and blood thinners to prevent clots.

Outlook (Prognosis)

The outcome may be poor without surgical repair. The outcome is good with successful surgery.

Possible ComplicationsHeart failureRight ventricular hypertrophy (enlargement)


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COARCTATION OF AORTA1. 0.2-0.6 / 1000 live births2. 5-8 % of all congenital heartdisease3. associated congenital heartlesions

a. PDAb. VSDc. Bicuspid Aortic valved. Mitral valve stenosis

4. Congenital narrowing of upperdescending thoracic aorta5. Infolding of media, mostprominent opposite ductus > 50%narrowing needed to be significant6. 6.5% of CHD7. Isolated: M/F = 2:18. Coexisting anomalies: M/F =1:19. RAS plays important role inHTN


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Coarctation of the aorta


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S ymptomsS ymptoms depend on how much blood can flow through the artery.Other heart defects may also play a role. In severe cases, symptomsare seen when the baby is very young.In milder cases, symptoms may not develop until the child has reached adolescence.S ymptoms include :D izziness or faintingS hortness of breathPounding headacheChest painCold feet or legsNosebleedLeg cramps with exerciseHypertension (high blood pressure) with exerciseD ecreased ability to exerciseFailure to thrivePoor growthNote: There may be no symptoms


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CL A SSIFIC AT IO N OF CO A RC

1 . G roup I. isolated coarctation

2. G roup II. C oarctation and V SD

3 . G roup III. C oarctation and complex congenital heartdisease

4. O ther coarctationsa. pseudocoarctation : tortuous aorta but normal blood

flow surgery for compression or aneurysm only b. Abdominal coarctation : .5-2% of all coarcts

O LDE R C LA SS IFI C AT IO N1 . P reductal coarctation2. D uctal coarctation3 . P ostductal coarctation : most common in adults.

Diagnosis


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DiagnosisAsymptomatic mostly, sometimes

complain of intermittent claudicationHeadache and ventricular failureUsually manifests between 20-30

years of age and if uncorrected hi risk of mortality

Diagnosis


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DiagnosisPhysical Findings

marked development of the upper half of the body ascontrasted with the lower half.Normally, leg systolic blood pressure is 10-20 mm Hghigher than arm pressure. In patients with COARC, armsystolic blood pressure is much higher than leg pressure.

Simultaneous palpation of the brachial and femoral arteriesdemonstrates that the femoral pulse is delayed and of smaller amplitude.Auscultatory findings: early systolic ejection click associated with systolic, diastolic, or continuous murmursbest heard over the back in the vicinity of the fourth or fifththoracic vertebra either in the middle or just to the left of the spine.Continuous murmurs generated by turbulent flow indilated intercostal collateral vessels can be heard at variouslocations over the entire chest, but they are usually bestheard posteriorly. S3 and S4 are often encountered as well.


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E CG, CXR and Other Diagnostics

Electrocardiographic findings: LVH, Incomplete RBBB, Complete RBBB,Complete LBBB

Roentgenographic findings : Left ventricular enlargement,Rib notching,D ilated left sublclavian artery and aortic knob forming a silhouette of the numeral3Echocardiography demonstrates increased LV wall thickness and dilatation of the aortic root. Left ventricular and left atrial dilatation may be present. A bicuspidaortic valve may be observed. The coarctation itself is usually difficult to visualizewith transthoracic echocardiography in adults. However, the COARC can oftenbe visualized by transesophageal echocardiography. Largeamplitude carotidarterial pulsations often are recorded in patients with COARC.

Magnetic resonance imagingRadionuclide studies . No L-R shunt.Catheterization and angiography. No L-V shunt in uncomplicated COARC.

Left ventricular filling pressures may be elevated in patients with markedhypertrophy or failure. Aortography in the left anterior oblique projectionvisualizes the COARC and numerous dilated, tortuous collateral vessels. Abicuspid aortic valve can be identified by aortography.

Criteria for D iagnosis: HTN c higher systolic pressures in the arm than in the leg,a systolic or continuous murmur heard over the midthoracic spine, and ribnotching noted on the chest x-ray film and Aortography.


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M R A ngiography


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M R A , AN GIOGR AP HY


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CXR

LS A left S ubclavian arteryC O AR C coarctationD A D escending Aorta

White Arrows point to ribnotching, typical of C O AR C


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Exams and Tests

The health care provider will perform a physical examand take your blood pressure in your arms and legs.Your pulse will be checked.The pulse in the femoral (groin) area is weaker than the

carotid (neck) pulse.S ometimes, the femoral pulse may not be felt at all.

The doctor will use a stethoscope to listen to your heartand check for murmurs.People with aortic coarctation have a harsh-sounding

murmur that can be heard from the back.Other types of murmurs may also be present. A systolic murmur, caused by flow through collateralvessels, may be heard in the back.In about 30 percent of patients with aortic coarctation, asystolic murmur indicating an associatedbicuspid aortic valve is audible at the base.

Coarctation is often discovered during a newborn's firstexamination or a well-baby exam.Taking the pulses in an infant is an important part of theexaminationsince there may not be any other symptoms or findingsuntil the child is older.


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TreatmentS urgery is usually recommended. The narrowed part of the aorta will be removed or opened.

If the problem area is small, the two free ends of the aorta may be re-connected.This is called anastomosis. If a large part of the aorta was removed, a D acron graft (a syntheticmaterial) or one of the patient's own arteries is used to fill the gap. A tube graft connecting two parts of theaorta may also be used.

S ometimes, balloon angioplasty may be done instead of surgery.

Outlook (Prognosis)Coarctation of the aorta can be cured with surgery. S ymptoms quickly get better after surgery.

However, there is an increased risk for death due to heart problems among thosewho have had their aorta repaired. But, without treatment, most people die before age 40.

For this reason, doctors usually recommend that the patient has surgery before age 10.Most of the time, surgery to fix the coarctation is done during infancy.


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Possible Complications Aortic aneurysm Aortic dissection Aortic ruptureS evere hypertension

EndocarditisIntracerebral hemorrhageS trokeHeart failurePremature development of coronary artery

disease (CA D )Paraplegia (rare complication of surgery to repair

coarctation)Injury to the nerve to the larynxResidual narrowing of the aorta


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A trioventricular canal defect


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Atrioventricular canal defectThis is a combination of defects, including a large hole in the center of theheart and a single common valve instead of the separate tricuspid andmitral valves. Also called atrioventricular septal defect, this defect isclassified by whether it's only partial, involving only the upper chambers othe heart, or complete, in which blood can travel freely among all four chambers of the heart. Both forms allow extra blood to circulate to the

lungs, causing the heart to enlarge.The condition is often associated with D own syndrome. Infants may alsohave trouble breathing and not grow well. S urgery is often done in infancyto close the hole and reconstruct the valve s.


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Cyanotic ConditionsPatients with cyanotic congenital heart disease have arterialoxygen desaturation resulting from the shunting of systemicvenous blood to the arterial circulation.The magnitude of shunting determines the severity of desaturation. Most children with cyanotic heart disease

do not survive to adulthood without surgical intervention.In adults, the most common causes of cyanotic congenitalheart disease are tetralogy of Fallot61 and Eisenmenger'ssyndrome.


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T etralogy of Fallot


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Tetralogy of Fallot is classified as a cyanotic heart defectbecause the condition causes too little oxygen levels in the blood,which leads to cyanosis (a bluish-purple coloration to the skin).

The classic form of Tetralogy includes 4 defects within the heart structures:Ventricular septal defect (hole between the right and left ventricles)Narrowing of the pulmonary outflow tract (tube that connects the heart with the lungs)

An aorta (tube that carries oxygenated blood to the body) that grows from both ventricles,rather than exclusively from the left ventricle A thickened muscular wall of the right ventricle (right ventricular hypertrophy) At birth, infants may not show the signs of the cyanosis, but later may developsudden frightening episodes (called "Tet spells") of bluish skin from crying or feeding.

Tetralogy of Fallot occurs in approximately 5 out of 10,000 infants.

The cause of most congenital heart defects is unknown. Multiple factors seem to be involved.Prenatal factors associated with higher than normal risk for this condition include maternalrubellaor other viral illnesses during pregnancy, poor prenatal nutrition, maternal alcoholism,

mother over 40 years old, and diabetes.

There is a high incidence of chromosomal disorders in children with tetralogy of Fallot,such as D own syndrome and D i George's syndrome(a partial gene deletion that results in heart defects, low calcium levels, and immune deficiency.)

S ymptoms


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y pD ifficult feeding (poor feeding habits)Failure to gain weightPoor developmentCyanosis which becomes more pronounced during periods ofagitation

Passing outS udden deathClubbing of fingers (skin or bone enlargement around the finger nails)S quatting during episodes of cyanosis


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Exams and TestsPatients with tetralogy of Fallot have cyanosis and digital clubbing, the severity of which isdeterminedby the degree of obstruction of the right ventricular outflow tract. The peripheral pulses are

normal. A right ventricular lift or tap is palpable. In some patients, a systolic thrill (caused by turbulentflowacross the right ventricular outflow tract) is palpable. The first heart sound is normal,but the second heart sound is single, since its pulmonary component is inaudible. An aortic ejection click (due to a dilated, overriding aorta) may be heard.

A systolic ejection murmur, audible along the left sternal border, is caused by theobstruction of right ventricular outflow. The intensity and duration of the murmur are inverselyrelatedto the severity of the obstruction of right ventricular outflow; a soft, short murmur suggests that severe obstruction is present.

Tests may include:

EKG (electrocardiogram) may show the thickening of the right ventricle muscleCBC may show an increase in red blood cellsChest x-ray may show a "boot shaped" heart and dark lungsCardiac catheterization helps show blood vessels in the lungs and heartEchocardiogram provides a definite diagnos


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T ransposition of the great vessels


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D efinition

Transposition of the great vessels is a congenital heart defect in whichthe 2 major vessels that carry blood away from the heart -- the aorta and the pulmonary artery-- are switched (transposed)

Transposition of the great vessels is a cyanotic heart defect.This means there is too little oxygen in the blood that is pumped from the heart to the rest of the body.Low blood oxygen leads to cyanosis (a bluish-purple color to the skin) and shortness of

breath.

In normal hearts, blood goes through the lungs, then to the rest of the body, then back to thelungs again.In transposition of the great vessels, the blood does not travel from the lungs to the body and

back to the lungs again. Instead, blood flow in the lungs and blood flow in the body occursindependently.S o the blood with oxygen from the lungs does not get to the heart, where it feeds the rest of the body.The blood that goes through the body lacks oxygen.

S ymptoms appear at birth or very soon afterwards. How bad the symptoms are depend on thetype andsize of the heart defect and how much oxygen moves through the body's general blood flow.The condition affects approximately 40 out of 100,000 infants.

It is the most common cyanotic heart defect identified in the first week of life


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S ymptomsBlueness of the skinS hortness of breathPoor feedingClubbing of the fingers or toes

Exams and TestsThe health care provider may detect a heart murmur (holosystolic) while listening to thechest with a stethoscope.The baby's mouth and skin would be a blue color.

Tests often include the following:

Chest x-rayCardiac catheterization

ECGEchocardiogram (if done before birth, it is called a fetal echocardiogram)Pulse oximetry (to check blood oxygen level)


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Treatment

A medicine called prostaglandin will be immediately given to the baby. The medicineis given to the baby through an IV (intravenous line). This medicine helps blood flowthrough the lungs and body.

S urgery to temporarily adjust the affected blood vessels may be needed shortly after birth. In most hospitals, a type of surgery called an arterial switch procedure can beused to permanently correct the problem within the first week of life.

Outlook (Prognosis)

Improvement in symptoms and growth and development is seen after surgicalcorrection of the defect. If corrective surgery is not performed, the life expectancy isshortened.


ArrhythmiasHeart valve problemsCoronary artery disease


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Eb stein's Anomaly


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clinicsThe severity of the hemodynamic derangements in patients with

Ebstein's anomaly dependson the degree of displacement and the functional status of thetricuspid-valve leaflets.

Patients with mild apical displacement of the tricuspid leaflets havenormal valvular function,whereas those with severe tricuspid-leaflet displacement or abnormal anterior leaflet attachment, with valvular dysfunction, have elevated right atrial pressure andright-to-left interatrial shunting.

Similarly, the clinical presentation of Ebstein's anomaly varies fromsevere heart failure in a fetusor neonate to the absence of symptoms in an adult in whom it is

discovered incidentally

On physical examination the severity of cyanosis depends on the magnitude of right-to-left


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On physical examination , the severity of cyanosis depends on the magnitude of right to leftshuntingThe first and second heart sounds are widely split, and a third or fourth heart sound is oftenpresent,resulting in a "triple" or "quadruple" rhythm. A systolic murmur caused by tricuspid regurgitation is

usually present at the left lower sternal border. Hepatomegaly(resulting from passive hepatic congestion due to elevated right atrial pressure) may be present.EcgTall and broad P waves are common on the electrocardiogram, as is right bundle-branch block.First-degree atrioventricular block occurs frequently. S ince about 20 percent of patients with Ebstein's anomaly have ventricular preexcitation by way of an accessory

electrical pathway between the atrium and ventricle (WolffParkinsonWhite syndrome), a deltawave may be present.

x -rayThe radiographic findings depend on the severity of the anatomical abnormality.In mild cases, the heart size and pulmonary vasculature are normal.In more severe cases, marked cardiomegaly, which is largely due to right atrial enlargement, ispresent.In severe cases (with little functional right ventricle and marked right-to-left shunting), pulmonary

vascular markings are decreased.Echo&doppler Echocardiography is used to assess right atrial dilatation, anatomical displacement and distortion

of the tricuspid-valve leaflets, and the severity of tricuspid regurgitation or stenosis; in addition, the presence and magnitude of interatrial shunting can be determined(by color D oppler imaging or bubble study), as can the presence of associated cardiac

abnormalities.Electrophysiologic evaluation is warranted in patients with atrial tachyarrhythmias.


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E valuation possi b le congenital


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E valuation possi b le congenital heart

AB C sE xam : rate, rhythm, impulse, murmur,

pulses (brachial and femoral) O

xygen saturation (pre and postductal) AB GC hest xrayHyperoxia testE chocardiogram


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Increased pulmonary flow (Qp)

Atrial septal defect (minimal) Ventricular septal defectT ransposition

T runcusD ouble outlet right ventricleAtrioventricular canalP

atent ductus arteriosus


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T runcus arteriosus


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Hypoplastic left heart syndrome


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Hypoplastic left heart syndromeIn this condition, the left side of the heart is underdeveloped (hypoplastic),including the aorta, aortic valve, left ventricle and mitral valve.

As a result, the body doesn't receive enough oxygenated blood.In the first few days after a baby is born, the ductus arteriosus remainsopen(patent), allowing normal circulation, so the baby may seem fine initially.But when the ductus arteriosus naturally closes, signs and symptoms

begin,including a bluish cast to the skin from lack of oxygen, difficulty breathing

and poor feeding. This condition may be accompanied by an atrial septal defect.

Treatment options for this life-threatening condition are a heart transplantor a multistage surgical procedure done during the first few years of life.

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[LEC_OLESON] CHD