CONGENITAL HEART DISEASES

PRESENTED BY DONA MATHEW

MSC(N)GOVT.COLLEGE OF NURSING

KOTTAYAM1

Congenital heart defect (CHD)

Anatomic malformation of the heart or greatvessels which occurs during intrauterinedevelopment, irrespective of the age atpresentation.

Congenital heart disease occurs inapproximately 0.8% of live births.

The incidence is higher in stillborns (3-4%),spontaneous abortuses (10-25%), andpremature infants (about 2% excludingpatent ductusarteriosus [PDA]

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Causes of congenital heart defects

Environmental factors

Viral Infections

rubella during the first three months of pregnancy

Medication

lithium (used to manage bipolor disorder), Accutane(acne medication), some anti-seizure medications

Alcohol

with fetal alcohol syndrome (FAS)

Smoking

Cocaine

Maternal chronic illnesses –diabetes, phenylketonuria(PKU) and a deficiency in the B vitamin folic acid.

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Genetic factors

Heredity –occur in siblings or offspring ofindividuals with heart defects than thosewithout.

Mutations –can affect the formation of theheart and lead to congenital heartmalformations

Linked with other birth defects – More thanone-third of children born with Downsyndrome have heart defects. About 25% ofgirls with Turner syndrome have heart defects

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Classification of congenital heart disease

• Acyanotic

• Cyanotic

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ACYANOTIC

INCREASED PULMONARY BLOOD FLOW

ASD,VSD,PDA,

ATRIOVENTRICULAR

CANAL MALFORMATOION

OBSTRUCTION TO BLOOD

FLOW FROM VENTRICLES

COARCTATION OF AORTA,AORTIC

STENOSIS, PULMONARY

STENOSIS

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CYANOTIC

DECRESED

PULMONARY BLODD FLOW

TETRALOGY OF FALLOT. TRICUSPID ATRESIA

MIXED BLOOD FLOW

TRANS POSITION OF GREAT

ARTERIES,TOTAL ANOMOLOUS

PULMONARY VENOUS RETURN,TRUNCUS

ARTERIOSUS,

HYPOLPLASTIC LEFT HEART SYNDROME

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RELATIVE FREQUENCY OF MAJOR CONGENITAL HEART LESIONS

LESION % OF ALL LESIONS

Ventricular septal defect 35-30

Atrial septal defect (secundum) 6-8

Patent ductus arteriosus 6-8

Coarctation of aorta 5-7

Tetralogy of Fallot 5-7

Pulmonary valve stenosis 5-7

Aortic valve stenosis 4-7

d-Transposition of great arteries 3-5

Hypoplastic left ventricle 1-3

Hypoplastic right ventricle 1-3

Truncus arteriosus 1-2

Total anomalous pulmonary venous return

1-2

Tricuspid atresia 1-2

Single ventricle 1-2

Double-outlet right ventricle 1-2

Others 5-10 8

ACYANOTIC CONGENITAL HEART DISEASE

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DEFECTS WITH INCREASED PULMONARY BLOOD FLOW

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Atrial Septal Defect (ASD)

An opening in the atrial septum

An atrial septal defect allows oxygenated (red) blood to pass from the left atrium, through the opening in the septum, and then mix with unoxygenated (blue) blood in the right atrium

During fetal heart devt. The partitioning process does not occur completely, leaving an opening in the atrial septum

Occur in 4-10% of all infants w/ CHD

Effects: when blood passes through the ASD from the left atrium to the right atrium a larger volume of blood than normal must be handled by the right side of the heart extra blood then passes through the pulmonary artery into the lungs pulmonary hypertension and pulmonary congestion

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If the ASD is left uncorrected-->pulmonaryhypertension progresses -->pressure in the rightside of the heart will become greater than theleft side of the heart.

This reversal of the pressure gradient across theASD causes the shunt to reverse --> a right-to-left shunt will exist. This phenomenon is knownas Eisenmenger's syndrome

Once right-to-left shunting occurs, a portion ofthe oxygen-poor blood will get shunted to theleft side of the heart and ejected to theperipheral vascular system.

This will cause signs of cyanosis12

Atrial Septal Defect (ASD)

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Types of atrial septaldefects

Ostium secundum atrial septal defect most common type of atrial septal defect comprises 6–10% of all congenital heart

diseases. Opening near the center of the septum The secundum atrial septal defect usually arises

from an enlarged foramen ovale, inadequategrowth of the septum secundum, or excessiveabsorption of the septum primum.

If the ostium secundum ASD is accompanied byan acquired mitral valve stenosis, that is calledLutembacher's syndrome.

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Patent foramen ovale

A small channel that has some hemodynamic consequence

It is a remnant of the fetal foramen ovale.

On echocardiography, there may not be any shunting of blood noted except when the patient coughs.

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Ostium primum atrial septal defect

Opening at the lower end of the septum

A defect in the ostium primum is occasionallyclassified as an atrial septal defect but it ismore commonly classified an atrioventricularseptal defect. Ostium primum defects are lesscommon than ostium secundum defects

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Sinus venosus atrial septal defect

Opening near the junction of superior venacava and right atrium, may be associatedwith partial anomalous pulmonary venousconnection

Common or single atrium

It is a failure of development of theembryologic components that contribute tothe atrial septal complex. It is frequentlyassociated with heterotaxy syndrome.

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CLINICAL MANIFESTATIONS

History.

Infants and children with ASDs are usuallyasymptomatic

Physical Examination

A relatively slender body build is typical.

A widely split and fixed S2 and a systolic ejectionmurmur are characteristic findings of ASD in olderinfants and children. .

Classic auscultatory findings of ASD are notpresent unless the shunt is reasonably large

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Electrocardiography

Right axis deviation of +90 to +180 degreesand mild right ventricular hypertrophy (RVH)or right bundle branch block (RBBB) with anrsR' pattern in V1 are typical findings

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X-ray Studies

Cardiomegaly with enlargement of the RA andright ventricle (RV) may be present.

A prominent pulmonary artery (PA) segment andincreased pulmonary vascular markings are seenwhen the shunt is significant

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Echocardiography

A two-dimensional echo study is diagnostic.

shows the position as well as the size of thedefect,

In secundum ASD, a dropout can be seen in themidatrial septum.

The primum type shows a defect in the lower atrialseptum

Indirect signs of a significant left-to-right atrialshunt include RV enlargement and RAenlargement, as well as dilated PA

M-mode echo may show increased RV dimensionand paradoxical motion of the interventricularseptum, which are signs of RV volume overload.

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ASD on 2D echo

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ASD flow by colour Doppler

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Echocardiogram in apical four chamber view of primum

ASD

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CONTRAST ECHO

If agitated saline is injected into aperipheral vein during echocardiography,small air bubbles can be seen onechocardiographic imaging.

It may be possible to see bubbles travelacross an ASD either at rest or during acough. (Bubbles will only flow from rightatrium to left atrium if the RA pressure isgreater than LA).

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NATURAL HISTORY OF ASD spontaneous closure of the secundum defect

occurs in about 40% of patients in the first 4years of life

Most children with an ASD remain active andasymptomatic.

Rarely, congestive heart failure (CHF) candevelop in infancy.

If a large defect is untreated, CHF andpulmonary hypertension develop in adults whoare in their 20s and 30s

With or without surgery, atrial arrhythmias(flutter or fibrillation) may occur in adults.

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MANAGEMENT

Medical

Exercise restriction is unnecessary.

Prophylaxis for infective endocarditis indicatedin patients with primum ASD.

In infants with CHF, medical management isrecommended because of its high success rateand the possibility of spontaneous closure ofthe defect.

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Nonsurgical Closure of ASD

catheter-delivered closure device has becomea preferred method

Devices available for clinical use haveincluded the Sideris buttoned device, theAngel Wings device, the CardioSEAL device,and the Amplatzer ASD Occlusion Device.

The amplatzer septal occluder has thewidespread use.

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Use of the closure device may be indicated In

To close a secundum ASD, measuring 5 mmor more in diameter (but less than 32 mm),

A significant left-to-right shunt with clinicalevidence of right ventricular volume overload

There must be enough rim (4 mm) of septaltissue around the defect for appropriateplacement of the device.

The timing of the device- because of thepossibility of spontaneous closure, it is notused in infancy unless the patient issymptomatic with heart failure.

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Advantages of nonsurgical closure

Complete avoidance of cardiopulmonarybypass

Avoidance of pain and residual thoracotomyscars

A less than 24-hour hospital stay

Rapid recovery

All these devices are associated with a higherrate of small residual leak than is operativeclosure.

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Post-device closure follow-up

The patients are administered aspirin 81mg/day for 6 months.

Postprocedure echo studies

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Surgical ClosureIndications and Timing

A left-to-right shunt with a pulmonary-to-systemic blood flow ratio ( p/ s) of ≥1.5:1 .

Surgery is usually delayed until 2 to 4 years ofage because the possibility of spontaneousclosure.

Surgery is performed during infancy-if CHFdoes not respond to medical managementinfancy,.

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Infants with associated bronchopulmonarydysplasia and the device closure is notconsidered appropriate, surgery is performedduring infancy.

High pulmonary vascular resistance may be acontraindication for surgery

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Procedure.

For secundum ASD, the defect is traditionallyrepairedwith a simple suture or a pericardialor Teflon patch through a midsternal incisionunder cardiopulmonary bypass by either

For sinus venosus defect without associatedanomalous pulmonary venous return, thedefect is closed using an autologouspericardial patch.

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When it is associated with a pulmonaryvenous anomaly, a tunnel is created betweenthe anomalous pulmonary vein and the ASDby using a Teflon or pericardial patch

For coronary sinus ASD, the ostium of thecoronary sinus is closed with an autologouspericardium

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Mortality. Fewer than 0.5% of patients die

Complications. Cerebrovascular accident postoperative arrhythmias

Postoperative Follow-up 1. Cardiomegaly on x-ray film and enlarged RV

dimension on echo as well as the wide splitting ofthe S2 may persist for 1 or 2 years postoperatively.

The ECG typically demonstrates RBBB (or RVconduction disturbance).

2. Atrial or nodal arrhythmias occur in 7% to 20%of postoperative patients.

3. Rarely, patients with residual shunt may beadministered aspirin 81 mg to prevent paradoxicalembolization 41

Ventricular Septal Defect (VSD)

an opening in the ventricular septum

allows oxygenated blood to pass from the leftventricle, through the opening in the septum, andthen mix with unoxygenated blood in the rightventricle.

VSDs are the most commonly occurring type ofcongenital heart defect, occurring in 14-17 % ofbabies born each year.

occur when the partitioning process does not occurcompletely, leaving an opening in the ventricularseptum.

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EFFECTS:

When blood passes through the VSD from theleft ventricle to the right ventricle a largervolume of blood than normal must be handledby the right side of the heart extra blood thenpasses through the pulmonary artery into thelungs pulmonary hypertension and pulmonarycongestion pulmonary arteries becomethickened and obstructed due to increasedpressure

.

Ventricular Septal Defect (VSD)

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If VSD is not repaired, and lung disease begins to occur pressure in the right side of the heart will eventually exceed pressure in the left R to L shunt cyanosis

Due to high pressure --- tissue damage may eventually occur in the right ventricle bacteria in the bloodstream can easily infect this injured area bacterial endocarditis

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CLINICAL MANIFESTATIONS History

With a small VSD, the patient is asymptomaticwith normal growth and development.

With a moderate to large VSD, delayedgrowth and development, decreased exercisetolerance, repeated pulmonary infections, andCHF are relatively common during infancy.

With long-standing pulmonary hypertension,a history of cyanosis and a decreased level ofactivity may be present.

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Physical Examination Infants with small VSDs are well developed and

acyanotic. Before 2 or 3 months of age, infants with large

VSDs may have poor weight gain or show signsof CHF.

Cyanosis and clubbing may be present inpatients with Eisenmenger's syndrome

A systolic thrill may be present at the lower leftsternal border.

Precordial bulge and hyperactivity are presentwith a large-shunt VSD.

The S2 is loud and single in patients withpulmonary hypertension or pulmonary vascularobstructive disease.

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A grade 2 systolic murmur is audible at thelower left sternal border .It may beholosystolic or early systolic.

An apical diastolic rumble is present with amoderate to large shunt because of increasedflow through the mitral valve during diastole.

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Electrocardiography

With a small VSD, the ECG is normal.

With a moderate VSD, left ventricularhypertrophy (LVH) and occasional left atrialhypertrophy (LAH) may be seen.

With a large defect, the ECG showsbiventricular hypertrophy (BVH) with orwithout LAH

If pulmonary vascular obstructive diseasedevelops, the ECG shows RVH only

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VSD (6 month old child)

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X-ray Studies Cardiomegaly of varying degrees is present and

involves the LA, left ventricle (LV), and sometimes RV.

Pulmonary vascular markings increase.

The degree of cardiomegaly and the increase inpulmonary vascular markings directly relate to the

magnitude of the left-to-right shunt.

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Echocardiography

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NATURAL HISTORY Spontaneous closure occurs in 30% to 40% of

patients with membranous VSDs and muscularVSDs during the first 6 months of life.

CHF develops in infants with large VSDs butusually not until 6 to 8 weeks of age.

Pulmonary vascular obstructive disease maybegin to develop as early as 6 to 12 months ofage in patients with large VSDs, but the resultingright-to-left shunt usually does not develop untilthe teenage years.

Infective endocarditis rarely occurs.

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Medical Management

Treatment of CHF if it develops, (digoxinand diuretics for 2 to 4 months )

Addition of spironolactone may be helpfulto minimize potassium loss.

Concomitant use of an afterload-reducingagent, such as captopril,

Frequent feedings of high-calorieformulas, by either nasogastric tube ororal feeding, may help.

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Anemia, if present, should be corrected by oraliron therapy..

No exercise restriction is required in the absenceof pulmonary hypertension.

Maintenance of good dental hygiene andantibiotic prophylaxis against infectiveendocarditis are important

Nonsurgical closure of selected muscular VSDs ispossible using the “umbrella” device, but this isstill in the experimental stage.

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Surgical-Indications and Timing

Small infants who have large VSDs and developCHF and growth retardation - If growth failurecannot be improved by medical therapy, the VSDshould be operated on within the first 6 monthsof life.

if the PA pressure is greater than 50% ofsystemic pressure, surgical closure should bedone by the end of the first year.

After 1 year of age, a significant left-to-rightshunt with p/ s of at least 2:1 indicates thatsurgical closure is needed, regardless of PApressure.

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Surgical treatment

Palliative – pulmonary artery banding

Placing a band around the pulmonary arteryto decrease the pulmonary blood flow

It increases the resistance to blood flowthrough the pulmonary artery. Pressureincreases in the right ventricle and preventsexcess shunting from left to right

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Complete repair-small defects are repairedwith a purse-string approach.

Large defects usually require a KnittedDacron patch sewn over the opening

Both procedures require CPB

The repair is generally approached throughthe right atrium and tricuspid valve

Post operative complications include residualVSD and conduction disturbances

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Mortality.

Surgical mortality is less than 1%. Mortality ishigher for small infants younger than 2months of age, infants with associateddefects, or infants with multiple VSDs

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Postoperative Follow-up Activity should not be restricted unless complications

have resulted from surgery.

The ECG shows RBBB in 50% to 90% of patients who hadVSD repair through right ventriculotomy and up to 40%of the patients who had repair through a right atrialapproach.

Bacterial endocarditis prophylaxis may be discontinued6 months after surgery. If a residual shunt is present,endocarditis prophylaxis should be continued indefinitelywhen the indications arise.

A patient with a postoperative history of transient heartblock with or without pacemaker therapy requires long-term follow-up.

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Patent Ductus Arteriosus (PDA)

characterized by a connection between the aorta and the pulmonary artery

All babies are born with a ductus arteriosus.

As the baby takes the first breath, the blood vessels in the lungs open up, and blood begins to flow the ductus arteriosus is not needed to bypass the lungs

Most babies have a closed ductus arteriosus by 72 hours after birth.

In some babies, however, the ductus arteriosus remains open (patent) .

The opening between the aorta and the pulmonary artery allows oxygenated blood to pass back through the blood vessels in the lungs.

PDA occurs in 6-11 % of all children with CHD61

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In many children, there is no known reason for the ductusarteriosus remaining open. However, PDA is seen more often in the following:

premature infants

infants born to a mother who had rubella during the first trimester of pregnancy

EFFECTS:

PDA oxygenated blood passes from the aorta to the pulmonary artery & mixes w/ the unoxygenated blood w/c goes to the lungs blood volume to the lungs pulmonary hypertension & congestion

Further, because blood is pumped at high pressure through the PDA, the lining of the pulmonary artery will become irritated and inflamed. Bacteria in the bloodstream can easily infect this injured area bacterial endocarditis.

Patent Ductus Arteriosus (PDA)

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CLINICAL MANIFESTATIONS

History

Patients are usually asymptomatic when theductus is small.

A large-shunt PDA may cause a lowerrespiratory tract infection, atelectasis, andCHF (accompanied by tachypnea and poorweight gain).

Exertional dyspnea may be present inchildren with a large-shunt PDA

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Physical Examination

Tachycardia and tachypnea may be present ininfants with CHF.

Bounding peripheral pulses with wide pulsepressure are characteristic findings.

A systolic thrill may be present at the upper leftsternal border.

A grade 1 to 4/6 continuous (“machinery”)murmur is best audible at the leftinfraclavicular area or upper left sternalborder..

If pulmonary vascular obstructive diseasedevelops, a right-to-left ductal shunt results incyanosis only in the lower half of the body

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Electrocardiography. The ECG findings in PDA are similar to those in

VSD. A normal ECG or LVH is seen with small to

moderate PDA. BVH is seen with large PDA. If pulmonary vascular obstructive disease

develops, RVH is present.X-ray Studies.

X-ray findings are also similar to those of VSD. Chest x-ray films may be normal with a small-

shunt PDA. Cardiomegaly of varying degrees occurs in

moderate- to large-shunt PDA with enlargementof the LA, LV, and ascending aorta. Pulmonaryvascular markings are increased.

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Echocardiography

Its size can be assessed by two-dimensionalecho in a high parasternal view or in asuprasternal notch view

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NATURAL HISTORY

Spontaneous closure of a PDA does notusually occur in full-term infants andchildren. This is because the PDA in terminfants results from a structural abnormalityof the ductal smooth muscle

CHF or recurrent pneumonia or bothdevelop if the shunt is large.

Pulmonary vascular obstructive disease maydevelop if a large PDA with pulmonaryhypertension is left untreated.

Infective endocarditis may occur. Although rare, an aneurysm of PDA may

develop and possibly rupture in adult life.

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MANAGEMENT- IN TERM NEONATES

Medical

Indomethacin is ineffective in term infantswith PDA and should not be used.

Standard anticongestive measures withdigoxin and diuretics are indicated when CHFdevelops.

No exercise restriction is needed in theabsence of pulmonary hypertension.

Prophylaxis for subacute bacterialendocarditis (SBE) is indicated whenindications arise.

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Nonsurgical Closure. Small ductus less than 4 mm in diameter are

closed by coils

Larger ones by an amplatzer PDA device.

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SURGICAL CLOSURE Surgical closure is reserved for patients in whom

a nonsurgical closure technique is not consideredapplicable

Procedure

Ligation and division through left posterolateralthoracotomy without cardiopulmonary bypass isthe standard procedure.

The technique of video-assisted thoracoscopicclip ligation has become the standard of care forsurgical management of a ductus with adequatelength

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Patent Ductus Arteriosus in Preterm Neonates

Clinical evidence of PDA appears in 45% ofinfants with birth weight less than 1750 g andin about 80% of infants with birth weight lessthan 1200 g.

Significant PDA with CHF occurs in 15% ofpremature infants with birth weight less than1750 g and in 40% to 50% of those with birthweight less than 1500 g

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MANAGEMENT- IN PRE TERM NEONATES

Medical

Fluid restriction to 120 mL/kg per day and adiuretic (e.g., furosemide, 1 mg/kg, two to threetimes a day) may be tried for 24 to 48 hours

Pharmacologic closure of the PDA can heachieved with indomethacin (a prostaglandinsynthetase inhibitor).

The dose is given intravenously every 12 hoursfor a total of three doses. For infants less than 48hours old, 0.2 mg/kg is followed by 0.1 mg/kg2 times .

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For those 2 to 7 days old, 0.2 mg/kg times 3, andfor infants older than 7 days, 0.2 mg/kg followedby 0.25 mg/kg times 2

Contraindications to the use of indomethacin High blood urea nitrogen (>25 mg/dl) or creatinine

(>1.8 mg/dl) levels Low platelet count (<80,000/mm3) Bleeding tendency (including intracranial hemorrhage) Necrotizing enterocolitis, and hyperbilirubinemia.

A multicenter prospective study from Europeshowed that intravenous ibuprofen (10 mg/kg,followed at 24-hour intervals by two doses of 5mg/kg) starting on the third day of life was aseffective as indomethacin in closing the ductus inpreterm newborns..

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ATRIOVENTRICULAR CANAL DEFECT

Also known as endocardial cushion defects

They account for about 5 percent of all congenital heart disease, and are most common in infants with down syndrome.

(About 15 percent to 20 percent of newborns with down syndrome have atrioventricularseptal defects).

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Complete atrioventricularcanal (CAVC)

Complete atrioventricular canal (CAVC) is asevere defect in which there is a large hole inthe the septum that separates the left andright sides of the heart.

The hole is in the center of the heart, wherethe upper chambers and the lower chambersmeet.

In a child with a complete atrioventricularcanal defect, there is one large valve, and itmay not close correctly.

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Partial atrioventricularcanal defects The hole does not extend between the lower

chambers of the heart and the valves arebetter formed.

Partial atrioventricular canal is also calledatrioventricular septal defect, or AVSD.

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PATHOPHYSIOLOGY defect in the septum

blood to travel from the left side of the heart to the right side of the heart, or the other way around.

The oxygenated and unoxygenated blood being mixed up

The extra blood being pumped into the lung arteries makes the heart and lungs work harder and the lungs can become congested.

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CLINICAL MANIFESTATION

Dyspnea

MILD CYANOSIS

A newborn baby will show signs of heart failure such as edema, fatigue, wheezing, sweating and irregular heartbeat

CHARACTERSTIC MURMUR

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MANAGEMENT

Palliative-pulmonary artery banding

Complete repair –

Patch closure of septal defect

Reconstruction of AV valve tissue

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OBSTRUCTIVE DEFECTS

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Coarctation of the Aorta

Narrowing of the aorta

can occur anywhere, but is most likely to happen in the segment just after the aortic arch.

This narrowing restricts the amount of blood to the lower part of the body

occurs in about 8-11 % of all children with CHD

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EFFECTS:

The left ventricle has to work harder to try to move blood through the narrowing in the aorta left-sided heart failure

BP is higher above the narrowing, and lower below the narrowing.

Older children may have headaches from too much pressure in the vessels in the head, or cramps in the legs or abdomen from too little blood flow in that region.

The walls of the arteries may become weakened by high pressure spontaneous tears cause a stroke or uncontrollable bleeding.

risk for bacterial endocarditis.

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SYMPTOMATIC INFANTS History.

Poor feeding, dyspnea, and poor weight gainor signs of acute circulatory shock maydevelop in the first 6 weeks of life.

Physical Examination

Infants with COA are pale and experiencevarying degrees of respiratory distress.

Oliguria or anuria, general circulatory shock,and severe acidemia are common.).

Peripheral pulses may be weak and threadyas a result of CHF.

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A blood pressure differential may becomeapparent only after improvement of cardiacfunction with administration of rapidly actinginotropic agents.

The S2 is single and loud; a loud S3 gallop isusually present.

No heart murmur is present in 50% of sickinfants.

A nonspecific ejection systolic murmur isaudible over the precordium.

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Electrocardiography.

A normal or rightward QRS axis and RVH orright bundle branch block (RBBB) are present

X-ray Studies.

Marked cardiomegaly and pulmonary edemaor pulmonary venous congestion are usuallypresent

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The blue arrow to the actual coarctationand the green arrow to the post-stenotic dilation of the

descending aorta.

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Echocardiography

Two-dimensional echo and color flowDoppler studies usually show the site andextent of the coarctation.

In the suprasternal notch view, a thin wedge-shaped “posterior shelf” is imaged in theposterolateral aspect of the upperdescending aorta

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Natural History

About 20% to 30% of all patients with COA develop CHF by 3 months of age.

If it is undetected or untreated, early death may result from CHF and renal shutdown in symptomatic infants

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Management

Medical

In symptomatic neonates, PGE1 infusion shouldbe started to reopen the ductus arteriosus andestablish flow to the descending aorta and thekidneys during the first weeks of life.

Intensive anticongestive measures with short-acting inotropic agents (e.g., dopamine,dobutamine), diuretics, and oxygen should bestarted.

Balloon angioplasty can be a useful procedurefor sick infants in whom standard surgicalmanagement carries a high risk.

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Surgical

Indications and Timing

If CHF or circulatory shock develops early in life,surgery should be performed on an urgent basis.

Procedures

Resection and end-to-end anastomosis consistsof resecting the coarctation segment andanastomosing the proximal and distal aortas .

Subclavian flap aortoplasty consists of dividingthe distal subclavian artery and inserting a flap ofthe proximal portion of this vessel between thetwo sides of the longitudinally split aortathroughout the coarctation segment.

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With patch aortoplasty, the aorta is openedlongitudinally through the coarctationsegment and extending to the left subclavianartery, and the fibrous shelf and any existingmembrane are excised. An elliptic wovenDacron patch is inserted to expand thediameter of the lumen.

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ASYMPTOMATIC INFANTS AND CHILDREN

Management

Medical

Children with mild COA should be watchedclosely for hypertension in the arm or forincreasing pressure differences between thearm and leg.

Balloon angioplasty

A balloon-expandable stainless-steel stentimplanted concurrently with balloonangioplasty

An absorbable metal stent is in theexperimental stage

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Surgical

Indications and Timing

COA with hypertension in the upperextremities or with a large systolic pressuregradient equal to or greater than 20 mm Hgbetween the arms and the legs indicates thatelective surgical correction is necessarybetween the ages of 2 and 4 years.

Reduction of aortic diameter by 50% at thelevel of COA is also an indication for surgery.

Older children are operated on soon after thediagnosis is made.

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In asymptomatic children, surgery isperformed by age 4 to 5; late surgery mayincrease the risk of developing early essentialhypertension.

If severe hypertension, CHF, or cardiomegalyis present, surgery is performed at an earlierage.

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Surgical Procedures

Resection of the coarctation segment andend-to-end anastomosis

Occasionally, subclavian artery aortoplasty orcircular or patch grafts may be performed.

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AORTIC STENOSIS

Narrowing or stricture o the aortic valve

Resistance to blood flow in the left ventricle,decreased cardiac out put, left ventricularhypertrophy and pulmonary vascularcongestion

Valvular stenosis is the most common typeand is usually caused by malformed cusps

Sub valvular stenosis is a stricture caused bya fibrous ring below the normal valve

Supra valvular stenosis occurs infrequently

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Pathophysiology

A stricture in the aortic outflow tract -->resistance to ejection of blood from leftventricle -> extra work load of the leftventricle -> hypertrophy -> left ventricularfailure -> left atrial pressure increases ->increased pressure in the pulmonary veins ->pulmonary edema

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CLINICAL MANIFESTATIONS

History

Neonates with critical or severe stenosis of theaortic valve may develop signs of hypoperfusionor respiratory distress related to pulmonaryedema within days to weeks after birth.

Most children with mild to moderate AS areasymptomatic. Occasionally, exerciseintolerance may be present.

Exertional chest pain, easy fatigability, orsyncope may occur in a child with a severedegree of obstruction

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Physical Examination

Infants and children with AS are acyanoticand are normally developed.

blood pressure is normal in most patients, buta narrow pulse pressure is present in severeAS.

A systolic thrill may be palpable at the upperright sternal border, in the suprasternalnotch, or over the carotid arteries.

An ejection click may be heard with valvularAS.

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Newborns with critical AS may develop signsof reduced peripheral perfusion (with weakand thready pulses, pale cool skin, and slowcapillary refill)

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Electrocardiography.

In mild cases the ECG is normal. LVH with orwithout strain pattern may be present insevere cases

X-ray Studies

The heart size is usually normal in children,but a dilated ascending aorta or a prominentaortic knob may be seen occasionally invalvular AS, resulting from poststenoticdilatation.

Significant cardiomegaly does not developunless CHF occurs later

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Echocardiography

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NATURAL HISTORY

Chest pain, syncope, and even sudden death(1% to 2% of cases) may occur in childrenwith severe AS.

Heart failure occurs with severe AS during thenewborn period or later in adult life.

Mild stenosis becomes more severe with timein a significant number of patients

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MANAGEMENT

Medical

For critically ill newborns with CHF patients are stabilized before surgery

balloon valvuloplasty

use of rapidly acting inotropic agents and diureticsto treat CHF

intravenous infusion of PGE1 to reopen theductus.

Percutaneous balloon valvuloplasty is nowregarded as the first step in the managementof symptomatic neonates

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Surgical

Valvular AS

Closed aortic valvotomy, using calibrateddilators or balloon catheters withoutcardiopulmonary bypass, may be performedin sick infants if balloon valvuloplasty hasbeen unsuccessful or if it is not available

Aortic valve commissurotomy is usually triedif stenosis is the predominant lesion

Aortic valve replacement may be necessary ifAR is the predominant lesion

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Subvalvular AS- Excision of the membrane isdone for discrete subvalvular AS

Supravalvular AS- a reconstructive surgery isdone using aY-shaped patch

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Postballoon and Postoperative Follow-up

An annual follow-up examination is necessary for allpatients who have the aortic valve balloon procedure orsurgery in order to detect development of stenosis orregurgitation.

Anticoagulation is needed after a prosthetic mechanicalvalve replacement.

The International Normalized Ratio (INR) should bemaintained between 2.5 and 3.5 for the first 3 monthsand 2.0 to 3.0 beyond that time.

Low-dose aspirin (75 to 100 mg/day for adolescents) isindicated in addition to warfarin (American College ofCardiology, 2006).

After aortic valve replacement with a bioprosthesis andno risk factors, aspirin (75 to 100 mg), but not warfarin, isindicated.

Restriction from competitive, strenuous sports may benecessary for children with moderate residual AS or AR,or both 119

PULMONARY VALVE STENOSIS

Narrowing at the entrance of pulmonary artery

Resistance to blood flow causes right ventricular hypertrophy

Pulmonary atresia is the extreme form – total fusion of commissures and no blood flows to the lungs

rt . Ventricle may be hypoplastic

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Pathophysiology

PS--> Resistance to blood flow-->RVH

If Rt . Ventricular failure develops-->increase in rt, atrial pressure -->re-openingof foramen ovale-->shunting of un oxy. bloodin to the Lt.atrium--> systemic cyanosis

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Clinical manifestations

May be asymptomatic

Some had mild cyanosis

Newborn with severe narrowig- cyanotic

Characteristic murmur

Cardiomegaly on chest radiograph

Pt are at risk for infective endocarditis

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Management

Surgical

In infants trans ventricular valvotomy (brockprocedure)

In children pulmonary valvotomy with CPB

Non surgical treatment

Balloon angioplasty

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DEFECTS WITH DECREASED PULMONARY BLOOD FLOW

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Tetralogy of Fallot (TOF)

a complex condition of several congenital defects that occurdue to abnormal devt. of the fetal heart during the first 8weeks of pregnancy. These problems include the following:

1. ventricular septal defect (VSD)

2. Pulmonary valve stenosis

3. overriding aorta - The aorta sits above both the left andright ventricles over the VSD, rather than just over the leftventricle. As a result, oxygen poor blood from the rightventricle can flow directly into the aorta instead of into thepulmonary artery to the lungs.

4. Right ventricular hypertrophy - The muscle of the rightventricle is thicker than usual because of having to workharder than normal.

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EFFECTS:

If the right ventricle obstruction is severe, or if thepressure in the lungs is high a large amount ofoxygen-poor (blue) blood passes through the VSD, mixeswith the oxygen-rich (red) blood in the left ventricle, andis pumped to the body cyanosis

The more blood that goes through the VSD, the lessblood that goes through the pulmonary artery to thelungs oxygenated blood to the left side of theheart.

Soon, nearly all the blood in the left ventricle is oxygen-poor (blue). This is an emergency situation, as the bodywill not have enough oxygen to meet its needs.

Tetralogy of Fallot (TOF)

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CLINICAL MANIFESTATIONS

History

A heart murmur is audible at birth.

Most patients are symptomatic with cyanosisat birth or shortly thereafter. Dyspnea onexertion or hypoxic spells develop later, evenin mildly cyanotic infants.

Immediately after birth, severe cyanosis isseen in patients with TOF and pulmonaryatresia.

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Physical Examination

Varying degrees of cyanosis, tachypnea, andclubbing (in older infants and children) arepresent.

An RV tap along the left sternal border and asystolic thrill at the upper and mid-left sternalborders are commonly present (50%).

An ejection click that originates in the aortamay be audible

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Electrocardiography

Right axis deviation (RAD) (+120 to +150 degrees) is present in cyanotic TOF.

RVH is usually present

BVH may be seen in the acyanotic form. RAH is occasionally present.

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X- ray

Decreased pulmonary markings

Black lung fields

Boot shaped heart

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NATURAL HISTORY

Infants with acyanotic TOF gradually becomecyanotic.

Patients who are already cyanotic become morecyanotic as a result of the worsening condition ofthe infundibular stenosis and polycythemia.

Polycythemia develops secondary to cyanosis.

Hypoxic spells may develop in infants.

Growth retardation may be present if cyanosis issevere.

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HYPOXIC SPELL

Hypoxic spells are characterized by aparoxysm of hyperpnea (i.e., rapid and deeprespiration), irritability and prolonged crying,increasing cyanosis, and decreasing intensityof the heart murmur.

Hypoxic spells occur in infants, with a peakincidence between 2 and 4 months of age.

These spells usually occur in the morningafter crying, feeding, or defecation.

A severe spell may lead to limpness,convulsion, cerebrovascular accident, or evendeath.

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Treatment of the hypoxic spell

The infant should be picked up and held in aknee-chest position.

Morphine sulfate, 0.2 mg/kg administeredsubcutaneously or intramuscularly, suppressesthe respiratory center and abolishes hyperpnea

Oxygen is usually administered, but it has littledemonstrable effect on arterial oxygensaturation.

Acidosis should be treated with sodiumbicarbonate (NaHCO3), 1 mEq/kg administeredintravenously. The same dose can be repeated in10 to 15 minutes..

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MANAGEMENT OF TOF Medical educate parents to recognize the spell and know

what to do. Oral propranolol therapy, 0.5 to 1.5 mg/kg every

6 hours, is occasionally used to prevent hypoxicspells while waiting for an optimal time forcorrective surgery

Balloon dilatation of the right ventricular outflowtract and pulmonary valve, it is not widelypracticed,

Maintenance of good dental hygiene andpractice of antibiotic prophylaxis against SBE areimportant .

A relative iron deficiency state should bedetected and treated

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Surgical

Palliative Shunt Procedures

Classic Blalock-Taussig shunt, anastomosedbetween the subclavian artery and the ipsilateralPA, is usually performed for infants older than 3months because the shunt is often thrombosedin younger infants with smaller arteries

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Modified Blalock-Taussig (BT) shunt. AGore-Tex interposition shunt is placedbetween the subclavian artery and theipsilateral PA. This is the most popularprocedure for any age, especially for smallinfants younger than 3 months of age

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The Waterston shunt, anastomosed between the ascending aorta and the right PA, is no longer performed because of a high incidence of surgical complications

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The Potts operation

Anastomosed between the descending aortaand the left PA, is no longer performed

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Complete Repair Surgery

Usually done in the first year of life

Total repair of the defect is carried out undercardiopulmonary bypass

The procedure includes patch closure of theVSD, preferably through a transatrial andtranspulmonary artery approach

widening of the RVOT by division and/orresection of the infundibular tissue; andpulmonary valvotomy,).

The operative mortality for total correction ofTOF is 5%

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Tricuspid Atresia

In this condition, there is no tricuspid valve, therefore, no blood flows from the right atrium to the right ventricle.

Blood in right atrium foramen ovale left atrium and left ventricle aorta

There is complete mixing of the oxy. Blood and un oxy. blood

Tricuspid atresia defect is characterized by the following:

a small right ventricle

a large left ventricle

Small VSD and PDA

diminished pulmonary circulation

cyanosis - bluish color of the skin and mucous membranes caused from a lack of oxygen.

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CLINICAL MANIFESTATIONS History

Cyanosis is usually severe from birth. Tachypnea and poor feeding usually manifest. History of hypoxic spells may be present in infants

with this condition. Physical Examination

Cyanosis, either with or without clubbing, is always present.

A systolic thrill is rarely palpable when associated with PS.

A grade 2 to 3/6 holosystolic (or early systolic) murmur of VSD is usually present at the lower left sternal border

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Electrocardiography

LVH is usually present;

RAH or biatrial hypertrophy (BAH) iscommon.

X-ray Studies.

The heart size is normal or slightly increased,with enlargement of the RA and LV

Echocardiography.

Absence of the tricuspid orifice, markedhypoplasia of the RV, and a large LV can beimaged in the apical four-chamber view.

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MANAGEMENT

Initial Medical Management

PGE1 should be started in neonates with severecyanosis to maintain the patency of the ductusbefore planned cardiac catheterization orcardiac surgery.

The Rashkind procedure (balloon atrialseptostomy) may be performed as part of theinitial catheterization to improve the RA-to-LAshunt, especially when the interatrialcommunication is considered inadequate byecho studies.

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Surgical.

Most infants with tricuspid atresia requireone or more palliative procedures before aFontan-type operation

Ideal candidates for a Fontan-type operationare those who have normal LV function andlow pulmonary resistance

Stage I

Blalock-Taussig shunt, when PBF is small

This procedure results in the volume load onthe LV because the LV supplies blood to boththe systemic and pulmonary circulations.

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Damus-Kaye-Stansel and shunt operation

the aorta and pulmonary artery are joined using a patch (pink). The blue tube is known as a Modified Blalock-Taussig Shunt.

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Pulmonary artery banding. PA banding is rarely necessary for infants with CHF resulting from increased PBF. PA banding protects the pulmonary vasculature from developing pulmonary hypertension

Medical follow-up after stage I. Watch for:

Cyanosis (O2 saturation <75%)—cardiac catheterization or MRI to find out its cause.

Poor weight gain (CHF from too much PBF)—tightening of PA band may be necessary.

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Stage II (at 3 months or by 6 months).

Bidirectional Glenn operation (BDG).

also called bidirectional superiorcavopulmonary shunt

An end-to-side SVC-to-RPA shunt (alsocalled bidirectional superior cavopulmonaryshunt) can be performed by 2.5 to 3 monthsof age

This procedure satisfactorily increasesoxygen saturation

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The hemi-Fontan operation.

SVC is connected to PA.

Blood coming back from the upper body nowflows directly to the lungs without going throughthe heart

The Blalock-Taussig (BT) shunt is removed.

A patch is placed over the top part of the heart'sright upper chamber . This prevents blood fromthe upper body from entering the heart andblood from the lower body from entering thelungs. It also maintains a connection that is usedfor the final stage of the repair and greatlysimplifies the last operation.

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Medical follow-up after stage II. Watch for the following:

A gradual decrease in O2 saturation (<75%) may be caused by: .

Opening of venous collaterals

Pulmonary AV fistula (due to the absence of hepatic inhibitory factor)

Transient hypertension—1 to 2 weeks postoperatively—may use ACE inhibitors

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Stage III ( Fontan operation)—within 1 to 2 years after stage II operation

The whole premise of the Fontan operation isdirecting the entire systemic venous blood tothe pulmonary arteries without anintervening pumping chamber.

The Fontan operation is usually completedwhen the child is around 2 years of age. Thisprocedure can even be performed on infants.

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Postoperative Medical Follow-up

Patients should maintain a low-salt diet. Medications: Some patients need continued digoxin and diuretic

therapy. An angiotensin-converting enzyme (ACE) inhibitor is

generally recommended. Aspirin (or even warfarin) is used to prevent

thrombus formation in the RA. Patients should not participate in competitive,

strenuous sports. Antibiotic prophylaxis against SBE should be

observed when indications arise.165

MIXED BLOOD FLOW

Difference in the PA pressure and aortic pressure--> pulmonary congestion

Volume load in ventricles -->decresed cardiac out put

Relative desaturation of systemic blood,

Mixing of blood from pulmonary and systemic circulation within the heart chambers

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Transposition of the Great Arteries (TGA)

the aorta is connected to the right ventricle, and the pulmonary artery isconnected to the left ventricle

Oxygen-poor (blue) blood returns to the right atrium from the body passes through the right atrium and ventricle, into the misconnectedaorta back to the body.

Oxygen-rich (red) blood returns to the left atrium from the lungs passes through the left atrium and ventricle, into the pulmonary arteryand back to the lungs.

Other heart defects are often associated with TGA

- atrial or ventricular septal defect

- may be necessary in order for the infant with TGA to survive

- Allow mixing of blood – providing at least smaller amounts of oxygento the body

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CLINICAL MANIFESTATIONS

History

History of cyanosis from birth is alwayspresent.

Signs of congestive heart failure (CHF) withdyspnea and feeding difficulties developduring the newborn period.

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Physical Examination

Moderate to severe cyanosis is present,

The S2 is single and loud..

If CHF supervenes, hepatomegaly anddyspnea develop.

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Electrocardiography

There is a rightward QRS axis (i.e., +90 to+200 degrees).

Right ventricular hypertrophy (RVH) isusually present

Biventricular hypertrophy (BVH) may bepresent

Occasionally right atrial hypertrophy (RAH) ispresent.

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Echocardiography.

In the parasternal short-axis view, the “circleand sausage” appearance of the normal greatarteries is not visible. Instead, the greatarteries appear as “double circles”

3. In the apical and subcostal five-chamberviews, the PA arises from the LV, and theaorta arises from the RV.

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X-ray Studies

Cardiomegaly

An egg-shaped cardiac silhouette

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NATURAL HISTORY

Without surgical intervention, death occurs in 90% of patients before they reach 6 months

of age.

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MANAGEMENT Medical

Arterial blood gases and pH should beobtained and metabolic acidosis should becorrected

PGE1 infusion should be started to improvearterial oxygen saturation by reopening theductus.

Oxygen administration

A balloon atrial septotomy (Rashkindprocedure) may be performed to increase themixing by opening the atrial septum

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Surgical

An arterial switch procedure- performed infirst week of life

Transecting the great arteries andanastomosing the main pulmonary artery tothe proximal aorta and anastomosing theascending aorta to the proximal pulmonaryartery

The coronary arteries are switched fromproximal aorta to the proximal pulmonaryartery to create a new aorta

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Intra atrial baffle repairs –An intraatrial baffle is created to divert the venous blood to the mitral valve and pulmonary venous blood to the tricuspid valve using using patients atrialseptum or a prosthetic valve.

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Rastelli procedure-operative choice in patients with TGA,VSD, and severe pulmonicstenosis.

It involves closure of VSD with baffle->LV blood directed through VSD in to aorta

Pulmonic valve is then closed , and a conduit is placed from RV to PA

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Total Anomalous Pulmonary Venous Return

No direct communication between the pulmonary

veins and the left atrium

Drain anomalously into the systemic venous

tributeries or into right atrium

4 types

Supracardiac:( 50% of TAPVR ). The commonpulmonary venous sinus drains into the right SVC

Cardiac: (20% of TAPVR) .The commonpulmonary venous sinus drains into the coronarysinus

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Infracardiac:( 20% of TAPVR patients) Thecommon pulmonary venous sinus drains tothe portal vein, ductus venosus, hepatic vein,or inferior vena cava (IVC).

Mixed type: This type, which is acombination of the other types, accounts for10% of TAPVR patients

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TOTAL ANOMALOUS PULMONARY VENOUS

RETURN –Clinical ManifestationsHistory

CHF with gowth retardation

Frequent pulmonary infection

Mild cyanosis

Physical examination

Undernourished

Signs of CHF

Precordial bulge

ECG

RVH and occational RAH

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TOTAL ANOMALOUS PULMONARY VENOUS

RETURN –Clinical Manifestations

X- ray studies

Cardiomegaly

Kerley B lines

Snowmans sign/ figure of 8

Echo

A large RA and a small LA, with deviation of the atrial septum to the left and dilated PAs, are also present.

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MANAGEMENT

medical

Intensive anticongestive measures

Metabolic acidosis should be corrected,

Infants with severe pulmonary edema-shouldbe intubated and receive ventilator support

In some patients with pulmonaryhypertension, PGE1 can increase systemicflow by keeping the ductus open

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Surgical treatment

Supracardiac Type.

A large, side-to-side anastomosis is madebetween the common pulmonary venoussinus and the LA. The ASD is closed with acloth patch.

TAPVR to the Right Atrium.

The atrial septum is excised and a patch issewn in such a way that the pulmonaryvenous return is diverted to the LA . TheASD may have to be enlarged.

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TAPVR to the Coronary Sinus.

An incision is made in the anterior wall of thecoronary sinus (“unroofing”) to make acommunication between the coronary sinusand the LA. A single patch closes the originalASD and the ostium of the coronary sinus. Thisresults in the drainage of coronary sinus bloodwith low oxygen saturation into the LA

Infracardiac Type.

A large vertical anastomosis is made betweenthe common pulmonary venous sinus and theLA. The common pulmonary vein, whichdescends vertically to the abdominal cavity, isligated above the diaphragm

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Truncus Arteriosus The aorta and pulmonary artery start as a single blood vessel, which

eventually divides and becomes two separate arteries.

Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery.

Usually accompanied by a ventricular septal defect

EFFECTS:

oxygen-poor (blue) and oxygen-rich (red) blood mix back and forth through the ventricular septal defect.

This mixed blood then flows through the common truncal vessel. Some of it will flow to pulmonary artery and on to the lungs, and some of the mixed blood will go into the aortic branch and to the body.

The mixed blood that goes to the body does not have as much oxygen as normal, and will cause varying degrees of cyanosis

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CLINICAL MANIFESTATIONS

History

Cyanosis may be seen immediately afterbirth.

Signs of CHF develop within several days toweeks after birth.

History of dyspnea with feeding, failure tothrive, and frequent respiratory infections isusually present in infants.

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Physical Examination

Varying degrees of cyanosis and signs of CHFwith tachypnea and dyspnea are usuallypresent.

The peripheral pulses are bounding, with awide pulse pressure.

A systolic click is frequently audible at theapex and upper left sternal border.

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Electrocardiography.

BVH is present in 70% of cases

X-ray Studies.

Cardiomegaly is usually present

Echocardiography.

A large, single great artery arises from theheart .The type of persistent truncusarteriosus can be identified, and the size ofthe PAs can be determined.

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Medical

Vigorous anticongestive measures withdigitalis and diuretics

surgical

Corrective repair

Closing the VSD

Excising the pul. Arteries from aorta , andattaching them to the RV by means ofhomograft

Prognosis

Mortality greater than 10%

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Hypoplastic Left Heart Syndrome

HLHS occurs in 1% of all congenital heartdefects or 9% of such defects in critically illnewborns

Underdevelopment of the left side of the heartresulting in a hypoplastic left ventricle andaortic atresia

Most of the blood from LA Flows across PFOto RARVPA

The descending aorta receives blood from pdasupplying systemic blood flow

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Effects

PFO allows saturated blood from LA to mixwith desaturated blood from RA-->RV -->PA

from PA blood flows to lung and thenthrough ductus arteriosus in to the aorta andout f the body

The coronary and cerebral vessels receiveblood by retrograde flowthrough thehypoplastic ascending aorta

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CLINICAL MANIFESTATIONS

A neonate with HLHS becomes critically ill withinthe first few hours to the first few days of life.

Tachycardia, dyspnea, pulmonary crackles, weakperipheral pulses, and vasoconstrictedextremities are characteristic.

The patient may not have severe cyanosis buthas a grayish blue color of the skin with poorperfusion.

The S2 is loud and single.. The ECG almost always shows RVH. Chest x-ray films characteristically show

pulmonary venous congestion or pulmonaryedema

Arterial blood gas levels reveal a slightlydecreased Po2 and a normal Pco2.

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Therapeutic management

PGE1 infusion to maintain ductal patency

Surgical procedure

Several staged approach

1st stage- Norwood procedure

Anastomosis of main PA to the aorta to create anew aorta, placement of a shunt or inserting aconduit from the RV to PA to provide pulmonaryblood flow , and creation of a large ASD.

2nd stage-Bidirectional Glenn Shunt

Done at 6-9 months of age to relieve cyanosis and reduce overload in RV

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Final repair-Modified Fontan Procedure

Transplantation

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Ebstein's Anomaly

Ebstein anomaly is a congenital heartdefect in which the septal leaflet ofthe tricuspid valve is displaced towards theapex of theright ventricle of the heart.

The valve leaflets, however, are to a varyingdegree, attached to the walls and septum ofthe right ventricle.

There is subsequent 'atrialization' of a portionof the morphologic right ventricle

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Pathophysiology

The right ventricle is thus divided into 2 parts by the abnormal tricuspid valve

the 1st, a thin-walled “atrialized” portion, is continuous with the cavity of the right atrium;

the 2nd, often smaller portion consists of normal ventricular myocardium.

The right atrium is enlarged as a result of tricuspid valve regurgitation,

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In more severe forms of Ebstein anomaly, theeffective output from the right side of theheart is decreased due to a combination ofthe poorly functioning small right ventricle,tricuspid valve regurgitation, and obstructionof the right ventricular outflow tractproduced by the large, sail-like, anteriortricuspid valve leaflet.

The increased volume of right atrial bloodshunts through the foramen ovale (orthrough an associated atrial septal defect) tothe left atrium and produces cyanosis

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CLINICAL MANIFESTATIONS

History

In severe cases, cyanosis and CHF developduring the first few days of life.

Children with milder cases may complain ofdyspnea, fatigue, cyanosis, or palpitation onexertion.

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Physical Examination

Mild to severe cyanosis is present, as well asclubbing of the fingers and toes in older infantsand children.

Characteristic triple or quadruple rhythm isaudible.

Electrocardiography

1. Characteristic ECG findings of RBBB and RAHare present in most patients with this condition

x-ray Studies.

. In severe cases, an extreme cardiomegaly witha balloon-shaped heart and decreasedpulmonary vascular markings are present

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Echocardiography

The tricuspid valve leaflets are elongated, redundant, and dysplastic

A large RA, including the atrialized RV, and a small functional RV represent anatomic severity

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MANAGEMENT

Medical

In severely cyanotic newborns, intensivetreatment with mechanical ventilation,

PGE1 infusion

inotropic agents

correction of metabolic acidosis

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Surgical

Palliative procedures

Blalock-Taussig shunt

Starnes operation- a procedure to reduce the RV or RA

Classic Glenn anastomosis or its modification may be considered in severely cyanotic infants

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Definitive procedures

Two-ventricular repair

Danielson technique: For repair of thetricuspid valve

Carpentier technique: This repair alsoplicates the atrialized portion of the RV andthe tricuspid annulus

One-ventricular repair:

For patients with inadequate size of the RV,a Fontan-type operation is usuallyperformed in stages following the initialpalliative procedures such as bidirectionalGlenn operation

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Nursing diagnosis

Impaired gas exchange related to altered pulmonary blood flow or oxygen deprivation

Altered cardiac output related to specific anatomic defect

Activity intolerance related to decreased oxygenation in blood and tissues

Altered Nutrition: less than body requirements related to the excessive energy demands required by increased cardiac workload

Increased potential for infection related to poor nutritional status

Anxiety related to diagnostic procedures and hospitalization

Developmental delay related to decreased energy, inadequate nutrition, physical limitations and social isolation

Alteration in parenting related to parental perception of the child as vulnerable

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NURSING INTERVENTIONS

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Relieve the respiratory distress associated with increased pulmonary blood flow or oxygen deprivation Determine degree of respiratory distress Position child at 45 degree angle to decrease pressure of the

viscera on the diaphragm and increase lung volume Pin diapers loosely and provide loose-fitting pajamas for older

children Feed slowly Tilt infant’s head slightly Suction the nose and throat if unable to cough out secretions Provide oxygen therapy as needed Improve oxygenation o that the body functions may be

maintained Provide effective oxygen environment Observe response to oxygen therapy Observe response to oxygen weaning therapy

Relieve Hypoxic spells associated with cyanotic types of Congenital heart disease

Observe for “tet” spells Encourage fluid intake Obtain vital signs

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Nursing interventions

A. Provide adequate nutritional and fluidintake to maintain the growth anddevelopmental needs of the child

Feed in semi-erect position

Provide small frequent feedings

Provide foods with high nutritional value

Determine child’s likes and dislikes

Strict input and output

Daily weight

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B. Prevent infection

Prevent exposure to communicable diseases

Immunizations should be up-to-date

Handwashing should be observed

Be certain that the child receives prophylactic medication for infective endocarditis

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C. Reduce the workload of the heart since decreased activity and expenditure of energy will decrease oxygen requirements

Uninterrupted rest

Avoid unnecessary activities

Prevent excessive crying

Provide diversional activities

Prevent constipation

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D. Observe child for symptoms of CongestiveHeart Failure that occur frequently as acomplication of Congenital Heart Disease

E. Observe for the development of symptoms ofinfective endocarditis that may occur as acomplication of congenital heart disease

F. Observe for the development of thrombosisthat may occur as a complication of congenitalheart disease

G. Prepare the child for diagnostic and treatmentprocedures

H. Explain cardiac problems to child and parents

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Health Education

A. Instruct the family in necessary measures to maintain the child’s health

B. Teach the family about the defect and its treatment

C. Encourage the parents and other persons to treat child in a normal manner as possible

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