Clinicopathologic conference- Ventricular septal defect

8/14/2019 Clinicopathologic conference- Ventricular septal defect

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khaikwan, nophawanlariosa, michael jeromele, manliao, karen isabellibres, kimberlypandac, therese rondellpangan, efrea lyndee

perocho, zichri kerensantos, edilberto de belenthanathada, sunklangtonagaya, sarahyee, ma. stephaniezamora, mary rose sugar



CASE 1

During a routine athletic physical, a 15year old boy is found to have a systolicthrill that is palpable at the lower left

sternal border accompanied by a harsh,pansystolic murmur that is heard best atthe site of the thrill. He is asymptomaticand has no evidence of hypertension,

cyanosis or edema. An electrocardiogramand chest radiograph are normal.



Clinical Scenario

Male, 15 year old

Presence of systolic thrill palpable atthe lower left sternal border

Accompanying sign: harsh, pansystolicmurmur heard best at site of thrill

Asymptomatic

No hypertension, cyanosis or edema ECG Normal

Chest radiography Normal



Position of the Heart



Pulmonary and Systemic

Circulation



Pericardium

The pericardium is the set of membranesaround the heart. It is actually composedof three layers of membranes.

Visceral pericardium- the innermost Parietal pericardium- the middle,

Fibrous pericardium- the outer one is theextra one and is tough.

Pericardial Activity- tiny space betweenthe visceral pericardium and the parietalpericardium.



Chambers of the Heart



Valves of the Heart

4 valves

One way flow

Leaky valve=heart murmur

2 atrioventricular valves

>Left AV valve-bicuspid or mitral

>Right AV valve-tricuspid

2 semilunar valves>Pulmonic semilunar valve

>Aortic Semilunar valve



Valves of the Heart



Atrioventricular Valves

1. Right AV valve

Between the right atrium and right ventricle

Also called the tricuspid valve because it has threecusps

Cusps close when right ventricle contracts…preventingblood from going back up into the right atrium

2. Left AV valve

Between the left atrium and the left ventricle

Also called the bicuspid valve because it only has 2

cusps Also called the mitral valve

Cusps close when left ventricle contracts…preventingblood from back-up into the left atrium



Semilunar Valves

1. Pulmonary semilunar valve

When right ventricle contracts,

blood is forced through this valve toenter pulmonary trunk.

2. Aortic semilunar valve

When left ventricle contracts, bloodis forced through this valve to enterthe aorta.



Cardiac Conduction

System The cardiac conduction system

generates and transmits impulsesthat stimulate contraction of themyocardium.

Under normal circumstances, theconduction system first stimulate the

contraction of the atria and then theventricles.



Cardiac Cycle

Refers to the events of one complete heartbeat. The length of the cardiac cycle is usually about0.8 sec.

Systole (contraction of the muscle)- there isventricular pumping, the chambers of the heartbecome smaller as the blood is ejected. Occurssecondary to the depolarization of cells.

Diastole (relaxation of the muscle)- there isventricular filling, the heart chambers fill withblood in preparation for subsequent ejection.



Cardiac Output

Volume of blood ejected per minute

-Each ventricle ejects approximately

70ml of blood/beat Averages between 4-8L/min

CO=Stroke volume x Heart rate

=70ml x 60 beats/min=4,200 ml/min



Anatomy of the

Interventricular Septum The interventricular septum is the wall of the heart intermediate to

the right and left ventricles. Its surface markings correspond to theanterior and posterior interventricular grooves. It runs obliquely tothe left and slightly inferiorly from posterior to anterior. Due to thisangulation, the right ventricle tends to lie anteriorly and the leftventricle posteriorly.

Throughout most of its surface area, the septum is as muscular asthe left ventricle. It tends to bulge into the chamber of the rightventricle producing a concavity on the left ventricular side. Nearerto the aortic valve orifice beneath the margins of the right andposterior leaflets, the septum becomes thinner and more fibrous. This region is termed the pars membranacea septi, or themembranous part of the interventricular septum. It is oval in shape.

The muscular and membranous parts of the interventricularseptum have different developmental origins. The membranouspart of the septum is the most frequent site for ventricular septaldefects which are usually congenital.



a. Patent ductus arteriosus (PDA)b. Tetralogy of fallotc. Tricuspid regurgitation

Ventricular septal defect Patent ductus arteriosus Tetralogy of fallot Tricuspid regurgitationMurmur Timing Primary four malformations Murmur



Murmur

Location: 3rd, 4th and 5th left

interspaces

Radiation: often wide

Intensity: often very loud, with

a thrill

Pitch: high holosystolic

Quality: often harsh

Timing

Continuous murmur in

both systole and diastole,

often with a silent interval

late in diastole. Loudest in

late systole, obscures S2, and

fades in diastole.

Primary four malformations

•Pulmonary stenosis

•Overriding aorta

•Right ventricular hypertrophy

•Ventricular septal defect(VSD)

Murmur

Location: lower left sternal

border

Radiation: to the right of

the sternum, to the xiphoid

area, and perhaps to the left

midclavicular line, but not the

axilla

Intensity: variable Pitch: medium

Quality: blowing

holosystolic

Aids: unlike mitral

regurgitation, the intensity

may increase slightly with

inspiration.

Associated findings

•

S2 may be obscured by the loudmurmur

• Findings vary with the severity of

the defect and with associated

lesions

Location

Left 2nd

interspaceRadiation

Toward the left clavicle

Intensity

Usually loud, sometimes

associated with a thrill

Quality

Harsh, machinery-like

Pitch

Medium

Additional anomalies

1.stenosis of the left pulmonary artery,in 40% of patients

2.a bicuspid pulmonary valve, in 40% of patients

3.right-sided aortic arch, in 25% of patients

4.coronary artery anomalies, in 10% of patients

5.a foramen ovale or atrial septaldefect, in which case the syndrome issometimes called a pentalogy of

Fallot6.an atrioventricular septal defect

7.partially or totally anomalouspulmonary venous return

8.forked ribs and scoliosis

Associated findings

•

the right ventricle impulse isincreased in amplitude and

may be sustained.

•An S3 may be audible along

the lower left sternal boarder.

The jugular venous pressure is

often elevated, with large v

waves in the jugular veins.

Mechanism

A VSD is a congenital

abnormality in which blood flows

from the relatively high-pressure

left ventricle into the low-pressureright ventricle through a hole.

Mechanism

Low oxygenation of blood due to the

mixing of oxygenated and

deoxygenated blood in the left ventricle

via the VSD and preferential flow of themixed blood from both ventricles

through the aorta because of the

Mechanism

When the tricuspid valve

fails to close fully in systole,

blood regurgitates from the

right ventricle to right atrium,producing a murmur. The most

common cause is right



Ventricular septal

defect• A ventricular septal defect (VSD) is a defect inthe ventricular septum, the wall dividing the left andright ventricles of the heart.

• The ventricular septum consists of an inferior muscularand superior membranous portion and is extensively

innervated with conducting cardiomyocytes. Themembranous portion, which is close tothe atrioventricular node, is most commonly affected inadults and older children.

• Congenital VSDs are collectively the mostcommon congenital heart defects.



Diagnosis

• Detected by cardiac auscultation(due topathognomonic holo- or pansystolic murmur)-- murmur depends on the abnormal flow of blood from the left ventricle, through the

VSD, to the right ventricle.• Confirmation of cardiac auscultation can be

obtained by non-invasivecardiac ultrasound (echocardiography).

• More accurately measure ventricularpressures-cardiac catheterization.



PDA



Patent ductus arteriosus

heart condition that is normal but reverses soon after birth.

Persistent PDA, there is an irregular transmission of bloodbetween two of the most important arteries in close proximity tothe heart.

Ductus arteriosus normally seals off within a few days, in PDA,the newborn's ductus arteriosus does not close but remainspatent.

common in neonates with persistent respiratory problems suchas hypoxia, and has a high occurrence in premature children. Inhypoxic newborns, too little oxygen reaches the lungs to produce

sufficient levels of bradykinin and subsequent closing of the DA. Premature children are more likely to be hypoxic and thus have

PDA because of their underdeveloped heart and lungs.



MOA of PDA

allows a portion of the oxygenated blood from the leftheart to flow back to the lungs (following the pressuregradient from the higher pressure aorta to thepulmonary arteries).

If this shunt amount is substantial, the neonate becomesshort of breath because there is not only the normalamount of unoxygenated blood that has returned fromthe body to go to the lungs but in addition there is theamount shunted through the PDA.

The neonate's work of breathing is increased, using upmore calories and often interfering with feeding ininfancy. This condition as a constellation of findings iscalled congestive heart failure.



In some cases, such as in transposition of the greatvessels (the pulmonary artery and the aorta), aPDA may need to remain open. In this

cardiovascular condition, the PDA is the only waythat oxygenated blood can mix with deoxygenatedblood. In these cases, prostaglandins are used tokeep the patent ductus arteriosus open.

Exemptions:



Diagnosis

Usually diagnosed using non-invasive techniques.

Echocardiography, in which sound waves are used to capturethe motion of the heart, and

Associated Doppler studies are the primary methods of detecting PDA.

Electrocardiography (ECG), in which electrodes are used torecord the electrical activity of the heart, is not particularlyhelpful as there are no specific rhythms or ECG patterns whichcan be used to detect PDA.

Chest X-ray may be taken, which reveals the overall size of neonate's heart (as a reflection of the combined mass of the

cardiac chambers) and the appearance of the blood flow to thelungs ---small PDA most often shows a normal sized heart andnormal blood flow to the lungs --- large PDA generally shows anenlarged cardiac silhouette and increased blood flow to thelungs.



Tetralogy of Fallot

Accounting for about 5% of all congenitalcardiac malformations

is the most common cause of cyanotic

congenital heart disease The four features of the tetralogy are

(1)VSD,

(2) obstruction to the right ventricular

outflow tract (subpulmonic stenosis),(3) an aorta that overrides the VSD, and

(4) right ventricular hypertrophy



Clinical features

- right-to-left shunting,

- decreased pulmonary blood flow, and increased aorticvolumes.

- If the pulmonic obstruction is mild, the conditionresembles an isolated VSD, because the high left-sidedpressures on the left side cause a left-to-right shunt with nocyanosis.

- , marked stenosis causes significant right-to-leftshunting and consequent cyanosis early in life.

- As patients with tetralogy grow, the pulmonic orificedoes not enlarge, despite an overall increase in the size of theheart.

- patients develop erythrocytosis with attendanthyperviscosity, and hypertrophic osteoarthropathy;

- the right-to-left shunting also increases the risk forinfective endocarditis, systemic emboli, and brain abscesses.



Tricuspid Regurgitation

In this condition, the valve doesnot close properly and blood flows

back into the right atrium.



CAUSE



CAUSE

Right ventricle enlarges and Increase resistance of blood flow from the right ventricle to the lungs

Resistance may be increased by a severe, long-standing lung disorder

Emphysema

Pulmonary Hypertension pulmonic stenosis

left side of the heart disorder (such as mitral valvestenosis)

To compensate, the right ventricle enlarges, stretchingthe tricuspid valve and causing regurgitation.



Weakness and fatigue.

Pulsations in the neck from theelevated Right Atrial pressure

Discomfort in the right upper part of the abdomen due to an enlarged liver

Heart failure results in accumulation

of fluid in the body, mainly in the legs

Signs and Symptoms



Physical Diagnosis

Echocardiography

Chest x-ray



Morphology

- heart is large and "boot shaped" as a result of rightventricular hypertrophy

- the proximal aorta is typically larger than normal,with a diminished pulmonary trunk.

- The left-sided cardiac chambers are normal sized,

while the right ventricular wall is markedly thickened andmay even exceed that of the left.

- The VSD lies in the vicinity of the membranousportion of the interventricular septum, and the aortic valvelies immediately over the VSD.

- The pulmonary outflow tract is narrowed, and, in a

few cases, the pulmonic valve may be stenotic. - Additional abnormalities are present in many cases,

including PDA or ASD; these are actually beneficial inmany respects, because they permit pulmonary bloodflow.



Three Major Categories:

Malformations causing a left-to-rightshunt

Malformations causing a right-to-leftshunt

Malformations causing anobstruction



A.) Left-to-right shunts

Atrial Septal Defect

Ventricular Septal Defect

PDA (Patent Ductus Arteriosus) Arterioventricular Septal Defect




Three major types:

Secumdum ASD – 90% of most cases

Primum ASD – 5% of anomalies Sinus venosus



Ventricular Septal

Defect



Ventricular Septal

Defect Membranous VSD – 90% involves themembranous septum

Infundibular VSD – lies below thepulmonary valve

Swiss-cheese septum – multiplemuscular VSD



PDA (Patent Ductus

Arteriosus)



Arterioventricular

Septal Defect



Arterioventricular

Septal Defect Partial AVSD – consist of primum ASDand a cleft anterior mitral leaflet

Complete AVSD – large combined AVseptal defect and a large commonAV valve



B.) Right-to-left shunts

(cyanotic) Tetralogy of Fallot

Transposition of the Great Arteries

Persistent Truncus Arteriosus Tricuspid Atresia

Total Anomalous Pulmonary VenousConnection



Tetralogy of Fallot



Tetralogy of Fallot

Four features of the Tetralogy of Fallot:

VSD

Obstruction to the right ventricularoutflow tract (subpulmonarystenosis)

Aorta that overrides the VSD

Right ventricular hypertrophy



Transposition of the

Great Arteries



Truncus Arteriosus



Tricuspid Atresia



C.) Obstructive

Congenital Disease Coarctation of the Aorta

Pulmonary Stenosis and Atresia

Aortic Stenosis and Atresia



Coarctation of the Aorta



Aortic Stenosis and

Atresia



Natural History

Small (& even some larger)

lesions often close spontaneously either by

muscular growth or plugging with tricuspidvalve tissue. Larger lesions will eithercause problems due to the size of theshunt (failure to thrive & recurrent chest

infections) or cause irreversible pulmonaryvascular disease. Life expectancy andexercise capacity will therefore berestricted.



Congenital VSDs are frequently associated

with other congenital conditions, such asDown syndrome.

A VSD can also form a few days after amyocardial infarction (heart attack) due tomechanical tearing of the septal wall,before scar tissue forms, when

macrophages start remodelling the deadheart tissue.

http://en.wikipedia.org/wiki/File:Heart_right_vsd.jpg



Epidemiology and

Etiology VSDs are the most common

congenital cardiac anomalies. They are found in

30-60% of all newborns with a congenital heartdefect, or about 2-6 per 10,000 births. It isdebatable whether all those defects are trueheart defects, or if some of them are normalphenomena, since most of the trabecular VSDsclose spontaneously. Prospective studies give aprevalence of 2-5 per 100 births of trabecularVSDs that closes shortly after birth in 80-90% of the cases.

http://en.wikipedia.org/wiki/File:Heart_right_vsd.jpg



Prognosis

Transcatheter closure has been undertakenfor muscular defects and a device has just

become available for perimembranousdefects and is under evaluation. This isexcellent for most patients. The vastmajority are able to live a normal and

unrestricted life. Re-operations for residualVSDs are now uncommon.



Congenital Heart

defects(VSD) Caused by developmental abnormalities. However the genes involved in these defects

have been identified in only a minority of conditions.

Well defined genetic or environmentalinfluence are identifiable in only 10% of thecases of congenital heart ds (VSD…).

There is an association of congenital cardiacmalformations with certain chromosomal

abnormalities: trisomies 13, 15, 18 and 21and the Turner Syndrome.



VSD

Mutation of the gene that encodes thetranscription factor TBX5 has been shown tocause VSD and ASD observed in Holt-Oramsyndrome which is a rare condition associated

with heart, arm and hand defects. Developmental errors in mesenchymal tissue

migration: anomalies of outflow tract, failure of fusion and failure of septation.

Other mechanisms include extracellular matrix

abnormalities and situs and looping defects andendocardial cushions defects.



S d b th l ft



Second, because the leftventricle normally has amuch higher systolicpressure (~120 mm Hg) thanthe right ventricle (~20 mmHg), the leakage of bloodinto the right ventricletherefore elevates rightventricular pressure andvolume, causing

pulmonary hypertension with

Thi ff t i

http://en.wikipedia.org/wiki/Pulmonary_hypertension

http://en.wikipedia.org/wiki/Pulmonary_hypertension



This effect is morenoticeable in patients withlarger defects, who maypresent withbreathlessness, poorfeeding and failure tothrive in infancy. Patientswith smaller defects may beasymptomatic.



Symptoms

VSD is an acyanotic congenitalheart defect, aka a Left-to-rightshunt, so there are no signs of cyanosis.

V t i l t l



Ventricular septal

defect is usuallysymptomless at birth. Itusually manifests a few

weeks after birth.

Si



Signs

Pansystolic /Holosystolic murmur(depending upon the size

of the defect)

CLINICAL MANIFESTATIONS



CLINICAL MANIFESTATIONS

T b i t f VSD



Two basic types of VSD

Perimembranous VSD - an opening inthe upper section of the ventricularseptum, near the valves, occurs in 75%of all VSD cases.

Muscular VSD - an opening in the lowersection of the ventricular septum

occurs in up to 20% of all VSD cases.



NORMAL HEART SOUND

CLICK ME!!!

VENTRICULAR SEPTAL DEFECTSOUND

CLICK ME TOO!!!

P t ti



Presentation The size of the ventricular septal opening will

affect the type of symptoms noted, theseverity of symptoms, and the age at whichthey first occur. A VSD permits extra blood topass from the left ventricle through to the

right side of the heart, and the right ventricleand lungs become overworked as a result.

The larger the opening, the greater theamount of blood that passes through and

overloads the right ventricle and lungs.

M t t f



Most common symptoms ofVSD fatigue sweating rapid breathing

heavy breathing congested breathing disinterest in feeding, or tiring while

feeding poor weight gain

Signs of VSD



Signs of VSD

Murmur- due to the sound of bloodas it passes between the left andright ventricles.

Thrill- palpable murmur



VSD will produce a

systolic murmur. Theclassic murmur is grade¾, holosystolic, harshand blowing incharacter.It will peak in

midsystole. It is bestheard along the lowerleft sternal border andapex. Smaller defects of small and mediumdefects will typically

produce a louder soundthan a larger defect asthe blood velocity ishigher.

Small left to right



Small left-to-rightshunt 1st heart sound at the apex is normal;

2nd sound at pulmonary area is splitphysiologically

Grade 2-4/6,medium-to high-pitched,harsh pansystolic murmur heard bestat the left sternal border in the 3rd

and 4th

ICS

Moderate left to right



Moderate left-to-rightshunt Slight prominence of precordium

Moderate left ventricular heave

Systolic thrill palpable at the lowerleft sternal border between the 3rd and 4th ICS

Grade 3-4/6, harsh pansystolic

murmur

Large ventricular septal



Large ventricular septaldefects w/ pulmonary

hypertension Precordium is prominent, sternum bulges

Left ventricular thrust and rightventricular heave are palpable

Thrill may or may not be present at thelower left sternal border

S2 usually single or narrowly split, w/

acdentuation of pulmonary component

Harsh, pansystolic murmur



SIMPLE RECALL



The most common formsare in the muscular portionof the septum where they



of the septum where theymay lie posterior (1),apically (2) or anteriorly(3). The perimembranous

(4) form is the next mostcommon. It may extendposteriorly in the septum(inlet) or anteriorly towardsthe aortic valve (subaortic).More rarely (except inAsians) it is situated below

both the aortic andpulmonary valves doublycommitted (5).



The diagnosis of a VSD is usually



suspected clinically by hearing acharacteristic heart murmur. A murmuris a sound generated by abnormallyturbulent flow of blood through theheart. This murmur is the result of blood

being shunted through the VSD from thehigher-pressure left ventricle into thelower-pressure right ventricle.

The loudness of the murmur is relatedto the size of the defect and amount of blood crossing the defect.

Chest x-ray

http://www.medicinenet.com/script/main/art.asp?articlekey=3673

http://www.medicinenet.com/script/main/art.asp?articlekey=3673



yChest X-ray looks to see if there is a large heart with fluid

in the lungs.

Possible result: Small defects have a normal chest X-ray. Large defects with a big shunt have cardiomegaly and

pulmonary plethora.

A chest X-ray can also help follow the progression of

congestive heart failure by looking at the size of theheart and the amount of blood flow to the lungs. Thismay be normal at birth and change with time.

Electrocardiogram-- ECG shows signs of an enlarged left ventricle



ECG shows signs of an enlarged left ventricle.

Possible result: Small defects have a normal ECG. Large defectswith a big shunt have biventricular hypertrophy due to thepressure load on the RV and the volume load on the LV. If the

shunt decreases due to either the development of pulmonarystenosis or vascular disease then the volume load decreases onthe LV and the left ventricular hypertrophy resolves to leaveright ventricular hypertrophy.

echocardiogramEchocardiogram is used to make a definite diagnosis



Echocardiogram is used to make a definite diagnosis.

This shows size & position of VSD (arrow) and thus assists with theprognosis. It also allows identification of associated defects (ASD,pulmonary stenosis, aortic, mitral valve & arch lesions) when present.Colour Doppler is especially useful in identifying small defects.

The size of the shunt can be numerically estimated by Doppler flowtechniques but the accuracy is poor and the method rarely usedclinically. A reasonable subjective impression can however be madebased on the LA and LV size which will be enlarged in a large shunt. This may stretch the mitral valve ring causes mitral regurgitation

(LAVVR).

The pulmonary artery pressure can usually accuratelybe assessed using Doppler across the VSD In the



be assessed using Doppler across the VSD. In theexample the VSD velocity which using the Bernoulliequation (4V2) is equivalent to a pressure difference

between the LV and RV of 64 mm predicating anormal PA pressure in most children.

Cardiac catheterization

http://www.nlm.nih.gov/medlineplus/ency/article/003419.htm

http://www.nlm.nih.gov/medlineplus/ency/article/003419.htm



- (rare) concerns of high blood pressure in the lungs esp. moderate-sized defectsCardiac catheterization is used to study the various functions of theheart. Using different techniques, the coronary arteries can be viewedby injecting dye or opened using balloon angioplasty. The oxygenconcentration can be measured across the valves and walls (septa) of the heart and pressures within each chamber of the heart and acrossthe valves can be measured. The technique can even be performed in

small, newborn infants.



A VSD can be detected by



A VSD can be detected by… cardiac auscultation.

Classically, a VSD causes a pathognomonicholo- or pansystolic murmu

Auscultation is generally consideredsufficient for detecting a significant VSD.

The murmur depends on the abnormal flowof blood from the left ventricle, through the

VSD, to the right ventricle

http://en.wikipedia.org/wiki/Auscultation

http://en.wikipedia.org/wiki/Heart_murmur

http://en.wikipedia.org/wiki/Heart_murmur

http://en.wikipedia.org/wiki/Auscultation



Normal section through the



Normal section through themiddle

Normal heart front view



Normal heart front view

VSD



VSD



CHF1 knockout mice have ventricular septal defects but no other organpathology



Membranous VSD

http://www.embryology.ch/anglais/pcardio/patholcardio03.html#AtrioventrikularerSeptum

defekt



Muscular VSD



Muscular VSD

Histological sections of mousehearts

VSD



VSD

VSD

Cardiomyopathy



y p y

cardiomyocyte

multinucleation.

Increased size of both cytoplasmand nuclease

Myocyte hypertrophy



Histology of myocyte



Clinical Features



Small VSDs may beasymptomatic

VSDs in themuscular portion of the septum mayclose spontaneouslyduring infancy orchildhood

Large defects causesevere left to rightshunts

ASDs often lead toother cardiacmalformations orsecondary defects

Sequelae of Untreated Large



gVentricular Septal Defects

Hypertrophied and dilated right ventricle Progressive pulmonary hypertension

Shunt reversal

Infective endocarditis

Vascular changes leading to cardiac failure High output

Subvalvular aortic stenosis (aortic valve incompetence)

Hypertrophied and Dilated



Right Ventricle

Right QRS axisdeviation

Ejection isforceful but

ineffective

Ventriculararrhythmias

Myocardialischemia leadingto anginal pain

Progressive Pulmonary



Hypertension

Vascular hyperactivity that leads tochronic vasoconstriction

Dyspnea on exertion

Cyanosis

Severe respiratory insufficiency

Chest pain

Shunt reversal

Effects of Shunt



Reversal Condition similar to the Tetralogy of

Fallot

Decreased blood flow to the lungs

Cyanosis Increased size of heart

Increased risk of infective

endocarditis

Infective Endocarditis



High pressure shunts increasehemodynamic trauma to the endocardialsurface

The creation of jet lesions in the rightventricle

Infection of the mural surface of theendocardium

Formation of vegetation (mass of thrombotic debris and organism)

Vascular Changes Leading to



Cardiac Failure

Heart is enlarged, flabby and itspumps are inefficient

Development of progressive

congestive heart failure Patient experience episodes of

angina pectoris

As disease progresses, sudden deathis common



T R E A T M E N T

The medical treatment of infants with



ventricular septal defect (VSD) is directed at

the control of congestive heart failure.

The goals of therapy are:

To relieve symptoms.

To minimize frequency and severity of respiratory infections.

To facilitate normal growth.

Ventricular septum defect in infants is initiallyt t d di ll ith



treated medically with:

Cardiac glycosides (e.g. 10-20mcg/kg/day)

- increases the strength of the heart muscle todeal with the greater blood volume.

Loop diuretics (e.g. furosemide 1-3 mg/kg/day)

- help remove excess fluid from the body so the

heart doesn't have to work as hard and thepatient feels much better.

ACE inhibitors (e.g. captopril 0.5-2 mg/kg/day)

- used to decrease the work load on the left

ventricle.



A VSD that either decreases in size or



closes completely during the first year

of life presents no problems to thepracticing physician.

Older children with VSDs are seldomsymptomatic and require little in theway of medical therapy.

Surgical Therapy



Surgical closure is typically done before thechild begins preschool.

Surgery is indicated if medications do notwork in the first few months or years of life,especially if the child is not growingadequately even with medications.

Surgery is more urgent if evidence of

pulmonary hypertension has developed.

VSD surgery involves making a cut in the



g y gchest so a surgeon can stitch the hole

closed or sew a patch of manmadesurgical material (such as Dacron orGore-Tex) over the defect. This preventsshunting (the movement of oxygenatedblood from the left to the right ventricle).Eventually, the tissue of the heart healsover the patch or stitches, and by 6

months after the surgery, the hole will becompletely covered with tissue.



Surgery is not usually performed in



g y y pnewborns because small defects will

close spontaneously in 20-25%.

The surgery also is more risky in the

first few months of life; the risk of death from the operation is higher inthe first 6 months of life than later.



Medical management includes endocarditisantibiotic prophylaxis for all patients with VSDs



antibiotic prophylaxis for all patients with VSDs.

Respiratory infections require promptevaluation and treatment.

Evaluate children with VSD at least once ortwice yearly to detect changes in the clinicalpicture that suggest the development of pulmonary vascular obliterative changes.

Patients with VSD and pulmonaryl b t ti di h



vascular obstructive disease who are

deemed inoperable because of irreversibly elevated resistance requiremore intensive support andsymptomatic therapy as cyanosis

progresses and activity becomes morelimited.

Improvement in the symptomsi t d ith th l th i f



associated with the polycythemia of

Eisenmenger complex (headache,extreme fatigue, and extremedyspnea) may be provided by partialexchange transfusion for RBC volume

reduction.



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Clinicopathologic conference- Ventricular septal defect

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