-DR DHEERAJ SHARMA(RESIDENT CTVS)

Ventricular septal defects

CONTENTS

1. Classification2.Presentation3. Clinical examination4. Diagnosis

Etiology

Most common congenital heart defect. accounting for up to 40 % of cardiac anomalies .

Chromosomal disorders associated with an increased incidence of VSD , (Down syndrome), (Di George syndrome), (Turner syndrome).

Familial forms , TBX5, GATA4, and NKX2.5 mutations . Children from an adult with a VSD that is not associated

with a genetic disorder may have a risk of VSD as high as 3 % if the father is affected and a 6 % risk if the mother is affected.

CLASSIFICATION

1. SUGICAL2.MORPHOLOGICAL3.SIZE4.EUROPEAN5.VAN PRAAGH

Ventricular septum

Surgical Classification

TYPE 1 : subarterial defect: (conal,subpul, infundibular, supracristal, doubly commited, outlet), 5-10%

TYPE 2 : perimembranous defect, membranous septum ( outlet, trabecular and inlet subtype), 80%

TYPE3 : inlet or atrioventricular defect, < 5%

TYPE 4 : muscular defects, 5%

TYPE 1

Conal,subpul, infundibular, supracristal, doubly commited ( 4 ) outlet Aortic regurgitation (87% IN 20Y)Prolapse of the anterior aortic valve leaflet. ( LCC,RCC 6 % of defects 30% in AsianSpontaneous closure of this type of defect is uncommonDoubly committed subarterial :

More common in Asian patients, In the outlet septum, Bordered by fibrous continuity of the aortic and pulmonary valves.

TYPE 2

Synonyms: perimembranous, paramembranous,conoventricular

SUBTYPES : Inlet, trabecular, outlet, and confluent. (multiple areas of the septum)

Most common VSD, (80 % of defects) Bordered by fibrous continuity between the leaflets of an AV

valve and an arterial valve. AI (Prolapse of,RCC,NCC)

TYPE 3

Synonyms: inlet, AV canal type, endocardial cushion

May be associated with AV canal defect. Trisomy 21 syndrome. 5–8 % of VSDs .

Type 4(MUSCULAR)

Rim totally composed of septal muscle Subclassified as inlet, trabecular, outlet, or confluent . 20 % of VSDs in infants Spontaneous closure is common,. Frequently multiple. “Swiss-cheese” septum

MORPHOLOGICAL CLASSIFICATION

CLASSIFICATION EXTENTION

PERIMEMBRANOUS Inlet, anterior, outlet

MUSCULAR Outlet, trabecular, inlet, anterior, apical

DOUBLY COMMITTED SUBARTERIAL

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INLET SEPTAL Atrioventricular septal type

MALALIGNED Anterior (tof), posterior(CoA, interrupted aortic arch), rotational(taussig bing anomaly)

CLASSIFICATION BY SIZE

TYPE FEATURES

LARGE Size is >75% of aortic annulus, flow velocity less than 1 m/s, VSD resistance index < 20 u/m2

MODERATE Size 33- 75% of aortic annulus, flow velocity 1-4 m/s,

SMALL Size <33% of aortic annulus, flow velocity > 4 m/s, VSD resistance index more than 20 u/m2

EUROPEAN CLASSIFICATION

According to borders of VSD.

TYPE FEATURES

PERIMEMBRANOUS Bordered directly by fibrous continuity between leaflets of AV valves and arterial valves

DOUBLY COMMITTED Bordered by fibrous continuity between leaflets of aortic and pulmonary valves

MUSCULAR Completely embedded in muscular septum

VAN PRAAGH CLASSIFICATION

4 TYPES . The only difference from other classifications is that Van

Praagh used the term PARAMEMBRANOUS instead of perimembranous. He told that these defects besides involving membranous septum involved the tissue around them and are confluent with them.

Gerbode defect

This is direct LV to RA shunt.Membranous septum has 2 parts – (a)

atrioventricular part , (b) ventricular part.Defects through atrioventricular part leads to

shunting of blood from LV to RA directly (true gerbode shunt).

Defects in ventricular part leads to shunting of blood from LV to RV and then through perforation in septal leaflet goes to RA . It is indirect LV to RA shunt ( false gerbode defect).

Gerbode defect

Gerbode defect

PRESENTATION OF DISEASE

spectrum of disease

Asymptomatic patient patients with eisenmenger disease

presenting with marked cyanosis.

Presentation contd….

The variation in the spectrum of disease is due to the physiologic consequences depending on:

a) size of VSD b) pulmonary vascular resistance As these variable changes with time the presentation and

clinical features changes resulting in different spectrum of disease.

The presentation and spectrum of disease can be easily understood by following the ANATOMIC PHYSIOLOGIC CLASSIFICATION OF VSD.

Presentation contd…

ANATOMIC PHYSIOLOGIC CLASSIFICATION OF VSD: ( based on size of VSD and PVR):

A. TYPE 1 (RESTRICTIVE VSD) B. TYPE 2 (MODERATELY RESTRICTIVE VSD) C. TYPE 3 (NONRESTRICTIVE VSD) D. TYPE 4 (VSD WITH REVERSAL OF SHUNT)

Presentation contd….

TYPE 1 ( RESTRICTIVE VSD): Resistance that limits the left to right shunt resides at the

level of VSD. Normal PVR Normal PA pressure and RVSP LV pressure > RV pressure

1. Produces a significant pressure gradient between the left ventricle and the right ventricle

2. Pulmonary-to-aortic systolic pressure ratio < 0.33. Small (≤1.4 : 1) shunt. 4. Less than 5mm, or defect size <=25% of annulus diameter5. Normal PA and branches 6. Normal LV, LA size

Presentation contd….

TYPE 2 ( moderately restrictive VSD ) Higher than normal RV AND PA pressure but with low and

variable PVR LV pressure > RV pressure

1. Qp/Qs of 1.4 to 2.2 2. pulmonary-to-aortic systolic pressure ratio less than 0.66.3. Diameter of defect >25% <75% of annulus size or 5-10

mm4. RVP,PAP normal or near normal5. Mild to moderate PA,LA,LV dilation

Presentation contd….

TYPE 3( NON RESTRICTIVE VSD): Large left to right shunt, identical LV to RV pressure RV and LV behave as single chamber with direction of flow

determined by resistance in pulmonary and systemic circulation

PVR is high but subsystemic.1. Qp/Qs > 2.22. pulmonary-to-aortic systolic pressure ratio greater than

0.66. 3. Defect diameter >75% of aortic diameter4. PH in less than 2years

Presentation contd…

TYPE 4(VSD WITH REVERSAL OF SHUNT):

Identical RV and LV pressure Suprasystemic PVR and reversal of shunt across VSD. PAP/systolic pressure ratio of 1 Qp/Qs less than 1 : 1 Net right-to-left shunt and cyanosis.

Natural history of disease

1. A restrictive VSD may close spontaneously during childhood or may go unnoticed as it hardly produces symptoms or may lead to infective endocarditis.

2. A perimembranous defect ,doubly committed VSD, 1. Progressive AR. 2. Subaortic and subpulmonary stenosis 3. Left ventricular to right atrial shunt

3. A moderately restrictive VSD 1. Left atrial and ventricular dilation due to volume overload. There is pressure

overload on RV to which they adapt.2. Variable increase in pulmonary vascular resistance occurs with time and may lead to

CHF in adult life.3. Infective endocarditis.

4. A large or nonrestrictive VSD 1. Ventricular volume overload early in life leading to CHF in childhood.2. Progressive rise in pulmonary artery pressure 3. A fall in left-to-right shunting.4. Finally the reversal of shunt

Spontaneous diminution in size

Occurs in both perimembranous and muscular types. In this study 15.8% of defects < 3 mm remained patent

in comparison to 71.4% > 3mm at 1yr (Nir A et al.PediatrCardiol1990; 11: 208–10.)

Isolated VSD ( 124 pts) -34% at 1 yr & 67% at 5 yr Female predominance Decreases substantially after 1 year of age.(Mehta AV et al.

TennMed 2000; 93: 136–8). Rare in malaligned VSD and In outlet VSD closure only in 4% 80% of the patients with VSD seen at 1 month age, 60% of

the infants seen at 3 months age, 50% of the patients seen at 6 months of age, 25% of those seen at 12 months have spontaneous closure.

Mechanism of closure

A.Closure of a perimembranous defect by adhesion of the tricuspid leaflets to the defect margin and by formation of aneurysm of ventricular septumB.Closure of a small muscular defect by a fibrous tissue plug. C.Closure of a muscular defect by hypertrophied muscle bundles in the right ventricleD.Closure of a defect in subaorticlocation by adhesion of the prolapsed aortic valve cusp

Right ventricular outflow tract obstruction

Incidence 3% to 7%. Mechanism:- Hypertrophy of malaligned infundibular septum Hypertrophy of right ventricular muscle bundles Prolapsing aortic valve leaflet Obstruction of outflow tract by ventricular septal aneurysm.

Aortic valve prolapse

VSD with direct contact with the aortic valve are most prone to develop AVP:

1. the perimembranous defects2. doubly committed juxtaarterial defects (RCC prolapse)3. Some of muscular outlet defects Characteristic deformity of aortic cusp-nadir of the cusp is

elongated. RCC (60-70%) ,NCC (10-15%) , both in 10-20% Non-coronary cusp prolapse in perimembranous type Left coronary cusp prolapse extremely rare Presentation is rare before 2 yrs and after 10yrs. It peaks

between 5 to 9 yrs.

Aortic valve prolapse

Pathogenesis:Hemodynamic factor ‘’Venturi effect’’

Aortic valve proplapse

the perimembranus defects tends to decrease following closure by prolapsed aortic leaflet and volume overload on LV is due to AR only.

The subarterial defects donot usually decrease in size thus the volume overload on LV is sum of shunt volume and AR.

Infective endocarditis in VSD

18.7 per 10000 person-years in non operated cases Occurs at the rate of 0.15 – 0.3 % per year. Its [revalance is more in males that to of age more than 20

years. Operated VSD 7.3 per 10000 person-years(Gersony WM et

al.Circulation1993; 87(Suppl. I):I-121–I-126.) Higher in small defect. Patients with a proven episode of endocarditis are considered

at increased risk for recurrent infection so surgical closure may be recommended

Arrhythmias in VSD

Patients with VSD have a high incidence of arrhythmia A. Ventricular tachycardias in 5.7% B. Sudden death is 4.0% C. SVT, mostly AF, is also prevalent Age and pulmonary artery pressure are the best predictors of

arrhythmias The odds ratio of serious arrhythmias increases A.1.51 for every 10-year increase in age B.1.49 for 10mm Hg increase in mean PA pressure(Wolfe RR et al. Circulation. 1993;87:I89-101)

Improvement in symptoms of VSD

Closing defect -soft S2, high frequency & shorter murmur

Increasing PVR : S2 loud & narrow split

Infundibular hypertrophy & resulting decreased L to R shunt : S2 decreases in intensity ,crescendo-decrescendo systolic murmur in the ULSB

Pulmonary vascular disease

Patients with large VSD are at increased risk of developing progressively increasing pulmonary vascular resistance owing to high pulmonary artery pressure and flow leading to permanent changes in pulmonary vasculature.

Once developed these changes seldom regress.

A pathological classification of pulmonary artery disease is given by Heath and Edwards.

Heath and Edwards classification of PVD

GRADE FEATURES

GRADE 1 Medial hypertrophy without intimal proliferation

GRADE2 Medial hypertrophy with intimal reaction

GRADE3 Medial hypertrophy with intimal fibrosis

GRADE4 Generalised vascular dilatation, areas of vascular occlusion by intimal fibrosis

GRADE5 Other dilatation , plexiform lesions like cavernous and angiomatoid lesions

GRADE6 Necrotising arteritis with grade 5 changes.

CHF IN VSD

Rare in small VSD as size limits the L-R shunt After birth decline in PVR to adult level by 7to 10 days: In

large VSDs, the rate of this process is delayed. Small VSD the shunt is small & remain asymptomatic. Moderate sized VSD symptoms by 1to 6 months. Large VSD congestive heart failure in first few weeks Risk for recurrent pulmonary infection high If survives without therapy -pulmonary vascular disease

develop in the first few years of life Symptoms “get better” as Qp/Qs returns to 1:1

Left ventricular outflow tract obstruction

Subvalvar stenosis is more common than valvar type and is due to displacement of infundibular septum into LV side (posterior), discrete fibromuscular bar lying caudal or downstream to VSD.

PREMATURE DEATH

9 % of patients with large VSD die with in 1 yr due to CHF which may develop with in 2-3 months of life.

Death in large VSD may also result from recurrent pulmonary infections secondary to pulmonary edema and pulmonary congestion.

After age of 1 yr few death may occur upto second decade of life and these patients may succumb to complications of eisenmenger syndrome like hemoptysis, polycyathemia, cerebral abscess and infarction, right sided heart failure.

Patients with small VSD die infrequently as a result of infective endocarditis.

HISTORY AND PRESENTATION

1. RESTRICTIVE VSD:I. May remain asymptomatic.II. Systolic murmur heard incidentally during examination by

doctorIII. Infective endocarditis: restrictive VSD is a risk factor for IE

but it rarely occurs before the occurance of secondary teeth. Tricuspid valve septal leaflet is site of infection in most cases as it is the site where jet hits.

IV. Longevity of patient is near normal.

HIRTORY AND PRESENTATION

2. MODERATELY RESTRICTIVE VSD: I. Escapes detection in early neonatal period as shunt is

delayed due to delay in fall of PVR.II. CHF occurs after few months in infancy when PVR falls.III. Infant cough and fatigue after feeding, sweats excessively

and become restless when recumbent, sleeps poorly.IV. Parents detects a thrill when they hold the infant against

their chest or by noticing a hyperactive precordium.V. spontaneous improvement is seen due to closure or

reduction in size of VSD or by increase in Pulmonary vascular resistance resulting in reduced shunt .

HISTORY AND PRESENTATION

3. NONRESTRICTIVE VSD:I. Present in early infancy with CHF and it seldom reduces in size.II. Poor growth and development, laboured breathing, frequent

episodes of URTI, difficult feeding and excessive diaphoresis.III. Dyspnoea and irritability are most pronounced when the infant is

supine and get improved when infant is held upright.IV. Feeding patterns are typical: a hungary infant awakes from fretful

sleep and feeds vigorously only to stop due to dyspnoea, then falls to sleep to be awaken due to hunger due to effort exhaustion .

V. Improvement in symptoms is always due to rise in PVR.VI. When the PVR become suprasystemic the reversal of shunt occurs

presenting with cyanosis. It is known as Eisenmenger complex.

HISTORY AND PRSENTATION

FEATURES OF EISENMENGER SYNDROME:I. ERYTHROCYTOSIS: due to chronic hypoxia stimulated rise in

erythropoitin .II. Intracranial venous thrombosis: due to high vicousity of blood.III. Platelet counts are in lower rangeIV. Clotting factors especially Wonvillebrand factor are deficient leading

to pulmonary haemorrhage, increased traumatic bleeding, menorrhagia, easy brusing, gingival bleeding.

V. Increased incidence of calcium bilirubinate gall stones. VI. Paradoxical embolism leading to TIA .VII. Clubbing : systemic venous megakaryotypes are released into arterial

circulation and get impacted in digits and subperiosteum . They release PDGF leading to synthesis of connective tissue.

VIII. Sudden death due to massive intrapulmonary haemorrhage , rupture of dilated hypertensive pulmonary trunk

IX. Cerebral abscess may result in seizure disorder.

PHYSICAL APPEARANCE

POOR GROWTH AND DEVELOPMENT CACHEXIA IN INFANTS DUE TO CATABOLIC EFFECTS OF

CHF. CYANOSIS DUE TO REVERSAL OF SHUNT CLUBBING HARRISONS GROOVES ARE DUE TO THORACIC

RETRACTINS CAUSED BY CHRONIC DYSPNOEA.

HARRISONS GROOVE

ARTERIAL PULSE

IN RESTRICTIVE VSD: Normal arterial pulse is seen. MODERATELY RESTRICTIVE VSD: the arterial pulse is brisk

because of vigorous ejection from volume loaded LV. NONRESTRICTIVE VSD : nonrestrictive VSD with large left

to right shunt and congestive heart failure are associated with diminished arterial pulse and pulsus alternance.

EISENMENGER SYNDROME: arterial pulse is normal because the systemic output is maintained.

JUGULAR VENOUS PULSE

Moderate and non restrictive VSD with congestive heart failure are associated with raised JVP with increase in A and V waves.

In eisenmenger syndrome the JVP is nearly normal with exceptional large A wave. Large A wave is exceptional as RVSP is never more than systemic level so RV requires little support from its atrium.

PRECORDIUM

Restrictive VSD: only harsh thrill maximum in left 3rd or 4th intercoastal space at sternal border is only sign. In case the VSD is subarterial which directs the flow directly into pulmonary trunk the thrill is maximum in left 1st or 2nd intercoastal space with radiation upward and left into the neck.

Moderately restrictive VSD: hyperdynamic left ventricular apex is palpable, dilated pulmonary trunk is palpable in left 2nd intercoastal space, thrill of VSD is present.

Nonrestrictive VSD: hyperdynamic volume overloaded left ventricle is palpable, dilated pulmonary trunk is palpable, palpable pulmonary component of second heart sound in addition to characterstic thrill.

Eisenmenger syndrome: only dilated hypertensive pulmonary trunk with palpable pulmonary component of second heart sound is present.

AUSCULTATION

RESTRICTIVE VSD:I. MURMUR: soft, highly localized, high frequency, early

systolic in very restrictive defect to holosystolic in restrictive VSD . Early systolic timing is due to fact that small perimembranous and muscular defects tends to close in late systole. The early systolic murmur teds to be longer during premature ventricular beat as reduced contractility cannot close the defect. It is maximum in left 3rd or 4th ICS at sternal border. Grade 4/6 or louder in intensity.

II. When the chordae tendinae of tricuspid valve bridge the defect the murmur have muscal overtones assuming the pitch of aeolian harp.

AUSCULTATION

Moderately restrictive VSD:I. Loud harsh holosystolic murmurs when the PVR is below the

systemic levels. The shape of murmur is cresendo or cresendodecresendo.

II. When the shunt is subarterial the murmur is heard in 1st or 2nd left intercostal space with radiation upwards and to left.

III. When VSD is spontaneously closes the holosystolic murmur becomes early systolic before disappearing .

IV. Septal aneurysm when present leads to late systolic accentuation of holosystolic murmur due to stretching of aneurysmal pouch and may lead to mid systolic clicks and may give rise to midsystolic murmur in 2nd left intercostal space due to RVOTO caused by septal aneurysm.

V. Middiastolic murmur at apex: increased flow through mitral vave.VI. Third heart sound in case of Left heart failure.

AUSCULTATION

NONRESTRICTIVE VSD:I. As the PVR increase and reaches the systemic levels the

holosystolic murmur softens and shortens, it becomes early systolic and shape changes to decresendo before disappearing altogether as shunt is reversed.

II. Second heart sound increase in intensity as pulmonary component becomes loud. As the PVR increase the splitting decreases .

III. Other signs are similar to moderately restrictive VSD.

AUSCULTATION

Eisenmenger syndrome:I. Second heart sound becomes single as both pulmonary and

aortic valves closes simultaneously.II. Auscultatory signs of pulmonary hypertention persists: Pulmonary ejection sound due to flow in dilated

hypertensive pulmonary trunk. It also produces a soft midsystolic murmur.

High frequency mid diastolic graham steel murmur of pulmonary regurgitation .

ECG

RESTRICTIVE VSD:I. Near normalECGII. RSR pattern in V1III. Increased incidences of conduction disturbances and

rhythm disturbances especially AF, PAT,CHB , atrial flutter, junctional rhythm are seen when septal aneurysm is present with perimembranous conduction defects.

ECG

Moderately restrictive VSD:I. Broad notched left atrial P waves in lead 1 and 2.II. QRS axis is normal. VSD with left axis deviation are seen in

association with AV septal defects and with septal aneurysms.

III. Volume overload of LV is seen as tall R waves and tall T waves in leads 2,3 and aVF. Tall R waves are also seen in V5 and V 6.

ECG

Nonrestrictive VSD:I. Right atrial or combind atrial P wave abnormality in lead 2

and V1 and V2.II. QRS axis shift moderately towards right.III. Biventricular hypertrophy : large R wave in V1, large R

wave in V5,V6, tall T waves in V5 V6.

ECG

Eisenmenger syndrome:I. P wave is normal in young patients.II. Right axis deviation is moderateIII. Tall R waves in V1IV. Prominent S waves in left precordial leads.

ECG of moderately restrictive VSD.

Showing left axis deviation.

Notched left atrial p waves in lead 1,2

ECG of non restrictive VSD

Peaked rt atrial p waves are seen in v1-v4

RVH – prominent R waves in rt precordial leads and prominent S waves in v4, v5

LVH: prominent R waves and tall T waves in lt precordial leads.

ECG of VSD with eisenmenger syndrome

Normal p wavesRVH: tall R waves in

v1Showing pure

pressure load of RV.

X RAY

RESTRICTIVE VSD:I. Near normal chest X rayII. Defects which are previously large but later reduces shows

signs of initial larger shunts like enlarged LV and dilated pulmonary truck and branches.

X RAY

MODERATELY RESTRICTIVE VSD:I. When PVR is low there is increased pulmonary vascularity

with vascular congestion.II. Lungs are hyperinflated with flat hemidiaphragmIII. Right atrial dilatation with development of congestive heart

failure.IV. Enlarged pulmonary artery and branches shows the

magnitude and chronicity of pulmonary blood flow.V. Left atrial enlargement is seen in lateral view.VI. Ascending aorta is not enlarged as left to right shunt is

intracardiac.

X RAY

NONRESTRICTIVE VSD:I. Radiologic features of enlargement of all four chamber

when associated with heart failure in infancy.II. X ray resembles that of moderately restrictive VSD III. Exceptionally there is aneurysmal enlargement of

pulmonary artery and branches.IV. As the PVR increases the congested heart failure is

ameliorated and size of heart decreases but enlargement of pulmonary trunk and branches persisit.

X RAY

Eisenmenger syndrome:I. The lung fields are oligamicII. Right atrial, left atrial, left ventricular sizes are normalIII. Hypertrophied but non dilated right ventricle occupies the

apexIV. Cardiac size is normalV. Pulmonary artery and branches are dilated.

ECHOCARDIOGRAPHY

TTE, TEE with colour flow imaging and doppler gives presize location and physiologic characters of VSD. Gradient across the VSD can be measured.

Small multiple defects in septum, septal aneurysm, type of ventricular septal defects can be seen.

Left ventricular function, RVSP,PASP can be seen. Associated anomelies like AV septal defects, PDA can be

seen. Assessment of all the valves especially aortic valve can be

done. Associated right and left ventricular outflow tract obstruction

can be seen.

The sensitivity of echocardiography is maximum for inlet and outlet defects (100%), slightly less for perimembranous defects(80-90%), and least for trabecular defects.

Typically apical and parasternal views are used to look for different types of ventricular septal defects.

The membranous septum is closely related to the aortic valve. In the apical and subcostal “five-chamber”views, it is seen in the LV outflow tract just under the aortic valve (see Fig. 12-11, C3 ).

In the parasternal short-axis view at the level of aortic valve, it is seen adjacent to the tricuspid valve (see Fig. 12-11, B1 ).

These are the best views to confirm the membranous VSD. The membranous VSD is not visible in the standard parasternal long-axis view.

The inlet septum is best imaged in the apical or subcostal four-chamber view beneath the AV valves (see Fig. 12-11, C2 and D1 ).

It can also be seen equally well in the parasternal short-axis view in the posterior interventricular septum at the levels between the mitral valve and the papillary muscle (see Fig. 12-11, B2 ).

The infundibular (or outlet) septum lies inferior to the semilunar valves. The subpulmonary, supracristal infundibular VSD lies under the pulmonary valve (see Fig. 12-11, A2 and D3 ), and the subaortic infracristal VSD (TOF type, also called conoventricular VSD) lies under the aortic valve (see Fig. 12-11, A2 and D2 ).

From the RV side, if the outlet septum lies inferior to the pulmonary valve, it is supracristal. The infracristal VSD lies much closer to the aortic valve but away from the pulmonary valve (see Fig. 12-11, A1 and C3 ), and the supracristal is closer to the pulmonary valve (see Fig. 12-11, A2, D3, and E1 ).

The trabecular septum is the largest portion of the ventricular septum and extends from the membranous septum to the cardiac apex.

Four types of trabecular VSD are (1) anterior, (2) midmuscular, (3) apical, and (4) posterior.

Echo views that show the locations of different types of trabecular VSDs are shown in Figure 12-11 .

The apical VSD occurs near the cardiac apex (see Fig. 12-11, A1, A2, C2, C3, D1, and D2 ).

The entire ventricular septum seen at the papillary muscle level is the trabecular septum (see Fig. 12-11, B3 and E2 ).

Catheterization study

Not done routinely Indicated in cases where echocardiography is doubtfully or

uncertain In cases where there is suspecision of high pulmonary

vascular resistance and to make decision of whether to operate or not.

In cases where device closure of VSD is planned. In cases of elderly patients to look for coronary artery status. Quantification of left to right shunts by measuring the ratio of

pulmonary blood flow versus the systemic blood flow can be calculated (QP/QS

Catheterization study

Calculation of Pulmonary Blood Flow (Qp)

Calculation of Systemic Blood Flow (Qs)

Catheterization study

Qp/Qs can be a very useful tool in making decisions about the need for repair of a shunt

Qp/Qs of 1–1.5 – observation is generally recommended.

Qp/Qs ratio of 1.5–2.0 – significant enough that closure (either surgically or percutaneously) should be considered if the risk of the procedure is low

Qp/Qs ratio of greater than 2 – closure (either surgically or percutaneously) should be undertaken unless there are specific contraindications

Pulmonary Vasoreactivity

Pulmonary circulation is characterized by high flow, low pressure and low resistance system

Normal pulmonary systolic pressures are 18-25 mm Hg, end diastolic pressure ranges from 6-10 mm Hg and mean pulmonary arterial pressures of 10-16 mm Hg

Pulmonary hypertension is define as mean pulmonary artery pressure (MPAP) >25 mm Hg at rest or > 30mmHg on exercise or systolic pulmonary artery pressure >30 mm Hg

Pulmonary artery pressure increase in response to increase on LA pressures, pulmonary vascular resistance and cardiac output

Expressed in Woods unit (1WU=1mm Hg/L = 80 dynes/cm3)

Normal value is < 3 WU or 150 – 250 dynes/sec/cm3

PVR is one sixth SVR

Factors increases PVR Hypoxia Hypercapnia Increased sympathetic tone Polycythemia local release of serotonin Mechanical obstruction by multiple pulmonary emboli Precapillary pulmonary edema Lung compression (pleural effusion, increased

intrathoracic pressure via respirator)

Factors that decreases PVR: Oxygen Adenosine Isoproterenol Inhaled nitric oxide Prostacyclin infusions High doses of calcium channel blockers

Pulmonary Vasoeactivity

Pulmonary vasoreactivity can be checked with the help of

100% oxygen Adenosine Epoprostenol Inhaled nitric oxide

Criteria for Positive Responders

Positive response is define as: 20% fall in pulmonary artery pressure and PVR or

decrease in mean pulmonary artery pressure of 10 mm Hg to an absolute value of less than 40 mm Hg without in decrease in cardiac output

These are the patient who are most benefited from corrective procedure and calcium channels blockers

Resistance Ratio

The ratio between pulmonary vascular resistance and systemic vascular resistance (resistance ratio) can be used as a criterion for operability in dealing with congenital heart disease

Normally, this ratio is <0.25 Values of 0.25 to 0.50 indicate moderate pulmonary

vascular disease Values greater than 0.75 indicate severe pulmonary

vascular disease When the PVR/SVR resistance ratio equals 1.0 or more,

surgical correction of the congenital defect is considered contraindicated because of the severity of the pulmonary vascular disease

Angiographic assessment of VSD

Best done by using biplane technique.Interrelationship of LV, PA, RV and Aortic root

is assessed.3 views – 40 degree RAO, 40 degree Cr-LAO,

50 degree LAO view.Perimembranous VSD: LAO view shows VSD

just below parietal bandDoubly committed VSD: RAO view shows

defect below the aortic and pulmonary valves.

Angiography

Inlet VSD: LAO view shows defect between two AV valves.

Muscular VSD: appreciated in LAO view.

Diagnostics of noncyanotic heart disease

features ASD VSD PDA

X RAY Cardiomegaly with enlargement of the RA and right ventricle (RV) may be present. A prominent pulmonary artery (PA) segment and increased pulmonary vascular markings are seenwhen the shunt is significant.

Cardiomegaly of varying degrees is present and involves the LA, left ventricle (LV)increase inpulmonary vascular markings .

Cardiomegaly of varying degrees occurs in moderate- to large-shunt PDA with enlargement of theLA, LV, and ascending aorta. Pulmonary vascular markings are increased.

ECG Right axis deviation of +90 to +180 degrees and mild right ventricular hypertrophy (RVH) orright bundle branch block (RBBB) with an rsR' pattern in V1 are typical findings. In about 50% of thepatients with sinus venosus ASD, the P axis is less than 30 degrees.

With a moderate VSD, left ventricular hypertrophy (LVH) and occasional left atrial hypertrophy(LAH) may be seen.With a large defect, the ECG shows biventricular hypertrophy (BVH) with or without LAHIf pulmonary vascular obstructive disease develops, the ECG shows RVH only

A normal ECG or LVH is seen with small tomoderate PDA. BVH is seen with large PDA. If pulmonary vascular obstructive disease develops, RVH ispresent

Treatment and results

TOMMORROW ………..by

DR GAURAV GOYAL……………………..

THANK YOU………………………