APPROACH TO ACYANOTIC CONGENITAL HEART DISEASES

Dr.k. Nagendra prasadAPPROACH TO ACYANOTIC CONGENITAL HEART DISEASES

Congenital cardiovascular defects, also known as congenital heart defects, are structural problems that arise from abnormal formation of the heart or major blood vessels. Defects range in severity from tiny pinholes between chambers that may resolve spontaneously to major malformations that can require multiple surgical procedures before school age and may result in death in utero, in infancy, or in childhood.AMERICAN HEART ASSOCIATION INTRODUCTION

Congenital heart diseaseis a problem with the heart's structure and function that is present at birth.

DEFINITION

Approximately 5 to 8 per 1000 live births . About 2 or 3 in 1000 infants will be symptomatic during the first year of life with significant heart diseases that will require treatment. CHD is the major cause of death in the first year of life. There are more than 35 well recognized cardiac defects the most common heart anomaly is VSD.INCIDENCE

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MULTIFACTORIAL

GENETIC - 90%ENVIRONMENTAL - 10%PREDISPOSING FACTORS -Drugs / abortificient ! Infection! Radiation! Systemic disease (maternal)Etiology

The systematic approach to CHD includes:-

1.Detailed antenatal and postnatal history. 2.Systematic physical examination: Which includes general examination, followed by detailed examination of the cardiovascular system by inspection, palpation, percussion and auscultation. The examination of the respiratory system and abdomen should be done in a methodical manner. 3. Electrocardiography (ECG) 4. Chest X-ray 5. Echocardiography 6.Computed tomography (CT) angiogram and cardiac magnetic resonance imaging (MRI) in selected cases 7.Invasive procedures like cardiac catheterizationandangiocardiography, if needed.

Approach to CHDIs the Heart Disease Congenital?CONGENITALRecognition early in ageMurmurs more parasternally Presence of central cyanosisPresence of extra-cardiac anomaliesACQUIREDH/o. joint pains, feverApical murmurs

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Does the child have Heart Disease? NADAS Criteria

MAJORSystolic murmur Gr. III or > intensityDiastolic murmurCyanosisCHFMINORSystolic murmur Gr. II intensityAbnormal 2nd Sound Abnormal ECGAbnormal Chest X RayAbnormal Blood Pressure

ACYANOTIC vs CYANOTICNormal saturations are >94%.Between 93 and 85 human eye cannot detect desaturation.Below 85% we can visually diagnose cyanosis.

Central cyanosis is characterized by blueness of the tongue and oral mucosa. Central cyanosis most likely is related to cardiac or respiratory disease. If the source of cyanosis is cardiac, it is constant. Children in shock also may appear cyanotic owing to venous stasis, right-to-left intrapulmonary shunting, and increased peripheral oxygen extraction.

Classification CHDAcyanoticCyanotic

Left-to-right shuntsOutflow obstruction- Ventricular Septal Defect (VSD)- Patent Ductus Arteriosus (PDA)- Atrial Septal Defect (ASD- Pulmonary Stenosis- Aortic Stenosis-Coarctation of aorta Teralogy of fallot Tricuspid atresia Transposition of great vesselsTruncus arteriosusTotal anomalous pulmonary venous return (TAPVR)Ebsteins anomaly

Lesions% of all Lesions- Ventricular septal defect25-30- Atrial septal defect (Secundum)10- Patent ductus arteriosus10- Coarctation of aorta7- Tetralogy of Fallot6- Pulmonary Valve Stenosis5-7- Aortic Valve Stenosis4-7- Transposition of great arteries4- 0thers20

Acyanotic congenital heart diseases - present with signs of congestive heart failure and/or heart murmurs that are heard during physical examination and can manifest any time during infancy or early childhood. Most of these patients present during the first 6 months of life.

PRESENTING COMPLAINTS1.Feeding difficulties 2. Tachypnea 3. Sweating 4. Subcostal recession5. Recurrent respiratory infections 6. Growth impairment in the infant 7. Exercise intolerance 8. Easy fatigability or murmur in the older childThe magnitude of the shunt or the severity of the obstruction determines the clinical presentation and symptoms.

FEEDING DIFFICULTIESIt is a common symptom in significant acyanotic CHDs. In infants, feeding itself is a form of exercise or effort. An infant with heart failure has the inability to complete feeds within 15 to 20 minutes, sucks less volume of milk (< 3.5 ounces per feeding), gets tired easily and takes frequent feeds (less than 2 hours). As the feeds are inadequate they become irritable and cry excessively. This suck-rest-suck cycle continues round the clock. As pulmonary vascular resistance declines, irritability and fussiness with feeding may indicate angina and ischemia in a child with an anomalous left coronary artery from the pulmonary artery.

RESPIRATORY DISTRESSIt is the most prominent sign of heart failure caused by significant lefttoright shunting in infancy.Symptoms of respiratory distress include tachypnea or rapid breathing (respiratory rate > 60/min in newborn and > 50/min in infants) and intercostal and subcostal chest retractions. In very sick infants,grunting and nasal flaring can also be present. Unlabored tachypnea often accompanies cyanotic heart disease, whereas grunting and dyspnea are associated with left-sided obstructive lesions or respiratory illness.Leftsided heart failure produces tachypnea with or without dyspnea.Wheezing or persistent cough at night early sign of CHF.

Easy Fatigability In an infant, it is seen as poor ability to suck and feed. In older children, heart failure may be manifested as exercise intolerance;difficulty in keeping up with peers during sports or need for a nap after coming home from school and poor growth.

Repeated Lower Respiratory infectionsRecurrent pneumonia - two or more episodes of RTI in one year or more than three in any time frame requiring hospitalization or IV antibiotics.Frequent upper respiratory tract infection are not related to CHD.

Growth Retardation or Failure to Thrive Failure to thrive is definedas weight < 3rd percentile for age.Infants should gain about 20 g/day. A normal infant can grow while receiving 100 calories/kg/day; infants whose growth is impaired by heart disease typically require 130 to 140 calories/kg/dayAcyanotic patients - weight is more affected due to high catabolic rate & poor feeding due to dyspnea ,fatigue.Cyanotic patients disturbances in both height and weight.Pressure overload lesions does not affect.

CHEST PAINChest pain or angina is rare, but not unknown in infants and children.Pain associated with palpitations, dizziness and panic attacks - mitral valve prolapse (Barlow syndrome). LVOT obstruction stenosis of the aortic valve, subaortic valve area, supra-aortic valve area or coarctation of the aorta, may present with chest pain and associated with dizziness and fatigue - due to demand-supply mismatch resulting in myocardial ischemia. Chest pain in association with exercise intolerance and fatigue - hypertrophic or dilated cardiomyopathy. Chest pain due to myocardial ischemia - abnormal coronary artery anatomy, including congenital anomalies of the coronary artery, coronary artery fistulas and stenosis or atresia of the coronary artery ostium and have been recognized in infants withan aberrant left coronary artery.Chest pain can also occur with very rapid paroxysmal tachycardia.

SyncopeHistory of syncope on mild to moderate exertion indicates severe aortic stenosis, hypertrophic cardiomyopathy (HOCM), severe pulmonary hypertension or complete transpositionof the great vessels (CTGV).In CTGV , syncope is due to significant bradycardia.long QT syndrome A uncommon cause of syncope.

Peripheral EdemaPretibial and presacral edema are late developments in the child with congestive circulatory failure, apparently due to the difference in tissue turgor.

When peripheral edema due to heart failure does develop in an infant, it first appears periorbitally, andis usually preceded byothermanifestations such as tachypnea, tachycardia, dyspnea and liver enlargement.

Sweat on forehead low cardiac output increased sympathetic activity- seen in CHF.

Family HistoryIf one parent has a congenital heart anomaly, the risk of the child having one 10 percent.When a firstcousinhas a congenital heart anomaly, the risk of a sibling having one is approximately 2 percent.Withno family history of CHD, if the firstborn hasa congenital heart lesion, the risk of a second child having a congenital heart lesion is 2 to 3 percent.

Maternal History

BIRTH WEIGHTHIGH BIRTH WEIGHT TGALOW BIRTH WEIGHT Intra uterine infections,drugs( alcohol)

CYANOSISonset at birth / several days after birthSeverity of cyanosisPermanent /paroxysmal natureParts of body that is cyanoticWhether cyanosis becomes worse after feedingCyanotic spells time of appearance , duration of spell, frequency of spell Should be differentiate from breath holding spellHistory of squatting.

HEART MURMURHeard within few hours after birth stenotic lesion / AV valve regurgitation, small left to right shunt lesions as their flow characteristics is independent of PVR changes.Murmurs of large left to right shunts are delyed due to slow regression of pulmonary vascular resistence. Murmur that is first noticed in a routine examination of a healthy boy innocent

How to assess PBFSYMPTOMS Increased respiratory rate, retractions , Increased respiratory infections,Sweating while feedingFailure to thriveHarrison sulcusInfants with lesions associated with shunting at the ventricular or great vessel level are generally more symptomatic than those with only atrial-level shunting..

GENERAL APPEARANCEA wide spectrum of extracardiac malformations occur in 15-45% of cases with CHD. Extracardiac malformations can give a clue towards certain CHD.

PULSE Character of the pulse 1. Bounding pulses PDA , BAV with severe AR , large systemic AV Fistulas , persistent truncus arteriosus.2. Pulsus parvus et tardus- severe AS.3. Weak ,thready pulse cardiac failure /circulatory shock ,myocarditis, cardiomyopathy, legs in patients with CO A, arm pulses on the side of classic BT shunt / subclavian flap angiplasty.

Difference between upper and lower limb pulses CO A/ interrupted aortic arch.Discrepancy in both radial pulses - supravalvular aortic stenosis, aortic isthmus stenosis or pre-ductal CoA with the left subclavian arising below the coarctation & the aortoarteritis with obstruction to left subclavian artery.

BLOOD PRESSUREUpper extremity hypertension first sign of CoA.A narrow pulse pressure - low cardiac output or severe aortic stenosis. Pulse pressure widens in conditions with an elevated cardiac output (anemia and anxiety) or with abnormal runoff of blood from the aorta during diastole (PDA or aortic insufficiency).

Jugular Venous Pressure

TheJVP is difficultto interpret in newborns and infants(due to short neck and tachycardia).ASD- V A large shunt A> V small shunt , associated PHT , LV failure increased mean pressure LV dysfunction.VSD- JVP elevated with incresed A and V in patients with moderately restrictive ,non restrictive VSD eith CHF. EISENMENGER normal/ small dominant A wave.PDA sameAS increase of A wave amplitude in the absence of PHT.CO A- normal except in when biventricular failure present.

PS prominent A wave and a presystolic liver pulse , With the advent of right ventricular failure and tricuspid regurgitation, the v wave increases, the Y descent becomes brisk and the liver manifests presystolic and systolic pulsations.

INSPECTIONPrecordial bulge suggests chronic cardiac enlargementPectus excavatum pumonary systolic murmur and cardiomegaly in PA viewHarrisons groove large left to right shunts leading to HF > 2 months .left precordial bulge - left atrial enlargement in post-tricuspid shunts. The upper sternum may bulge in children with a large left-to-right shunt and pulmonary hypertension or with elevated pulmonary venous pressure. Seen after 3 months of age. In older patients withright ventricular(RV) hypertrophy secondary to pulmonary hypertension there can be a prominant precordium.

Hyper active precordium heart diseases with volume overload large left to right shunts , AV valve regurgitation.Leftparasternal pulsations and lift can be seen in large atrial septal defects and in cases of RV hypertrophy.In patients with PDA, aortopulmonary window, aortic insufficiency,aorticstenosis and CoA, suprasternal pulsations can be visible.

GENERAL APPEARANCE - SCARRight thoracotomy - right Blalock Thomas Taussig shunt, atrial septal defect repair, or mitral valve surgery. Midline sternotomy - most heart operations, especially if cardiopulmonary bypass is used. A left thoracotomy - patent ductus ligation, coarctation repair, and placement of a left Blalock Thomas Taussig shunt.

Apex BeatIn infants and children below 4 years, the apex beat is located in the fourth intercostal space (ICS).

Between 4 and 7 years it can be either in the fourth or fifth ICS and thereafter inthe fifth ICS.

Thedisplacement of the apex beat laterally or inferiorly indicates cardiac enlargement. A hyperdynamic apical impulse is seen in volume overload conditions like post-tricuspid shunts and A sustained heaving apical impulse is seen in pressure overload conditionslikeinLVOTO.

ThrillThrills at the lower sternal borderare more likelyto be associated with VSDs than mitral or tricuspid regurgitation.

Thrills at the right upper sternal border (RUSB) or suprasternal notch are most likely to be due to severe aortic stenosis.

Other less common cause of thrill in the suprasternal notch is pulmonary stenosis. Diastolic thrills are less common

Auscultationloud S1 can occur with increased flow across the AV valves fromlarge lefttoright shunts, suchas ASD,VSD,PDA.Normal splitting: - small VSD, mild aortic or pulmonic stenosisWide splitting- Volume overload (e.g., ASD, PAPVR)Pressure overload (e.g., PS)Electrical delay (e.g., RBBB)Early aortic closure (e.g., MR)Occasional normal child.

Paradoxical splitting or reversed splitting PDA, severe aortic stenosis, complete left bundle branch block, WPW syndrome Type 2.Single S2Pulmonary hypertensionOne semilunar valve (e.g., pulmonary atresia, aortic atresia, persistent truncus arteriosus)P2 not audible (e.g., TGA, TOF, severe PS)Severe ASOccasional normal childNarrowly Split S2Pulmonary hypertensionAS

Third Heart Sound

The S3 - low-frequency sound in early diastole and is related to rapid filling of the ventricle . It is best heard at the apex or lower left sternal border. It is commonly heard in normal children and young adults. A loud S3 is abnormal and is audible in conditions with dilated ventricles and decreased ventricular compliance (e.g., large-shunt VSD or CHF). When tachycardia is present, it forms a Kentucky gallop.

Fourth Heart Sound or Atrial Sound

The S4 is a relatively low-frequency sound of late diastole (i.e., presystole) and is rare in infants and children. When present, it is always pathologic and is seen in conditions with decreased ventricular compliance or CHF. With tachycardia, it forms a Tennessee gallop.

SYSTOLIC and DIASTOLIC SOUNDSAn ejection click (or ejection sound) follows the S1 very closely and occurs at the time of the ventricular ejection's onset. It is usually audible at the base (either side of the upper sternal border), whereas the split S1 is usually audible at the lower left sternal border. 1. Stenosis of semilunar valves (e.g., PS or AS). 2. Dilated great arteries, which are seen in systemic or pulmonary hypertension, idiopathic dilatation of the PA, TOF (in which the aorta is dilated), and persistent truncus arteriosus.Midsystolic click with or without a late systolic murmur is heard at the apex in mitral valve prolapse(MVP)

SYSTOLIC MURMURSMid-systolic murmurs- crescendo-decrescendo or diamond-shaped murmurs-

Bicuspid aortic valve - murmur is brief with an aortic ejection sound. The murmur is best heard over the right second interspace with little or no radiation.

valvular aortic stenosis- the maximum intensity is appreciated over the right second interspace; murmur is harsh and rough and a thrill may be present. The murmur radiates up into the neck and over both carotid arteries. In patients with aortic stenosis, the longer and later peaking murmur is usually associated with hemodynamically significant obstruction; The intensity of the murmur is variable and may not correlate with the severity of stenosis.

In supravalvular aortic stenosis, the murmur may be loudest at a slightly higher location than in valvular aortic stenosis. In subvalvular left ventricular outflow obstruction (hypertrophic cardiomyopathy), the maximum intensity of the murmur is usually located along the lower left sternal border or over the cardiac apex. It radiates poorly to the base and neck.

FIXED LVOTODYNAMIC LVOTOPULSEPARVUS AT TARDUSSharp upstroke with normal volume with STANDING - MURMURDECREASEINCREASESTRAIN phase of VALSAVA MURMUR intensity INCREASEDECREASECAROTID UNCHANGED or decreaseDECREASE

The murmur of valvular pulmonary stenosis is harsh and best heard over the left second interspace and radiates to the left side of the neck and is frequently accompanied by a palpable thrill.

A pulmonary ejection sound at the onset of the murmur may be heard and S2 is widely split with a decreased intensity of P2.

The murmur duration correlates reasonably well with the severity.

Holosystolic (pansystolic) murmursThis type of murmur is typically heard in AV valve regurgitation. Mitral and tricuspid regurgitation murmurs are high pitched, with variable intensity and blowing quality.

Diastolic Murmurs

Diastolic murmurs are usually abnormal and may be early, mid or late diastolic. Early diastolic murmurs - aortic and pulmonary regurgitation.Mid-late diastolic murmurs occur due to stenosis or increased flow across the mitral or the tricuspid valves. These murmurs can occur in mitral stenosis, tricuspid stenosis, or as flow murmurs of ASD, VSD and PDA. Late diastolic (presystolic) murmurs - pathological narrowing of the AV valves. The pulmonary regurgitation murmur in patients with normal pulmonary artery pressure, is low-pitched and early diastolic because of the low-pressure gradient. In patients with pulmonary hypertension, the murmur, known as the Graham-Steell murmur is heard, which is also early diastolic but is high pitched and decrescendo, because of the high pressure gradient between the pulmonary artery and the right ventricle in diastole.

The continuous murmur from aortopulmonary communications is loudest around the S2.

The arterial continuous murmur is characterized by a more pronounced systolic component while a venous continuous murmur is characterized by a more pronounced diastolic component.

Abdominal Examination- Normally liver is palpable (23 cm below costal margin) at midclavicular line up to 4 to 5 years of the age, after that it remains palpable up to 1 cm till late childhood. If liver is palpable further downwards and the infant is irritable it probably indicates presence of congestive heart failure. If the liver is in midline and palpable symmetrically, it indicates cardiac malpositions and underlying complex heart disease.The site of the gastric fundus is detected by percussion, which determines visceral situs. Spleen is not normally palpable; if palpable it indicates possibility of infective endocarditis.Respiratory System ExaminationUnlike adults, where crepitations is commonly basal, in neonates and infants it is more diffusely heard and often associated with rhonchi.

CXRFeature of increased PBF 1. Visibility of pulmonary vascularity in lateral 1/3rd 2. Vascularity extending below diaphragm.3. End on views 3 on oneside / 5 on both sides4. Trachea /right MPA >1.5 in children 16 mm in male and >14 mm in female), it indicates a shunt lesion. In normal children diameter of RDPA is less than that of trachea. If the ratio of RDPA to trachea is more than 1 it indicates significant left to right shunt.

Plethora occurs in left to right shunts, admixture lesions and transposition of the great arteries (d-TGA) without PS. Increased pulmonary blood flow in an acyanotic child - ASD, VSD, PDA, ECD, partial anomalous pulmonary venous return (PAPVR).

Prominent Main Pulmonary Artery Segment-1. Poststenotic dilatation (e.g., pulmonary valve stenosis)2. Increased blood flow through the PA (e.g., atrial septal defect [ASD], ventricular septal defect [VSD])3. Increased pressure in the PA (e.g., pulmonary hypertension)4. Occasional normal finding in adolescents, especially girlsDilatation of the Aorta- patent ductus arteriosus (PDA), coarctation of the aorta (COA), Marfan syndrome, or systemic hypertension.

PULMONARY VENOUS CONGESTIONPulmonary venous congestion is characterized by a hazy and indistinct margin of the pulmonary vasculature.This is caused by pulmonary venous hypertension secondary to left ventricular failure or obstruction to pulmonary venous drainage (e.g., mitral stenosis, TAPVR, cor triatriatum).NORMAL PULMONARY VASCULATUREPulmonary vascularity is normal in patients with obstructive lesions such as pulmonary stenosis or aortic stenosis. Patients with small left-to-right shunt lesions also show normal pulmonary vascular markings.

Associated anomalies -hypoplasia of the right lung and right pulmonary artery, sequestration of right lung tissue receiving arterial supply from the aorta, and other congenital heart diseases such as VSD, PDA, COA, or TOF.

ASD - CXR

1. increased PBF 2. dilated MPA, both PA3. right atrail enlargementLAE rare , seen in older adults with AF , associated MSRVH acute angle at apexLVH if MR is present in OP- ASD.scimitar syndrome- A vertical vascular shadow along the lower right cardiac border - PAPVR from the lower lobe ,pulmonary veins drain in to IVC.SV ASD localized dilatation of SVC at junction with RA.

SCIMITAR SIGN

The scimitar sign is produced by an anomalous pulmonary vein that drains any or all of the lobes of the right lung.

VSD

SMALL VSD - N , mild dilatation of pulmonary trunkMOD VSD - cardiomegaly , enlarged LA SPLAYING OF CARINA., increased PBF featuresLARGE VSD cradiomegaly , globular silhoutte- LA,LV,RV ,occ RA dilatation With increasing PVR , left to right shunt decresese , cardiac size decrease , but enlargement of pulmonary trunk persists.VSD with AR LVH is disproportionate to shunt with dilated aortic root.VSD Is a part of many congenital defects. So Xray feature is suggestive of the original defect that VSD.

PATENT DUCTUS ARTERIOSUSEnlargement of the left heart chambers.Enlargement of the ascending aorta or aortic arch.Pulmonary plethora with enlarged central and peripheral artery.Filling up of the angle between the aortic arch and the pulmonary artery : Most specific sign.Possible PDA calcification in adults.

CO AIn a heavily exposed film, the precoarctation and postcoarctation dilatation of the aorta may be seen as a figure of 3. This may be confirmed by a barium esophagogram with E-shaped indentation.Rib notching is a specific finding of COA in an older child (usually older than 5 years) and is usually found between the fourth and eighth ribs.

Left anterior oblique view of chest, obtained with barium esophagographyUnilateral rib notching :left rib:anamolous origin of right subclavian artery distal to coarctationRight rib:if it involves origin of left subclavian artery

DDS FOR RIB NOTCHINGChronic superior venacava obstructionChronic inferior venacava obstructionAortoarteritisIntercostal AV malformationsPost BT shunt

PRE TRICUSPID SHUNTPOST TRICUSPID SHUNTCARDIOMEGALYMORE( RA, RV )LESS (LA, LV)With development of PAHPERSISTREGRESSPULMONARY VASCULARITYMORE - PERIPHERAL PRUNNING +MPA DILATATIONMORELESSAORTA DILATATIONABSENTPDA & VSD with AR

ECG

ASD

Severity of shuntSize of ShuntMildModerate SevereQp/Qs2RAENoYesYesRV volume overloadNoYesYesNrsrrsR/evidence of PAHCrochetageNo No YesEvidence of PAHNo No YesR > 7mm in V1

CROCHETAGEIn inferior leads, a notch near the apex of the R wave,coined crochetage, has been correlated with ASDSeen in 73.1%, specificity - 92% (all 3 inf leads),86.1%(atleast 1 inf lead)

ASSOCIATED CONDITIONSLutembachers syndrome LAE,RVH more common,Atrial arrhythmias more common

TAPVR-resembles OS ASD,PR prolonged, AF in older personsPFO no hemodynamic changes ECG is normal or shows crochetageCommon Atrium Absent atrial septum ECG shows features of combination of OS, OP, Sinus venosus ASD

2. VSD Small shunt Normal / mild LVHModerate shunt - LVH, LAH ()Large shunt - BVH, LAHKatzwatchal phenomenon- R+S in mid precordial leads >60 / >50 in single leadInlet VSD LAD , counter clock wise loopPVOD (Eisenmenger's syndrome) RVH, RAE, R in v1, RAD >120.SIZE OF THE SHUNT 1.restrictive N / Rsr in v12. mod restrictive volume overloaded LV with BVH ,LAE3. large non restrictive BVH ,LAE , PAH Number of VSDs- multiple VSD left axis deviation with clockwise loop.GERBODE defect RAE with LV volume overload.VSD with AR deep Q ,tall R , deep inverted T ,coved ST segment in lateral precordial leads.

SITE OF VSD AxisLoopInletLADCounterclockwiseMultiple muscularLAD ClockwiseL TGA with VSDLADClockwiseDORV with VSDLADCounter clockwise

SUMMARY

AV SEPTAL DEFECTSMarked left to right shuntEarly development of PAHAssociated MR determines outcomes

AV SEPTAL DEFECTSCharacteristic finding : - left axis deviation - QRS vector counter clockwise loopMech : due to posterior and inferior displacement of the AV node and the left bundleS waves in leads II, III, and aVF have a characteristically notched upstrokeQRS pattern in right leads resembles OS-ASD-Due to longer than normal Rt bundle

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PDADiagnosis by -LV Volume overloadLAE+/- RV pressure overloadQRS axis normal

SHUNT SEVERITYSmall normalModerate LAE + LV Vol overload q & Tall R in V5-6,deep S in V1-2, T remain uprightLarge Biatrial enlargement + BV hypertrophyEisenmenger RAE + RVH

COMPLICATIONSPAHBUT Q IN V5-V6 PERSIST EVEN AFTER DEVELOPMENT OF PAHPR interval prolongation -10 20%AF in older persons with large shunt

ASSOCIATED CONDITIONSRAD Neonates , LAD- Rubella, sometimes superior axisCoA LVH with secondary ST- T changes

Pulmonary stenosisObstruction to RVOT increases afterload on RV RV systolic overloadRVH With or without RAEGood correlation with hemodynamics and ECG

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Normal ht of R in V1 =20mmShift of transition zone to leftQR in V1T waveInverted in V1/V2 onlyUpright T in V1 V2 after 4 days may be only sign Inverted upto V4-V5,In 50% cases upright in right leadsDeep symmetric inversions,upto V6T inversions II,III,AVFST depressionNoNoYes

RVSP MEASUREMENTSHt of R in V1/V4R x 5,between 2 20 yrs age, if pure R wave is present.Ht of R in V1 + 27Ht of R in V1 x 3 + 47Pressure gradient across pulmonary valve= 10.5(ISM)+2.6 (S1)+S2 Score+T score ISM=intensity of systolic murmur(gr 1-6) S1=S in lead I in mm S2 score= -10 if P2 is normal,+2 if P2 is audible but diminished,+15 if P2 is inaudible T score= +15 if T in V1 is diphasic with R in V1 >10mm,otherwise T score = 0

TYPES OF PSValvular PS As describedSub/Supra valvular sameSub infindibular PS/DCRV Degree of RVH in V1-3 less than anticipated, Upright T in V3R may be the only finding in 40% casesDysplastic Pulmonary valvular stenosis as a part of Noonan syndrome Extreme axis deviation, QRS prolonged and splintered, QS in inferior and left precordial leads

Pure PSRAD,But not markedTall R in Rt precordial leads

Deeply inverted symmetric T wavesTOF(VSD + PS)Marked RADTall R only in V1,with sudden transitionLess deeply inverted T waves/upright T in V1 & V2

EXCEPTION-Pentology LA or LV enlargementTriology marked increase in RVSP ~PS + LAEPulmonary atresia marked increase in RVSP

4. ASMild to moderate- Normal or LVHSevere - LVH with or without strain5. COAInfants younger than 6 mo- RBBB or RVHOlder children- LVH, normal, or RBBB6. Anomalous origin of the left coronary artery from the PA - Myocardial infarction, anterolateral

COR TRIATRIATUMRAE, RVH,RAD are commonLAE may be seen due to prolonged conduction in proximal accessory chamber

REGURGITANT LESIONSCONGENITAL PRNormal in mild-mod casesRV volume overload in severe casesAF unlikely

CONGENITAL ARLV volume overload with LV q waves

APPROACH

Introduction- ASD

Incidence is 1 child per 1,500 live births

More frequent in females than males by about 2:1

Most ASDs occur sporadically; however, a few families have the defect as a genetic abnormality.Mutation in-TBX5, NKX2.5 , GATA4, Myosin heavy chain 6

Samanek M et al. Pediatr Cardiol1999;20:4117

Small ASD ASYMPTOMATICMODERATE ASD- symptoms starts at 2nd decadeSymptoms after 40 yrs 1. Decrese in LV distensibility -> increased left to right shunt2. Age related increase in atrial arhhythmias precipitates CHF3. Development of PAH .By 4th decade 50% will became symptomatic, 5th decade 75% will become symptomatic.Depending on ASD size and PBF 7% will develop Eisenmengerization in 1st decade and 8% in 2nd decade.Infective endocarditis does not occur in patients with isolated ASDs.

SURGICAL CLOSURE -1. Primum septal defects, sinus venosus and coronary sinus defects and defects with associated anomalous pulmonary venous drainage.2. Secundum atrial defects that are larger than 38 mm in diameter or defects that have insufficient rims (< 5 mm) are also not suitable for transcatheter device closure.DEVICE CLOSURE- Devices available for clinical use have included the Sideris buttoned device, the Angel Wings device, the CardioSEAL device, and the Amplatzer ASD Occlusion Device.There must be enough rim (4 mm) of septal tissue around the defect for appropriate placement of the device.The patients are administered aspirin 81 mg/day for 6 months.

VSDThe incidence of spontaneous closure in perimembranous and muscular VSDs is high, while it is low in outlet defects and inlet defects do not close.60 percent close before 3 years and 90 percent by 8 years of age.Surgery for Ventricular Septal Defect-

RECOMMENDATIONS FOR DEVICE CLOSURE OF MUSCULAR VSDS-Class IIa - Class IIA is reasonable for infants who weigh 5 kg, children and adolescents with hemodynamically significant muscular ventricular septal defect (MVSD) to undergo percutaneous VSD device closure (level of evidence: B).

Exclusion criteria - Weight less than 3.0 kg (unless the hybrid perventricular approach is used); distance of less than 4 mm between the VSD and the aortic, pulmonic, mitral or tricuspid valves. Except the subpulmonic and inlet VSDs, most of the other VSDs can be closed without surgery.

PDAINDICATIONS FOR PDA CLOSURE Premature InfantsPDA closure is indicated in all patients less than 10 days of age with symptomatic PDA, who had no response to indomethacin and for those who are more than 10 days old. Symptomatic PDA is defined as respiratory rate of more than 70 per minute, heart rate more than 160 per minute, liver enlargement more than 3 cm below costal margin and cardiomegaly more than 60 percent on chest X-ray.Children and AdultsSymptomatic and even asymptomatic PDA should be closed to avoid CHF, pulmonary vascular disease, IE and aneurysm formation.

Gianturco coils- The coil diameter should be at least two times the minimum PDA diameter and yet fit within the ampulla of the ductus.The immediate success rate using single or multiple coils ranges from 67 to 95 percent, almost invariably related to ductus size.Although ducts as large as 7 mm have been closed with coils, a larger ductus diameter is an unfavorable factor.Sideris Buttoned Device-Gianturco-Grifka Vascular Occlusion Device- ideal for long tubular PDADuct-occlud and Nit-occlud Device- for 40 mm Hg4. Clinically significant pulmonary valvar obstruction in the presence of RV dysfunction

A balloon is chosen that is 20 to 40 percent larger than the angiographically measured pulmonary valve annulus and it is positioned over a guidewire with the valve at its midpointSurgical Technique - Indications1. Dysplastic pulmonary valve with valve ring hypoplasia.2. Fixed infundibular and supravalvar stenosis with pulmonary valvar stenosis.

Natural history- CO ABimodal presentation. produces significant symptoms in early infancy and after age 20 to 30 years. Neonates with severe coarctation become acutely symptomatic when the ductus closes. Most who survive the hazards of infancy reach adulthood25% die by age 20 years,50% die by age 30 years75% die by age 50 years.Survival has been reported at age 74 years and 76 years.natural history of acynotic chd

CO AIndications for treatment- 1. In infants with hemodynamic compromise .2. In older patients a consistent systolic blood pressure gradient between the arms and legs of more than 20 mm Hg 3. Systemic hypertension at rest or following provocation with exercise greater than the 97th percentile for age along with evidence of important luminal stenosis.Surgical treatments-End-to-End Anastomosis Including Extended RepairSubclavian Flap RepairInterposition Grafts, Bypass Tube, Extra-anatomic Bypass GraftsPatch AortoplastyInterventional treatments-1. Balloon Angioplasty-sick hemodynamically compromised infantas a bridge to definitive surgical repair , children weighing less than 25 kg when faced with significant residual or early recurrence of gradient2. Stent Placement- Covered stents preferred where possible.

RSOVM:F- 4:1More in asiansPresentationAcute tearing chest pain/interactable sobSmall shunt asymptomatic 20% Small shunt incidious onset sob 45%Large shunt acute hf 35%

natural history of acynotic chd

RSOVnatural history of acynotic chdInvolvement ofRCC 67%NCC - 25%LCC 8%Rupture in to RV - 70-75%RA 15-20%LA 5-10%LV -