Acyanotic; LEFTRIGHT SHUNT (VOLUME OVERLOAD)

VSD
Incidence - 30% of all CHD (commonest) Site/Type site: - single defect in membranous part of ventricular septum adjacent to tricuspid valve - less commonly; single/multiple defects in muscular part of septum types: Ostium secundum common affects upper part of the septum

ASD
- 7% of all CHD - in female (F:M = 2:1)

PDA
- in female (F:M = 2:1) - in prematures - usually assctd with maternal rubella

Ostium Primum

* C/P of VSD depends on 2 factors: i- Size of VSD ( large VSD appear earlier) ii - Presence of PHTN Symptoms Small VSD - Asymptomatic Large VSD - tachpnea : very early - excessive sweating on feeding - repeated chest infection (pulm congest) - failure to thrive (poor syst circulation) *in CHF, baby looks dyspneic and seriously ill - failure to thrive - labored respiration with tachypnea - prominent precordium, intercostals retraction, left parasternal pulsation small peripheral pulse (poor systemic circulation) cardiomegaly ++ laterally displaced hyperkinetic apex S2 - split & 2nd pulmonary sound + gallop rhythm (if there is HF)

less common affects lower part of septum, may involve mitral valve incompetence (dt cleft in anterior cusp) less serious more serious small : asymptomatic large : - retarded growth ( syst BF) - repeated chest infection ( pulm BF) - dyspnea + easy fatigue on effort

Signs - weight - respiration - chest exam i) Pulse & BP

- normal growth - weight not affected - normal respiration - normal chest exam normal

small : asymptomatic large : - underweight - repeated chest infection - dyspnea on exertion - left HF (may develop in infancy in severe cases) - small and thin child, underweight - pink in colour (NO cyanosis)

normal

ii) Heart size

- normal size - normal apical thrust normal

RV enlargement (right ventricular pulsation at left sternal border) S1: normal S2 over pulmonary area: wide split & fixed in all phase of respiration - dt constantly RV diastolic volume & long ejection time

iii) HS

large PDA: - collapsing pulse (Water hammer) - low diastolic BP + wide pulse press small : normal size + prominent apical impulse large : enlarged + dynamic LV apex Pulmonary S2 is loud.

iv) Thrills -systolic-thrills

felt at lower left sternal border

continuous/systolic thrill on pulmonary area

v) Murmur

harsh pansystolic murmur rd th - on lower left sternal border (3 & 4 intercostals space)

pulmonary soft ejection-systolic murmur of grade 2-3 - dt BF thru pulm valve - heard at left middle & upper sternal border

CXR

normal

ECG

normal

i) cardiomegaly (both ventricles & LA) ii) prominent PA iii) plethoric lungs ( pulm BF prominent pulm artery branch) biventricular + LA hypertrophy

i) Enlarged RV ii) Large PA iii) vascularity of the lungs O.secundum: partial right br. block, mild RV hypertrophy, PR interval prolongation O. primum : as in o.sec + left axis deviantion + biventricular hypertrophy shows the site & size of the defect

- harsh or machinery in character - continuous (syst+diastolic) or may be systolic only nd - heard on 2 left parasternal i/space - radiates to left clavicle + down to left sternal border i) Normal size/ LV enlargement ii) Prominent PA iii) Plethoric lung ( pulm vascularity) normal/left or biventricular enlargement

Echo-Doppler Complications

shows small defect infective endocarditis

shows large defect + PA dilatation i) repeated chest infection ii) congestive HD (in early infancy) iii) infective endocarditis iv) Eisenmenger syndrome

Prognosis (natural history)

Rx

i) spontaneous closure of small defects during childhood (more than 60% of i) spontaneous closure rare cases) ii) ii) relative closure : VSD becomes relatively smaller when the heart grows O. Secundum O. Primum iii) pulmonary HTN : marked left to right shunt press in pulm circultn HF develops in HF develops in excessive VC of pulm arterioles muscularization of pulm artery adulthood (rare in infancy/childhood obliteration of pulm arterioles irreversible in pulm vascular bed children) reversed shunt (Eisenmenger syndrome; cyanosis, inoperable) *may cause death Actual closure or relative closure Develops complication death before 40 y/o. i) follow up in childhood i) antibiotic prophylaxis against Medical : Medical : ii) antibiotic prophylaxis against bacterial endocarditis - same as VSD - same as VSD bact endocarditis ii) initial medical Rx of CHF Transcatheter : Transcatheter : iii) elective surgical closure if iii) surgical closure in early infancy or - closure of some ostium secundum - occlusion of PDA by a coil device spontaneous closure doesnt occur childhood Surgical : Surgical : - patch closure of ostium primum defect, - ligation & division of PDA a.s.a.p in Medical (for complications & prevention of bacterial endocarditis): multiple defects or large ostium secundum large PDA i) for CHF = furosemide + captopril + digoxin ii) prophylactic antibiotic against bacterial endocarditis Before tooth extract, oral, upper resp tract, GIT or urinary procedures Surgical: 1. ampicillin + gentamycin, IM/IV, 30mins before & 6 hrs after procedure i) Corrective surgery : complete repair (patching) Or ii) Palliative surgery : banding of pulmonary artery 2. vancomycin (1hr IV infusn) + gentamycin IM/IV, 30 mins before & 8 hrs after procedure Or iii) Transcatheter
3.amoxicillin OR erythromycin, oral, 1 hr before & 6hrs after procedure

shows size of the ductus i) Infective endocarditis (at any age) ii) Pulmonary/systemic emboli iii) Congestive HF (with large PDA) iv) PHTN reversal of shunt Eisenmenger syndrome small : normal life style but late complications large : early complications

Acyanotic; STENOTIC (PRESSURE OVERLOAD)

PS
Incidence Type i) Valvular : fusion of malformed semilunar pulm. cusps ii) Subvalvular (infundibular) : hypertrophy of muscular RVOT (infundibulum) --> obstruction of flow iii) Supravalvular : narrowing of main PA or its branch Mild : Asymptomatic Severe : symptoms related to RV failure (cyanosis, edema etc)

Cyanotic CONGENITAL HD
Fallots tetralogy
50% of cyanotic congenital HD Component of classic F4: 1. Pulm stenosis, of infundibular type severe obstruction of RVOT hypoplastic main pulm artery +pulm. arterial tree 2. VSD; large VSD, in membranous part. 3. Overriding of aorta; aorta receives blood from both ventricles cyanosis 4. RV hypertrophy - pink (birth) cyanosis later by time - clubbing of fingers & toes (long case) - retarded growth - intolerance to effort - squatting after exercise (symptomatic relief) dt: > prevention of unsaturated venous blood from legs to return to heart > obstruction of femoral artery aortic pressure right to left shunt - cyanotic spells (hypoxic, blue spells) = spasm of infundibulum minimal pulm BF deep cyanosis, dyspnea, irritability, unconsciousness, convulsion, death *pulm murmur diasappear during the spells no clinical enlargement, but RV is easily felt (left parasternal pulsation)

AS
in boys i) Valvular : cusps are fused at their edges (stenosis) ii) Supravalvular : narrowed ascending aorta above the valve iii) Subvalvular (subaortic) : fibrous ring around the LVOT

Coarctation of Aorta
= constriction (narrowing) of varying degree of a segment of aorta - 98% occurring just below the origin of left subclavian artery at the origin of ductus arteriousus (Juxtaductal Coarctation)

Symptoms

Signs

- NO cyanosis

- pulse - BP

normal normal

- Mild & moderate : asymptomatic - few : anginal pain & feeling of dizziness on effort - some: fainting or unconsciousness on effort - Severe cases : CHF - mildly affected children usually grow well - NO cyanosis anacrotic (plateau type) & of small volume low PP and systolic BP

most cases : asymptomatic st severe : CHF in 1 few days/wks of life may present by complication as bacterial endocardiitis

- baby is well developed and pink

- heart

- RV pulsation over left parasternal

enlarged with heaving LV impulse

- brachial pulse : normal - femoral pulse : weak/absent - systemic HTN is BP measured in UL - BP in LL is < than BP in UL (usually 10mmHg) - forcible apex (dt LV hypertrophy) *collateral arteries to bypass the constrictions around the scapula (visible/palpable)

- systolic thrill - HS

2 left space

nd

- murmur

P2- more weak & more delayed as stenosis increases * in severe cases, it is not heard & S2 is single (aortic component only) harsh ejection-systolic murmur - max at 2nd left interpace - propagated to the back

palpable at upper sterna border + in suprasternal notch + along carotid arteries A2 may be weak or absent

may be palpated at pulm area

S2 single S2 on pulmonary area (aortic component only)

harsh ejection-systolic murmur - best heard at 1st & 2nd aortic area - transmitted upwards to the neck & frequently to the apex

ejection systolic murmur - may be heard at 2nd aortic area + suprasternal notch +interscapular area

*murmur of PS - harsh ejection-systolic murmur - max at 2nd left interspace *NO murmur of VSD (equal pressure)

CXR

- post stenotic dilatation of PA (in valvular stenosis) - oligemic lung field - severe cases : enlarged RV & RA

- normal heart or LV enlargement - poststenotic dilatation of aorta

ECG

Etc

- right axis deviation - RV hypertrophy - cor-pulmonale Prognosis: mild : compatible with long life severe : HF before middle age

LV hypertrophy

- NO abnormality or LV dilatation - notch in aortic shadow at the site of coarctation + post stenotic dilatation - rbd notching dt erosion by dilated i/costal arteries (collateral circulation, >10 yrs) normal or LV hypertrophy

- heart is normal in size + boot-shaped - apex is raised over diaphragm (RVH), concavity in region of pulmonary artery - oligemic lungs - large ascending aorta + right aortic arch (25% cases) RV hypertrophy + RA hypertrophy

Complications: - bacterial endocarditis - HF - risk of sudden death on strenuous exercise

Rx

- Transcatheter : balloon dilatation (valvuloplasty) in moderate cases - Surgical : pulmonary vulvotomy if PS is severe or subsalsular

Medical: - prevention against bact endocarditis - diuretic for HF Transcatheter : balloon dilatation (valvuloplasty) in some selected cases - Surgical (severe cases) : open aortic vulvotomy *aortic valve replacement postponed until late in adolescence

Surgical : resection & anastomosis by aortic graft or subclavian flap angioplasty *done at 4 y/o t prevent restenosis Transcatheter : balloon dilatation with/out stent implantation

Echo : - aorta overriding the VSD in varying degree - RV infundibular hypertrophy & degree of RVOT is measured - small sized main PA & its branches Complications: - severe spells loss of consciousness & occasionally hemiparesis * seldom death - polycythemia 2ry to hypoxemia (even in the presence of iron-def anemia Hct blood viscosity CNS thromboembolism) - brain abscess (<2 y/o children) - bacterial endocarditis - extremely rare HF (NO overload) Medical 1. antibiotic proph to bact endocarditis (like vsd) 2. Rx of cyanotic spells - administer O2 - knee-chest position (squatting) - morphine sulphate ( hyperstimulatn of RC) - IV B-blocker ( infundibular spasm) - IV sodium bicarbonate (if acidosis is present) - emergency systemicpulm shunt surgery Surgery 1. Palliative (Taussig-Blalock operation = subclav artery is anastomosed to PA by graft insertion) - when effort intolerance is evident/ breathless child after short walk/arterial O2 sat <75% 2. Total correction - closure by patch/ resection of infundibular muscle/widening of PA by transannular patch