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