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CONGENITAL HEART DISEASES. ACYANOTIC HEART DISEASE. Acyanotic Heart Diseases. LEFT TO RIGHT SHUNTS. OBSTRUCTIVE LESIONS. REGURGITANT LESIONS. LEFT TO RIGHT SHUNT. Determinants of L to R shunting. Size Of the Defect. Relative Compliance of the Right and Left Ventricle. - PowerPoint PPT Presentation
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CONGENITAL HEART DISEASES
ACYANOTIC HEART DISEASE
Acyanotic Heart DiseasesLEFT TO RIGHT
SHUNTS
OBSTRUCTIVE LESIONS
REGURGITANTLESIONS
Acyanotic Heart Disease
Increased Volume Load
LVE
VSD, PDA, AVSD
RVE
ASD, PAPVR
Increased Pressure Load
LVH
Coarcation of the Aorta, Mitral regurgitation
Aortic Stenosis
RVH
Pulmonary Stenosis,
Mitral Stenosis
LEFT TO RIGHT SHUNT
Determinants of L to R shunting
Size Of the Defect
Relative Compliance of the
Right and Left VentricleRelative Vascular
Resistance in the Pulmomary and
Systemic Circulations
Communication between the pulmonic and
systemic circulation
LEFT TO RIGHT SHUNT
Increase blood flow to the lungs
HEART FAILURE SIGNS
Heart Remodeling(dilatation of the heart)
Increase Sympathetic nervous system
Increase pulmonary vascular resistance(EISENMENGER
PHYSIOLOGY)
Left to Right Shunt• Atrial Septal Defect• Atrioventricular Septal Defect• Partial Anomalous Pulmonary Venous
Retur• Ventricular Septal Defect• PDA• Coronary-AV Fistula• Ruptured Sinus of Valsalva Aneurysm
Atrial Septal Defect
Atrial Septal Defect•Secundum
Most common form and is associated with structurally normal atrioventricular valves
Region of the fossa ovalis May be single or fenestrated, openings ≥2cm
in largest diameter are common in symptomatic older children
Atrial Septal Defect
Normal Left Ventricle and Aorta
Enlargement of the right atrium
Enlargement of the pulmonary artery
Atrial Septal Defect•Clinical Manifestations and findings
▫Wide and fixed splitting 2nd heart sound▫Mild left precordial bulge▫Right ventricular systolic lift on the left sternal
border▫Systolic ejection murmur at the left middle and
upper sternal border▫Loud 1st heart sound and sometimes a pulmonic
ejection click▫Short rumbling Mid-diastolic murmur on the
lower left sternal border
Atrial Septal Defect•Diagnostics
▫Chest Xray:RVE, RAE, pulmonary artery is large and pulmonary vascularity is increased
▫ECG: normal or right axis deviation and a minor right ventricular conduction delay (rsR pattern in the right precordial leads
Atrial Septal Defect•Diagnostics
▫2D echo increased right ventricular-end-diastoic
dimension and flattening and abnormal motion of the ventricular septum (anterior movement in systole or it remains straight.
ASD confirmed by pulsed and color flow Doppler
Atrial Septal Defect•Treatment
▫Surgical or transcatheter device closure is advised for all symptomatic patients and also for asymptomatic patients with a Qp:Qs ratio of at least 2:1
Sinus Venosus Atrial Septal Defect•Upper part of the atrial
septum in close relation to the entry of the SVC
•May be related to Partial Anomalous Pulmonary Venous return
•Sometimes the superior vena cava straddles the defect (rarely involves the IVC)
•Tx: Surgical
Partial Anomalous Pulmonary Venous Return•May drain into the: SVC or IVC, Right
atrium, Coronary Sinus•May involve some or all of the veins from
only 1 lung (right>left)•Scimitar Syndrome
▫An anomalous vein draining into the IVC is visible on Chest Xray as a crescentic shadow of vascular density along the right border of the cardiac silhouette
Scimitar Syndrome
Partial Anomalous Pulmonary Venous Return•Dx: 2D Echo•Cardiac catheterization
▫Selective pulmonary arteriography: presence of anomalous pulmonary veins
▫Descending aortography: anomalous pulmonary arterial supply to the right lung
•Prognosis: Excellent•Tx: Surgical if large left-to-right shunting
Atrioventricular Septal Defects(Ostium Primum and AV Canal Defects)
•Situated in the lower portion of the atrial septum and overlies the mitral and tricuspid valve
•A cleft in the anterior leaflet of the mitral valve can be seen
Atrioventricular Septal Defects(Ostium Primum)
•Pathophysiology: left to right shunt across the atrial defect and mitral (or occasionally tricuspid insufficiency
Pulmonary arterial pressure is typically normal or only mildly increased
Atrioventricular Septal Defects(Ostium Primum)
•Clinical Manifestations▫Asymptomatic▫History of exercise intolerance, easy
fatigability and recurrent pneumonia (with large defects and severe mitral insufficiency)
▫Harsh or occasionally high-pitched apical holosystolic murmur (due to mitral insufficiency)
▫Cardiac enlargement and hyperdynamic precordium
Atrioventricular Septal Defects(Ostium Primum)
•Clinical Manifestations▫Other findings: normal or accentuated 1st
heart sound; wide, fixed splitting of the 2nd sound; pulmonary ejection murmur sometimes preceeded by a click; low-pitched and diastolic rumbling murmur at te lower left sternal edge or apex or both
Atrioventricular Septal Defects(AV Canal Defects)
•AV canal defect or endocardial cushion defect
•Contiguous AV septal defects with markedly abnormal AV valves
Atrioventricular Septal Defects(Ostium Primum and AV Canal Defects)
•Complete form: single AV valve common to both ventricles and consists of an anterior and a posterior bridging leaflet related to the ventricular septum with a lateral leaflet in each ventricle▫Common to Down
syndrome
Atrioventricular Septal Defects(AV Canal Defects)
•Left or right dominant AVSD due to hypoplasia of one of the ventricles
Atrioventricular Septal Defects(AV Canal Defect)
•Pathophysiology▫L to R shunting occurs at both atrial and
ventricular levels; with shunting from the left ventricle to the right atrium (due to the absence of the AV septum)
▫Pulmonary hypertension and increased tendency to develop pulmonary vascular resistance right to left shunting cyanosis (Eisenmenger syndrome)
▫AV valvular insufficiency
Atrioventricular Septal Defects(AV Canal Defect)
•Clinical Manifestations▫Heart failure and intercurrent pulmonary
infection▫Enlarged liver▫Failure to thrive
Atrioventricular Septal Defects(AV Canal Defect)•PE findings
▫Cardiac enlargement▫Systolic thrill at the lower left sternal border▫Precordial bulge and lift▫Normal or accentuated 1st heart sound▫Widely split 2nd heart sound▫Low pitched, mid-diastolic rumbling murmur,
lower left sternal border▫Pulmonary systolic ejection murmur▫Harsh apical holosystolic murmur
Atrioventricular Septal Defects(AV Canal Defect)
•CXR: prominent ventricles and atrium• 2D echo
▫RVE with encroachment of the mitral valve echo on the left ventricular outflow tract
▫“gooseneck” deformity of the left ventricular outflow tract
▫Both valves insert at the same level▫Common AV valve
Atrioventricular Septal Defects(AV Canal Defect)
•Treatment▫Ostium septum defect: Patch prosthesis for
the closure of ASD and direct suture for the cleft in the mitral valve
▫AV septum defect: surgical operation during infancy (due to the risk of pulmonary vascular disease as early as 6-12 months of age)
Patent foramen Ovale•Not an ASD•Left to right shunting is unusual but may
occur in the presence of a large volume load or hypertensive left atrium
•Does not require surgical treatment but may be a risk for paradoxical systemic embolization.▫Device closure is considered if there is a
history of thromboembolic stroke
Ventricular Septal Defect•Most common cardiac malformation (25%
of CHD)•Different types:
▫Membranous type: most common▫Infundibular types▫Muscular▫Supracrista
Ventricular Septal Defect•Determinants of the magnitude of the L
to R shunting▫Size
restrictive VSD (<5mm)▫Level of Pulmonary resistance in
relation to systemic resistance Nonrestrictive VSD (>10mm)
Ventricular Septal Defect•At birth, the pulmonary vascular resistance
is elevated, thus the size of the left to right shunting is limited
•Few weeks after birth, there is decrease in pulmonary resistance
•With continued exposure of the pulmonary vascular bed to high systolic pressure and high flow, pulmonary vascular obstructive disease develops Eisenmenger syndrome
Ventricular Septal Defect•Clinical Manifestations
▫Small VSDs Asymptomatic Loud, harsh or blowing holosystolic murmur
on the left lower sternal border (frequently with thrill)
In neonates with VSD on the apical muscular septum, murmur heard on the apex
Ventricular Septal Defect•Clinical Manifestations
▫Large VSD Dyspnea, feeding difficulties, poor growth,
profuse perspiration, recurrent pulmonary infections and cardiac failure
Cyanosis Prominence of the left precordium (palpable lift) Laterally displaced apical impulse and apical
thrust Systolic thrill
Ventricular Septal Defect•Clinical Manifestations
▫Large VSD Less harsh but more blowing systolic murmur Increased pulmonic component of the 2nd
heart sound Mid-diastolic, Low pitched rumble at the
apex Increased blood flow to the mitral valve Qp:Qs ratio ≥2:1
Ventricular Septal Defect•Diagnosis
▫Chest Xray Small VSDs: normal or minimal cardiomegaly
and borderline increased in pulmonary vasculature
Large VSDs Gross cardiomegaly with prominence of both
ventricles, left atrium and pulmonary artery Pulmonary vascular markings are increased Pulmonary edema
Ventricular Septal Defect•Clinical Manifestations
▫2D echo Helpful in estimating shunt size, the degree
of volume overload, the increased dimensions of chambers and presence of other valve defects (including aortic valve insufficiency or prolapse)
Pulse doppler examination calculates pressure gradient across the defect
Ventricular Septal Defect•Treatment
▫30-50% close spontaneously, most frequently during the 1st 2 years of life Small muscular VSDs > membranous
Ventricular Septal Defect•Treatment
▫Indications for surgical closure Large defects in whom clinical symptoms and
failure to thrive cannot be controlled medically Infants between 6 and 12 months with large
defects associated with pulmonary hypertension Patients older than 24 months with Qp:Qs ratio
greater than 2:1 Supracristal VSD
▫Contraindication to surgery: severe pulmonary vascular disease nonresponsive to pulmonary vasodilators
Patent Ductus Arteriosus•Location: the aortic end is distal to the origin
of the left subclavian artery and enters the pulmonary artery at its bifurcation
•Associated with maternal rubella infection•Preterm: the smooth muscle in the wall is less
responsive to high PO2 and less likely to constrict after birth; normal structure PDA
•Term: the wall is deficient in both the mucoid endothelial layer and the muscular media
Patent Ductus Arteriosus•Clinical Manifestations
▫Bounding peripheral pulses and a wide pulse pressure, due to runoff of blood into the pulmonary artery during diastole
▫Machinery like murmur▫Thrill, maximal in the 2nd left interspace
with radiation toward the left clavicle, left sternal border and apex
Patent Ductus Arteriosus•Treatment
▫Surgical or catheter closure
Aorticopulmonary Window Defect•Consists of a communication between the
ascending aorta and the main pulmonary artery
•Unlike truncus arteriosus, there is presence of pulmonary and aortic valves and an intact ventricular septum
•Systolic murmur with an apical mid-diastolic rumble (due to increased blood flow across the mitral valve)
•Tx: surgical
Coronary-Cameral Fistula•A congenital fistula existing between a
coronary artery and an atrium, ventricle or pulmonary artery
•Clinical signs may be similar to PDA but diffuse
•Diagnosis: doppler echocardiography and cardiac catheterization
•Treatment: small fistuals may close spontaneously, larger fistulas may require catheter intervention or surgical closure of the fistula
Ruptured Sinus of Valsalva Aneurysm•Happens when one of the valsalva of the
aorta is weakened by congenital or acquired disease and ruptures in to the right atrium or ventricle
•Acute heart failure with new loud to and fro murmur
•Left to right shunt at the area of the atrium or ventricle
•Urgent surgical repair is required
Acyanotic Heart Disease
Increased Volume Load
LVE
VSD, PDA, AVSD
RVE
ASD, PAPVR
Increased Pressure Load
LVH
Coarcation of the Aorta, Mitral regurgitation
Aortic Stenosis
RVH
Pulmonary Stenosis,
Mitral Stenosis
Increased Pressure Overload•Cardiac output is maintained•Increased wall thickness (hypertrophy)
Acyanotic Heart Diseases
• Pulmonary Stenosis• Coarctation of the
Aorta• Pulmonary Venous
Hypertension
OBSTRUCTIVE LESIONS
REGURGITANTLESIONS
• Pulmonar Valve Insufficiency
• Congenital Mitral Insufficiency
• Mitral Valve prolapse
• Tricuspid Regurgitation
Acyanotic Congenital Heart Disease: Obstructive Lesions
Pulmonary valve stenosis •The valve cusps are deformed to various degrees
thus the valve opens incompletely during systole•May be severely fused or if not, may produce a
dome like obstruction to right ventricular outflow tract during systole
•May be a result of valve dysplasia seen in Noonan syndrome
•May be also associated with Algallie syndromwe when the pulmonary stenosis is either of the valve or the branch pulmonary arteries
Pulmonary valve stenosis•Severity depends on the size of the
restricted valve openinng▫Severe: increased right pulmonary pressure
•Pulmonary artery pressure is normal•Arterial oxygenation will be normal even in
cases of severe stenosis except if with intracardial communication
•In neonates, decreased right ventricular compliance leads to cyanosis due to right to left shunting through a patent foramen ovale
Pulmonary valve stenosis•Clinical Signs
▫Mild: sharp pulmonic ejection click after the 1st heart sound and split 2nd heart sound
▫Severe stenosis: The pulmonary component of the 2nd sound is inaudible; harsh systolic ejection murmur on the pulmonic area with radiation over the entire precordium, to both lung fields, neck and back
▫Enlarged right ventricle and right atrium▫Prominence of the pulmonary artery segment
due to post-stenotic dilatation
Pulmonary valve stenosis•Tx:
▫Initial treatment: Balloon valvuloplasty▫Surgical
Aortic Stenosis•Valvular: the leaflets are thickened and
the commisures are fused to varying degrees
•Subvalvular (subaortic): discrete fibromuscular shelf below the aortic valve▫Associated with mitral valve stenosis and
coarctation of the aorta (Shone syndrome)•Supravalvular aortic stenosis
▫Least common; associated with Williams syndrome
Aortic Stenosis•Clinical findings
▫Early systolic ejection click, best heard at the apex and left sternal edge
▫The click does not vary with respiration▫If severe, the 1st heart sound is diminished▫Paradoxical splitting of the 2nd heart sound
Aortic Stenosis•Diagnosis
▫Xray: Normal or prominent ascending aorta▫ECG: left ventricular hypertrophy▫2D echo: left ventricular hypertrophy
May shows the presence of the number of leaflets of the aortic valve and their morphology and other associated abnormalities
In neonates with critical aortic stenosis, presence of endocardial fibroelastosis (bright in 2D echo, indicative of scarring of the endocardium)
Aortic Stenosis•Treatment
▫Balloon valvuloplasty: moderate to severe valvular aortic stenosis
▫Ross procedure: aortopulmonary translocation
Coarctation of the Aorta• Juxtaductal coarctation
▫Most common▫Just below the left
subclavian artery at the origin of the ductus arteriosus
• Turner’s syndrome• Shone complex: when
associated with mitral valve abnormalities and subaortic stenosis
Coarctation of the Aorta•A tubular hypoplasia of the transverse
aorta starting at one of the head or neck vesssels and extending to the ductal area (preductal or infantile type coarctation)
Coarctation of the Aorta
Coarctation of the Aorta•Discrepancy in blood
pressure and pulses of the amrs and legs
•Radial-femoral delay▫occurs when blood
flow to the descending aorta is dependent on collaterals
▫femoral pulse is felt after the radial pulse
Coarctation of the Aorta•Notching of the
inferior border of the ribs due to enlarged collateral vessels
•Other dx:▫2D echo▫CT and MRI▫Cardiac
Catheterization
Coarctation of the Aorta•Tx:
▫Prostaglandin E: to reopen the ductus and re-establish adequate lower extremity blood flow
▫Surgical
Coarctation of the Aorta•Tx:
▫Prostaglandin E: to reopen the ductus and re-establish adequate lower extremity blood flow
▫Surgical
Acyanotic Congenital Heart Disease: Regurgitant Lesions
Pulmonary valvular Insufficiency•Usually rare•Clinical signs
▫Descrescendo diastolic murmur at the upper and midleft sternal border
•Dx:▫Chest Xray: Right ventricular enlargement
and prominence of the main pulmonary artery
▫2D Echo
Mitral valve insufficiency•Usually associated with other cardiac
anomalies•Isolated cases: mitral valve annulus is
usually dilated, the chordae tendinae are short and may insert anomalously and the valve leaflets are deformed
Mitral valve insufficiency•High-pitched apical holosystolic
murmur•If in severe cases: may be associated with
mid-diastolic rumbling murmur•Enlarged left atrium and Left ventricular
hypertrophy•Tx: mitral valvuloplasty
Mitral valve prolapse•Caused by billowing of one or both mitral
leaflets especially the posterior cusp, into the left atrium toward the end of systole
•Prolapse: defined by single or bileaflet prolapse of ≥2mm beyond the long axis of the annular plane with or without leaflet thickening▫Classic: >5mm valve thickening
Mitral valve prolapse•Common in patients with Marfan
syndrome, straight back sydrome, pectus excavatum, scoliosis, Ehlers-Danlos syndrome, Osteogenesis Imperfecta, and pseudoxanthoma elasticum
Mitral valve prolapse•Apical murmur is late systolic and may be
preceded by a click•2D echo: posterior movement of the
posterior mitral leaflet during mid-or late systole or pansystolic prolapse of both the anterior and posterior leaflets
Tricuspid Regurgitation•Isolated tricuspid regurgitation occurs
with ebstein anomaly of the tricuspid valve•This often accompanies right ventricular
dysfunction•May be related with perinatal aspyxia due
to increased susceptibility of the papillary muscles to ischemic damage and subsequent transeint papillary muscle dysfunction