congenital heart diseases. acyanotic heart disease

CONGENITAL HEART DISEASES

ACYANOTIC HEART DISEASE

Acyanotic Heart Diseases

LEFT 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


•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


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


•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


•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


•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


•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


•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


•Clinical Manifestations▫Heart failure and intercurrent pulmonary

infection▫Enlarged liver▫Failure to thrive


•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


•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


•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


•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)


•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


•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


•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


•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


•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


•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


•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


•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


•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


•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•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


•Notching of the inferior border of the ribs due to enlarged collateral vessels

•Other dx:▫2D echo▫CT and MRI▫Cardiac

Catheterization


•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


•Common in patients with Marfan syndrome, straight back sydrome, pectus excavatum, scoliosis, Ehlers-Danlos syndrome, Osteogenesis Imperfecta, and pseudoxanthoma elasticum


•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

congenital heart diseases. acyanotic heart disease

Documents

atrial septal defect

right shunt slide

surgical slide

pulmonary artery slide

right border

right precordial leads

acyanotic heart disease

mitral stenosis slide