Transcript Acyanotic Congenital Heart Disease

Cardiovascular disease
in children
Prepared by
Dr.Aso Faeq Salih
Pediatric cardiologist
2013-2014
Incidence & Epidemiology :
 0.5 – 0.8 % of a live births (5 – 8/1000 live births ).
 3 – 4 % of still born.
 10 – 25 % of abort uses
 2 % in premature babies .
 Associated non cardiac malformation noted in identifiable
syndromes may be seen in as many as 25 % .
 Leading cause of death in children with congenital
malformation
Etiology :
 The cause of most CHD is unknown .
1) Primary genetic factors ( 10 %): e.g. chromosomal
abnormalities or single gene abnormalities.
Down syndrome (50 %),
Edward (90 %),Turner(40%)
Marfan syndrome , William syndrome .
2) Environmental factors : (2 – 4 %)
 Adverse maternal conditions:
D.M, SLE , phenylketonuria ,congenital Rubella &
coxsackie B viral infections.
 Maternal ingestion of drugs:
1. Anticonvulsants (phenytoin , Na valproate ).
2. Lithium. 3. Warfarin. 4. Antimetabolites.
5. Aspirin . 6. Trimethadione 7. Ethanol.
8. Isotretinoin
3) Multifactorial (85 % )
 Gender role :
TGA , left sided obstructive lesions  > in males .
VSD , ASD , PS  > in females .
 Race :
No racial difference .
TGA > in white infants .
supracristal VSD > in Asian children .
Diff Dx: Congenital Heart Disease
Atrial
Shunts
ACYANOTIC
Obstruction
Ventricular
Great Art.
AS, Coa, PS
Both
CHD
Tetralogy of Fallot
CYANOTIC
Transposition of the GA's
TAPVC
Tricuspid atresia
Truncus arteriosus
Terminology
 Cyanosis vs hypoxemia
A Perception
Produced by 4.5gms
desaturated Hb in the
systemic arterial circ.
A Quantity
Measured arterial pO2 < normal
given altitude when measured.
Ductal -dependent Physiology
ToF
/PA
HLHS
LV
RV
LV
RV
PA
PA
Ao
= direction of flow
Atrial Septal defect
( ASD )
 10 % of CHD .
 Majority (sporadic )
 AD inheritance reported .

Types :
 Ostium primum .
 Ostium secundum .
 Sinus venosus .
 Nearly absent Atrial septum  single atrium .
Ostium Secundum :
 Most common , 6 – 8 % of CHD .
 The hole is being in the region of fossa ovalis (mid
Septal ) .
 Associated with structurally normal AV valve
 Usually single , may be multiple (fenestrated
septum ).
 F:M3:1.
 usually associated with MV prolapse .
Pathophysiology :
Clinical manifestations :
 Most often asymptomatic .
 1 – 5 years  asymptomatic murmur .
 Few have subtle FTT , or symptoms of HF .
 Older children  varying degree of exercise
intolerance .
Physical Examination :
 RV systolic lift (impulse ) at L sternal border .
 Loud 1st heart sound .
 wide & fixed splitting 2nd heart sound .
 Soft ejection systolic murmur ( grade I , II )
Investigation :
 CXR :
• Cardiomegaly  RA , RV .
• pulmonary artery is enlarged .
• Pulmonary vascularity is increased .
 ECG :
 Volume overload of RV .
 RAD .
 Minor RV conduction delay  rsR pattern in R
precordial leads .
Echocardiography :
confirmation of diagnosis
Cardiac Catheterization
Hemodynamics
Closure by device
Treatment :
 Surgery :
closure of the defect either by direct
suturing or using a patch .
 Trans catheter device closure .
 Timing of surgery  after 1st year & before entry in to
school , preferably in early childhood .
 Indications :
 All symptomatic patients .
 All a symptomatic patients with Qp : Qs of at least
2 : 1 or > .
Prognosis & Complications :
• Secundum ASD usually well tolerated during childhood ,
symptoms usually appear in 3rd decade .
• Majority of defects seen in neonates , close spontaneously
as well as defects < 6 mm .
• Defects > 8mm are unlikely to close spontaneously .
• Complications :
 Pulmonary HT .
 Atrial dysrhythmias .
 Tricuspid or mitral insufficiency .
 HF .
 Post operative complication  late HF , AF & SVT .
Sinus venosus ASD
 Situated in upper part of Atrial septum in close relation
to the entry of SVC , rarely involve IVC .
 Often one or more pul. Vein (usually from R lung )
drains anomalously to SVC .
 Similar to Secundum ASD .
 Dx  2 – dimensional Echo .
 Cardiac Catheterization  for a better evaluation of
pulmonary venous drainage .
 Rx  surgery to close the defect while incorporating
the entry of the anomalous vein into LA .
Ostium Primum ASD
I. Isolated type .
II. Part of the complete AV Septal defect ( AV canal
, Endocardial Cushion defect ) .
Isolated Ostium primum :
• Situated in the lower portion of Atrial septum &
overlies mitral &tricuspid valve .
• Cleft in the anterior leaflet of mitral valve , tricuspid
valve is functional .
• The ventricular septum is intact .
AV canal :
 Atrial & ventricular Septal defects with markedly
abnormal AV valve .
 Common in patients with Downs syndrome .
AV Septal defect :
 L  R shunt occur at both Atrial & ventricular
level .
 AV valve insufficiency
 Pulmonary HT & increase pul. vascular resistance
is common  R  L shunt & clinical cyanosis
(Eisen menger physiology ).
Ventricular Septal
Defect
( VSD )
Most common cardiac malformation  25 %
Types of VSD :
 According to position  membranous , muscular .
 According to size  small , large .
Membranous :
most common , are usually single ,( peri membranous )
may extend into adjacent muscle :
 Posteriorly into muscular inlet between AV valve .
( endocardial cushion defect ) .
• Inferiorly into trabecular portion of interventricular septum
 Anterioly into the muscular outlet between R & L
ventricular outflow tract (TOF ) .
 Supracristal : superior to cristasupraventricularis 
AI
Muscular :
 mid portion of septum to the apex .
 Single or multiple (Swiss cheese septum )
 Size of defect :
 Small (restrictive ) :
 < 0.5 cm2 .
 Trivial L  R shunt . (LV pressure > RV )
 Qp : Qs < 1.75 : 1.
 Normal pulmonary arterial &RV pressure .
 Normal Pulmonary vascular bed .
 Normal cardiac chambers .