Congenital Cardiac

Anomalies
Jaime S. Nuevo MD
 Acyanotic heart
diseases
 Left to right shunts
 PDA
 ASD
 VSD
 AP WINDOW
 COMBINATION
 Clinical
manifestations
 Failure to thrive
 Heart murmur
 RV failure in older age group
 Infective endocarditis
 Diagnostic work up
 Non invasive
 ECG

 Chest X Ray

 2D Echo with doppler

 Cine CT Scan
 Invasive
 Hemodynamic studies

 Angiogram

 Electrophysiology study
 management
 Medical mangement
 Treatment for heart failure
 Lower pulmonary hypertension
 Treatment for infective
endocarditis
 Prepare patient for surgery
 Catheter base treatment
 Coil closure of PDA
 Umbrella closure of ASD
 Umbrella closure of VSD
 Surgical treatment
 PDA ligation/transection
 Open heart srgery
 ASD closure
 VSD closure
 AP window patching
common complex cardiac
anomalies
 Tetralogy of fallot
 Transposition of great
arteries
 Total anomalous pulmonary
venous return
 Pulmonary valve atresia with
intact septum
 Hypoplastic left heart
syndrome
 tetralogy of fallot
 RV hypertrophy
 Infundibulovalvar stenosis
 Overriding of the aorta
 VSD
 indications for total
correction
Pulmonary artery size
2D Echo or Angiogram
Age
6 months or older
Cyanotic spells
 Palliative Procedures
 Increase flow to the lungs
 PA growth
Blalock Taussig Shunt
Waterston Shunt
 Result
 Mortality
 <5 % in total correction

 >10% in palliative operation

 Transposition of the
Great arteries
 TGA intact septum with PDA
 TGA VSD,ASD and PDA
 TGA VSD with Coarctation of
Aorta
 Other combinations
 Natural Course
 5 % will reach 1year old

 95 % will die before the

reach the age of one year
 Indication for
Arterial Switch
operation
 Age
 5 - 15 days old - ASO
 >21 days - BAS
 30 days - 60 days for TGA with
VSD
LV Mass - 70 grams
With other anomalies - stage
procedure or other types of
repair
 Other Procedures
 Mustard operation
 Senings operation

 Both are atrial switch or

physiologic operation
 Total Anomalous
Pulmonary Venous
Return
 Intracardiac or coronary
sinus drainage
 Supracardiac type
 Infracardiac type
 Mixed type [mixture of any
anomalous drainage]
 Indications for
Surgery
 Upon diagnosis
 Urgent correction if venous
drainage are obstructed
 Pulmonary Valve
Atresia Intact Septum
 Usually associates with
obligatory shunts [PFO or
PDA]
 Types
 Membranous valve atresia
 Muscular type
tripartite RV
bipartite RV
monopartite RV
 Types of Correction
 Biventricular repair
 Membranous types
 Tripartite RV
Univentricular repair [Fontan
operation}
bipartite RV
monopartite RV
Hypoplastic Left Heart
Syndrome
 Underdeveloped LV
 Hypoplastic aortic arc
 Mitral valve atresia
 VSD
 PDA
 Norwood Operation
 Stage I - 1 month
 Stage II - 6 months to pre
school
 Glen Shunt
 Stage III - school age
 Fontan operation
 Norwood operation
 Result
 Stage I - 8 % - 40 %

Stage II - 8% - 20 %

Over all survival usually 20- 30

%