Tcvs Congenital Cardiac Anomalies

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

 Age

Indication for Arterial Switch operation

 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 %