TCVS CONGENITAL CARDIAC ANOMALIES Acyanotic heart diseases: Left to right shunts Patent ductus arteriosus Atrial septal

defect Ventricular septal defect

Pulmonary valve atresia with intact septum Hypoplastic left heart syndrome Double outlet ventricle Truncus arteriosus Tetralogy of fallot Components

Aortopulmonary window Combination (ASD & PDA, VSD &

PDA)

RV hypertrophy Infundibulovalvar stenosis Overriding of the aorta VSD Indications for total correction Pulmonary artery size: 2D older

Clinical manifestations Depends on the volume of the shunt Failure to thrive Heart murmur

Echo or Angiogram

age group Infective endocarditis Diagnostic work up Non invasive

Right ventricular failure in older

Age: 6 months or (Younger: Risk of destroying pulmonary valves Cyanotic spells Nakata: 330 surface????

mm2/body

ECG Chest X Ray

2D Echo with Doppler Cine CT Scan Invasive Hemodynamic studies Angiogram Hemodynamic studies angiogram: Gold standard Electrophysiology study Management Medical mangement

McGun Criteria ?? Size of right + left pulmonary = or 2x aorta Level of diaphragm

Palliative Procedures: When young, pulmonary artery not yet developed Increase flow to the lungs PA growth Blalock Taussig Shunt: Subclavian artery connect to pulmonary artery (Modified Blalock Taussig Shunt: Graft which will connect subclavian artery to pulmonary artery)

&

Treatment for heart failure

Lower pulmonary hypertension: Milrinone, nitric oxide Treatment for infective endocarditis Prepare patient for surgery (Identify other problems like neurological or bleeding) Catheter base treatment

Waterston Shunt: Connect right pulmonary artery to ascending aorta Mortality <5 % in total correction >10% in palliative operation Transposition of the Great arteries Aorta arises from the right ventricle, pulmonary artery arises from the left ventricle 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

years Surgical treatment

Coil closure of PDA: 2-3 Umbrella closure of ASD Umbrella closure of VSD Umbrella closure under trial PDA ligation/ transaction:

95 % will die before the reach the age of one year 65% will die within the 1st 6

may develop aneurysm

Open heart surgery ASD closure: 0 1% mortality rate VSD closure: <3% mortality rate AP window patching

Common complex cardiac anomalies (Cyanotic) Tetralogy of fallot Transposition of great arteries Total anomalous pulmonary venous return CHRABI Page 1 of 2

months of life 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 physiologic operation

Mustard operation Senings operation Both are atrial switch or

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: correction if venous drainage are obstructed

Urgent

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

school: Glen Shunt

Stage I: 1 month Stage II - 6 months to pre Stage III school age:

Fontan operation Result 30 %

Stage I - 8 % - 40 % Stage II - 8% - 20 % Over all survival usually 20CHRABI

CHRABI Page 2 of 2