Atrioventricular Septal Defects

• AV canal defect/AV septal defect/ Endocardial cushion defect

• Terdiri dari ASD dan VSD + abnormal AV valve• Total AVSD a single AV valve is common to

both ventricels• Common in Down Syndrome

Patofisiologi

• Complete AVSD: Left-right shunt across atrial and ventricle level

• Left ventricle-right atrium karena absen AV septum

• Resistensi vaskularisasi pulmonal meningkat• AV valvular insufucient increase volume load

on 1/both ventricle.• If defect >>, right-left shunt desaturastion

Manifestasi Klinis

• Exercise intolerance, easy fatigabilty, reccurent pneumonia

• Ausculation left-right shunt: normal/accentuated 1st heart sound, spliting 2nd heart sound, pulmonary systolic murmur, mid-diastolic murmur at apex/lower left sternal increase flow through AV valve

• Mitral insufficiency: apical holosyctolic murmur, radiate to left axilla

• Total AVSD:– Heart failure, pulmonary infection– Liver enlarge, failure to thrive– Systolic thrill at lower left sternal

Diagnosis• Xray: cardiomegaly prominence both ventricles and

atria, a.pulmonalis, increase pulmonary vascularity• EKG

– Superior frontaal QRS axis with left axis deviation– Q wave in lead I and aVL– Biventricular hypertrophy/isolated right ventricular

hypertrophy– Right ventricular conduction delay (rSR’ in V3, V1)– Normal/tall P waves– Prolonged PR interval

• Echocardography

Treatment

• Surgical incision in right atrium• Insertion patch protesa• Postoperative course prolonged in infant with

severe cardiac failure, pulmonary hypertension (develop at 6-12 months age)