Endocardial Cushion Defect

Endocardial Cushion Defect Types

Partial: Ostium primum

Complete: Atrioventricular canal

Figure 1: Diagrammatic illustrations of complete endocardial cushion defect viewed with the LA and LV posterior wall removed. Cleft mitral and tricuspid valves with resulting common AV valve are compared with normal AV valves.

Presentation

• 30% of the defects occur in Down's syndrome

• Partial defects are usually asymptomatic

• Complete- CHF

Physical Examination:

• Undernourished

• Hyperactive precordium with holosystolic murmur at LLSB

• Loud P2, mid-diastolic rumble at the apex

• Liver enlargement

Figure 2: Cardiac findings of complete endocardial cushion defects(ECD), which resemble that of VSD. A systolic thrill may be present at the LLSB (dotted area), where the systolic murmur is the loudest.

Investigations

ECG:

• Superior QRS axis; 1st degree AV block

• RVH or BVH

Chest X-ray:

• Heart size enlarged

• Pulmonary vascularity increased

Surgical

Closure of ostium primum defect at 2-5 years

Closure of complete defect at 6 months of age.