Atrial Septal Defect Disease

BASICS

DESCRIPTION

A defect or opening in the atrial septum allowing flow of blood between the two chambers. Shunting is typically left to right and occurs late in ventricular systole and early diastole. The degree of shunting depends on 1) the size of the defect, and 2) the relative compliance of the two ventricles. There can be minimal right to left shunting in early ventricular systole, especially during inspiration. Symptoms typically occur due to right ventricular and pulmonary vascular volume overload sometimes with resultant pulmonary hypertension.

Types:
- Ostium secundum - occurs in the region of the fossa ovalis (most common)
- Sinus venosus - occurs in the superior-posterior septum
- Ostium primum - occurs in the inferior portion of the septum (often involves mitral valve)
System(s) affected: Cardiovascular, Pulmonary
Genetics: Congenital, associated with multiple syndromes. rarely familial.
Incidence/Prevalence in USA: Accounts for 10% of congenital heart defects
Predominant age: Newborn, but may be diagnosed at any age
Predominant sex: Female > Male (2:1)

SIGNS AND SYMPTOMS

Childhood symptoms - usually minimal. Can include failure to thrive and frequent pulmonary infections
Adult symptoms - easy fatigability, dyspnea on exertion, heart failure (late)
Signs vary according to extent of shunting and include:
Right ventricular lift
Palpable pulmonary artery pulse
Fixed, widely-split S2
Pulmonic flow murmur
Low pitched diastolic murmur at left upper sternal border
Cyanosis and clubbing
Stroke due to paradoxical emboli

CAUSES

Unknown

RISK FACTORS

Congenital heart disease family history

DIAGNOSIS

LABORATORY

N/A

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Gross defect in atrial septum
Dilated right atrium
Enlarged pulmonary artery

SPECIAL TESTS

ECG findings:
- Ostium secundum - rightward axis, right ventricular hypertrophy, rSR' pattern
- Sinus venosus - leftward axis, inverted P wave in lead III
- Ostium primum - leftward axis

Note: All may be associated with PR prolongation

IMAGING

X-ray - varying degrees of cardiac enlargement
Cardiac catheterization (indicated in select patients) demonstrates right ventricle enlargement and location of the shunt
Echocardiography

DIAGNOSTIC PROCEDURES

Cardiac angiography
Echo and Doppler
Transesophageal echo in adults

TREATMENT

APPROPRIATE HEALTH CARE

Inpatient for work-up and when surgery is indicated

GENERAL MEASURES

N/A

SURGICAL MEASURES

Surgical repair (particularly when the pulmonary systemic flow ratio is ³ 1.5:1)
Surgical repair delayed until preschool age (2-4) except for large defects to be repaired earlier
Small ASD - primary closure with umbrella-like patch via cardiac catheter is experimental
Surgery if paradoxical emboli result in stroke

ACTIVITY

As tolerated

DIET

No special diet

PATIENT EDUCATION

For patient education materials on this topic, contact: American Heart Association, 7320 Greenville Avenue, Dallas, TX 75231, (214)373-6300

FOLLOW UP

PREVENTION/AVOIDANCE

Evaluation prior to pregnancy

POSSIBLE COMPLICATIONS

Congestive heart failure
Cyanosis
Late-onset arrhythmias 10-20 years after surgery (5%)
Stroke
Pulmonary hypertension
Eisenmenger's syndrome
Infective endocarditis

EXPECTED COURSE AND PROGNOSIS

Course - chronic
50% mortality by age 50 in untreated patients
Favorable in surgically treated symptomatic patients

MISCELLANEOUS

ASSOCIATED CONDITIONS

Mitral stenosis
Mitral regurgitation
Anomalous pulmonary venous return
Multiple congenital syndromes

AGE-RELATED FACTORS

Pediatric: Most frequently appears in this age group
Geriatric: Defects in older persons may still be closed surgically
Others: N/A

PREGNANCY

Evaluation prior to pregnancy, since condition may worsen

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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