Acyanotic Congenital Heart Disease

Acyanotic Heart Defects a congenital disorder manifested with left to right shunting and
obstructive lesions. Clinical signs are not always apparent at birth, they manifest anytime during
infancy or early childhood.
Defects: Left to right shunting lesions, increased pulmonary blood flow the blood is shunted
through an abnormal opening from the left side of the heart to the right side of the heart.
Pulmonary blood flow increases because of the extra volume in the right side. There is a stepup saturation in the right side of the heart (abnormal increased) because of the addition of more
highly saturated blood. Physiologic effects include increased pulmonary blood flow, increased
cardiac workload (including ventricular strain, dilation, and hypertrophy). Examples: Atrial Septal
Defect (ASD), Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA), and
Atrioventricular Septal Defect (AVSD).

Atrial Septal Defect (ASD)

Incidence and Pathophysiology:
ASD accounts for approximately 10% of all chronic heart disease. It is seen more
frequently in females than males.
The lesion consists of an abnormal opening between the atria
Chief complaint: most infants and children are asymptomatic
Pathologically, there is a deficiency of the septal tissue in the region of fossa ovalis.
Small ASDs result in trivial shunting and have no hemodynamic consequences.
Larger defects are associated with substantial shunting, which may lead to volume
overload of the right atrium, right ventricle, and pulmonary arteries.
The magnitude of left-to-right shunting depends on the size of the ASD, the relative
compliance of the 2 ventricles, and the pulmonary and systemic vascular resistance.
If left untreated, this may result in pulmonary hypertension, right ventricular failure,
decreased right ventricular compliance, and potentially right-to-left shunting.
However, Eisenmenger's syndrome secondary to ASDs is rare in the adult population
(5%).
- Eisenmengers Syndrome
The process in which a left to right shunt caused by a congenital heart defect in
the fetal heart causes increased flow through the pulmonary vasculature, causing
pulmonary hypertension, which in turn causes increased pressures in the right
side of the heart and reversal of the shunt into a right-to-left shunt.
Types of Atrial Septal Defect:
1. Ostium Secundum
Located at the middle of the atrial septum (fossa ovalis), the most common type.
2. Ostium Primum

 Located low in the atrial septum, results from a defect in endocardial tissue formation
and is often associated with a left mitral valve malformation.
3. Sinus Venosus
Which is located high in the septum close to the SVC
Altered Hemodynamics:
Lower right ventricular compliance which is the ease of ventricular diastolic filling,
compared with left ventricular compliance leads to left to right shunting at the atrial level
through the ASD. This increased blood flow through the ASD leads to an enlarge RA
and RV and increased pulmonary blood flow.

Manifestations:
Most infants and children are asymptomatic but over years to decades may experience:
1) Fatigue and SOB
2) Palpitations or atrial dysrhythmias result of atrial enlargement
3) Recurrent respiratory infections can occur when there is a large amount of pulmonary
blood flow
4) Systolic murmur is produced by increased blood flow across the pulmonary valve.
5) Diastolic murmur is present with large shunts
6) Stroke or major organ damage can occur because of embolization of thrombus, air or
other materials PARADOXIMAL EMBOLISM
Diagnostics:
1. Echocardiogram
2. EKG
3. CXR
4. Cardiac Catheterization
Therapeutic Management:
1. Asymptomatic child is followed by cardiologist. Spontaneous closure can occur in the first
years of life for smaller size secundum ASDs.
2. Elective surgical repair is performed around 2-5 years of age
3. Surgical repair is recommended for all sinus venosus and ostium primum defects.
Medical Management:
1. Asymptomatic patients with moderate size secundum ASDs are monitored for spontaneous
closure in the first years of life with medication.

2. Symptomatic infants and children are treated with diuretics and digoxin as indicated
3. Atrial dysrhythmias are treated with appropriate anti-dysrhythmics