by Nicole Evans M.D.

Created on: May 01, 2009   Last Updated: August 07, 2009

Congenital heart defects result from improper or incomplete development of the heart in babies. Some congenital heart defects are detected as a murmur at birth or become symptomatic within the first days to weeks of life. Other congenital heart defects may go unnoticed for years until a child begins to develop heart failure, while others may be asymptomatic for life.

There are many types of congenital defects but most can be categorized as either cyanotic or acyanotic heart lesions. Cyanotic heart lesions cause an infant to turn blue, usually beginning at the lips, oral mucosa and chest. Acyanotic lesions in the pediatric heart, on the other hand, do not cause a blue discoloration.

A congenital heart defect is diagnosed with radiographic imaging and an echocardiogram. An echocardiogram is essentially an ultrasound of the heart that provides useful information on both the anatomy of the heart and how well the heart is functioning.

Types of acyanotic congenital heart defects:

Acyanotic heart defects as a group can be further divided into those that create a shunt in the cardiovascular system and those that cause an obstruction. A shunt is an abnormal pathway that allows blood to flow directly between two chambers or vessels that are not normally connected in a healthy infant.

Congenital acyanotic defects that act as shunts include:

1. Atrial septal defect "ASD"

2. Patent ductus arteriosus "PDA"

3. Ventricular septal defect "VSD"

Whereas obstructive acyanotic defects in infants include:

1. Stenosis (narrowing) of the pulmonic valve

2. Stenosis of the aortic valve, including bicuspid aortic valve

3. Aortic coarctation "CoA"

Types of cyanotic congenital heart defects:

Pediatric heart defects that cause cyanosis can be divided into groups depending on whether they cause an increase or decrease in blood flow to the lungs.Furthermore, they may be grouped within these two categories by which ventricle of the heart is most affected:

Cyanotic defects that increase pulmonary blood flow:

A. Right ventricular enlargement:

1. Total anomalous pulmonary venous return

2. Transposition of the great arteries

B. Left and right ventricular enlargement:

1. Truncus arteriosus

2. Transposition of the great arteries with a ventricular septal defect

Cyanotic defects that decrease pulmonary blood flow:

A. Right ventricular enlargement:

1. Tetralogy of Fallot

B. Left ventricular enlargement:

1. Tricuspid atresia (tricuspid valve is not formed)

2. Pulmonary atresia (pulmonic valve is not formed) with abnormally small right ventricle

C. Neither ventricle predominant:

1. Ebstein anomaly

Complications of cyanotic congenital heart disease:

Cyanotic heart defects are generally more uncommon than acyanotic lesions in infants and children. These cyanotic heart defects are also associated with a wider variety of complications including increased numbers of red blood cells with a relative anemia (each cell contains less hemoglobin), brain abscesses and thromboembolic stroke, gum disease, gout, arthritis, clubbing of the digits, and failure to thrive.

Treatment of pediatric heart defects:

Each infant or child with a congenital heart lesion is managed on an individual basis. Treatment may include one surgery or multiple surgeries done with open heart procedures or through cardiac catheterization. Other heart defects are managed with medications. There is also a minority of lesions that are relatively harmless. The best management for these is simply to "watch and wait".

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