Congenital heart disease

Occurs in 1 infant per 100 live births.

Most common group of congenital malformations and leading cause of infant deaths in the developed world.

About 40,000 children are born with congenital heart disease in the United States each year.
Approximately 30,000 operations for congenital heart disease are performed in the US every year: the majority of cases involves infants younger than one year of age.

CHD is the leading cause of death in the first year of life, affects 1-22% of live births.

Congenital heart disease is the leading cause of infant mortality and mobility with many survivors manifesting physical, developmental, or cognitive problems.

Most forms of disease amenable to surgical repair.

About 25% of children with congenital heart disease require corrective surgery during infancy.

A major cause of infant mobility and mortality despite medical advancements.

CHD surgery in infants is high risk, with complications directly related to severe systemic inflammatory response syndrome after cardiopulmonary bypass.

Screening for CHD relies on midtrimester ultrasound and postnatal physical exam including assessment of pulses and heart sounds and inspection for cyanosis.

Most common anomalies in infants.

Screening techniques have fairly low detection rate for diagnosis of CHD.

However, large scale sequencing efforts suggest a distinct genetic basis can be identified in more than 50% of persons with congenital heart defects.

Pulse oximetry adds value to existing screening tests and can identify cases of critical CHD that goes undetected with antenatal ultrasound.

Refers to abnormalities of the heart or great vessels that are present from birth.

Most cases are a result of faulty embryological development during weeks 3-8, when major cardiovascular structures are being formed.

Risk factors in causes for congenital heart defects are mostly unknown with only 15% due to genetic anomalies.

May be a result of the maternal infections, drugs, or environmental exposures.

The most severe lesions are incompatible with intrauterine survival.

Generally lesions compatible with survivability are associated with defects of individual chambers or regions of the heart, while the remainder of the heart is structurally normal.

Increase of survival results in higher prevalence of heart defects in adults, approximately 5.7 per thousand and greater health care costs.

The development of the muscular aspects of the heart may be normal, but blood vessels arising from the heart may not have appropriate connections with specific cardiac chambers.

Manifestations of CHD may begin at birth when the change from fetal to postnatal circulation occurs with reliance of the lungs for oxygenation.

Manifestations of the process may not be noted until adulthood, depending upon the lesion.

Most congenital heart disease surgeries use cardiopulmonary bypass, which triggers endothelial, inflammatory, and coagulation system responses.

This response in combination with myocardial ischemia and reperfusion can cause low cardiac output syndromes.

A low output cardiac syndrome occurs in 25 to 40% of children undergoing cardiopulmonary bypass surgery and is associated with a higher mortality, prolonged mechanical ventilation, and an unfavorable long-term outcome.

Ventricular septal defect accounting for approximately 42% of CHD lesions and an incidence of 4482 per million live births.

Functionally single ventricle anatomic abnormalities represents approximately 8% of all patients with congenital heart disease, with the birth incidence of 4-8 per 10,000.

The Fontan operation is done in nearly all patients with functionally single ventricles and represents 4.2% of all congenital heart operations performed in the US.

The Fontan operation consists of surgery to reroute systemic bicavalvenous return directly into the pulmonary arterial confluence, bypassing the subpulmonary ventricle.

This procedure results in markedly increased central venous pressure which can increase the afterload experienced by the liver and with low cardiac output lead to congestive hepatopathy.

Atrial septic defect accounts for approximately 10% of cases of CHD with an incidence of 1043 cases per million live births.

Pulmonary stenosis accounts from approximately 8% of cases of CHS with an incidence of 836 cases per million live births.

Patent ductus arteriosus accounts for 7% of cases of CHD with 781 cases per million live births

Tetralogy of Fallot accounts for 5% of CHD cases and occurs in 577 live births per million.

Coarctation of the aorta accounts for 5% of cases of CHD and 492 cases per million live births.

Atrioventricular septal defect accounts for 4% of CHD cases with 398 cases per million live births.

Aortic stenosis accounts for 4% of CHD cases with 388 cases per million live births.

Transposition of great arteries accounts for 4% of CHD cases and 388 cases per million live births.

Truncus arteriosus accounts for 1% of CHD cases and account for 136 cases per million live births.

The number of adults surviving CHD is increasing rapidly and is related to surgical advances.

Following congenital heart surgery repair cardiac function may not be normal.

Cardiac hypertrophy, and other changes of cardiac remodeling from congenital defects may be irreversible or may be necessary for survival and growth.

The mortality rates for cardiac surgery with cardiopulmonary bypass in pediatric patients is about 3%, with major complications between 30 and 40%.

The use of prophylactic steroids does not reduce the likelihood of a worse outcome among infants undergoing surgery with cardiopulmonary bypass.

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