Innledning

Congenital heart disease (CHD) is the most common birth defect, although still relatively rare. The surgical and medical treatment of CHD has markedly improved over the last 50 years. Corrective surgery for intra-cardiac defects first began at the Mayo Clinic and the University of Minnesota in the 1950s. [1] It was the introduction of machines that perfused the vital organs while a surgeon carefully repaired a non-beating heart that revolutionised the field of corrective surgery. Survival well into adulthood is now expected for most babies born with CHD.

Epidemiology

Occurs in 0.8% of live births. In North America, it is estimated that there are more than 1 million adults with CHD; for the first time, the number of adults now exceeds the number of children with CHD.

Classification

Left-to-right shunts

• Lesions that allow blood to shunt from the left side to the right side of the heart. They are associated with varying degrees of increased pulmonary blood flow and are typically acyanotic. In some defects, the site of the shunt may not be within the heart itself.

• Cyanosis occurs only if the lesions are large and not repaired in childhood, and if the patient develops pulmonary vascular obstructive disease (Eisenmenger's physiology). Echocardiography is the primary imaging modality, and, in the current era, the role of cardiac catheterisation is primarily for intervention. [2]

• Examples include:

  • Ventricular septal defect (VSD)

  • Atrial septal defect (ASD)

  • Atrioventricular septal defect (AVSD)

  • Patent ductus arteriosus (PDA)

  • Partial anomalous pulmonary venous connection (PAPVC).

Right-to-left shunts

• Lesions that result in de-oxygenated blood reaching the aorta and are associated with an increased or decreased pulmonary blood flow.

• Examples include:

  • Tetralogy of Fallot (TOF)

  • Pulmonary valve atresia with or without a VSD

  • d-Transposition of the great arteries (d-TGA)

  • Truncus arteriosus

  • Ebstein's anomaly

  • Total anomalous pulmonary venous connection (TAPVC)

  • Hypoplastic left heart syndrome (HLHS).

Obstructive valvular and non-valvular lesions

• Left ventricular outflow tract (LVOT) obstruction

• Coarctation of the aorta

• Pulmonary valve stenosis (PS)

• Aortic valve stenosis (AS)