The initial presentation of congenital heart disease in adult life is now uncommon. The specialty of adult congenital heart disease is largely concerned with long term follow up of patients with previously diagnosed lesions, many of whom have undergone a palliative or theoretically corrective procedure, and who are best managed by an appropriate specialist in a tertiary centre. Although congenital heart disease is an uncommon problem for the general cardiologist, he or she may be faced with a patient with an undiagnosed defect, may be asked to look after a patient with a complex lesion for geographical or other reasons, or may have to manage a patient who usually attends a tertiary centre but is admitted acutely with an associated illness or is pregnant. Often the exact diagnosis and previous surgical treatment is not accurately known to the echocardiographer, who is then faced with the problem of determining the basic diagnosis and previous surgical treatment before assessing the cardiac status.

In pregnancy, deterioration in the patient with a significant cardiac lesion is a recognised complication. Reassessment of minor or previously corrected abnormalities may be required, and benign murmurs are more easily heard and their significance may be questioned.

The requirements for a service for adults with congenital heart disease has been well considered in the report commissioned by the Canadian Cardiovascular Society at the 1996 consensus conference on adult congenital heart disease. The most recent amendments to this were done in August 1998 and are available on the internet.1Recommendations are made on diagnosis, investigation, and follow up assessment. An important part is the requirement for echocardiography and in this review we will expand upon these guidelines.

Echocardiography is the mainstay in the diagnosis and follow up assessment of most patients with congenital heart disease. The literature in adults …