New Guidelines for the management of adults with congenital heart disease (ACHD)

Advances in medical treatment have meant that these days about 85% of children born with CoHD reach adulthood, whereas 20 years ago about 90% died before their 18th birthday. Because of this pleasing fact there are now many young people with complex cardiac diseases who are entering their adult life and starting families. In Europe they are named GUCH (Grown ups with CoHD), in the United states ACHD (adults with congenital heart disease). These young people have many psychological, social and financial issues that may present barriers to proactive health management.

Infrastructural and general problems

• Lack of case management by advanced practice nurses and social workers within the adult healthcare system
• Enhanced education of adult cardiovascular specialists and paediatric cardiologists
• Failure to provide a guided transition from paediatric to adult care
• Insufficient number of specialty clinics and regional centres
• Inadequate access to, or availability of, insurance
• Insufficient education of patients and caregivers regarding disease nature and follow-up
• Inadequate system of management of patient’s cognitive or psychosocial impairment

New practice guidelines

A joint task force between the American College of Cardiology (ACC) and the American Heart Association (AHA) has published best practice guidelines to assist healthcare providers in clinical decision-making. These provide:

• A range of generally acceptable approaches for diagnosis, management and prevention of specific diseases or conditions
• Recommendations on topics common to all lesions (infective endocarditis [IE] prophylaxis, pregnancy, physical activity and medical therapy)
• Definitions of when referral to a highly specialised centre is appropriate
• Recommendations for personnel and services for regional ACHD centres: at least two congenital heart surgeons, comprehensive multidisciplinary team and expert for vocational services
• Recommendations for transition of care: stepwise approach, should begin by the age of 12
• Definitions of surgical procedures for the management of adults with CoHD:
• 37 procedures, alphabetically organised from “arterial switch operation (Jatene Procedure)” to “Waterston Shunt”

These guidelines are intended to improve patient care. Because lack of patient understanding and adherence may adversely affect outcomes, physicians and other healthcare providers should make every effort to engage the patient’s active participation in prescribed medical regimens and lifestyles. The ultimate judgement regarding care of a particular patient must be made by the healthcare provider and the patient in light of all of the circumstances presented by that patient. There are circumstances in which deviations from these guidelines are appropriate.
The realisation of all recommendations, especially those concerning infrastructure, educational programs for patients, families and caregivers, and a complete medical ‘passport’ that outlines specifics of the patient’s medical history, seems very ambitious.

The guidelines will be reviewed annually by the ACC/AHA Task Force on Practice Guidelines and considered current unless they are updated, revised or withdrawn from distribution.