GENERAL RECOMMENDATIONS
Part I - Levels of Care for Adult Patients with Congenital Heart Disease
Care should be available at several different levels.
A national ACHD centre is one that can provide all needed services to ACHD patients. The CACH Network has recommended the maintenance or establishment of five 'national' centres in Canada (population 31 million), one in each of the five regions of the country.
A regional ACHD centre is one that has the essential resources required for an ACHD centre (two ACHD cardiologists and excellent echo facilities), plus any other resources they may have beyond this. Such centres would provide most patient care, but would refer to a national centre when their resources are required (e.g. congenital heart surgery; special electrophysiology services).
A national "full service" ACHD centre
- Should have all (or almost all) of the components described below in the ideal national centre.
- May provide care to any patient with congenital or heritable cardiovascular disease.
- Would usually serve a population base of 3-10 million.
A regional ACHD centre will have a minimum of two cardiologists (either adult or pediatric) with special skills/training/experience in the care of adult patients with CHD and highly skilled echocardiographic services.
- Beyond this, other components of the national ACHD centre described below may be available, depending on local resources and needs.
- May provide care to any patient with congenital or heritable cardiovascular disease within the constraints of available resources.
- Would usually serve a population base of up to 2 million.
An individual specialist/cardiologist
- Provides specialist care to the types of patients listed in Appendix I without there being a need to involve an ACHD centre. When such patients require special interventions [e.g. cardiac surgery, electrophysiology study (EPS)], referral to an national or regional ACHD centre is still recommended, unless these matters have nothing to do with the CHD.
- Participates in the care of the types of patients listed in Appendix II in collaboration with the staff of a regional or national ACHD centre.
An individual primary caregiver
- May reasonably provide cardiovascular follow up for some of these patients (see categories with asterisks in Appendix I) without specialist referral unless circumstances warrant.
- Should only manage other patients with congenital and heritable cardiovascular disease in collaboration with the staff in a regional or national ACHD centre.
Part II - Description of an Ideal National Adult Congenital Heart Disease Centre
Purpose:
- To optimize care for all adult patients with CHD and to reduce errors in care occurring in such patients.
- To consolidate specialized resources required for the care of adult congenital cardiac patients.
- To provide sufficient patient numbers to facilitate the training of personnel wishing to develop expertise in ACHD, and to maintain staff and faculty competence and special skills in the treatment of patients with ACHD.
- To facilitate research in this unique population in order to approach the ideal of evidence-based care, and to promote a more complete understanding of the processes affecting these patients.
- To offer educational opportunities to primary caregivers, cardiologists and surgeons so that they may contribute optimally to patient management.
- To provide a readily available source of information and expert opinion for patients and doctors.
- To help organize support groups for patients.
- To provide information for government and act as the representative of the specialty.
Resources:
Human Resources
These should include the following personnel, who have additional experience/training in the management of adults with CHD, as well as adult cardiology in general, and knowledge of the terminology and issues of concern in pediatric CHD patients:
- Two or more cardiologists trained in adult cardiology and/or pediatric cardiology and with special training/experience in the care of ACHD patients.
- At least two surgeons with experience in all aspects of CHD surgery (usually based in a pediatric unit).
- Two or more electrophysiologists with training/experience in congenital cardiac electrophysiology and with relevant pacemaker expertise.
- Two or more interventional cardiologists with training/experience in non-coronary interventional procedures.
- Two or more medical imaging specialists (e.g. MRI, CT, nuclear cardiology).
- Two or more cardiac anesthetists with special ACHD knowledge and skills.
- A well-functioning intensive care team.
- A well-functioning transplant team or a formal association with a transplant centre.
- A social work and vocational counselling service.
- Specialized nursing staff (e.g. nurse clinicians or specialists) with experience in dealing with CHD and adult cardiology.
- A cardiac pathologist with substantial experience in congenital heart malformations.
- Consultants in the fields of obstetrics and gynecology, genetics, nephrology, pulmonary medicine, hematology, neurology, rheumatology, cardiac rehabilitation, infectious diseases and psychiatry/psychology.
Technical Infrastructure
The following resources should be available:
- Echocardiography (including transesophageal, intra-operative, and fetal echocardiography).
- A cardiac catheterization laboratory with biplane angiography for both diagnostic and interventional procedures.
- An electrophysiology laboratory capable of sophisticated mapping and radiofrequency ablation.
- An operating room and team capable of providing both pump and non-pump facilities, both electively and as emergencies.
- Other appropriate inpatient facilities [intensive care unit (ICU), step-down and inpatient unit].
- Diagnostic imaging with full capabilities (including cardiovascular radiology, computerized tomography, magnetic resonance imaging, nuclear cardiology).
- Pacemaker clinic with expertise in advanced pacing and defibrillation technology.
- Holter monitoring.
- Cardiopulmonary function testing, exercise testing, and oxygen saturation capability.
- Cardiac pathology.
- Data collection system.
Functions:
- To provide optimal care to adults with CHD.
- To work with colleagues at the usually adjacent pediatric centres to optimize the transition and transfer of patient care from a pediatric to an adult facility.
- To hold regular conferences in which management of patients is discussed and decisions are made through consensus.
- To ensure appropriate and timely communication with referring physicians and their staff.
- To ensure appropriate links between the national ACHD centre and support services within the academic medical centre.
- To implement and ensure processes for evaluating feedback and continuous quality improvement in patient care and teaching within the national ACHD centre.
- To ensure co-operation and collaboration with other ACHD centres.
- To participate in clinical trials with other centres both nationally and internationally, and to help develop new knowledge through, when appropriate, the sharing of linked databases established in accordance with legal and ethical requirements.
- To establish and evaluate ongoing training programs both for cardiologists and surgeons interested in developing expertise in the treatment of patients with ACHD, and for all associated staff, including technologists, nurses, psychologists, physiotherapists, occupational therapists and others.
- To maintain a database on all patients managed through that centre.
- Assessment of suspected or known CHD.
- Follow up and continuing care of patients with lesions listed in Appendix II.
- Some types of surgical/non-surgical intervention.
- Assessment regarding non-cardiac surgery or pregnancy.
Part IV - Specialists Involved in the Management of ACHD Patients
Cardiac surgeons operating on adults and adolescents with CHD should have completed training in cardiothoracic or cardiac surgery to prevailing national standards, undergone formal training in surgery for congenital heart malformations, and obtained extensive experience in surgical management of adult patients with CHD.
Cardiac anesthetists involved in surgery on adults with CHD must have had specialized training and/or extensive experience in the treatment of patients with CHD, adult patients undergoing other types of cardiac surgery and the anesthetic management of problems such as cyanosis, elevated pulmonary vascular resistance, or severe outflow obstruction.
Adult ACHD cardiologists (especially those still to-be-trained) should have completed full adult cardiology training, and have taken at least one year of supplemental training in CHD as it applies to adolescents and adults. Guidelines have been published. Their ability to serve the interests of these patients will be in proportion to the amount of time they have spent in training, continuing education, and clinical experience in the management of these patients.
Pediatric ACHD cardiologists (especially those still to-be-trained) should have completed pediatric cardiology training, and have taken at least one year of supplemental training in adult cardiology and ACHD so as to be able to recognize and deal with non-congenital issues that will arise in these patients. Their ability to serve the interests of these patients will be in proportion to the amount of time they have spent in training, continuing education, and clinical experience in the management of these patients.
Echocardiographers responsible for recording and interpreting echocardiograms in adults with CHD should be appropriately trained (level 3 echocardiography training) and have a thorough understanding of the technical principles of echocardiography and a thorough knowledge of the anatomy, hemodynamics and pathology of both acquired and CHD in order to obtain, correlate, and record efficiently the echocardiographic findings. The Canadian Society of Echocardiography on Physician Training recommends 1 year of echocardiography fellowship to attain level 3 training (2). Training in transesophageal echocardiography is also vital.
Performance of any surgical procedures in most adult patients with CHD carries a greater risk than in the general population. Evaluation in an ACHD centre prior to surgery is recommended, and in the case of unoperated or complex ACHD, it is recommended (where feasible) that the surgery be carried out in the ACHD centre, utilizing experienced cardiac anesthetists. This is strongly recommended for cyanotic patients, patients with pulmonary hypertension, or with some rhythm abnormalities. Pregnant women with CHD should be managed by the patient's obstetrician and ACHD cardiologist together with a cardiac anesthetist if necessary. In most cases, an obstetrician knowledgeable in the management of ACHD is optimal. Postoperatively, the patients with CHD may need ICU/monitoring facilities even for relatively minor procedures.
Dental Care
Regular dental care, often in a hospital setting, is needed by most adult patients with CHD to decrease the likelihood of caries, abscesses or periodontal disease, all of which contribute to the increased incidence of infective endocarditis. There is justification for government subsidization of dental care in those patients unable to afford it. Endocarditis prophylaxis, both antibiotics and daily teeth/gum care, are recommended.
Informed Consent
Despite its lifelong presence, most adolescents and young adults with CHD have inadequate knowledge about their cardiac conditions. Health care providers must assess each patient's knowledge of his or her condition and give appropriate information to enable independent decision-making about choices in care. Adults with CHD should be encouraged to understand not only their disease, but the medications they use. They should be involved in major management decisions or decisions involving invasive procedures. Patients should be encouraged to inform their specialists of any new events which may occur. Further involvement of patients in the evaluation of processes, programs, and in the planning of research trials within the constraints of their motivation and capacity to understand them is ideal.
Advance Directives and Palliative Care
Patients should be made aware of the availability of advance directives which are legally binding. Their use may reduce uncertainty when caring for critically ill individuals. Likewise the role of non-intervention, or of palliative care, as a treatment modality should be presented in a realistic, unbiased, and acceptable manner as one of the options to patients making decisions about interventions or procedures. The probable result of this clinical pathway should be objectively explained with comparison of outcomes with other interventions when this information is known.
Auto-Donation of Blood
Patients should be made aware of the possibility of autologous donation or directed donation (from family members) of blood prior to cardiac surgery if such facilities exist.
