Jack M. Colman MD, FRCPC, FACC
*Colman JM. Noncardiac Surgery in Adult Congenital Heart Disease, Chapter 12 from Gatzoulis MA, Webb GD and Daubeney P, Eds. Diagnosis and Management of Adult Congenital Heart Disease. Elsevier 2003.
Excellent guidelines have been published (1,2) that assist the cardiology consultant in assessing a patient with heart disease before noncardiac surgery. The consultant should evaluate the severity and stability of the cardiac problem, clarify options for optimizing pre-operative status, recommend pre-operative testing if it might modify the contemplated procedure or the nature of monitoring, and make the assessment far enough in advance to allow critical assessment of the data. However, these guidelines were developed for acquired heart disease, and although they are helpful in assessing patients with adult congenital heart disease (ACHD), they do not address many of the issues unique to this population.
The ACHD patient's history may be incomplete or misleading, especially concerning the nature of the underlying cardiac condition and the type of repair. Often, the patient cannot provide this information from personal experience because the relevant events occurred in infancy or childhood. Parents may have misunderstood, forgotten details, or not transmitted their knowledge to growing children. Surgical treatment in childhood may have led to misapprehension of "cure" when in fact active issues remain. Because of chronic limitation, functional status assessed from history alone may not quantify the magnitude of disability; formal testing may be needed.
In addition to such patient-related factors, the consultant works in an adult medical environment where understanding of the anatomy and physiology of patients with ACHD may be quite limited. Eponymous descriptions of syndromes and operations contain little helpful information for those unfamiliar with the terminology. Further, there are more than 100 distinct diagnostic categories. Details about the era in which the cardiac surgery was done, the exact techniques applied, and the types and severity of residua and sequelae may impact importantly on management plans.
The cardiology consultant needs to interpret the ACHD terminology for the surgeon and the anesthesiologist and to explain how the cardiovascular system works in the patient at hand. To develop an accurate understanding of current status, it is often crucial to review old cardiac operative and catheterization reports as well as notes from prior clinical assessments. A Health Care Passport containing "important historic information including comprehensive diagnostic data, procedures, operations, and medications … kept by patients as a summary of past and present important health issues" has been recommended, (3) and would be invaluable at the time of pre-operative assessment for noncardiac surgery, were its use to become widespread.
Patients with congenital heart disease (CHD) are also susceptible to the acquired cardiovascular and non-cardiovascular diseases that accrue with age. Such conditions must be evaluated on their own account, and additionally for their potential impact on cardiac function. For example, if left ventricular diastolic dysfunction develops as a result of coronary artery disease (CAD) in a patient with an uncorrected atrial septal defect (ASD), the magnitude of the systemic-to-pulmonary shunt will increase.
