Care of Adults With Congenital Heart Disease: Key Points

The following are key points to remember from a state-of-the-art review on the care of adults with congenital heart disease for noncongenital cardiologists:

The authors identify seven guiding principles for the care of adult patients with congenital heart disease.

1. Principle 1: Track medical and surgical history.An accurate understanding of the underlying anatomical diagnoses and previous procedures is key to understanding a patient’s current physiology and to guide appropriate testing and patient care.
   
   
2. Principle 2: Look for the expected. Know basic outcomes for the specific congenital heart defects. Each congenital heart lesion is associated with a number of long-term issues requiring monitoring and follow-up. A knowledge of these issues allows for a high index of suspicion for certain long-term complications, such as pulmonary valve regurgitation and ventricular tachyarrhythmias in patients with tetralogy of Fallot. The authors provide a concise summary of common lesions and their associated issues.
   
   
3. Principle 3: Unravel the unexpected. Patients with complications not consistent with their underlying anatomy require additional evaluation to identify co-existing congenital or acquired issues. There should be a low threshold for expert adult congenital heart disease consultation in these situations.
    
4. Principle 4: Employ appropriate diagnostic tests and interpret accordingly.Echocardiography, computed tomography, cardiac magnetic resonance imaging, and cardiac catheterization all play important roles in the evaluation of adults with congenital heart disease. Multiple congenital cardiac anomalies may co-exist in the same patients. Congenital expertise should be employed in the interpretation of noninvasive and invasive testing when necessary. 
   
   
5. Principle 5: Deal appropriately and promptly with emergencies. Common emergencies include arrhythmias, systemic right ventricular failure, endocarditis, and complications of right-to-left shunting. Management of specific emergencies:
   1. Atrial arrhythmias should be suspected in any tachycardiac adult congenital heart disease patients. There should be a low threshold for prompt cardioversion to restore sinus rhythm.
   2. “Never trust a systemic or single right ventricle.” These patients are at risk for sudden decompensation, particularly in the setting of arrhythmias.
   3. Be aware of right-sided endocarditis, particularly in patients with bioprosthetic valves.
   4. Be aware of paradoxical embolism in systemic vasodilation in patients with obligate right-to-left shunts.
      
      
6. Principle 6: Provide advice regarding everyday life: education, work, sport, and pregnancy.Counseling regarding appropriate vocations, exercise prescription and restrictions if necessary, and pregnancy risks and management are key parts of the management of adults with congenital heart disease.
   
   
7. Principle 7: Seek expert adult congenital heart advice. Regular follow-up at specialized centers for more complex congenital heart disease is required. Even patients with simple lesions benefit from intermittent evaluation at experienced adult congenital heart disease centers. Direct communication between local and tertiary centers with a collaborative approach allows for optimal patient care.
   
   
   

https://academic.oup.com/eurheartj/article-abstract/45/45/4783/7841876