With exceptions, medical management is largely supportive for heart failure, arrythmias, pulmonary and systemic arterial hypertension, prevention of thrombo-embolic events, or endocarditis. Significant structural abnormalities usually require interventional treatment.
Heart failure is a frequent problem in the grown-up congenital heart disease population. However, as the pathophysiology of cardiorespiratory dysfunction is often very different from the failing normal circulation, particularly in settings such as transposition of the great arteries with arterial switch repair
Arrhythmias and sudden cardiac death:
Arrhytmias are the main reason for the hospitalization of grown-up congenital heart disease patient and they are an increasingly frequent cause of morbidity and mortality. Furthermore, the onset of arrythmyas may be a signal of haemodynamic decompensation, and the risk associated with arrhytmias may be amplified in the presence of the often abnormal underlying circulation.
Sudden cardiac death is of particular concern in grown-up congenital heart disease. The defects with the greatest known risk of late sudden cardiac death are tetralogy of fallot, transposition of the great arteries, aortic stenosis and univentricular heart. Unexplained syncope is an alarming event.
Many grown-up congenital heart disease patients will have undergone intervention in childhood, but surgery during adulthood may be required in various situation:
- Patients with prior repair and residual or new haemodynamic complication
- Patient with conditions not diagnosed or not considered severe enough to require surgery in childhood
- Patients with prior palliation
There has been a marked increase in the number and range of interventional catheterization procedures in grown-up congenital heart disease, which in some patients obviates the need for surgery. In others, treatment of congenital cardiac malformations is best achieved by a collaborative approach involving interventional catheterization and surgery. Newer techniques include stenting of systemic or pulmonary vessels and percutaneous valve implantation.
The endocarditis ris in grown-up congenital heart disease patients is substantially higher than in the general population, with marked variation between lesions. The approach to antibiotic endocarditis prophylaxis has changed for several reason. In short, transient bacteraemia occurs not only after dental procedures but frequently in the context of daily routine activities such as tooth brushing, flossing or chewing. The recommendation is limited to dental procedures requiring manipulation of the ginggival or periapical region of the teeth or perforation of the oral mucosa.