The survey focused on the management of patients with at least one of the eight following defects:
- Atrial Septal Defect (ASD) (22%)
- Ventricular Septal Defect (VSD) (15%)
- Tetralogy of Fallot (20%)
- Aortic Coarctation (13%)
- Transposition of Great Arteries (TGA) (9%)
- Marfan Syndrome (7%)
- Fontan Circulation (5%)
- Cyanotic defect (9%)
At baseline, most patients were in their twenties or thirties. The overall median age was 27.9 years and 79% of the patients were aged < 50 years. Median age varied per defect, reflecting the severity of the condition.
The majority of patients had no or only mild functional limitations, but a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. Overall, supraventricular arrhythmia’s had occurred in 18% of patients vs. 5% for ventricular arrhythmia’s, 3% were reported to have experienced endocarditis, 4% stroke or transient ischemic attack, and 1% had a previous myocardial infarction or had undergone PCI or CABG.
There were important differences between the eight defects, with worst outcomes in cyanotic defects and in the Fontan Circulation. Arrhythmias were the most common symptom, with, for example, supraventricular arrhythmia's in 28% of ASD patients vs. 9% in VSD patients. More than half of the patients were using chronic medication. Among Fontan, only 10% did not use any drugs. However, even among ASD patients, a significant proportion did use drugs. A total of 2.8% died during the study period. All-cause mortality was lowest in patients with CoA (0.7%), ASD (1.1%), Tetralogy of Fallot (1.3%), and VSD (1.6%) and highest in those with cyanotic defect (12.6%) or Fontan Circulation (8.2%).
Guidelines showed to be reasonably applied in clinical practice, with variation per type of defect and with a better adherence for operative procedures and prophylactic medication than for diagnostic work-up.62 For example, according to guidelines, all patients aged 40 years or older should have a coronary angiography (CAG) before undergoing a cardiac operation, while angiography is not indicated in patients younger than 40 years of age. The data showed that in 567 patients operated on during follow up, angiography was under-used in 43% and over-used in 15% of patients.
Conclusion: (1) The spectrum of ACHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period, with important differences between the eight defects studied.(2) Adherence to guidelines was good for operative procedures and prophylactic drug treatment. There was a gap between clinical practice and guidelines for angiographic work-up in patients older than 40 years.
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