One of the focus points of Concor is the prevention of premature death in adults with congenital heart defects. The second most common cause of death –the first being heart failure– is sudden cardiac death. In the majority of cases, sudden cardiac death is caused by dangerous rhythm disorders in the chambers of the heart (ventricular fibrillation). When this happens, countless chaotic electrical currents go through the heart chambers, causing the heart to stop pumping. This is also called cardiac arrest. An implantable defibrillator (ICD) can detect this heart rhythm disorder and stop it with an electric shock. Yet, it is not known which patients should receive an ICD.
Recently, new guidelines were released that attempted to create some clarity in this issue. However, it is not yet evident whether these quidelines can actually select the right patients who need an ICD. Because of this, Concor researcher Jim Vehmeijer examined whether the recommendations in these guidelines are correct using data from Concor, Leuven (Belgium) and Toronto (Canada). Patients who died from sudden cardiac death were compared to control patients who were still alive at the same age. In the study, 35-40% of patients should have received an ICD according to these guidelines. Most of these patients would therefore not have received protection from sudden cardiac death, even with these new guidelines. In addition, about 15% of control patients should also have received an ICD according to the guidelines. This may cause unnecessary complications related to the implant surgery and the device itself.
Altogether, the ability of the guidelines to determine who will actually die from sudden cardiac death was mediocre.
In short, the guidelines are a step towards creating more clarity about which patients have a high risk of sudden cardiac death, and therefore should receive an ICD. However, more research on the matter is still required to refine this. Until that is achieved, it remains important to not only follow the guidelines, but also regard each patient individually.
During the Congress of the American Heart Association in New Orleans in november 2016 presented research physician Jouke Bokma results of an investigation by the international Indicator Registry. It is the largest international registration of patients with tetralogy of Fallot. Using MRI scans they looked at the results of this patient group. The research that Dr. Bokma presented focused on the effect and usefulness of the operative pulmonary-valve replacement in patients with this congenital heart disease. There was not a clearly improved prognosis after undergoing this operation, but there may be a selective group of patients who will benefit from it. The results of this research can help make the indication and timing of this operation. It is expected that the findings of this study shortly to be published in an international scientific journal.
Adults with congenital heart disease (CHD) often have atrial arrhythmias; about 23% at an average age of 28 years. The high risks for stroke or thrombosis are well-known in adults with atrial arrhythmias without a CHD. Therefore, these patients need to receive blood thinners to prevent stroke or thrombosis. Previous research has shown that (relatively) new blood thinners (NOACs) are just as effective and safe as the older blood thinner (Sintrom) in patients with an acquired atrial arrhythmias. Furthermore NOACs have two advantages compared with Sintrom, namely 1. Thanks to the more stable working of the drug, the regular blood monitoring at the thrombosis service is not needed, 2. The chance of getting brain hemorrhage (bleeding), one of the most serious complications of blood thinners is decreased by 50% compared with using Sintrom. Unfortunately, adults with a CHD are not examined in these studies. So it is not yet known whether these new blood thinners (NOACs) are also effective and safe in adults with CHD and atrial arrhythmias while they may offer to be a better alternative for Sintrom.
This study investigates how often strokes, thrombosis and bleeding occur under the use of NOACs in 2 years in adults with CHD and atrial arrhythmias. Furthermore, we measure the quality of life and treatment compliance. Any adult with CHD who uses NOACs can join this study. We contact the participating patient once a year to ask about the experience with the NOACs and you will also be asked to fill in questionnaires about the quality of life and treatment compliance.
Contact person: Hayang Yang, firstname.lastname@example.org, tel: 020-5668679
Contact moments: first telephone contact after 6 months and then once a year during the regular outpatient visits at your own cardiologist.