Objective

Nowadays, most patients with Congenital Heart Disease survive to adulthood. Numerous adults with Congenital Heart Disease (ACHD) are prone to thromboembolic events. A study from our institution showed that 22 % of 821 ACHD were on oral anticoagulation ¹ .

Although novel oral anticoagulants (NOAC′s) have emerged for treatment or prevention of thromboembolic disease, there is a lack of data in ACHD.

Aim of this study is to collect data of characteristics, therapy and clinical course of ACHD treated with NOACs.

Methods

68 ACHD from our institution were included in the questionnaire-based study.

The questionnaire contained questions about the CHD, medication, indications for anticoagulation, and adverse effects. The refusal to consent and lack of cognitive competency to understand and complete the questionnaire were the exclusion criteria.

Methods

68 ACHD from our institution were included in the questionnaire-based study.

The questionnaire contained questions about the CHD, medication, indications for anticoagulation, and adverse effects. The refusal to consent and lack of cognitive competency to understand and complete the questionnaire were the exclusion criteria.

Results

55 patients were treated either with the direct factor Xa-inhibitor rivaroxaban (n = 41) or apixaban (n = 9) or with the direct thrombin-inhibitor dabigatran (n = 5).

In 13 patient the family physician did not implement the proposed NOAC-treatment.

In the treatment group (n = 55) the underlying heart anomaly was in 27 a complex (TGA, TOF, DORV, PA, Ebstein, HLH, DIV, cc-TGA), in 23 a pre- or posttricuspid shunt (PFO, ASD, VSD, PDA, TAPVR,) or others in 5 (AS, CoA, Ao) Median patient age was 49 years (range 22 – 74 years), 51 % females. Renal function was impaired in only 1 (2 %), hepatic function in only 3 (6 %). In 40 patients with atrial arrhythmias the CHA2DS2-VASc was 1 in 15 (38 %), and ≥ 2 in 14 (5,0 %). A HAS-BLED-score ≥ 3 had only 2 (5,0 %) patients. The patients were followed up to 28 months (median: 6 months).Almost all patients were free from clinically relevant complications. In only one NOAC therapy was stopped because of a bleeding (rupture of muscle fibers).