Abstract
Adults with congenital heart disease (ACHD) experience more thromboembolic complications than the general population. We systematically searched and critically appraised all studies on the safety and efficacy of non-vitamin-K oral anticoagulants (NOACs) in adult patients with various forms of congenital heart disease. PubMed and the Cochrane Central Register of Controlled Trials (CENTRAL) were used, with duplicate extraction of data and risk of bias assessment. The Newcastle-Ottawa quality assessment scale was used to assess study quality. Three studies fulfilled the inclusion criteria and were analyzed. The total number of participants was 766, with a total follow-up of 923 patient-years. The majority of patients (77%) received a NOAC for atrial arrhythmias, while the remainder were prescribed NOACs for secondary (19%) or primary (4%) thromboprophylaxis. The annual rate of thromboembolic and major bleeding events was low: 0.98% (95% CI: 0.51–1.86) and 1.74% (95% CI: 0.86–3.49) respectively. In Fontan patients, the annual rate of thromboembolic and major bleeding events was 3.13% (95% CI: 1.18–8.03) and 3.17% (95% CI: 0.15–41.39) respectively. NOACs appear safe and effective in ACHD without mechanical prostheses. Additional studies are, however, needed to confirm their efficacy in complex ACHD, especially those with a Fontan-type circulation.
Highlights
Adults with congenital heart disease (ACHD) are a rapidly growing population, both in number and age, and are at increased risk of thromboembolic complications, especially strokes, compared to the general population [1,2,3,4]
Supraventricular arrhythmias are common in ACHD patients, and the rise in prevalence is strongly related to aging, as well as to the number and type of previous interventions, and the effects of long-standing hemodynamic lesions [10,11]
Most major bleeding events (9 out of 16) occurred in patients treated with rivaroxaban (9/349), followed by those on apixaban (n = 6 major bleeds, 6/293), one major bleed occurred in a patient anticoagulated with edoxaban (1/35) and none in those treated with dabigatran (0/89)
Summary
Adults with congenital heart disease (ACHD) are a rapidly growing population, both in number and age, and are at increased risk of thromboembolic complications, especially strokes, compared to the general population [1,2,3,4]. The Pediatric and Congenital Electrophysiology Society and Heart Rhythm Society (PACES/HRS) Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adults with Congenital Heart Disease advises long-term anticoagulation in patients with recurrent or sustained intra-atrial reentrant tachycardia, or atrial fibrillation (AF) in the presence of moderate or complex CHD (class IIa and I indication respectively) [12]. The anticoagulants of choice in these situations are vitamin K antagonists (VKA), as data on the safety and efficacy of non-vitamin K oral anticoagulants (NOACs) are limited. This consensus statement did, recommend the use of NOACs in patients with intra-atrial reentrant tachycardia or atrial fibrillation and simple nonvalvular CHD (class IIb indication). The CHA2DS2-VASc (congestive heart failure, hypertension, age 65–74 years, diabetes mellitus, sex-female, (1 point for the presence of each) and age >75 years, Stroke/TIA (2 points); range from 0 to 9) and HAS-BLED (uncontrolled systolic blood pressure >r160 mm Hg, abnormal renal and/or liver function, previous stroke, bleeding history or predisposition, labile international normalized ratios, elderly, and concomitant drugs and/or alcohol excess (1 point for the presence of each); range from 0 to 9) scores, which are validated to assess stroke and bleeding risk respectively in patients with nonvalvular AF in the general population, are of unclear value in the ACHD population [13,14,15]
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