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Review
. 2021 Dec;11(6):1324-1333.
doi: 10.21037/cdt-20-631.

Anticoagulation management in adult patients with congenital heart disease: a narrative review

Christoph Sinning #  1   2   3 , Elvin Zengin #  1   3 , Stefan Blankenberg  1   2 , Carsten Rickers  3 , Yskert von Kodolitsch  1 , Gerhard Diller #  4 , Paulus Kirchhof #  1   2   5
Affiliations
Review

Anticoagulation management in adult patients with congenital heart disease: a narrative review

Christoph Sinning et al. Cardiovasc Diagn Ther. 2021 Dec.

Abstract

With improvements in treatment of congenital heart disease more paediatric patients are surviving with palliative or corrective interventions during childhood, thus becoming adults with congenital heart disease (ACHD). Overall, the ACHD population is at a higher risk of arrhythmias and stroke. The abnormal structure and function of their corrected hearts suggests that in addition to established stroke risk factors, such as prior stroke or older age, additional stroke risk factors need to be considered to determine the risk and establish the indication for oral anticoagulation (OAC) in ACHD patients. In structurally normal hearts non-vitamin-K oral anticoagulants (NOACs) offer at least equal stroke prevention with a better safety profile compared to vitamin K antagonists (VKA) in patients with atrial fibrillation (AF) or pulmonary embolism. Current guidelines recommend NOACs in ACHD patients with simple lesions and indication for OAC, while there is less certainty about their safety in ACHD patients with moderate or complex congenital heart disease such as patients with transposition of the great arteries (TGA) after atrial switch operation (Senning or Mustard operation), Fontan circulation or congenital corrected transposition of the great arteries (ccTGA). This review summarises the available evidence characterising stroke risk in patients with ACHD and the use of anticoagulants and interventional therapies to reduce that risk.

Keywords: Adults with congenital heart disease (ACHD); CHA2DS2-VASc Score; arrhythmias; non-vitamin-K oral anticoagulants; oral anticoagulation (OAC); vitamin K antagonists.

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Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/cdt-20-631). The series “Current Management Aspects in Adult Congenital Heart Disease (ACHD): Part IV” was commissioned by the editorial office without any funding or sponsorship. SB reports grants and personal fees from Abbott Diagnostics, grants and personal fees from Bayer, grants from SIEMENS, grants from Singulex, grants and personal fees from Thermo Fisher, personal fees from Abott, personal fees from Astra Zeneca, personal fees from AMGEN, personal fees from Medtronic, personal fees from Pfizer, personal fees from Roche, personal fees from Novartis, personal fees from Siemens Diagnostics, outside the submitted work. PK reports non-financial support from European Union, non-financial support from British Heart Foundation, non-financial support from Leducq Foundation, non-financial support from Medical Research Council (UK), non-financial support from German Centre for Cardiovascular Research, outside the submitted work; In addition, Dr. Kirchhof has a patent WO 2015140571 issued, and a patent WO 2016012783 issued and research support for basic, translational, and clinical research projects from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last three years. The authors have no other conflicts of interest to declare.

Figures

Figure 1
Figure 1
Grading the complexity of congenital lesions with the prevalence and morbidity/mortality of ACHD patients. ACHD, adults with congenital heart disease.
Figure 2
Figure 2
Practical considerations regarding anticoagulation in ACHD patients. The decision algorithm is suggesting a treatment choice shown on the right of the figure depending on the “yes” or “no” box in conjunction with the underlying pathology in the blue boxes. The black arrows indicate if a statement is not true, if a statement is true the white arrows shows the suggested treatment choice. The blue arrows between the statements indicate the next statement which should be checked if it is true for the patient. ACHD, adults with congenital heart disease.
See this image and copyright information in PMC

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