Management of Adults with Congenital Heart Disease

Sammanfattning: Adults with congenital heart disease (ACHD) are a completely new and rapidly growing specialty comprising patients who require specialist knowledge regarding their care and treatment. Challenges involve diagnostics and treatment as studies are scarce and include small and heterogenous study populations.The first aim with this thesis was to evaluate the relationship between the systemic right ventricular function determined by cardiac magnetic resonance (CMR) imaging, biomarkers, echocardiographic parameters, New York Heart Association class and performance on stress test.The second aim of this thesis was to evaluate anticoagulation treatment with vitamin K antagonists (VKA) in ACHD. This involved determining the quality of VKA therapy by evaluating time in therapeutic range (TTR) and variability of international normalised ratio of prothrombin time. Furthermore, studying the efficacy and safety of VKA and non-vitamin K oral antagonists (NOAC) in ACHD by evaluating the incidence of thromboembolism (TE), major bleeding and potential risk factors.Paper I A correlation was observed between the systemic ventricular function determined with CMR and echocardiographic ventricular global longitudinal strain in patients with systemic right ventricle.Paper II-III Anticoagulation with VKA was of high quality in ACHD in the South of Sweden. A low incidence of TE and major bleeding events was seen in ACHD patients with high quality VKA treatment. Younger age, female gender and cardiac failure were risk factors for poor anticoagulation and thus may be cause for cautiosness and when suitable, consideration of alternative non-VKA anticoagulants. History of TE was associated with complcations (TE and major bleeding).Paper IV In this retrospective study reports a single-center experience of NOAC use in ACHD patients we found no thromboembolic and one major bleeding events during a median duration of 17 months of therapy.In conclusion, right ventricular global longitudinal strain by echocardiography may be useful in the evaluation of the systemic right ventricular function in patients with congenitally corrected transposition of the great arteries (TGA) or TGA after atrial switch.Anticoagulation therapy with oral VKA is of good quality in ACHD patients with a high TTR, low INR variability, low incidence of TE and major bleeding events. We recommend the applicable target of TTR>65% for ACHD patients. Young age and female gender may be considerable factors to consider when initiating anticoagulation therapy. NOAC therapy may be considerable in the absence of highly specialized organization in regard to VKA medication where close monitoring of the patient is not possible. NOAC appear safe and effective in ACHD patients without mechanical valve protheses.Larger prospective trials on efficacy and safety of VKA and NOAC in ACHD would be needed to develop and further improve the treatment guidelines.