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Management of Adults with Congenital Heart Disease

Samarai, Daniel LU orcid (2022) In Lund University, Faculty of Medicine Doctoral Dissertation Series
Abstract (Swedish)
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... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
supervisor
opponent
  • Professor Lindahl, Bertil, Uppsala Clinical Research center
organization
publishing date
type
Thesis
publication status
published
subject
keywords
Adults with Congenital Heart Disease, Congenital Heart Disease, Congenital heart defect (CHD), Anticoagulation, Anticoagulants, anticoagulant treatment, Thromboembolic events, Major bleeding, Diagnostic Imaging/methods
in
Lund University, Faculty of Medicine Doctoral Dissertation Series
issue
2022:12
pages
55 pages
publisher
Lund University, Faculty of Medicine
defense location
Segerfalksalen, BMC A10, Sölvegatan 17 i Lund. Join by Zoom: https://lu-se.zoom.us/j/66991273977?pwd=WHBidlB5NW1IZlVwc295L1BjeUVUQT09
defense date
2022-02-04 13:00:00
ISSN
1652-8220
ISBN
978-91-8021-173-4
language
Swedish
LU publication?
yes
id
012fd4c9-87c2-48e4-92ee-f4ee5ed79266
date added to LUP
2022-01-21 14:50:37
date last changed
2022-01-21 15:20:03
@phdthesis{012fd4c9-87c2-48e4-92ee-f4ee5ed79266,
  abstract     = {{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.<br/>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.<br/>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.<br/>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.<br/>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).<br/>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.<br/>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.<br/>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&gt;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.<br/>Larger prospective trials on efficacy and safety of VKA and NOAC in ACHD would be needed to develop and further improve the treatment guidelines.}},
  author       = {{Samarai, Daniel}},
  isbn         = {{978-91-8021-173-4}},
  issn         = {{1652-8220}},
  keywords     = {{Adults with Congenital Heart Disease; Congenital Heart Disease; Congenital heart defect (CHD); Anticoagulation; Anticoagulants; anticoagulant treatment; Thromboembolic events; Major bleeding; Diagnostic Imaging/methods}},
  language     = {{swe}},
  number       = {{2022:12}},
  publisher    = {{Lund University, Faculty of Medicine}},
  school       = {{Lund University}},
  series       = {{Lund University, Faculty of Medicine Doctoral Dissertation Series}},
  title        = {{Management of Adults with Congenital Heart Disease}},
  url          = {{https://lup.lub.lu.se/search/files/112307790/e_spik_ex_Daniel.pdf}},
  year         = {{2022}},
}