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Long-term outcome and exercise capacity in adults with congenital heart disease with focus on tetralogy of Fallot
Umeå universitet, Medicinska fakulteten, Institutionen för folkhälsa och klinisk medicin, Kardiologi.ORCID-id: 0000-0002-3350-9001
2022 (Engelska)Doktorsavhandling, sammanläggning (Övrigt vetenskapligt)Alternativ titel
Långtidskomplikationer och arbetsförmåga hos vuxna med medfödda hjärtfel med focus på Fallotstetrad (Svenska)
Abstract [en]

Background: Congenital heart disease (CHD) includes a wide range of malformations, from simple lesions, e.g. ventricular septal defect, that resolve spontaneously, to severe lesions, e.g. univentricular heart, not compatible with life without intervention. Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease.

In Sweden, approximately 1% of the children are born with CHD each year of which about 30 have ToF. The prognosis has improved remarkably due to advances in surgical techniques as well as peri- and postoperative care. Nowadays, 97% of babies with CHD born in Sweden can be expected to survive into adulthood. Despite huge improvements in prognosis, the relative mortality is still 7 to 97 times higher in patients with CHD, depending on lesion severity, compared with matched controls. Therefore, it is important to improve long-term follow-up and to identify robust and easily available tools to predict poor outcome and optimize timing of possible reinterventions. Addressing quality of life (QoL) has also become more important when survival increases.

The main focus of this thesis was on QoL and long-term outcome in patients with repaired tetralogy of Fallot (rToF). In studies I, II and III, we analysed health related quality of life (HRQoL), prevalence of cardiac devices (i.e. pacemakers and implantable cardioverter defibrillators), and exercise capacity, respectively, in patients with rToF. In study IV, the aim was to broaden the perspective and include patients with a wide range of CHD, and to investigate whether exercise capacity, measured as workload could be of help in predicting mortality.

Methods: The studies were performed as nationwide register studies. Data from the Swedish registry of congenital heart disease (SWEDCON) were used in studies I-IV. Study II also included data from the Swedish ICD and Pacemaker registry. In studies I-III, only patients with rToF were included, whilst study IV included patient with a broad spectrum of CHD diagnoses.

Results: More than half (57%) of the patients with rToF reported best possible HRQoL. New York Heart Association (NYHA) class I and being physically active were associated with best possible HRQoL. Fourteen percent of patients with rToF had a cardiac device, either a pacemaker or an implantable cardioverter defibrillator (ICD). The prevalence of having a cardiac device was lower compared with reports from previous studies, especially for ICDs. Exercise capacity was 75% of predicted in patients with rToF and there was no decline in percent of predicted exercise capacity with age. Low heart rate reserve and low percent of predicted exercise capacity were associated with higher mortality.

In study IV, including a wide range of lesions, percent of predicted exercise capacity was associated with mortality. Patients with percent of predicted exercise capacity between 50 and 69.9% had 1.7 times, and patients with exercise capacity <50% of predicted had 2.2 times higher risk of being deceased, over a median follow-up time of 9 years. NYHA class III-IV and ongoing cardiovascular medication were also associated with increased risk of death when adjusted for sex, physical activity and impaired left ventricular function.

Conclusions: HRQoL in our patients with rToF was good, despite a fairly high prevalence of cardiac devices. Impaired exercise capacity was associated with an increased mortality. Our results reinforce the need for regular evaluation of exercise capacity in patients with CHD to identify patients at risk of worse outcome. Furthermore, the results in this thesis emphasize the importance of assessing NYHA class and physical activity as part of the follow-up since these parameters are easily attainable and strongly associated with HRQoL and exercise capacity.

Ort, förlag, år, upplaga, sidor
Umeå: Umeå University , 2022. , s. 75
Serie
Umeå University medical dissertations, ISSN 0346-6612 ; 2180
Nyckelord [en]
Adults congenital heart disease, tetralogy of Fallot, exercise test, exercise capacity, mortality, register, physical activity, pacemaker, implantable cardioverter defibrillator, quality of life
Nyckelord [sv]
Medfödda hjärtfel, Fallots tetrad, arbetsprov, fysisk prestationsförmåga, mortalitet, register, fysisk aktivitet, pacemaker, implanterbar defibrillator, livskvalitet
Nationell ämneskategori
Kardiologi
Forskningsämne
kardiologi; epidemiologi
Identifikatorer
URN: urn:nbn:se:umu:diva-194585ISBN: 978-91-7855-790-5 (digital)ISBN: 978-91-7855-789-9 (tryckt)OAI: oai:DiVA.org:umu-194585DiVA, id: diva2:1657411
Disputation
2022-06-10, Sal B Tandläkarhögskolan, 9tr / Zoom, Umeå, 09:00 (Svenska)
Opponent
Handledare
Anmärkning

Zoom för disputation: https://umu.zoom.us/j/68136604319Kod: 112233

Tillgänglig från: 2022-05-20 Skapad: 2022-05-10 Senast uppdaterad: 2023-06-10Bibliografiskt granskad
Delarbeten
1. Factors associated with health-related quality of life among adults with tetralogy of Fallot
Öppna denna publikation i ny flik eller fönster >>Factors associated with health-related quality of life among adults with tetralogy of Fallot
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2019 (Engelska)Ingår i: Open heart, E-ISSN 2053-3624, Vol. 6, nr 1, artikel-id e000932Artikel i tidskrift (Refereegranskat) Published
Abstract [en]

Background: Due to improved care, the numbers of patients with tetralogy of Fallot (ToF) are increasing. However, long-term morbidity and need for reinterventions are concerns and also address issues of quality of life (QoL).

Methods: Patients with ToF and valid EuroQol-5 dimensions questionnaire (EQ-5D) were identified in the national Swedish register on congenital heart disease. EQ-5Dindex was calculated and dichotomised into best possible health-related QoL (EQ-5Dindex=1) or differed from 1.

Results: 288 patients met the criteria and were analysed. Univariate logistic regression showed a positive association between New York Heart Association (NYHA) class I (OR 8.32, 95% CI 3.80 to 18.21), physical activity >3 h/week (OR 3.34, 95% CI 1.67 to 6.66) and a better right ventricular function (OR 2.56, 95% CI 1.09 to 6.02). A negative association between symptoms (OR 0.23, 95% CI 0.13 to 0.42), cardiovascular medication (OR 0.31, 95% CI 0.18 to 0.53), age (OR 0.97, 95% CI 0.96 to 0.99) and EQ-5Dindex was observed. In multivariate logistic regression, NYHA I (OR 7.28, 95% CI 3.29 to 16.12) and physical activity >3 h/week (OR 2.27, 95% CI 1.07 to 4.84) remained associated with best possible health-related QoL. Replacing NYHA with symptoms in the model yielded similar results.

Conclusion: In this registry study, self-reported physical activity, staff-reported NYHA class and absence of symptoms were strongly associated with best possible health-related QoL measured by EQ-5D. Physical activity level is a potential target for intervention to improve QoL in this population but randomised trials are needed to test such a hypothesis.

Ort, förlag, år, upplaga, sidor
BMJ Publishing Group Ltd, 2019
Nationell ämneskategori
Kardiologi
Identifikatorer
urn:nbn:se:umu:diva-161553 (URN)10.1136/openhrt-2018-000932 (DOI)000471922200023 ()30997127 (PubMedID)2-s2.0-85062271422 (Scopus ID)
Forskningsfinansiär
Västerbottens läns landstingHjärt-Lungfonden
Tillgänglig från: 2019-07-10 Skapad: 2019-07-10 Senast uppdaterad: 2023-03-23Bibliografiskt granskad
2. Implantable cardiac devices in adult patients with repaired tetralogy of Fallot
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2021 (Engelska)Ingår i: Scandinavian Cardiovascular Journal, ISSN 1401-7431, E-ISSN 1651-2006, Vol. 55, nr 1, s. 22-28Artikel i tidskrift (Refereegranskat) Published
Abstract [en]

Objectives: Implantable cardiac devices are common in patients with tetralogy of Fallot (ToF) (18.3–21.3%) according to previous reports from large centres. We conducted this study to investigate the prevalence and incidence of cardiac devices in a less selected population of patients with ToF and assess factors other than arrhythmia associated with having a device. 

Design: 530 adult (≥18 years) patients with repaired ToF were identified in the national registry of congenital heart disease (SWEDCON) and matched with data from the Swedish pacemaker registry. Patients with implantable cardiac devices were compared with patients without devices. 

Results: Seventy-five patients (14.2%) had a device; 51 (9.6%) had a pacemaker and 24 (4.5%) had an implantable cardioverter defibrillator. The incidence in adult age (≥18 years) was 5.9/1000 patient years. Estimated device free survival was 97.5% at twenty, 87.2% at forty and 63.5% at sixty years of age. Compared with previous studies, the prevalence of devices was lower, especially for ICD. In multivariate logistic regression, cardiovascular medication (odds ratio [OR] 3.5, 95% confidence interval [CI] 1.8–6.8), impaired left ventricular function, (OR 2.6, 95%CI 1.3–5.0) and age (OR 1.02, 95%CI 1.002–1.05) were associated with having a device. 

Conclusion: The prevalence of devices in our population, representing a multicenter register cohort, was lower than previously reported, especially regarding ICD. This can be due to differences in treatment traditions with regard to ICD in this population, but it may also be that previous studies have reported selected patients with more severe disease.

Ort, förlag, år, upplaga, sidor
Taylor & Francis, 2021
Nyckelord
Tetralogy of Fallot, pacemaker, implantable cardioverter defibrillator, congenital heart disease, register
Nationell ämneskategori
Kardiologi
Identifikatorer
urn:nbn:se:umu:diva-173769 (URN)10.1080/14017431.2020.1792973 (DOI)000548989400001 ()32672076 (PubMedID)2-s2.0-85088051820 (Scopus ID)
Forskningsfinansiär
Hjärt-LungfondenRegion VästerbottenCancerforskningsfonden i NorrlandVisare Norr
Tillgänglig från: 2020-07-31 Skapad: 2020-07-31 Senast uppdaterad: 2023-03-23Bibliografiskt granskad
3. Exercise capacity in adult patients with tetralogy of Fallot
Öppna denna publikation i ny flik eller fönster >>Exercise capacity in adult patients with tetralogy of Fallot
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2021 (Engelska)Ingår i: International Journal of Cardiology Congenital Heart Disease, ISSN 2666-6685, Vol. 5, artikel-id 100204Artikel i tidskrift (Refereegranskat) Published
Abstract [en]

Background: On a group level, patients with repaired tetralogy of Fallot have impaired exercise capacity. Since exercise capacity is related to prognosis, and more patients survive into higher age, it is important to know the expected exercise capacity at different ages. Furthermore, factors associated with exercise capacity and the relation between exercise capacity and mortality need further evaluation.

Methods: The national register of congenital heart disease was searched for exercise tests in adults with repaired tetralogy of Fallot. The results from exercise tests were compared with national reference data.

Results: 314 patients were identified (median age 33.2 [IQR 24.8-44.7], 40.8% women). The mean percent of predicted workload was 74.8 (±19.6) % without change across ages. In multivariable analysis, NYHA class I (odds ratio [OR]4.2, 95% confidence interval [CI]1.7-10.0) and higher physical activity level (>3 h/week) (OR 3.6, 95%CI 1.8-7.3) were positively associated with higher exercise capacity, while ongoing cardiovascular therapy (OR 0.4, 95%CI 0.2-0.7) and male sex (OR 0.3, 95%CI 0.2-0.6) were negatively associated with higher exercise capacity. Both exercise capacity (HR 0.96, 95%CI 0.93-0.98) and heart rate reserve (HR 0.96, 95%CI 0.94-0.98) were associated with mortality.

Conclusions: In patients with repaired tetralogy of Fallot the exercise capacity was approximately 75% of expected, regardless of age. Patients with better NYHA class and high physical activity level had a higher exercise capacity. Low exercise capacity and low heart rate reserve were associated with higher mortality. Therefore, evaluating exercise capacity as part of follow up is of importance.

Ort, förlag, år, upplaga, sidor
Elsevier, 2021
Nyckelord
Adult congenital heart disease, Tetralogy of Fallot, Exercise test, Exercise capacity, Register
Nationell ämneskategori
Kardiologi
Identifikatorer
urn:nbn:se:umu:diva-189921 (URN)10.1016/j.ijcchd.2021.100204 (DOI)
Tillgänglig från: 2021-11-25 Skapad: 2021-11-25 Senast uppdaterad: 2024-04-22Bibliografiskt granskad
4. Impaired exercise capacity is associated with higher mortality in adults with congenital heart disease
Öppna denna publikation i ny flik eller fönster >>Impaired exercise capacity is associated with higher mortality in adults with congenital heart disease
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(Engelska)Manuskript (preprint) (Övrigt vetenskapligt)
Nationell ämneskategori
Kardiologi
Forskningsämne
kardiologi
Identifikatorer
urn:nbn:se:umu:diva-194581 (URN)
Tillgänglig från: 2022-05-10 Skapad: 2022-05-10 Senast uppdaterad: 2022-05-11

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