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Coarctation of the aorta: register and imaging studies
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine.
2016 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Background Coarctation of the aorta (CoA) constitutes 5-8 % of all congenital heart disease (CHD) and is associated with long-term complications such as hypertension (HTN) and left ventricular hypertrophy (LVH). Factors associated with HTN, LVH, and diffuse myocardial fibrosis, are not yet fully explored in this population.

Methods Papers I-III: The Swedish national register of congenital heart disease (SWEDCON) was used to identify adult patients with repaired CoA.

Paper IV: Data on 2,424 adult patients with CHD was extracted from SWEDCON and compared to controls (n = 4,605) regarding height, weight and body mass index (BMI).

Paper V: Adults with CoA (n = 21, age 28.5 (19.1-65.1) years, 33.3 % female) referred for CMR were investigated with T1 mapping to determine left ventricular extracellular volume fraction (ECV).

Results Papers I-II: Out of 653 patients, 344 (52.7 %) had HTN. In a multivariable model, age (years) (OR 1.07, CI 1.05-1.10), sex (male) (OR 3.35, CI 1.98-5.68) and BMI (kg/m2) (OR 1.09, CI 1.03-1.16) were associated with having HTN, and so was systolic arm-leg blood pressure (BP) gradient where an association was found at the ranges (10, 20] mmHg (OR 3.58, CI 1.70-7.55) and > 20 mmHg (OR 11.38, CI 4.03-32.11), in comparison to the range [0, 10] mmHg.

When investigating 243 patients with diagnosed HTN, 127 (52.3 %) had elevated BP (≥ 140/90 mmHg). Age (years) (OR 1.03, CI 1.01-1.06) was associated with elevated BP, and so was systolic arm-leg BP gradient in the ranges (10, 20] mmHg (OR 4.92, CI 1.76-13.79), and > 20 mmHg (OR 9.93, CI 2.99-33.02), in comparison to the reference interval [0, 10] mmHg.

Patients with elevated BP had more classes of anti-hypertensive medication classes prescribed (1.9 vs 1.5, p = 0.003).

Paper III: Out of 506 patients, 114 (22.5 %) were found to have LVH. Systolic BP (mmHg) (OR 1.02, CI 1.01-1.04), aortic valve disease, (OR 2.17, CI 1.33–3.53), age (years) (OR 1.03, CI 1.01–1.05), and HTN (OR 3.02, CI 1.81-5.02), were associated with LVH, while sex (female) (OR 0.41, CI 0.24-0.72) was negatively associated with LVH.

Paper IV: There was no difference in height, weight, or BMI between patients with CoA (n = 414) and the reference population.

Paper V: In the population of 21 patients, an increased left ventricular myocardial ECV was found in 6 cases (28.6 %). Of the patients with increased ECV, 5/6 (83.3 %) were female (p = 0.002). Patients with increased ECV did not otherwise differ from the rest of the study population. iv

Conclusions In adults with repaired CoA, HTN and LVH were common, and many patients with HTN had elevated BP despite treatment. The potentially modifiable factors BMI and systolic arm-leg BP gradient were associated with HTN, and the gradient was also associated with elevated BP among patients with diagnosed HTN. The gradient’s significance remained even within what the current guidelines consider acceptable ranges. Potentially modifiable factors associated with LVH were systolic BP and aortic valve disease. We found no general difference in height, weight, or BMI between patients with CoA and the reference population. While LVH was more common among men, increased myocardial ECV was more common among women.

Place, publisher, year, edition, pages
Umeå: Umeå universitet , 2016. , 60 p.
Series
Umeå University medical dissertations, ISSN 0346-6612 ; 1842
Keyword [en]
coarctation of the aorta, adult congenital heart disease, hypertension, left ventricular hypertrophy, body mass index, height, weight, register, CMR
National Category
Cardiac and Cardiovascular Systems Public Health, Global Health, Social Medicine and Epidemiology
Identifiers
URN: urn:nbn:se:umu:diva-126428ISBN: 978-91-7601-557-5 (print)OAI: oai:DiVA.org:umu-126428DiVA: diva2:1033126
Public defence
2016-10-28, Hörsal B Unod T 9, Norrlands Universitetssjukhus, 13:00 (English)
Opponent
Supervisors
Available from: 2016-10-07 Created: 2016-10-05 Last updated: 2016-10-06Bibliographically approved
List of papers
1. Hypertension in adults with repaired coarctation of the aorta
Open this publication in new window or tab >>Hypertension in adults with repaired coarctation of the aorta
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2016 (English)In: American Heart Journal, ISSN 0002-8703, E-ISSN 1097-6744, Vol. 181, 10-15 p.Article in journal (Refereed) Published
Abstract [en]

Aims: In adults with coarctation of the aorta (CoA), hypertension (HTN) is a common long-term complication. We investigated the prevalence of HTN and analyzed factors associated with HTN.

Methods and results: In the national register for congenital heart disease, 653 adults with repaired CoA were identified (mean age 36.9 ± 14.4 years); 344 (52.7%) of them had HTN, defined as either an existing diagnosis or blood pressure (BP) ≥140/90 mmHg at the clinical visit. In a multivariable model, age (years) (odds ratio [OR] 1.07, CI 1.05-1.10), sex (male) (OR 3.35, CI 1.98-5.68), and body mass index (kilograms per square meter) (OR 1.09, CI 1.03-1.16) were independently associated with having HTN, and so was systolic arm-leg BP gradient where an association with HTN was found at the ranges of (10, 20] and N20 mmHg, in comparison to the interval ≤10 mmHg (OR 3.58, CI 1.70-7.55, and OR 11.38, CI 4.03-32.11). This model remained valid when all patients who had increased BP (≥140/90 mmHg) without having been diagnosed with HTN were excluded from the analyses.

Conclusions: Hypertension is common in patients with previously repaired CoA and is associated with increasing age, male sex, and elevated body mass index. There is also an association with arm-leg BP gradient, starting at relatively low levels that are usually not considered for intervention.

Place, publisher, year, edition, pages
Elsevier, 2016
National Category
Cardiac and Cardiovascular Systems Public Health, Global Health, Social Medicine and Epidemiology
Identifiers
urn:nbn:se:umu:diva-126422 (URN)10.1016/j.ahj.2016.07.012 (DOI)
Available from: 2016-10-05 Created: 2016-10-05 Last updated: 2017-11-30Bibliographically approved
2. Poor blood pressure control in adults with repaired coarctation of the aorta and hypertension: a register-based study of associated factors
Open this publication in new window or tab >>Poor blood pressure control in adults with repaired coarctation of the aorta and hypertension: a register-based study of associated factors
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2017 (English)In: Cardiology in the Young, ISSN 1047-9511, E-ISSN 1467-1107, Vol. 27, no 9, 1708-1715 p.Article in journal (Other academic) Published
Abstract [en]

Arterial hypertension is common in adults with repaired coarctation of the aorta, and is associated with several severe complications.

This study aimed to investigate the prevalence of poorly controlled (⩾140/90 mmHg) blood pressure among patients with diagnosed hypertension and to identify associated factors.

In the national register for CHD, adults with repaired coarctation of the aorta and diagnosed hypertension – defined as a registry diagnosis and/or use of anti-hypertensive prescription medication – were identified. Logistic regression analysis was used to identify variables associated with poorly controlled blood pressure.

Of the 243 included patients, 27.2% were female, the mean age was 45.4±15.3 years, and 52.3% had poorly controlled blood pressure at the last registration. In a multivariable model, age (years) (OR 1.03, CI 1.01–1.06, p=0.008) was independently associated with poorly controlled blood pressure and so was systolic arm–leg blood pressure gradient in the ranges [10, 20] mmHg (OR 4.92, CI 1.76–13.79, p=0.002) to >20 mmHg (OR 9.93, CI 2.99–33.02, p<0.001), in comparison with the reference interval [0, 10] mmHg. Patients with poorly controlled blood pressure had, on average, more types of anti-hypertensive medication classes prescribed (1.9 versus 1.5, p=0.003).

Poorly controlled blood pressure is common among patients with repaired coarctation of the aorta and diagnosed hypertension, despite what seems to be more intensive treatment. A systolic arm–leg blood pressure gradient is associated with poorly controlled blood pressure, even at low levels usually not considered for intervention, and may be an indicator of hypertension that is difficult to treat.

Place, publisher, year, edition, pages
Cambridge University Press, 2017
Keyword
Coarctation of the aorta, hypertension, adult CHD, register
National Category
Public Health, Global Health, Social Medicine and Epidemiology Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-126424 (URN)10.1017/S1047951117001020 (DOI)000412664000009 ()28703088 (PubMedID)
Note

Originally published in thesis in manuscript form.

Available from: 2016-10-05 Created: 2016-10-05 Last updated: 2017-12-15Bibliographically approved
3. Left ventricular hypertrophy in adults with previous repair of coarctation of the aorta: association with systolic blood pressure in the high normal range
Open this publication in new window or tab >>Left ventricular hypertrophy in adults with previous repair of coarctation of the aorta: association with systolic blood pressure in the high normal range
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2016 (English)In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 218, 59-64 p.Article in journal (Refereed) Published
Abstract [en]

Background: Arterial hypertension is common in adults with repaired coarctation of the aorta (CoA). The associations between the diagnosis of hypertension, actual blood pressure, other factors affecting left ventricular overload, and left ventricular hypertrophy (LVH) are not yet fully explored in this population.

Material and results: From the national register for congenital heart disease, 506 adult patients (≥18 years old) with previous repair of CoA were identified (37.0% female, mean age 35.7 ± 13.8 years, with an average of 26.8 ± 12.4 years post repair). Echocardiographic data were available for all patients, and showed LVH in 114 (22.5%) of these. Systolic blood pressure (SBP) (mm Hg) (OR 1.02, CI 1.01–1.04), aortic valve disease, (OR 2.17, CI 1.33–3.53), age (years) (OR 1.03, CI 1.01–1.05), diagnosis of arterial hypertension (OR 3.02, CI 1.81–5.02), and sex (female) (OR 0.41, CI 0.24–0.72) were independently associated with LVH. There was an association with LVH at SBP within the upper reference limits [130, 140] mm Hg (OR 2.23, CI 1.05–4.73) that further increased for SBP N 140 mm Hg (OR 8.02, CI 3.76–17.12).

Conclusions: LVH is common post repair of CoA and is associatedwith SBP even belowthe currently recommended target level. Lower target levels may therefore become justified in this population.

Keyword
CoA, Hypertension, Left ventricular hypertrophy, Adult congenital heart disease
National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-126425 (URN)10.1016/j.ijcard.2016.05.033 (DOI)000377856300010 ()27232912 (PubMedID)
Available from: 2016-10-05 Created: 2016-10-05 Last updated: 2017-11-30Bibliographically approved
4. Height, weight and body mass index in adults with congenital heart disease
Open this publication in new window or tab >>Height, weight and body mass index in adults with congenital heart disease
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2015 (English)In: International Journal of Cardiology, ISSN 0167-5273, E-ISSN 1874-1754, Vol. 187, 219-226 p.Article in journal (Refereed) Published
Abstract [en]

Background: High BMI is a risk factor for cardiovascular disease and, in contrast, low BMI is associated with worse prognosis in heart failure. The knowledge on BMI and the distribution in different BMI-classes in adults with congenital heart disease (CHD) are limited. Methods and results: Data on 2424 adult patients was extracted from the Swedish Registry on Congenital Heart Disease and compared to a reference population (n = 4605). The prevalence of overweight/obesity (BMI >= 25) was lower in men with variants of the Fontan procedure, pulmonary atresia (PA)/double outlet right ventricle (DORV) and aortic valve disease (AVD) (Fontan 22.0% and PA/DORV 15.1% vs. 43.0%, p = 0.048 and p < 0.001) (AVD 37.5% vs. 49.3%, p < 0.001). Overt obesity (BMI >= 30) was only more common in women with AVD (12.8% vs. 9.0%, p = 0.005). Underweight (BMI < 18.5) was generally more common in men with CHD (complex lesions 4.9% vs. 0.9%, p < 0.001 and simple lesions 3.2% vs. 0.6%, <0.001). Men with complex lesions were shorter than controls in contrast to females that in general did not differ from controls. Conclusion: Higher prevalence of underweight in men with CHD combined with a lower prevalence of over-weight/obesity in men with some complex lesions indicates that men with CHD in general has lower BMI compared to controls. In women, only limited differences between those with CHD and the controls were found. The complexity of the CHD had larger impact on height in men. The cause of these gender differences as well as possible significance for prognosis is unknown.

Place, publisher, year, edition, pages
Elsevier, 2015
Keyword
congenital heart disease, body mass index, height, weight, adult
National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-105260 (URN)10.1016/j.ijcard.2015.03.153 (DOI)000354905600063 ()25838217 (PubMedID)
Available from: 2015-06-22 Created: 2015-06-22 Last updated: 2017-12-04Bibliographically approved
5. High prevalence of increased left ventricular myocardial extracellular volume fraction in adult women with coarctation of the aorta
Open this publication in new window or tab >>High prevalence of increased left ventricular myocardial extracellular volume fraction in adult women with coarctation of the aorta
(English)Manuscript (preprint) (Other academic)
Abstract [en]

Background: Left ventricular hypertrophy (LVH) is common among patients with coarctation of the aorta (CoA). Cardiovascular magnetic resonance imaging (CMR) can be used to accurately measure left ventricular mass (LVM), but also the myocardial extracellular volume fraction (ECV), which reflects the degree of diffuse myocardial fibrosis.

Aims: This study aimed to investigate the prevalence of increased left ventricular myocardial ECV in adults with CoA, and to assess the relationship between increased ECV and LVH.

Material and methods: Adult patients with CoA (n = 21, age 28.5 (19.1-65.1) years, 33% female, 86% with prior CoA repair) referred clinically for CMR were investigated with T1 and ECV mapping. Clinical and echocardiographic data were retrieved from medical records.

Results: Median ECV was 29.0 (22.0-35.0) %; 29.0 (22.0-33.0) % for men versus 32.0 (27.0-35.0) % for women, p = 0.026). Median LVM indexed to body surface area (BSA) was 71.5 (44.0-99.6) g/m2, and LVM/BSA did not correlate with ECV (r = -0.184, p = 0.450). An increased myocardial ECV exceeding the upper normal limit (30.6 %) was found in 6/21 (29 %) of the patients. Of the patients with increased ECV, 5/6 (83 %) were female (p = 0.002). Patients with increased ECV did not differ from the rest of the study population in terms of age, age at intervention, blood pressure, or functional parameters such as left ventricular volumes or ejection fraction (p > 0.05 for all).

Conclusion: In a clinical population of adults with CoA, increased myocardial ECV was common, and associated with female sex, but not with LVM.

National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-126426 (URN)
Available from: 2016-10-05 Created: 2016-10-05 Last updated: 2017-02-13

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