| 1. |
- Lam, Y-Y, et al.
(author)
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Left ventricular long axis dysfunction in adults with "corrected" aortic coarctation is related to an older age at intervention and increased aortic stiffness.
- 2009
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In: Heart. - : BMJ. - 1355-6037 .- 1468-201X. ; 95:9, s. 733-739
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Journal article (peer-reviewed)abstract
- OBJECTIVES: This study examined the prevalence of left ventricular (LV) long axis dysfunction (LAD, septal annulus pulsed-wave (PW) tissue Doppler imaging (TDI) early diastolic velocity < or =8 cm/s) in patients with "corrected" aortic coarctation and its relationship to patient demographics and aortic elastic properties. METHODS: A retrospective study of 80 consecutive patients with "corrected" aortic coarctation (aged 27 (SD 6) years, seven postballoon aortoplasty, 41 poststenting and 32 postsurgical repair) was carried out. Patients' ages at intervention, comorbidities and medications were recorded. The LV long axis motions were recorded by M-mode and PW TDI. Aortic stiffness indices were calculated from the aortic diameters and pulse pressures. RESULTS: Forty-seven patients (59%) had LAD. They were older (28 (5) vs 9 (6) years) at treatment, had stiffer aorta (stiffness index 18.4 (6.0) vs 9.2 (2.3)), thicker LV walls (146.7 (59.7) vs 103.8 (44.9) g/m2), higher wall stress (80 (6) vs 70 (7) 10(3) dynes/cm2), larger left atria (31.7 (4.6) vs 24.5 (5.3) ml/m2) and higher LV filling pressures (p<0.01 for all) compared with those without LAD, despite a similar prevalence of antihypertensive use and bicuspid aortic valves. The age at intervention (OR 2.92, 95% CI 1.29 to 6.60, p<0.01) and aortic stiffness index (OR 1.98, 95% CI 1.41 to 2.79, p<0.001) were the two independent predictors for LAD in patients on multivariate analysis. A cut-off age of > or =25 year at intervention was 89% sensitive and 76% specific in predicting LAD (AUC = 0.90, p<0.001). CONCLUSIONS: LAD is common in adults with aortic coarctation despite apparently successful treatment. Its presence is related to older age at intervention and increased aortic stiffness.
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| 2. |
- Sliwa, Karen, et al.
(author)
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Risk stratification and management of women with cardiomyopathy/heart failure planning pregnancy or presenting during/after pregnancy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy.
- 2021
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In: European journal of heart failure. - : Wiley. - 1879-0844 .- 1388-9842. ; 23:4, s. 527-540
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Journal article (peer-reviewed)abstract
- This position paper focusses on the pathophysiology, diagnosis and management of women diagnosed with a cardiomyopathy, or at risk of heart failure (HF), who are planning to conceive or present with (de novo or previously unknown) HF during or after pregnancy. This includes the heterogenous group of heart muscle diseases such as hypertrophic, dilated, arrhythmogenic right ventricular and non-classified cardiomyopathies, left ventricular non-compaction, peripartum cardiomyopathy, Takotsubo syndrome, adult congenital heart disease with HF, and patients with right HF. Also, patients with a history of chemo-/radiotherapy for cancer or hematological malignancies need specific pre-, during and post-pregnancy assessment and counseling. We summarize the current knowledge about pathophysiological mechanisms, including gene mutations, clinical presentation, diagnosis, and medical and device management, as well as risk stratification. Women with a known diagnosis of a cardiomyopathy will often require continuation of drug therapy, which has the potential to exert negative effects on the fetus. This position paper assists in balancing benefits and detrimental effects.
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| 3. |
- Daelman, Bo, et al.
(author)
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Frailty and cognitive function in middle-aged and older adults with congenital heart disease
- 2024
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In: Journal of the American College of Cardiology. - : Elsevier. - 0735-1097 .- 1558-3597. ; 83:12, s. 1149-1159
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Journal article (peer-reviewed)abstract
- Background: Life expectancy of patients with congenital heart disease (CHD) has increased rapidly, resulting in a growing and aging population. Recent studies have shown that older people with CHD have higher morbidity, health care use, and mortality. To maintain longevity and quality of life, understanding their evolving medical and psychosocial challenges is essential.Objectives: The authors describe the frailty and cognitive profile of middle-aged and older adults with CHD to identify predictor variables and to explore the relationship with hospital admissions and outpatient visits.Methods: Using a cross-sectional, multicentric design, we included 814 patients aged ≥40 years from 11 countries. Frailty phenotype was determined using the Fried method. Cognitive function was assessed by the Montreal Cognitive Assessment.Results: In this sample, 52.3% of patients were assessed as robust, 41.9% as prefrail, and 5.8% as frail; 38.8% had cognitive dysfunction. Multinomial regression showed that frailty was associated with older age, female sex, higher physiologic class, and comorbidities. Counterintuitively, patients with mild heart defects were more likely than those with complex lesions to be prefrail. Patients from middle-income countries displayed more prefrailty than those from higher-income countries. Logistic regression demonstrated that cognitive dysfunction was related to older age, comorbidities, and lower country-level income.Conclusions: Approximately one-half of included patients were (pre-)frail, and more than one-third experienced cognitive impairment. Frailty and cognitive dysfunction were identified in patients with mild CHD, indicating that these concerns extend beyond severe CHD. Assessing frailty and cognition routinely could offer valuable insights into this aging population.
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| 4. |
- Engelfriet, P, et al.
(author)
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The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period - The Euro Heart Survey on adult congenital heart disease
- 2005
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In: European Heart Journal. - : Oxford University Press (OUP). - 1522-9645 .- 0195-668X. ; 26:21, s. 2325-2333
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Journal article (peer-reviewed)abstract
- Aims To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period. Methods and results Data collected as part of the Euro Heart Survey on adult CHD was analysed. This entailed information transcribed from the files of 4110 patients diagnosed with one of eight congenital heart conditions ('defects'), who consecutively visited the outpatient clinics of one of the participating centres in 1998. The patients were included retrospectively and followed until the end of 2003 for a median follow-up of 5.1 years. Notwithstanding their overall relatively good functional class and low mortality over the follow-up period, a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. There were major differences between the eight defects, both in morbidity and regarding specific characteristics. Outcomes were worst in cyanotic defects and in the Fontan circulation, but a considerable proportion of the other patients also suffer from cardiac symptoms. In particular, arrhythmias are common. Conclusion The spectrum of adult CHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period.
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| 5. |
- Hansmann, Georg, et al.
(author)
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2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT
- 2019
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In: The Journal of Heart and Lung Transplantation. - : Elsevier BV. - 1053-2498. ; 38:9, s. 879-901
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Research review (peer-reviewed)abstract
- © 2019 The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990–2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term “pulmonary hypertension” and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.
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| 6. |
- Hjortshøj, Cristel M Sørensen, et al.
(author)
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Past and current cause-specific mortality in Eisenmenger syndrome.
- 2017
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In: European Heart Journal. - : Oxford University Press (OUP). - 0195-668X .- 1522-9645. ; 38:26, s. 2060-2067
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Journal article (peer-reviewed)abstract
- Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015.Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001).Conclusion: The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.
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