| 1. |
- Diller, Gerhard-Paul, et al.
(författare)
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Exercise intolerance in adult congenital heart disease : comparative severity, correlates, and prognostic implication.
- 2005
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Ingår i: Circulation. - 0009-7322 .- 1524-4539. ; 112:6, s. 828-35
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Although some patients with adult congenital heart disease (ACHD) report limitations in exercise capacity, we hypothesized that depressed exercise capacity may be more widespread than superficially evident during clinical consultation and could be a means of assessing risk.METHODS AND RESULTS: Cardiopulmonary exercise testing was performed in 335 consecutive ACHD patients (age, 33+/-13 years), 40 non-congenital heart failure patients (age, 58+/-15 years), and 11 young (age, 29+/-5 years) and 12 older (age, 59+/-9 years) healthy subjects. Peak oxygen consumption (peak VO2) was reduced in ACHD patients compared with healthy subjects of similar age (21.7+/-8.5 versus 45.1+/-8.6; P<0.001). No significant difference in peak VO2 was found between ACHD and heart failure patients of corresponding NYHA class (P=NS for each NYHA class). Within ACHD subgroups, peak VO2 gradually declined from aortic coarctation (28.7+/-10.4) to Eisenmenger (11.5+/-3.6) patients (P<0.001). Multivariable correlates of peak VO2 were peak heart rate (r=0.33), forced expiratory volume (r=0.33), pulmonary hypertension (r=-0.26), gender (r=-0.23), and body mass index (r=-0.19). After a median follow-up of 10 months, 62 patients (18.5%) were hospitalized or had died. On multivariable Cox analysis, peak VO2 predicted hospitalization or death (hazard ratio, 0.937; P=0.01) and was related to the frequency and duration of hospitalization (P=0.01 for each).CONCLUSIONS: Exercise capacity is depressed in ACHD patients (even in allegedly asymptomatic patients) on a par with chronic heart failure subjects. Lack of heart rate response to exercise, pulmonary arterial hypertension, and impaired pulmonary function are important correlates of exercise capacity, as is underlying cardiac anatomy. Poor exercise capacity identifies ACHD patients at risk for hospitalization or death.
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| 2. |
- Lam, Yat-Yin, et al.
(författare)
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Left ventricular and ascending aortic function after stenting of native coarctation of aorta.
- 2010
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Ingår i: American Journal of Cardiology. - : Elsevier BV. - 0002-9149 .- 1879-1913. ; 105:9, s. 1343-1347
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Tidskriftsartikel (refereegranskat)abstract
- Patients with surgically corrected aortic coarctation have increased proximal aortic stiffness that might contribute to the known worse cardiovascular outcomes. We examined the effect of stenting on the mid-term ascending aortic elastic properties and its relation to cardiac structure and function in adults with native coarctation of the aorta. A total of 20 consecutive patients (13 men, age at stenting 30 + or - 8 years) were prospectively studied before and 14 + or - 2 months after coarctation stenting. The aortic stiffness index was calculated using the ascending aortic diameters and right arm blood pressure values. The ventricular long-axis function was assessed using pulsed-wave tissue Doppler imaging at the septal site. The results were compared to those from 31 normal controls. Statistically significant improvement was found in aortic narrowing (catheter-derived gradient 32 + or - 11 vs 10 + or - 6 mm Hg), left ventricular mass index (132.8 + or - 50.1 vs 114.7 + or - 47.7 g/m(2)), long-axis function, and left atrial volume index (26.5 + or - 5.3 vs 23.7 + or - 5.6 mm(3)/m(2)). The patients continued to have a thicker left ventricle, reduced long-axis function, and larger left atrium after intervention than did the controls. They also had impaired proximal aortic function with respect to the controls that remained unchanged after stenting (aortic stiffness index 10.7 + or - 4.5 to 10.1 + or - 3.0). The poststenting aortic stiffness index correlated modestly with the left ventricular mass index and reduced long-axis velocity. In conclusion, aortic stenting resulted in partial mid-term improvement in cardiac structure and function in adults with coarctation of aorta but the ascending aortic elastic properties remained abnormal. Such a degree of impairment was related to residual left ventricular hypertrophy and dysfunction. Early identification of such patients and optimum management might avoid these irreversible ventriculoaortic disturbances and their known consequences.
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| 3. |
- Babu-Narayan, Sonya V, et al.
(författare)
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Dyssynchrony and electromechanical delay are associated with focal fibrosis in the systemic right ventricle - Insights from echocardiography.
- 2016
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Ingår i: International Journal of Cardiology. - : Elsevier BV. - 0167-5273 .- 1874-1754. ; 220, s. 382-388
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Systemic right ventricular (RV) dysfunction and sudden cardiac death remain problematic late after Mustard operation for transposition of the great arteries. The exact mechanism for that relationship is likely to be multifactorial including myocardial fibrosis. Doppler echocardiography gives further insights into the role of fibrosis shown by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in late morbidity.METHODS AND RESULTS: Twenty-two consecutive patients, mean age 28±8years, were studied with 2D echocardiography, and also assessed by LGE CMR. The presence of LGE in 13/22 patients (59%) was related to delayed septal shortening and lengthening (P=0.002 &P=0.049), prolonged systemic RV isovolumic contraction time (P=0.024) and reduced systemic RV free wall and septal excursion (P=0.027 &P=0.005). The systemic RV total isovolumic time was prolonged but not related to extent of LGE. LGE extent was related to markers of electromechanical delay and dyssynchrony (delayed onset of RV free wall shortening and lengthening; r=0.73 &P=0.004 and r=0.62 &P=0.041, respectively, and QRS duration r=0.68, P<0.01) and was inversely related to systolic RV free wall shortening velocity (r=-0.59 &P=0.042). The presence of LGE was also related to lower exercise capacity, ≥mild tricuspid regurgitation and more arrhythmia (P=0.008, P=0.014 and P=0.040). RV free wall excursion and systolic tissue Doppler velocity were related to CMR derived RV ejection fraction (r=0.51, P=0.015, and r=0.77, P=<0.001, respectively).CONCLUSION: Post Mustard repair, myocardial fibrosis is related to dyssynchrony, RV long axis dysfunction and tricuspid regurgitation. Echocardiographic measurements of systemic RV function can be confidently used in serial follow-up following Mustard operation.
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| 4. |
- Daelman, Bo, et al.
(författare)
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Frailty and cognitive function in middle-aged and older adults with congenital heart disease
- 2024
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Ingår i: Journal of the American College of Cardiology. - : Elsevier. - 0735-1097 .- 1558-3597. ; 83:12, s. 1149-1159
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Tidskriftsartikel (refereegranskat)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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| 5. |
- Hansmann, Georg, et al.
(författare)
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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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Ingår i: The Journal of Heart and Lung Transplantation. - : Elsevier BV. - 1053-2498. ; 38:9, s. 879-901
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Forskningsöversikt (refereegranskat)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.
(författare)
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Past and current cause-specific mortality in Eisenmenger syndrome.
- 2017
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Ingår i: European Heart Journal. - : Oxford University Press (OUP). - 0195-668X .- 1522-9645. ; 38:26, s. 2060-2067
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Tidskriftsartikel (refereegranskat)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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| 7. |
- Lam, Y-Y, et al.
(författare)
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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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Ingår i: Heart. - : BMJ. - 1355-6037 .- 1468-201X. ; 95:9, s. 733-739
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Tidskriftsartikel (refereegranskat)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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| 8. |
- McMahon, Colin J, et al.
(författare)
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Adult congenital heart disease training in Europe: current status, disparities and potential solutions.
- 2023
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Ingår i: Open heart. - 2053-3624. ; 10:2
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Tidskriftsartikel (refereegranskat)abstract
- This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe.A questionnaire was sent to ACHD cardiologists from 34 European countries.Representatives from 31 of 34 countries (91%) responded. ACHD cardiology was recognised by the respective ministry of Health in two countries (7%) as a subspecialty. Two countries (7%) have formally recognised ACHD training programmes, 15 (48%) have informal (neither accredited nor certified) training and 14 (45%) have very limited or no programme. Twenty-five countries (81%) described training ACHD doctors 'on the job'. The median number of ACHD centres per country was 4 (range 0-28), median number of ACHD surgical centres was 3 (0-26) and the median number of ACHD training centres was 2 (range 0-28). An established exit examination in ACHD was conducted in only one country (3%) and formal certification provided by two countries (7%). ACHD cardiologist number versus gross domestic product Pearson correlation coefficient=0.789 (p<0.001).Formal or accredited training in ACHD is rare among European countries. Many countries have very limited or no training and resort to 'train people on the job'. Few countries provide either an exit examination or certification. Efforts to harmonise training and establish standards in exit examination and certification may improve training and consequently promote the alignment of high-quality patient care.
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| 9. |
- Sliwa, Karen, et al.
(författare)
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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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Ingår i: European journal of heart failure. - : Wiley. - 1879-0844 .- 1388-9842. ; 23:4, s. 527-540
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Tidskriftsartikel (refereegranskat)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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| 10. |
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