Pulmonary hypertension (PH) is an established risk factor in patients with atrial septal defect (ASD), and its persistence after ASD closure is associated with increased mortality. Therefore, predictors for PH normalization after defect closure are needed. Multiple hemodynamic types of PH exist, but little is known about their prevalence and prognostic value for PH normalization after ASD closure. We carried out a retrospective study on 97 patients (76% female, median age at ASD closure 58 years) with four types of PH determined predominantly by right heart catheterization: hyperkinetic, pulmonary arterial hypertension, isolated post-capillary, and combined pre- and post-capillary. We investigated the frequency of the PH types and their prognostic significance for PH normalization after ASD closure. Frequency of PH types before ASD closure in our study was: hyperkinetic 55%, pulmonary arterial hypertension 10%, isolated post-capillary PH 24%, and combined PH 11%. Hyperkinetic PH type was positively associated with PH normalization after ASD closure (78% patients normalized), remaining a significant independent predictor when adjusted for age at closure, sex, heart failure, and NYHA. Hyperkinetic PH patients also had significantly better survival prognosis versus patients with other PH types (p = 0.04). Combined PH was negatively associated with PH normalization, with no patients normalizing. Pulmonary arterial hypertension and isolated post-capillary PH had intermediate rates of normalization (60 and 52%, respectively). In summary, all four hemodynamic types of PH are found in adult patients with ASD, and they can be used to stratify patients by their likelihood of PH normalization and survival after ASD closure.
- Publikační typ
- časopisecké články MeSH
Background: Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether to perform ASD closure, especially in elderly patients with PH, is a complex dilemma. The aim of our study was to compare long-term survival in patients with closed and open ASD. Methods: A retrospective cohort study was performed on 427 patients with ASD (median age at diagnosis 38 years, IQR 18-56) out of which 186 patients (44%) manifested PH. ASD closure in patients with PH was only considered in patients without Eisenmenger syndrome with pulmonary vascular resistance < 5 WU. Median follow-up duration was 18 years (IQR 9-31 years). Kaplan-Meier and Cox proportional hazards survival analyses were performed to evaluate 12 potential predictors of survival. Results: Defect closure was associated with improved long-term survival in ASD patients both with (P < 0.001) and without PH (P = 0.01) and this association was present also in patients over 40 years. The 20-year survival since diagnosis was significantly higher in patients with PH and closed ASD compared to those with PH and open ASD (65% vs. 41%). ASD closure was a significant independent predictor of long-term survival (P = 0.003) after accounting for age at diagnosis, PH, NYHA class, Eisenmenger syndrome, and mitral regurgitation. Significant negative independent predictors of survival were older age at diagnosis (P < 0.001), Eisenmenger syndrome (P < 0.001), and PH (P = 0.03). Conclusion: ASD closure appears to be associated with improved long-term survival independently of age, PH, and other clinical variables.
- Publikační typ
- časopisecké články MeSH
AIMS: To develop and validate a clinically useful risk prediction tool for patients with adult congenital heart disease (ACHD). METHODS AND RESULTS: A risk model was developed in a prospective cohort of 602 patients with moderate/complex ACHD who routinely visited the outpatient clinic of a tertiary care centre in the Netherlands (2011-2013). This model was externally validated in a retrospective cohort of 402 ACHD patients (Czech Republic, 2004-2013). The primary endpoint was the 4-year risk of death, heart failure, or arrhythmia, which occurred in 135 of 602 patients (22%). Model development was performed using multivariable logistic regression. Model performance was assessed with C-statistics and calibration plots. Of the 14 variables that were selected by an expert panel, the final prediction model included age (OR 1.02, 95%CI 1.00-1.03, p = 0.031), congenital diagnosis (OR 1.52, 95%CI 1.03-2.23, p = 0.034), NYHA class (OR 1.74, 95%CI 1.07-2.84, p = 0.026), cardiac medication (OR 2.27, 95%CI 1.56-3.31, p < 0.001), re-intervention (OR 1.41, 95%CI 0.99-2.01, p = 0.060), BMI (OR 1.03, 95%CI 0.99-1.07, p = 0.123), and NT-proBNP (OR 1.63, 95%CI 1.45-1.84, p < 0.001). Calibration-in-the-large was suboptimal, reflected by a lower observed event rate in the validation cohort (17%) than predicted (36%), likely explained by heterogeneity and different treatment strategies. The externally validated C-statistic was 0.78 (95%CI 0.72-0.83), indicating good discriminative ability. CONCLUSION: The proposed ACHD risk score combines six readily available clinical characteristics and NT-proBNP. This tool is easy to use and can aid in distinguishing high- and low-risk patients, which could further streamline counselling, location of care, and treatment in ACHD.
- MeSH
- dospělí MeSH
- hodnocení rizik metody normy MeSH
- kohortové studie MeSH
- lidé MeSH
- následné studie MeSH
- prediktivní hodnota testů MeSH
- prospektivní studie MeSH
- reprodukovatelnost výsledků MeSH
- teoretické modely * MeSH
- vrozené srdeční vady diagnostické zobrazování epidemiologie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Nizozemsko MeSH
Úvod: Zajištění dobré kvality života je důležitý cíl léčby pacientů s komplexní cyanotickou vrozenou srdeční vadou (VSV). V literatuře však neexistuje shoda o kvalitě života těchto pacientů. Cílem naší studie bylo zhodnocení kvality života a výskytu depresí u dospělých s původně komplexní cyanotickou VSV v dlouhodobém odstupu od operace. Metody: Vyšetřili jsme celkem 212 dospělých po operaci komplexní cyanotické VSV a 86 zdravých kontrol pomocí dotazníku hodnocení kvality života SF-36 a Zungova dotazníku hodnocení deprese. Všichni pacienti byli dále dotázáni na subjektivní hodnocení vlivu VSV na jejich život, na fyzické aktivity, funkční třídu NYHA, vzdělání, zaměstnání, počet dětí a všichni podstoupili echokardiografické vyšetření. Výsledky byly srovnány se skupinou 32 neoperovaných pacientů s komplexní cyanotickou VSV, jejichž výsledky jsme publikovali v minulosti. Výsledky: Výskyt deprese se významně nelišil mezi skupinou operovaných s VSV a zdravými kontrolami. Těžká deprese byla u 8,5 % pacientů s operovanou komplexní cyanotickou VSV a u 7 % zdravých kontrol (p = 0,816). Mezi neoperovanými pacienty s perzistující cyanózou byl výskyt těžké deprese významně vyšší (28,1 %) oproti operovaným (p = 0,003). Kvalita života hodnocená pomocí dotazníku SF-36 se významně nelišila mezi pacienty s operovanými komplexními VSV a kontrolami, kromě horšího vnímání obecného zdraví, hodnocení fyzických funkcí a fyzického omezení činností. Závěr: Pacienti s operovanou komplexní cyanotickou VSV mají v dospělosti dobrou kvalitu života, srovnatelnou s kontrolní skupinou zdravých jedinců, zatímco neoperovaní pacienti s perzistující cyanózou u komplexní VSV mají významně vyšší počet depresí.
Background: Good quality of life is an important goal in treatment of complex cyanotic congenital heart disease (CHD); however, there is a lack of consensus concerning quality of life in complex CHD patients in the literature. The aim of this study was to assess quality of life and rate of depression in adults with complex cyanotic CHD late after repair. Method: The studied cohort consists of 212 adults with repaired complex cyanotic CHD and 86 healthy controls. Subjects filled in questionnaires evaluating quality of life (SF-36) and depression (Zung self-rating depression scale). Additional data on the influence of CHD on patient's life, physical activities, NYHA class, echocardiographic parameters, etc. were recorded. The results were compared with a previously published cohort of 32 cyanotic adults with unrepaired CHD. Results: The rate of depression did not differ significantly between repaired CHD patients and healthy controls. Severe depression was found in 8.5% of repaired complex CHD, in 7.0% of controls (p = 0.816), and in 28.1% of the unrepaired CHD with persistent cyanosis (p = 0.003). The quality of life measured by SF-36 was not significantly different between repaired CHD and controls, except for lower general health perception, physical functioning, and physical role limitation. Conclusion: The repaired complex cyanotic CHD patients have a good quality of life, similar to controls, while unrepaired patients have a significantly higher rate of depression.
- MeSH
- deprese MeSH
- kvalita života * MeSH
- lidé MeSH
- průzkumy a dotazníky MeSH
- vrozené srdeční vady * MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- práce podpořená grantem MeSH
