Summary
Background
Heart failure (HF) is the main cause of death in adult congenital heart disease (ACHD).
Aims
We aimed to characterize HF-related hospitalization of patients with ACHD, and to determine HF risk factors and prognosis in this population.
Methods
We prospectively included 471 patients with ACHD admitted to our unit over 24 months. Clinical and biological data and HF management were recorded. Major cardiovascular events were recorded for ACHD with HF.
Results
HF was the main reason for hospitalization in 13% of cases (76/583 hospitalizations). Patients with HF were significantly older (median age 44 ± 14 years vs. 37 ± 15 years; P < 0.01), with more complex congenital heart disease ( P = 0.04). In the multivariable analysis, pulmonary arterial hypertension (odds ratio [OR] 6.2, 95% confidence interval [CI] 3.5–10.7), history of HF (OR 9.8, 95% CI 5.7–16.8) and history of atrial arrhythmia (OR 3.6, 95% CI 2.2–5.9) were significant risk factors for HF-related admissions ( P < 0.001). The mean hospital stay of patients with HF was longer (12.2 vs. 6.9 days; P < 0.01), and 25% of patients required intensive care. Overall, 11/55 (20%) patients with HF died, 10/55 (18%) were readmitted for HF, and 6/55 (11%) had heart transplantation during the median follow-up of 18 months (95% CI 14–20 months). The risk of cardiovascular events was 19-fold higher after HF-related hospitalization.
Conclusions
HF is emerging as a leading cause of morbidity and mortality in the ACHD population. Earlier diagnosis and more active management are required to improve outcomes of HF in ACHD.
Résumé
Contexte
l’insuffisance cardiaque (IC) est la principale cause de décès chez les patients adultes atteints d’une cardiopathie congénitale (ACC).
Objectifs
Nous avons cherché à caractériser les hospitalisations en rapport avec l’IC du patient ACC et à déterminer les facteurs de risque et le pronostic de l’IC dans cette population.
Méthodes
Au total, 471 patients ACC admis dans notre unité sur une période de 24 mois ont été prospectivement inclus. Les données cliniques et biologiques, ainsi que la prise en charge de l’IC ont été recueillies. La survenue d’événements cardiovasculaires majeurs après IC au cours du suivi a été également relevée.
Résultats
l’IC était le motif d’hospitalisation principal dans 13 % des cas (76/583 hospitalisations). Les patients IC étaient significativement plus âgés (âge médian 44 ± 14 vs 37 ± 15 ans ; p < 0,01), avec des cardiopathies congénitales plus complexes ( p = 0,04). En analyse multivariée, l’hypertension artérielle pulmonaire (OR 6,2, IC 95 % 3,5-10,7), les antécédents d’insuffisance cardiaque (OR 9,8, IC 95 % 5,7–16,8) et d’arythmie auriculaire (OR 3,6 IC 95 % 2,2–5,9) étaient des facteurs de risque significatifs d’hospitalisation pour IC ( p < 0,001). La durée moyenne de séjour des patients IC était plus longue (12,2 jours contre 6,9 jours ; p < 0,01) et 25 % des patients ont nécessité une prise en charge en soins intensifs. Vingt pourcent (11/55 patients IC) sont décédés, 18 % (10/55) ont été ré-hospitalisés pour IC, et 11 % (6/55) ont eu une transplantation cardiaque sur un suivi médian de 18 mois (IC 95 % 14–20 mois). Le risque d’événements cardiovasculaires était 19 fois plus élevé après l’hospitalisation liée à l’IC.
Conclusions
L’IC devient l’une des principales causes de morbidité et de mortalité dans la population ACC. Un diagnostic précoce et une gestion plus active de l’IC dans cette population sont nécessaires pour améliorer les résultats.
Background
Owing to technical improvements in cardiac surgery and the medical management of congenital heart disease (CHD), more than 85% of the patients reach now adulthood . Consequently, the prevalence of CHD in the adult population has increased rapidly and is now higher than in the paediatric population . Heart failure (HF) is a serious and common complication in the long-term follow-up of adults with CHD, and is the main cause of death in these patients . A few studies have focused on the occurrence of HF in adult CHD (ACHD) . Although guidelines for management of HF in acquired heart diseases exist , there is no recommendation to prevent complications or to improve the management of HF in the CHD population. In patients with acquired heart disease, hospitalization for HF has a poor prognosis, with increased mortality and morbidity rates, despite current therapies . Twonationwide studies have focused on HF in ACHD, but little is known about the characteristics and prognosis of these patients hospitalized for HF, and the risk factors for HF in this population have not been described in depth .
The aims of the present study were to characterize HF-related hospitalization in an ACHD population in a French cohort; and to determine the risk factors for and the prognosis of HF in patients with ACHD who are hospitalized for HF.
Methods
Study population
Patients with CHD aged 18 years or older who were hospitalized in our tertiary referral centre were prospectively included in the study, from November 2013 to October 2015. This study was approved by the institution’s local ethics committee. Diagnosis, medical history, clinical events and procedure were collected from medical records. The main diagnosis was defined as the congenital heart lesion with the most severe haemodynamic anomaly, and the severity of the CHD diagnosis was assigned according to the Besthesda classification . We compared patients admitted for acute HF (with or without a history of HF) with patients hospitalized for other reasons. Acute and chronic HF were defined according to European Society of Cardiology guidelines . Cardiovascular risk factors (dyslipidemia, arterial hypertension, obesity [defined as body mass index ≥ 30.0)], diabetes mellitus and smoking) and common co-morbidities, including respiratory disease, renal dysfunction, arthritis, obesity, diabetes mellitus, cognitive dysfunction and depression, were also recorded. At admission, demographic data (age, sex, weight and height), clinical data (oxygen saturation, blood pressure, heart rate and rhythm) and biochemical data (natraemia, serum haemoglobin, creatinine and B-type natriuretic peptide [BNP] concentrations) were recorded. Treatment at admission was also reported. The systemic ventricle ejection fraction was obtained from echocardiographic and/or magnetic resonance imaging studies.
Outcomes and risk factors
Major outcomes occurring during the period following entry up to October 2016 were recorded systematically, and were defined as death, cardiac transplant or the need for a circulatory or ventricular assist device, readmission for HF, and a composite outcome, defined as the presence of at least one of those outcomes. Deaths outside the institution were based on physicians receiving a report from other healthcare providers. No patients were lost to follow-up during the 36-month study period.
We studied the predictive value of patient characteristics (sex, main defect, left-sided or right-sided cardiac lesions and history of cardiovascular complications and interventions), clinical and biological characteristics at admission and systemic ventricle ejection fraction, because these variables could potentially increase the risk of HF or were considered clinically important candidate risk factors for mortality and morbidity after HF admission; they were identified based on a review of the literature or clinical relevance.
Statistical analyses
Analyses were performed with the use of MedCalc ® statistical software (MedCalc Software, Mariakerke, Belgium). Data are presented as the mean value ± standard deviation when variables are normally distributed and the median value with 95% confidence interval (CI) when they are not. Comparison between patients admitted for HF and patients admitted for other reasons was made using a two-sample t test or the Mann–Whitney test for quantitative data and a χ 2 test or Fisher’s test for qualitative variables, when appropriate. The predictive value of patient characteristics for HF-related hospitalization was evaluated by univariate and multivariable logistic regression analysis. The multivariable logistic regression model included variables with the significance level P < 0.2 in the univariate analysis, after a backward selection of relevant variables, and excluding collinear variables from the model. For all analyses, a two-tailed P value < 0.05 was used as the criterion for statistical significance.
Results
Four hundred and seventy-one patients with ACHD were consecutively included, and 583 hospitalizations were recorded during the 24-month inclusion period months. HF was the main reason for hospitalization in 76 (13%) cases, concerning 55 (11.6%) patients with ACHD.
Characteristics of patients with HF ( Table 1 )
Patients admitted for HF were older and were mainly men compared with patients without HF ( P ≤ 0.04). Severe cardiac conditions, such as complex CHD, pulmonary arterial hypertension (PAH) and history of cardiovascular events (atrial arrhythmia, heart failure and pacing), were significantly associated with HF ( P ≤ 0.04). Complex CHDs were mainly represented by CHD with right ventricular outflow tract surgery, CHD associated with PAH, univentricular heart and systemic right ventricle ( Fig. 1 ). In patients with recurrent HF ( n = 25), median time between the acute HF preceding the study and the HF episode corresponding to the inclusion was 6.2 years (95% CI 1.9–13.7 years). Interestingly, patients with acute HF had significantly more co-morbidities than patients without HF ( P < 0.01; Table 2 ); they were mainly represented by stroke (16.3% of patients admitted for HF).
| HF group | No HF group | P | |
|---|---|---|---|
| ( n = 55) | ( n = 416) | ||
| Age (years) | 44 ± 14 | 37 ± 15 | <0.0001 |
| Men/women ( n / n ) | 35/20 | 204/212 | 0.04 |
| Syndrome | 6 (10.9) | 40 (9.6) | 0.78 |
| CHD complexity | 0.04 | ||
| Simple | 6 (10.9) | 54 (13.0) | |
| Moderate | 21 (38.2) | 221 (53.1) | |
| Severe | 28 (50.9) | 241 (33.9) | |
| Cardiac surgery | 0.1 | ||
| Palliation | 13 (23.6) | 123 (29.6) | |
| Repair | 16 (29.1) | 68 (16.3) | |
| No | 26 (47.3) | 225 (54.1) | |
| Number of cardiac surgeries | 0.27 | ||
| 0 | 13 (23.6) | 123 (29.6) | |
| 1 | 21 (38.2) | 120 (28.8) | |
| ≥2 | 21 (38.2) | 113 (27.2) | |
| CV risk factors | 28 (50.9) | 170 (40.8) | 0.1 |
| PAH | 16 (29.1) | 30 (7.2) | <0.0001 |
| Cardiac history | |||
| Atrial arrhythmia | 34 (61.8) | 118 (28.4) | <0.0001 |
| Ventricular arrhythmia | 5 (9.1) | 20 (4.8) | 0.2 |
| Pacemaker | 12 (21.8) | 50 (12.0) | 0.04 |
| Heart failure | 25 (45.5) | 26 (6.3) | <0.0001 |
| Infective endocarditis | 5 (9.1) | 25 (6.0) | 0.4 |
| Extracardiac medical history | |||
| History of pregnancy | 8/20 (40.0) | 24/212 (11.3) | 0.0004 |
| Co-morbidities | 34 (61.8) | 154 (37.0) | 0.0004 |
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