Adults With Congenital Heart Disease Patients Research Articles

Prevalence, Predictors, and Prognostic Value of Renal Dysfunction in Adults With Congenital Heart Disease

Renal insufficiency in patients with ischemic heart disease and acquired heart failure is associated with higher mortality and morbidity. We studied the prevalence of renal dysfunction in adult patients with congenital heart disease (ACHD) and its relation to outcome. A total of 1102 adult patients with congenital heart disease (age 36.0+/-14.2 years) attending our institution between 1999 and 2006 had creatinine concentration measured. Glomerular filtration rate (GFR) was calculated with the Modification of Diet in Renal Disease equation. Patients were divided into groups of normal GFR (> or =90 mL . min(-1) . 1.73 m(-2)), mildly impaired GFR (60 to 89 mL . min(-1) . 1.73 m(-2)), and moderately/severely impaired GFR (<60 mL . min(-1) . 1.73 m(-2)). Survival was compared between GFR groups by Cox regression. Median follow-up was 4.1 years, during which 103 patients died. Renal dysfunction was mild in 41% of patients and moderate or severe in 9%. A decrease in GFR was more common among patients with Eisenmenger physiology, of whom 72% had reduced GFR (<90 mL . min(-1) . 1.73 m(-2), P<0.0001 compared with the remainder), and in 18%, this was moderate or severe (P=0.007). Renal dysfunction had a substantial impact on mortality (propensity score-weighted hazard ratio 3.25, 95% CI 1.54 to 6.86, P=0.002 for moderately or severely impaired versus normal GFR). Deranged physiology in adult patients with congenital heart disease is not limited to the heart but also affects the kidney. Mortality is 3-fold higher than normal in the 1 in 11 patients who have moderate or severe GFR reduction.

Beta-Blockade Does Not Alter Plasma Cytokine Concentrations and Ventricular Function in Young Adults With Right Ventricular Dysfunction Secondary to Operated Congenital Heart Disease

Right heart failure is a major problem of young adults with congenital heart disease (ACHD) that has been corrected by cardiac surgery. In left ventricular dysfunction (LVD), beta-blocker therapy improved cytokine concentrations and clinical status. Thus, the effect of bisoprolol on clinical status and plasma cytokine concentrations in ACHD patients with right heart failure after surgical correction for Tetralogy of Fallot was investigated. A prospective, randomized, double-blind, placebo controlled study for a duration of 6 months was carried out. A total of 34 patients (30.9+/-9.5 years; New York Heart Association I or II) with brain natriuretic peptide plasma concentrations >100 pg/ml and peak oxygen uptake <25 ml . kg(-1) .min(-1) were recruited. Bisoprolol did not reduce plasma concentrations of soluble tumor necrosis factor receptors (sTNF-R) and interleukin-6 (IL-6) in ACHD patients (before/after: sTNF-R1: 750+/-131/802+/-130; sTNF-R2: 3,693+/-1,043/4,166+/-840; IL-6: 12.94+/-26.03/13.69+/-16.58 pg/ml). Likewise, peak oxygen uptake, right and left ventricular parameters (determined by magnetic resonance imaging) were not improved by bisoprolol treatment. In contrast to previously observed beneficial effect(s) of beta-blockade in patients with LVD, there were no beneficial therapeutic effects or cytokine reduction in asymptomatic or minimal symptomatic ACHD patients. These data point to a different pathophysiological role of cytokines in ACHD patients with right ventricular dysfunction, as compared to patients with LVD.

Smoking and its effects on mortality in adults with congenital heart disease

Aims To describe smoking habits in adults with congenital heart disease (ACHD) and to assess the relationship between smoking exposure and cardiovascular mortality. Methods Data on smoking history and cardiovascular mortality were extracted from the Euro Heart Survey on adult congenital heart disease — a retrospective cohort study, that included patients diagnosed with 1 of 8 subgroups of ACHD (Atrial Septal Defects, Ventricular Septal Defects, Marfan Syndrome, Aortic Coarctation, Tetralogy of Fallot (ToF), Transposition of the Great Arteries (TGA), Fontan circulation, and Cyanotic disease). Results Complete data of 3375 ACHD patients (median age 28 years) were available for analysis. At inclusion, 9.3% ( n = 314) were current smokers and 4.2% ( n = 142) of the patients had smoked in the past. During a median follow-up of 5.1 years, 101 patients (3%) died. In the majority of cases the cause of death was cardiovascular ( n = 81; 80%). Kaplan–Meier and Cox survival analysis for each of the defects separately showed a significantly increased age and sex-adjusted cardiovascular mortality associated with smoking exposure in TGA patients (Hazard ratio 4.2 (95% CI 1.0–16.8); P = 0.044). Also in ToF mortality was higher amongst smokers, though not significantly (HR 3.4 (95% CI 0.6–18.5); P = 0.15). In the remaining defects no relationship between smoking and cardiovascular mortality was observed. Conclusion The prevalence of smoking amongst ACHD patients is relatively low. Smoking exposure is associated with increased cardiovascular mortality in patients with TGA. Prospective long-term follow-up studies are necessary.

Is the Ability index superior to the NYHA classification for assessing heart failure?

Heart failure (HF) is a major problem in the long-term follow-up of adolescents and adults with congenital heart disease (ACHD) after cardiac surgery. The functional status of ACHD may be assessed in terms of the NYHA classification or the Ability index (ABILITY). The purpose of our study was to examine which of the two classification systems is more closely related to objectively defined HF. NT-pro brain natriuretic peptide (N-BNP) and maximal oxygen uptake (VO(2max)) were measured in 360 consecutive ACHD patients. HF was defined as an elevated N-BNP level > or =100 pg/ml combined with a reduced VO(2max) < or =25 ml/kg/min. There were no significant differences between the NYHA and ABILITY in grading HF in these patients. In both classifications, the risk of HF increases continuously over the classes and grades from odds ratio (OR) 1 in NYHA I/ABILITY 1 to an OR=3.4 in NYHA II/ ABILITY 2 up to 11.6 or 5.4 (ns) in NYHA III/ABILITY 3. Thus in the highest scores HF is found in 70-77% of the patients. The fact that in NYHA class I and ABILITY grade 1, 15% and 19% of the patients exhibited HF according to the measured indices underscores the discrepancy between subjective and objective assessment of the individual patients condition. The NYHA classification and the Ability index take different approaches to the patients with congenital heart defects but are equally suitable for the judgement of HF in post surgical ACHD.

Chronotropic Incompetence in Adolescents and Adults With Congenital Heart Disease After Cardiac Surgery

Chronotropic incompetence (CI) is one of the major problems in adults with congestive heart. Little is known about CI in adults with congenital heart disease (ACHD) after cardiac surgery. The purpose of our study was to investigate the presence and risk factors of CI in ACHD patients. Clinical and echocardiographic data, NT-pro brain natriuretic peptide (N-BNP), and peak oxygen uptake (VO2peak) during spiroergometry were obtained in 345 consecutive ACHD patients. CI was defined as the failure to achieve > or = 80% of the predicted maximal heart rate. A total of 117 (34%) of study patients fulfilled the CI criterion. These patients were in a higher New York Heart Association class (1.7 +/- 0.06 versus 1.4 +/- 0.03, P < .0001; mean +/- SEM), had significantly higher N-BNP levels (230 +/- 31 versus 121 +/- 10 pg/mL, P < .0001) and a more pronounced impairment of VO2peak (23.8 +/- 0.6 versus 28.4 +/- 0.5 mL x kg x min, P < .0001) than those without CI. Elevated odds ratios for CI were found in patients with a single ventricle (4.03), Mustard operation for transposition of the great arteries (3.11), and aortic coarctation (2.14). Our results indicate that CI in ACHD patients is a frequent problem and is related to the severity of the heart failure as measured by symptom assessment (New York Heart Association class), plasma N-BNP level and peak oxygen uptake.