Study participation and nonresponse in a population of adolescents and adults with operated congenital heart disease (GUCH patients).

OBJECTIVE: A group of patients after surgery of congenital heart defects was examined as to whether participants and nonparticipants (refusers, nonresponders, moved away, and deceased subjects) differed in terms of the type of congenital heart disease, the type of surgery (curative, reparative, and palliative), age and gender. DESIGN: A group of 698 subjects between 15 and 45 years were invited to participate in a study where a standardized interview was combined with a series of medical examinations. RESULTS: Finally, 361 patients participated, 121 explicitly refused, 92 did not respond, 91 had moved away, and 33 had died. Comparing participants and nonparticipants at the level of bivariate analyses using type of surgery, type of congenital malformation, gender, and age did not yield statistically significant differences. The final analysis using multivariate logistic regression revealed that individuals who underwent palliative treatment, the most severe type of surgery, had the same likelihood of not participating than patients with curative treatment. Classifying patients by the type of heart defect did not reveal group differences in the likelihood to participate. No differences for gender and age emerged. CONCLUSIONS: The analyses have shown that a worse health status may not lead to nonparticipation. As a consequence, we may conclude that selective nonparticipation because of the measures considered may not have occurred, and the likelihood for obtaining biased results can be considered as low.

A prospective, randomized, double-blind, placebo controlled trial of beta-blockade in patients who have undergone surgical correction of tetralogy of Fallot.

AIMS: Our purpose was to evaluate the effect of a treatment over six months with bisoprolol on the surrogate parameters of N-Terminal-pro brain natriuretic peptide, subsequently to be described as brain natriuretic peptide, peak uptake of oxygen, and ventricular function assessed by magnetic resonance imaging in grown ups and adults who had undergone surgical correction of tetralogy of Fallot. METHODS AND RESULTS: We designed a prospective, randomized, double-blind, placebo controlled trial. We enrolled 33 patients, aged 30.9 plus or minus 9.5 years in either class 1 or 2 of the grading of the New York Heart Association class with both levels of brain natriuretic peptide greater than 100 pg/ml and a reduced peak uptake of oxygen less than 25 ml/kg/min. During treatment with Bisoprolol, the levels of brain natriuretic peptide increased significantly from 206 plus or minus 95 to 341 plus or minus 250 pg/ml (p< 0.05), and those of atrial natriuretic peptide from 4117 plus or minus 1837 to 5340 plus or minus 2102 fmol/ml (p = 0.0005). These measures remained unchanged in the group of patients receiving the placebo. Peak uptake of oxygen did not differ significantly in either group, nor did treatment have any significant effect on right and left ventricular volumes and ejection fractions as determined by magnetic resonance imaging. The clinical state as judged within the grading system of the New York Heart Association was also unchanged by beta-blockade. CONCLUSION: Beta blockade with Bisoprolol seems to have no beneficial effect on asymptomatic or mildly symptomatic patients with right ventricular dysfunction secondary to repaired tetralogy of Fallot with residual pulmonary regurgitation and/or stenosis.

Is the Ability index superior to the NYHA classification for assessing heart failure?: comparison of two classification scales in adolescents and adults with operated congenital heart defects.

BACKGROUND: 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). OBJECTIVE: The purpose of our study was to examine which of the two classification systems is more closely related to objectively defined HF. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

BACKGROUND: 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. METHODS AND RESULTS: 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). CONCLUSIONS: 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.

Life chances after surgery of congenital heart disease: the influence of cardiac surgery on intergenerational social mobility. A comparison between patients and general population data.

BACKGROUND: To examine whether operated congenital heart disease (CHD) is setting preconditions for an active life by comparing intergenerational social mobility in patients with a population sample. DESIGN AND METHODS: The patient sample consisted of 314 females (42.1%) and males who underwent surgery for CHD (mean age at surgery 7.3 years, SD 7.8). According to the type of surgery, patients were classified as curative, reparative, or palliative. Consequently, the three groups reflect increasing severities of the initial heart defect. The mean age at examination was 26.9 years (7.6). Controls consisted of individuals who participated in the 2002 survey of the German Socio-Economic Panel. All individuals with complete sociodemographic information were included, and the final sample consisted of 4864 women (46.1%) and men (mean age 32.5 years, SD 7.2). RESULTS: Once having entered the labour market, intergenerational social mobility between patients and controls did not differ. For upward mobility respondents' social background was the key determinant; the respective standardized regression effect was beta=0.66. After dividing the study population, the social background turned out to having a slightly stronger effect in patients (beta=0.73) than in controls (beta=0.65). For downward mobility effects of the social background were smaller than for upward mobility (beta=0.19 in patients and beta=0.21 in controls). In economically active patients CHD severity did not determine social mobility. DISCUSSION: We conclude that a large proportion of patients could not or did not enter the labour market. Those who did experienced social mobility rates that are comparable with the general population.

Psychological symptoms in patients after surgery for congenital cardiac disease.

AIMS: We studied a population of patients with surgically corrected congenital cardiac disease to determine whether limitations in activity, impaired cardiac performance, and perception of body image have effects on psychological symptoms. METHODS: We undertook medical examinations, and carried out standardized interviews, in 361 patients aged between 14 and 45 years with surgically corrected congenital cardiac disease. From this data, findings from 343 patients were suitable for analysis. Subjectively reported limitations in activity were classified according to the system proposed by the New York Heart Association, while cardiopulmonary capacity was used as the indicator of cardiac performance. The Brief Symptom Inventory was used for assessing psychological symptoms, such as somatization, obsession-compulsion, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, and psychoticism. The Body Image Questionnaire was used to depict attitudes towards body image, which is assessed on the two subscales of rejection of the body and vitality. Multivariate regression analyses were conducted separately for females and males, taking into account age and socio-economic position. RESULTS: Impairments of everyday activities had only a few substantial associations with psychological symptoms. No significant effects of cardiac functional capacity as a standardized physiological measure emerged. Psychological symptoms were strongly influenced by perceptions of body image, particularly if they rejected it, this holding particularly for males. There were no gender differences in terms of psychological symptoms. CONCLUSIONS: Limitations of activity, and impaired cardiac performance, have only minor effects on psychological symptoms in patients with surgically corrected congenital cardiac disease. The perception of body image was the strongest predictor, especially if patients rejected their body as a result of disfigurement or perceived deficiency.

[Psychological symptoms and body image in patients after surgery of congenital heart disease]. / Psychische Symptome und Körperbild bei Patientinnen und Patienten nach der Operation angeborener Herzfehler.

In the last 20 years the survival rate of patients with congenital heart disease has increased considerably, thus psychological consequences of living with a heart defect have attracted considerable scientific attention. In our study psychological symptoms and the body image of patients with congenital heart disease were compared with the respective scores of general population samples (age: 14 - 45 years). Psychological symptoms were measured by means of the Brief Symptom Inventory (BSI; subscales: somatization, obsessive-compulsive thoughts, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, psychoticism). Body image was assessed with the FKB-20 body image questionnaire (subscales: rejection of the body, vitality). The patient group consisted of 361 women and men with congenital heart disease. For comparisons with the BSI, a sample of 1165 subjects was available. Comparisons with the FKB-20 were performed with data from a separate survey (N = 1169). After stratification for age and gender, in females only a few differences were found for both instruments. In males higher scores were obtained for "rejection of the body", and lower ones for "vitality". This does not apply to the highest age group (36 - 45 years). For all BSI-subscales except "depression", "phobic anxiety", and "psychoticism" marked differences between patients and controls were found in males and over all age groups. In women group differences emerged on some subscales (obsessive-compulsive thoughts, interpersonal sensitivity, anxiety, hostility, and phobic anxiety), but this does not hold for all age groups. These results do not apply to patients with the severest impairments due to congenital heart disease, because their number in our study was too low.

Incidence and risk distribution of heart failure in adolescents and adults with congenital heart disease after cardiac surgery.

Heart failure (HF) is a major problem in the long-term follow-up of adults with congenital heart disease (CHD) after cardiac surgery. The purpose of this study was to evaluate risk factors for HF in patients with CHD. N-terminal-pro-brain natriuretic peptide and maximal oxygen uptake (VO2max) were measured in 345 consecutive patients with CHD. HF was defined as an elevated N-terminal-pro-brain natriuretic peptide level (> or = 100 pg/ml) and reduced VO2max (< or = 25 ml/kg/min). The HF criteria were met by 89 patients. These patients were significantly older (mean +/- SEM 30.8 +/- 0.9 vs 24.8 +/- 0.5 years), had significantly lower maximal heart rates (149 +/- 3 vs 164 +/- 1 beats/min), and had larger end-diastolic right ventricular diameters (36 +/- 1 vs 27 +/- 1 mm) and right ventricular pressure estimated by Doppler flow velocities of tricuspid valve regurgitation (2.9 +/- 0.1 vs 2.3 +/- 0.03 m/s). Mean fractional shortening of the left ventricle was within the normal range. To estimate risk stratification, odds ratios for HF were determined for the most frequently occurring types of congenital heart defects and surgical procedures. In conclusion, HF in adults with CHD predominately depends on diagnosis, age, the frequency of reoperation, and right ventricular function and may be related to chronotropic incompetence indicated by lower maximal heart rates.

Elevated brain natriuretic peptide and reduced exercise capacity in adult patients operated on for tetralogy of fallot is due to biventricular dysfunction as determined by the myocardial performance index.

Although tetralogy of Fallot (TOF) can be repaired surgically, residual lesions that cause abnormal ventricular load can eventually lead to heart failure. Subclinical biventricular dysfunction in these patients may be detected only by using suitably sensitive indexes. The Tei index determined by the pulsed Doppler method enables the measurement of left ventricular (LV) and right ventricular (RV) function. This study was designed to evaluate the biventricular Tei indexes in adults with operated congenital heart disease and to correlate these indexes with cardiopulmonary capacity and neurohormonal activation. Fifty-nine patients with surgically corrected TOF and 52 patients with operated left-to-right-shunt defects were included in the study. Patients with TOF showed significantly greater LV and RV Tei indexes than those with left-to-right-shunt defects (LV Tei index 0.50 +/- 0.09 vs 0.34 +/- 0.05, RV Tei index 0.37 +/- 0.1 vs 0.25 +/- 0.06; p <0.0001). Peak oxygen uptake was significantly reduced in the patients with TOF (25 +/- 6 vs 32 +/- 6 ml x kg(-1) x min(-1), p <0.0001) and was correlated inversely with the LV Tei index (r = -0.61, p <0.0001). N-terminal-pro-brain natriuretic peptide was significantly increased in patients with TOF (150 +/- 141 vs 57 +/- 39 pg/ml, p <0.0001). In conclusion, in asymptomatic or minimally symptomatic patients with TOF, biventricular dysfunction is detected by the Tei index. Further indexes for heart failure in these patients are increased circulating plasma N-terminal-pro-brain natriuretic peptide and impaired peak oxygen uptake. The combined determinations of these 3 variables enable the identification of congenital heart disease with impaired cardiac function before they become clinically symptomatic.

The influence of congenital heart disease on psychological conditions in adolescents and adults after corrective surgery.

OBJECTIVE: The present study was designed to examine psychological characteristics of adolescents and adults with operated congenital heart disease (ACHD). Particularly it was to be examined whether cardiological parameters may be associated with subjectively perceived impairments and measures of psychological distress. PATIENTS: A total of 361 men (209) and women (152) between 14 and 45 years underwent medical checkups and an interview on psychological and sociological issues. SETTING: The medical part consisted of a complete cardiological examination including the classification of residual symptoms according to the New York Heart Association (NYHA), and spiroergometry. The Brief Symptom Inventory was used for depicting current psychological and somatic symptoms. These were assessed on 9 subscales: somatization, obsession-compulsion, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, and psychoticism. RESULTS: The analyses revealed statistically significant associations between the degree of NYHA class and psychological symptoms. These findings could not be reproduced for physical fitness as measured by peak oxygen consumption. No gender differences emerged. CONCLUSIONS: Our results suggest that psychological measures of ACHD are not directly dependent on their physical fitness or on the severity of residual symptoms. Instead, patients' subjective appraisal of their disease severity and the conviction to what degree one can depend on the operated heart may be important determinants of psychological states.

Relation of systemic ventricular function quantified by myocardial performance index (Tei) to cardiopulmonary exercise capacity in adults after Mustard procedure for transposition of the great arteries.

After Mustard repair for transposition of the great arteries, the right ventricle serves as a chronically overloaded systemic ventricle (SV). Thus, during long-term follow-up, dysfunction of the right ventricle with consecutive heart failure (HF) is not unusual. Early signs and symptoms of HF are depressed parameters of right ventricular (RV) function at rest and exercise intolerance. It was hypothesized that the measurement of RV function parameters, N-terminal pro-brain natriuretic peptide (NT-pro-BNP), and peak oxygen uptake (VO2max) during exercise testing were suitable for the early detection of subclinical HF. In 33 asymptomatic adolescents and adults who had undergone Mustard repair, RV function was analyzed by the myocardial performance index (Tei index). NT-pro-BNP and VO2max were also determined. The corresponding data from 52 patients operated on for left-to-right shunt defects without residual lesions served as reference data. In patients who underwent the Mustard procedure, the Tei index and NT-pro-BNP were elevated (mean Tei index of the SV 0.63 +/- 0.17 vs 0.34 +/- 0.05, p <0.002; mean NT-pro-BNP 240 +/- 230 vs 57 +/- 39 pg/ml, p <0.0001), and VO2max was reduced (27 +/- 6 vs 32 +/- 6 ml x kg(-1) x min(-1), p <0.002). A good correlation was found between the Tei index and VO2max (r = -0.83, p <0.0001). In conclusion, RV function is depressed in most patients with Mustard repair. Ventricular dysfunction in such asymptomatic or minimally symptomatic patients can be detected by measurement of the Tei index, NT-pro-BNP, and VO2max. These parameters are simple and reliable screening methods to stratify patients with impaired cardiac dysfunction before they become symptomatic.