Exercise systolic blood pressures are of questionable value in the assessment of the adult with a previous coarctation repair.

BACKGROUND: The role of exercise testing in the follow up of adults with a coarctation repair is unclear. Exercise induced systolic hypertension has been advocated as an indication for further investigation; however, the value of exercise testing in this role has been questioned, especially in paediatric populations. OBJECTIVE: To assess the value of resting and exercise blood pressure measurements in a cohort of adults with repaired coarctation. SETTING: Tertiary referral centre for adult congenital cardiac disease. PATIENTS: 56 patients (33 male, 23 female) with a previous coarctation repair, and 33 age matched controls. MAIN OUTCOME MEASURES: Resting and exercise blood pressures, including arm-leg systolic blood pressure gradients; standard echocardiographic measurements of left ventricular mass, aortic root diameter, and repair site gradient. RESULTS: The coarctation cohort had higher resting upper limb blood pressures than the controls (systolic: 129.7 v 120.7 mm Hg, p = 0.014; diastolic: 76.8 v 72.2 mm Hg, p = 0.02). Mean resting arm-leg systolic blood pressure gradient was also higher, at 3.6 v -2.2 mm Hg, p = 0.027. However, there were no differences between the peak exercise systolic blood pressures of the two groups. Peak exercise systolic blood pressure did not correlate with resting arm-leg blood pressure gradient (r = 0.24, p = 0.13) or with repair site gradient (r = 0.14, p = 0.39). Resting upper limb systolic blood pressure and resting arm-leg systolic blood pressure gradient were related to repair site gradient (r = 0.33, p = 0.03, and r = 0.47, p = 0.002). CONCLUSIONS: Measurements of upper limb blood pressure during exercise are of limited value in the assessment of the post-repair coarctation patient. If routine exercise testing is to be advocated in this population it must be for another indication.

Cardiopulmonary exercise performance in adult survivors of the Mustard procedure.

Most patients with the Mustard procedure are now adults. To date, however, there have been few reports on resting and exercise hemodynamics in a large population of adults with this circulation. The aim of this study is to describe such parameters in one of the largest and oldest populations of adults with the Mustard procedure. The database of the University of Toronto Congenital Cardiac Centre for Adults was examined to identify 84 adults with the Mustard procedure who have undergone cardiopulmonary exercise tests. Magnetic resonance imaging and echocardiography studies were obtained in order to assess right ventricular size, function and baseline hemodynamics. Patients achieved lower maximum uptake of oxygen, maximal heart rate, forced vital capacity, forced expiratory volume in 1 second, and oxygen saturations at maximal exercise compared to a healthy population. Magnetic resonance imaging showed significantly different right ventricular ejection fractions between patients and controls. There were no effects of operative variables or preoperative hemodynamics on current exercise capacity. Patients after the Mustard procedure have subnormal exercise capacities. Factors such as chronotropic incompetence, peripheral deconditioning, and impaired lung function may be responsible for these results.

Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure.

Angiotensin-converting enzyme inhibitors had no significant effect on cardiopulmonary exercise function in 14 patients who had undergone a Mustard operation for transposition of the great arteries. In some patients aerobic capacity improved and maximum systolic blood pressure decreased.

Aerobic capacity in adults with various congenital heart diseases.

As an increasing number of patients with congenital heart disease reach adulthood, more information is needed regarding outcomes. The first signs of impaired heart function may appear during exercise testing. The aim of the present study was to establish mean values for maximal oxygen uptake in adults with various congenital heart diseases. Patients from 6 major diagnostic groups were identified, including patients with atrial septal defect (ASD, n = 93), transposition of the great arteries corrected with the Mustard procedure (n = 84), congenitally corrected transposition of the great arteries (CCTGA, n = 41), Tetralogy of Fallot (n = 168), Ebstein's anomaly (n = 37), and Modified Fontan procedure (n = 52). Diminished maximal oxygen uptake was found in all diagnostic groups across age compared with healthy subjects. A significant decrease in maximal oxygen uptake with aging was found in those with ASD (p <0.0001), CCTGA (p = 0.01), and Tetralogy of Fallot (p <0.0001). There was no significant decline, however, in Ebstein's anomaly (p = 0.270), Fontan procedure (p = 0.182), and in the Mustard patients (p = 0.188). All patients achieved significantly lower heart rates than predicted (mean for all groups, p <0.0001). Forced vital capacity values (3.51 L, mean SD +/- 1.02) were lower than predicted values (4.10 L, mean SD +/- 0.90, p <0.0001) for all patients groups except those with ASD. Mean values, however, were within the accepted 20% range of variance. This study showed diminished aerobic capacity in all diagnostic groups when compared with a healthy population. The maximal oxygen uptake values across age groups can be used as reference values in patients with similar diagnoses and as the basis for further research.

Exercise capacity in adult patients with congenitally corrected transposition of the great arteries.

OBJECTIVE: To examine cardiopulmonary values, static lung function, and ejection fraction in adult patients with congenitally corrected transposition of the great arteries (CCTGA). PATIENTS AND METHODS: 41 patients who had undergone static lung function testing and cardiopulmonary exercise tests with measurements of ejection fraction were identified at the Toronto Congenital Cardiac Centre for Adults. RESULTS: Aerobic capacity in patients with CCTGA was severely diminished, varying from 30-50% of the results achieved by healthy subjects. Normal values of right ventricular and left ventricular ejection fraction were found. However, the systemic right ventricular ejection fraction increased by 2% from rest to exercise, as opposed to the expected > 5% increase in a healthy population. The pulmonary left ventricular ejection fraction decreased by 2% at peak exercise. CONCLUSION: Diminished values of heart rate, forced expiratory volume in one second (FEV(1)), forced vital capacity, and systolic blood pressure compared to the predicted values may contribute to the reduced maximal oxygen uptake (VO(2)max) found in patients with CCTGA. In addition, a limited increase in systolic right ventricular ejection fraction and a decrease in pulmonary left ventricle contractility suggest a dysfunction of both ventricles.

Life stories of families with a terminally ill child.

Family units with a terminally ill child have a tendency to withdraw and this isolation may lead to problems in their mental health. A tendency with psychologists, clergy and helpers from other professions is to act as ideal experts on the lives of saddened people. From painful personal experience, this does not seem to enable acquiescence. Therefore, the aim of research on families with terminally ill children, was to explore and describe their lives and to develop an approach to facilitate their families to obtain acquiescence. In this article however, attention will be given to the life-world of families with terminally ill children. The research consists of two phases. In phase one the experiences of four families with terminally ill children are explored and described by means of phenomenological, unstructured, in-depth interviews. In phase two an acquiescence approach, which was designed for educational psychologists to facilitate families with terminally ill children to achieve acquiscence, is described. This approach is based on results from phase one. This article focuses on phase one. In this phase four families were interviewed individually, in the privacy of their homes. The interviews were audiotaped, and were transcribed for the purpose of data gathering. The data was analysed according to Tesch's method and a literature control was performed to verify the results. Guba's model for the validity of qualitative research was used. Five recurrent themes were identified: 1. Families are able to choose their reactions to the crises of having a terminally ill child. 2. When there is a terminally ill child in the family, the family's values change. 3. Acceptance of the circumstances with a terminally ill child, makes life easier. 4. As families with a terminally ill child learn to live every moment to the full, their quality of life improves. 5. As people learn to accept support, their quality of life with a terminally ill child improves. The research indicated that families with terminally ill children move through a lonely and painful process, which is characterised by growth at the end. This growth implies that the life skills mentioned above, were obtained after years of unimaginable suffering. In order to reduce this period of suffering, an acquiescence approach was designed for educational psychologists to facilitate discovery and acceptance regarding the above life skills with family units and thus allow them to achieve acquiescence.

Varicocele is associated with abnormal retention of cytoplasmic droplets by human spermatozoa.

OBJECTIVE: To determine whether varicocele is associated with retention of sperm cytoplasmic droplets in infertile men. DESIGN: Retrospective study. SETTING: University infertility clinic. PATIENT(S): Nonazoospermic men with idiopathic (n = 69) and varicocele-associated infertility (n = 73), and 20 fertile controls presenting for vasectomy. INTERVENTION(S): None. MAIN OUTCOME MEASURES(S): Standard semen parameters and percentage of spermatozoa with cytoplasmic droplets on Papanicolaou smears. RESULT(S): No statistically significant differences were found between the fertile and infertile groups with respect to semen volume. Fertile controls had significantly greater mean percent sperm motility and normal morphology than infertile men. The mean percentage of sperm with residual cytoplasm was statistically significantly different in all three groups. Infertile men with varicocele had the highest percentage of sperm with cytoplasmic droplets, the next highest level being in men with idiopathic infertility and the lowest level in fertile controls (11.7 +/- 1.0, 8.1 +/- 0.9 and 3.2 +/- 0.4%, respectively, P<.0001). CONCLUSION(S): Our data show that idiopathic and even moreso, varicocele-related male infertility are conditions associated with impaired disposal of residual sperm cytoplasm by the testis and/or epididymis. These data provide a possible mechanism for the observed semen abnormalities and reduced fertility potential associated with varicocele and idiopathic male infertility.

Smoking is associated with the retention of cytoplasm by human spermatozoa.

OBJECTIVES: To determine whether cigarette smoking is associated with the abnormal retention of residual sperm cytoplasm in infertile men. METHODS: Semen samples were obtained from 87 consecutive non-azoospermic men with idiopathic infertility (18 smokers and 69 nonsmokers) and from 20 men presenting for vasectomy (fertile controls). Standard semen parameters and the percentage of spermatozoa with residual cytoplasm (on Papanicolaou smears) were recorded. RESULTS: Subject age, semen volume, and sperm density, motility, and morphology were not significantly different between the two groups of infertile men. However, a significant difference was found in the mean +/- SEM percentages of sperm with cytoplasm droplets between smokers and nonsmokers (12.9% +/- 1.7% and 8.1% +/- 0.9%, respectively; P < 0.001). CONCLUSIONS: Our data suggest that cigarette smoking is associated with retention of sperm cytoplasmic droplets in infertile men, a morphologic characteristic associated with impaired sperm function.

Outpatient clinics for adults with congenital heart disease: increasing workload and evolving patterns of referral.

OBJECTIVE: To examine the evolving role of specialised outpatient services for adult patients with congenital heart disease. DESIGN: A retrospective analysis of all patients attending the Toronto Congenital Cardiac Centre for Adults over three corresponding three month periods in 1987, 1992, and 1997. SETTING: A tertiary referral centre. MAIN OUTCOME MEASURES: Patient demographics, residence, medical and surgical history, type and source of referral, and investigations performed. RESULTS: In all, 570 patients were seen at the clinic during these three periods. There was a 44% and a 269% increase in workload between 1987 to 1992 and 1992 to 1997, respectively. There was a steady fall in mean age of patients seen at the clinic with time (38.5, 33.6, and 31.7 years in 1987, 1992, and 1997, respectively, p < 0.001). New referrals from community cardiologists and family physicians increased more in relative terms than did referrals from the Hospital for Sick Children, Toronto (6.7%, 15%, and 37.5%, p = 0.02). There was a steady increase in patients with previous reparative surgery (48.9%, 59.2%, and 69.2%, p < 0.002). The proportion of patients with previous reoperations also increased (2.3%, 10%, and 9.2%, p < 0.01). Echocardiography remained the predominant method of diagnosis. The diagnostic mix did not change with time. CONCLUSIONS: Over the past 10 years there has been a large increase in adults with congenital heart disease requiring and seeking specialised care in a tertiary health centre, with a concomitant evolution of referral patterns. These data may be helpful in planning of similar paediatric and adult cardiac services for this expanding population.

Surgery for partial atrioventricular septal defect in the adult.

BACKGROUND: We sought to determine the clinical profile, operative results, and long-term outcome for adult patients undergoing operations for partial atrioventricular septal defects. METHODS: Between 1976 and 1996, 50 adults (mean age, 36.6 +/- 13.2 years) underwent surgery for partial atrioventricular septal defects. Thirty-nine of them underwent primary repair for a substantial left-to-right shunt (> or =1.8), associated with symptoms in 29. The remaining 11 patients had previous atrioventricular septal defect repair in childhood, but required reoperation as adults for severe left atrioventricular valve regurgitation (6), subaortic (3) or mitral (1) stenosis, and a residual atrial shunt (1). RESULTS: No patients died in hospital. Of the 39 patients first repaired in adulthood, left atrioventricular valve repair was performed in 37, valve replacement in 1, and no repair in 1. In contrast, left atrioventricular valve replacement was necessary in 2 of the 6 adults undergoing reoperation for left atrioventricular valve regurgitation. At 7 years median follow-up, 8 patients have died (2 from noncardiac causes). Of 42 patients alive in 1997, 39 are New York Heart Association class I or II, and 3 were class III (class improved in 81%). Two patients required left atrioventricular valve replacement (1 week and 5 years after repair, respectively) for valvar failure. CONCLUSIONS: Low operative risk and excellent long-term results support repair of partial atrioventricular septal defect in adults.