Philip Moons

Temporal Trends in Survival to Adulthood Among Patients Born With Congenital Heart Disease From 1970 to 1992 in Belgium

Background— Over the past decades, the life expectancy of individuals with congenital heart disease (CHD) has increased significantly. However, precise estimates for survival to adulthood are scarce for patients with CHD. We investigated the proportion of CHD patients born between 1990 and 1992 who survived into adulthood. We also compared their survival with that of CHD patients born in earlier eras and evaluated survival as a function of the type of heart defect. Methods and Results— We reviewed the CHD program administrative and clinical database at the University Hospitals Leuven (Leuven, Belgium) and analyzed the records of 7497 CHD patients born from 1970 to 1992. Survival to 18 years of age in patients born between 1990 and 1992 was 88.6% (95% confidence interval [CI], 86.3% to 90.5%), which was significantly greater than that of patients born in previous decades (P<0.0001). For patients born between 1990 and 1992, survival into adulthood for those with mild heart defects was 98.0% (95% CI, 95.8% to 99.1%), whereas survival for those with moderate- and severe-complexity heart defects was 90.0% (95% CI, 86.8% to 92.5%) and 56.4% (95% CI, 47.4% to 64.5%), respectively. Analysis per heart defect confirmed these findings, demonstrating that patients with univentricular heart (49.1% [95% CI, 30.8% to 65.1%]) and hypoplastic left heart syndrome (7.5% [95% CI, 0.6% to 26.6%]) had the poorest survival rate. Conclusion— This study demonstrates that almost 90% of children with CHD have the prospect of surviving into adulthood.

Predictors of pregnancy complications in women with congenital heart disease

AIMS Data regarding pregnancy outcome in women with congenital heart disease (CHD) are limited. METHODS AND RESULTS In 1802 women with CHD, 1302 completed pregnancies were observed. Independent predictors of cardiac, obstetric, and neonatal complications were calculated using logistic regression. The most prevalent cardiac complications during pregnancy were arrhythmias (4.7%) and heart failure (1.6%). Factors independently associated with maternal cardiac complications were the presence of cyanotic heart disease (corrected/uncorrected) (P < 0.0001), the use of cardiac medication before pregnancy (P < 0.0001), and left heart obstruction (P < 0.0001). New characteristics were mechanical valve replacement (P = 0.0014), and systemic (P = 0.04) or pulmonary atrioventricular valve regurgitation related with the underlying (moderately) complex CHD (P = 0.03). A new risk score for cardiac complications is proposed. The most prevalent obstetric complications were hypertensive complications (12.2%). No correlation of maternal characteristics with adverse obstetric outcome was found. The most prevalent neonatal complications were premature birth (12%), small for gestational age (14%), and mortality (4%). Cyanotic heart disease (corrected/uncorrected) (P = 0.0003), mechanical valve replacement (P = 0.03), maternal smoking (P = 0.007), multiple gestation (P = 0.0014), and the use of cardiac medication (P = 0.0009) correlated with adverse neonatal outcome. CONCLUSION In our tertiary CHD cohort, cardiac, obstetric, and neonatal complications were frequently encountered, and (new) correlations of maternal baseline data with adverse outcome are reported. A new risk score for adverse cardiac complications is proposed, although prospective validation remains necessary.

The effectiveness of inpatient geriatric evaluation and management units: a systematic review and meta-analysis.

OBJECTIVES: To examine how geriatric evaluation and management units (GEMUs) are organized and to examine the effectiveness of admission on a GEMU.

Conversion from Cyclosporine to Tacrolimus Improves Quality of Life Indices, Renal Graft Function and Cardiovascular Risk Profile

Long‐term use of cyclosporine after renal transplantation results in nephrotoxicity and an increased cardiovascular risk profile. Tacrolimus may be more favorable in this respect. In this randomized controlled study in 124 renal transplant patients, the effects of conversion from cyclosporine to tacrolimus on renal function, cardiovascular risk factors, and perceived side‐effects were investigated after a follow‐up of 2 years. After conversion from cyclosporine to tacrolimus renal function remained stable, whereas continuation of cyclosporine was accompanied by a rise in serum creatinine from 142 ± 48 μmol/L to 157 ± 62 μmol/L (p < 0.05 comparing both groups). Conversion to tacrolimus resulted in a sustained reduction in systolic and diastolic blood pressure, and a sustained improvement in the serum lipid profile, leading to a reduction in the Framingham risk score from 5.7 ± 4.3 to 4.8 ± 5.3 (p < 0.05). Finally, conversion to tacrolimus resulted in decreased scores for occurrence of and distress due to side‐effects. In conclusion, conversion from cyclosporine to tacrolimus in stable renal transplant patients is beneficial with respect to renal function, cardiovascular risk profile, and side‐effects. Therefore, for most renal transplant patients tacrolimus will be the drug of choice when long‐term treatment with a calcineurin inhibitor is indicated.

Pregnancy and delivery in women after Fontan palliation.

Objectives: To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders. Design and patients: Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18–45 years): atriopulmonary anastomosis (n  =  23), atrioventricular connection (n  =  5) and total cavopulmonary connection (n  =  10). Results: Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live-birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non-specified secondary infertility (n  =  2), uterus bicornis (n  =  1) and related to endometriosis (n  =  1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n  =  15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population). Conclusion: Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.

Congenital heart disease in 111 225 births in Belgium: Birth prevalence, treatment and survival in the 21st century

Aim: To investigate the birth prevalence, treatment modalities and short‐term survival of children with congenital heart disease who were born in 2002.

What Does it Mean to Live with a Congenital Heart Disease? A Qualitative Study on the Lived Experiences of Adult Patients

Background: Adults with congenital heart disease constitute a relatively new and continuously growing patient population. In addition to medical problems, patients perceive specific psychosocial concerns that influence their lived experiences and quality of life. Aim: This study aimed to explore the lived experiences of adult congenital heart disease patients. Methods: Unstructured, in-depth interviews were performed with 12 patients, aged between 25 and 40 years and suffering from moderate or severe heart defect (tetralogy of Fallot, transposition of the great arteries). Interviews were tape recorded and transcribed verbatim. Data were analyzed using Grounded Theory procedures. Results: Feeling different was the central theme of the patients’ lived experience, as they are faced with physical limitations and visible signs due to the heart defect. The experienced discordance between their world and healthy individuals’ world implies that patients struggle constantly with themselves and with their environment to be accepted as normal. The feeling of being different was influenced by attitudes of the environment, health care, and patients personality. Moreover, it determined the perceived impact of the disease on the patients daily life. Conclusion: This study shows that normalisation is the most important process when dealing with patients suffering from a congenital heart disease.

Why Call it Health-Related Quality of Life When You Mean Perceived Health Status?

Health-related quality of life is a term frequently used in health services and nursing research. However, when using this term, researchers often refer to the self-perceived health status or the functional abilities of the patients. Since there is increasing evidence that quality of life and health status are distinct concepts, the appropriateness of the term ‘health-related quality of life’ is questionable. Therefore, researchers should consider whether health-related quality of life is actually measured if a health-status instrument is used. The scientific community should take up this conceptual discussion in order to make the concept of quality of life less ambiguous.

The effect of bosentan in patients with a failing Fontan circulation.

OBJECTIVES To investigate the effect of bosentan in patients with a failing Fontan circulation. DESIGN A multicentric open label, non-controlled study. SETTING 5 tertiary care centres for congenital cardiology. PATIENTS We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks. MAIN MEASURES OF OUTCOMES: We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded. RESULTS Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued. CONCLUSIONS Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.

Prevalence of cardiovascular risk factors in adults with congenital heart disease

Background Empirical evidence indicates that patients with congenital cardiac anomalies may be prone to developing coronary heart disease. Although primary prevention of ischaemic heart disease in patients with congenital heart defects is important, data on the prevalence of cardiovascular risk factors in these patients are not available. The aims of this study are therefore to describe the prevalence of risk factors for coronary heart disease in a large sample of adults with congenital cardiac anomalies, and compare this with the prevalence in the general population. Design A retrospective analysis of computerized patient records. Methods At our outpatient clinic, all patients are examined by an advanced practice nurse and a congenital heart disease cardiologist. Data on smoking behaviour, sports participation, blood pressure, body mass index, and the diagnosis of diabetes are recorded systematically. Data on the general population were derived from national health surveys. Results In a 4-year period, we collected data on 1976 individual patients. Male patients had a significantly higher prevalence of smoking and elevated blood pressure, whereas women were less engaged in sports activities and were more often obese. In comparison with the general population, our patients reported less smoking and more participation in sports, but presented more often with hypertension or diabetes. Only 20.4% of men and 21.0% of women have a fully heart-healthy lifestyle, as they presented without any risk factor. Conclusion A substantial number of patients had one or more cardiovascular risk factors. Therefore, primary prevention by strengthening educational efforts becomes critically relevant in patients with congenital heart disease, to avoid the additional burden of coronary events in this growing population of patients.