Folkert J. Meijboom

Preoperative Thresholds for Pulmonary Valve Replacement in Patients With Corrected Tetralogy of Fallot Using Cardiovascular Magnetic Resonance

Background— To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. Methods and Results— Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42±10% to 43±10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m2) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: &bgr;=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m2 for normalization of RV end-diastolic volume or 82 mL/m2 for RV end-systolic volume. Conclusions— Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m2 or RV end-systolic volume was <82 mL/m2.

Mortality in adult congenital heart disease

AIMS Mortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear. METHODS AND RESULTS The Dutch CONCOR national registry for adult congenital heart disease was linked to the national mortality registry. Coxs regression was used to assess mortality predictors. Of 6933 patients, 197 (2.8%) died during a follow-up of 24 865 patient-years. Compared with the general national population, there was excess mortality, particularly in the young. Median age at death was 48.8 years. Of all deaths, 77% had a cardiovascular origin; 45% were due to chronic heart failure (26%, age 51.0 years) or sudden death (19%, age 39.1 years). Age predicted mortality, as did gender, severity of defect, number of interventions, and number of complications [hazard ratio (HR) range 1.1-5.9, P < 0.05]. Several complications predicted all-cause mortality beyond the effects of age, gender, and congenital heart disease severity, i.e. endocarditis, supraventricular arrhythmias, ventricular arrhythmias, conduction disturbances, myocardial infarction, and pulmonary hypertension (HR range 1.4-3.1, P < 0.05). These risks were similar in patients above and below 40 years of age. Almost all complications predicted death due to heart failure (HR range 2.0-5.1, P < 0.05); conduction disturbances and pulmonary hypertension predicted sudden death (HR range 2.0-4.7, P < 0.05). CONCLUSION Mortality is increased in adults with congenital heart disease, particularly in the young. The vast majority die from cardiovascular causes. Mortality risk, particularly by heart failure, is increased by virtually all complications. Complications are equally hazardous in younger as in older patients.

The changing epidemiology of congenital heart disease

Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart failure and arrhythmias are the most prominent. Accordingly, these patients need frequent follow-up by physicians with specific knowledge in the field of congenital heart disease. However, planning of care for this population is difficult, because the number of patients currently living with congenital heart disease is difficult to measure. Birth prevalence estimates vary widely according to different studies, and survival rates have not been well recorded. Consequently, the prevalence of congenital heart disease is unclear, with estimates exceeding the number of patients currently seen in cardiology clinics. New developments continue to influence the size of the population of patients with congenital heart disease. Prenatal screening has led to increased rates of termination of pregnancy. Improved management of complications has changed the time and mode of death caused by congenital heart disease. Several genetic and environmental factors have been shown to be involved in the etiology of congenital heart disease, although this knowledge has not yet led to the implementation of preventative measures. In this Review, we give an overview of the etiology, birth prevalence, current prevalence, mortality, and complications of congenital heart disease.

Sudden cardiac death in athletes: the Lausanne Recommendations

Objectives This study reports on sudden cardiac death (SCD) in sport in the literature and aims at achieving a generally acceptable preparticipation screening protocol (PPSP) endorsed by the consensus meeting of the International Olympic Committee (IOC). Background The sudden death of athletes under 35 years engaged in competitive sports is a well-known occurrence; the incidence is higher in athletes (∼ 2/100 000 per year) than in non-athletes (2.5:1), and the cause is cardiovascular in over 90%. Methods A systematic review of the literature identified causes of SCD, sex, age, underlying cardiac disease and the type of sport and PPSP in use. Methods necessary to detect pre-existing cardiac abnormalities are discussed to formulate a PPSP for the Medical Commission of the IOC. Results SCD occurred in 1101 (1966–2004) reported cases in athletes under 35 years, 50% had congenital anatomical heart disease and cardiomyopathies and 10% had early-onset atherosclerotic heart disease. Forty percent occurred in athletes under 18 years, 33% under 16 years; the female/male ratio was 1/9. SCD was reported in almost all sports; most frequently involved were soccer (30%), basketball (25%) and running (15%). The PPSP were of varying quality and content. The IOC consensus meeting accepted the proposed Lausanne Recommendations based on this research and expert opinions (http://multimedia.olympic.org/pdf/en_report_886.pdf). Conclusion SCD occurs more frequently in young athletes, even those under the age of 18 years, than expected and is predominantly caused by pre-existing congenital cardiac abnormalities. Premature atherosclerotic disease forms another important cause in these young adults. A generally acceptable PPSP has been achieved by the IOCs acceptance of the Lausanne Recommendations.

Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. A prospective follow-up study of 21-33 years

AIMS Although studies have suggested good long-term results, arrhythmias, pulmonary hypertension and left ventricular dysfunction are mentioned as sequelae long-term after surgical atrial septal defect closure at young age. Most studies were performed only by questionnaire and in a retrospective manner. The long-term outcome is very important with regard to future employment and acceptance on insurance schemes. METHODS AND RESULTS One hundred and thirty-five consecutive ASD-patients, operated on in childhood, were studied longitudinally with ECG, echocardiography, exercise testing and Holter-recording 15 (10-22) and 26 (21-33) years after surgery. During follow-up no cardiovascular mortality, stroke, heart failure and no pulmonary hypertension occurred. Symptomatic supraventricular tachyarrhythmias were present in 6% after 15 years, and an additional 2% occurred in the last decade; 5% needed pacemaker implantation. No relation was found between arrhythmias and type of ASD, baseline data, right ventricular dimensions, or age at operation. Left and right ventricular function and dimension remained unchanged. Slightly more patients had right atrial dilatation at last follow-up. Exercise capacity was comparable with the normal Dutch population. CONCLUSIONS The long-term outcome after ASD closure at young age shows excellent survival and low morbidity. The incidence of supraventricular arrhythmias is lower than in natural history studies of ASD patients and also lower than after surgical correction at adult age.

Behavioural and emotional problems in children and adolescents with congenital heart disease

Behavioural/emotional problems were assessed at least nine years after surgical correction for congenital heart disease (ConHD) in childhood. Parents of 144 10-15-year-old ConHD-children completed the Child Behavior Checklist (CBCL) and 179, 11-17-year-old, ConHD-adolescents completed the Youth Self-Report (YSR). On the CBCL and YSR ConHD-children and adolescents obtained significantly higher problem scores than same-aged peers from normative reference groups. No significant differences were found between problem scores for different cardiac diagnostic groups. A negative correlation was found between CBCL total problem scores and IQ-scores of ConHD-children; for YSR total problem scores no such relationship was found.

Long-term follow-up (9 to 20 years) after surgical closure of atrial septal defect at a young age

To assess the long-term cardiac status after surgical closure of an atrial septal defect (ASD) at a young age, 104 of 135 children who consecutively underwent surgery (aged 0 to 14 years) at 1 institution between 1968 and 1980 participated in a follow-up study and underwent a complete cardiologic examination. Mean follow-up was 14.5 +/- 2.8 years. Most patients (87%) believed their health to be good or very good. At physical examination, all patients were in good health. Ninety-three patients (89%) were in sinus rhythm. Echocardiography showed that right ventricular dilatation was present in 27 patients (26%), 2 of whom had a residual ASD. Bicycle ergometry revealed that 88 patients (88%) had a normal exercise capacity. Both supraventricular and ventricular arrhythmias were observed in 67% of patients by 24-hour ambulatory electrocardiography, but only 3 (3%) had received antiarrhythmic medication, and 4 (4%) had needed a pacemaker. In the group of patients with right ventricular dilatation, the exercise capacity and prevalence of arrhythmias did not differ significantly from those in the group with a normal sized right ventricule. The outcome in patients with a secundum-type ASD was not different from that of those with a sinus venosus-type ASD. The finding of anatomic, functional or electrophysiologic abnormalities was not associated with a longer duration of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Long-term follow-up after surgical closure of ventricular septal defect in infancy and childhood

OBJECTIVES The purpose of this study was to assess the health-related quality of life of patients who underwent surgical closure of a ventricular septal defect at a young age between 1968 and 1980. BACKGROUND Since the beginning of open heart surgery for congenital cardiac malformations, the surgical techniques have continually improved. As a result, even infants have become eligible for surgical repair. Long-term follow-up data are not available on the health-related quality of life of nonselected patients after surgical repair at a young age. We therefore conducted a follow-up study of 176 infants and children consecutively operated on in one institution between 1968 and 1980. METHODS Patients who were alive and could be traced through the offices of local registrars received an invitation to participate in the follow-up study, consisting of an interview, physical examination, echocardiography, exercise testing and standard 12-lead and 24-h electrocardiography. RESULTS One hundred nine patients (78% of those eligible for follow-up) participated. The mean interval after operation (+/- SD) was 14.5 +/- 2.6 years. Eighty-four percent of the patients assessed their health as good or very good, and 89% had been free of any medical or surgical intervention since the operation. At physical examination all patients were in good health. Their mean exercise capacity was 100 +/- 17% (range 56% to 141%) of predicted values; 84% of the patients had a normal exercise capacity. Echocardiography demonstrated a small residual ventricular septal defect in seven patients (6%). There were no signs of pulmonary hypertension. No patient had symptomatic arrhythmias. CONCLUSIONS Long-term results of surgical closure of ventricular septal defect in infancy and childhood are good. Pulmonary hypertension is absent. Personal health assessment is comparable to that of the normal population, as is exercise capacity, even though many patients have anatomic, hemodynamic or electrophysiologic sequelae.

Psychosocial functioning of the adult with congenital heart disease: a 20–33 years follow-up

AIMS Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups. METHODS AND RESULTS Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results. CONCLUSIONS Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.

The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation.

PURPOSE The prevalence of adult patients with congenital heart disease (CHD) has been reported with a high degree of variability. Prevalence estimates have been calculated using birth rate, birth prevalence, and assumed survival and derived from large administrative databases. To report more robust prevalence estimate, we performed a systematic review for studies concerning CHD prevalence in adults. Moreover, to diminish bias of calculated estimates, we conducted an evidence-based calculation for the Netherlands. METHODS A systematic database search was performed to identify reports on the prevalence of adult CHD. Bicuspid aortic valve, mitral valve prolapse, Marfan syndrome, cardiomyopathy, congenital arrhythmia, and spontaneously closed defects were excluded. In addition, CHD prevalence was calculated using birth rate, birth prevalence, and survival estimates. RESULTS Our search yielded 10 publications on the prevalence of CHD in adults. Four reported results from population wide cross-sectional data, whereas in 6, prevalence was calculated. Mean prevalence reported by empirical studies was 3,562 per million when unspecified lesions were included and 2,297 per million when these were excluded. Mean prevalence derived from calculation was 3,536. Our calculated estimate was 3,228 per million adults. Taking these estimates as well as the limitations inherent to their derivation into consideration, the prevalence of CHD in the adult population is approximately 3,000 per million adults. CONCLUSION This systematic review presents a comprehensive overview of publications on the prevalence of CHD in adults. The best available evidence suggests that overall prevalence of CHD in the adult population is in the region of 3,000 per million.