International Journal of Cardiology 187 (2015) 175–182
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Sports participation in adults with congenital heart disease☆,☆☆ Petra Opić a,b, Elisabeth M.W.J. Utens b, Judith A.A.E. Cuypers a, Maarten Witsenburg a, Annemien van den Bosch a, Ron van Domburg a, Ad J.J.C. Bogers c, Eric Boersma a, Antonio Pelliccia d, Jolien W. Roos-Hesselink a,⁎ a
Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, The Netherlands d Institute of Sports Medicine and Science, Rome, Italy b c
a r t i c l e
i n f o
Article history: Received 9 November 2014 Received in revised form 4 February 2015 Accepted 7 March 2015 Available online 11 March 2015 Keywords: Sport Congenital heart disease Arrhythmia Safety Quality of life
a b s t r a c t Background: It is unclear whether sports participation in adults with repaired congenital heart disease is safe and has benefits. Methods: Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis, Tetralogy of Fallot or Transposition of the Great Arteries in our center between 1968 and 1980 were included, and participated in our longitudinal follow-up study with serial evaluations in 2001 and 2011. At both time points patients filled in questionnaires on sports participation, subjective physical functioning and quality of life. Exercise testing, echocardiogram and 24-hour continuous ambulatory ECG-monitoring were performed in both 2001 and 2011. All clinical events (re-intervention, arrhythmia, heart failure) were prospectively recorded. Results: No relationship was found between practicing sports and the occurrence of sudden death, PVCs or SVTs. Patients with moderate/complex forms of ConHD practiced fewer hours of sports compared with the general Dutch normative population. Patients with both simple and moderate/complex ConHD who practiced sports showed a higher exercise capacity. More favorable subjective physical functioning was found for moderate/ complex patients who practiced sports. Conclusions: Adults with repaired ConHD are less often involved in sports than the Dutch general population. The patients that were engaged in sports show a higher exercise capacity than those who did not. Sports participation in patients with ConHD was not associated with an increased incidence of adverse cardiac events. © 2015 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Since the first surgical techniques for patients with congenital heart disease (ConHD) became available some 55 years ago, virtually every area of medical care has evolved substantially. These improvements led to an increased survival for patients with ConHD, with over 90% of infants reaching adulthood nowadays [1]. Sports participation in adults with ConHD is a relatively new territory and many physicians are having difficulty in advising their patients.
Abbreviations: ASD, Atrial Septal Defect; CBS, Central Bureau of Statistics; CI, confidence interval; ConHD, congenital heart disease; LAS, Linear Analogue Scale; OR, odds ratio; PS, Pulmonary Stenosis; SVT, supraventricular tachycardia; TGA, Transposition of the Great Arteries; ToF, Tetralogy of Fallot; VSD, Ventricular Septal Defect; VT, ventricular tachycardia ☆ All authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation. ☆☆ Grant support: van de Hoop foundation, Dordrecht, The Netherlands. ⁎ Corresponding author at: Erasmus Medical Center, Department of Cardiology, Room Ba-583a, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands. E-mail address: [email protected] (J.W. Roos-Hesselink).
http://dx.doi.org/10.1016/j.ijcard.2015.03.107 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
The first concern is the safety of the patients, and the fear exists that exercise training and competitive sports participation may increase the risk of adverse events, including sudden death. In this field, however, there is a paucity of prospective data and controversial opinions still exist concerning the safety for these patients to be regularly engaged in sports [2,3]. Participation in sports can have beneficial effects on quality of life, as was recently shown in a multicenter randomized controlled trial for adolescents with ConHD, and also for ischemic heart disease and heart failure [4–6]. Moreover, adolescents overall do not achieve the 60 min of recommended daily moderate to vigorous physical activity [7]. The recent RCT showed that practicing sports reduced passive leisure time spending [4]. We therefore planned the present study to investigate the clinical consequences of sports participation in adults with repaired ConHD, with particular emphasis on the occurrence of adverse cardiac events, but also assessing the cardiovascular functional capacity, the patients' physical fitness and quality of life. To this scope, we took advantage of the long-standing follow-up program, which includes consecutive
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2. Methods
administered verbally for patients who had difficulty reading or understanding the written questions. The research protocol was approved by the institutional ethical committee of the Erasmus MC and all patients included in the present analysis provided written informed consent.
2.1. Inclusion criteria
2.3. Questionnaires and normative groups
The original cohort consisted of all consecutive patients who underwent surgical correction for Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD), Pulmonary Stenosis (PS), Tetralogy of Fallot (ToF) or Transposition of the Great Arteries (TGA) between 1968 and 1980 in the Erasmus MC, and were younger than 15 years at the time of surgery. This patient cohort has been investigated in 1991, in 2001, and again in 2011. The baseline characteristics and medical and psychosocial results of these investigations have been reported previously [8–13]. The target population of the third follow-up (conducted in 2011) consisted of the 362 patients who had previously participated in 2001. Of these patients, 10 died (i.e., 6 cardiac-related, 3 unknown, 1 accident), 1 underwent heart transplantation and 22 patients were lost to the follow-up. Of the remaining 329 eligible patients, 245 patients (already examined in 2001) agreed to participate and represent the study population of the current analysis in 2011. The flow-chart describing the plan of the study is shown in Fig. 1. No differences were found in baseline characteristics between the patients who participated again in 2011 and those who did not.
Sports participation was assessed using the same general questionnaire on leisure activities in 2001 and 2011 which is based on the Dutch Central Bureau of Statistics (CBS) [10,14,15]. In addition, in 2011, four items of the “Baecke questionnaire”, widely used for assessing habitual physical activity in epidemiological studies [16] were used. Sports participation was defined as any form of solitary or group physical activities outside of regular walking and cycling throughout the day. In order to make comparison with the general Dutch population possible, the types of sports were classified according to the CBS, which was also used to obtain normative values for the average Dutch population (data retrieved in 2012) [17]. Specifically, the categories “Extensive” (more than 5 h of sports per week), “Little/ Moderate” (in between 1 and 5 h of sports per week) and “None” (up to 1 h or less of sports per week) were used. The physiologic types of sports that patients in our study were practicing (listed in Table 3) were classified according to Mitchell et al. [18]. This classification shows the percentage of patients practicing dynamic and static sports in different intensities. According to the classification adopted at the American Heart Association Task Force on Adults with CHD [19], patients with repaired ASD, VSD and PS were classified as simple ConHD (unless they had complications such as severe ventricular dysfunction), while patients with ToF or TGA (all operated with a Mustard repair) were classified as moderate to complex ConHD. Clinical events were defined as surgical/transcatheter re-intervention, ICD implantation, pacemaker implantation, heart failure or symptomatic and clinically relevant arrhythmia. Arrhythmias (supraventricular tachycardia or ventricular tachycardia) were defined as clinically relevant if anti-arrhythmic medication was needed, cardioversion or catheter-based or surgical ablation had been applied, or hospitalization was necessary. Data on events were collected from patient records, and classified by the first and last authors. Maximal exercise capacity was assessed by bicycle ergometry and was compared with that of normal individuals corrected for age, gender, body height and weight [20]. Gradual increments of workload of 20 Watts per minute were used. Exercise capacity b 85% of the predicted value was considered to be decreased. VO2 max was also measured in the evaluation of 2011, but not in 2001. Subjective physical functioning [21] was assessed by the physical functioning scale of the SF-36. This scale measures the amount of limitation in physical activities due to health problems. Good reliability and validity for the Dutch version of the SF-36 have been reported [22]. Self-perceived quality of life was assessed by the Linear Analogue Scale (LAS) for Quality of Life. The LAS has been proven valid, reliable, and sensitive for the ConHD population [23]. This instrument was not used in 2001 and therefore no historical comparison can be made.
ConHD patients operated at the Erasmus Medical Center between 1968 and 1980, and extensively followed-up every 10 years.
2.2. Assessment procedure All patients were invited to the outpatient clinic for cardiac and psychological examination and were informed of the study project. During the outpatient visit, patients underwent a cardiac examination which consisted of 24-hour Holter monitoring, an electrocardiogram, an echocardiogram and exercise testing. The participation in exercise programs, sports and psychosocial characteristics were assessed by questionnaires, which are described in detail below. The questionnaires were
2.4. Statistical analyses
Fig. 1. Flow-chart of patient inclusion.
Continuous data are presented by means ± SD. In case of a skewed distribution (significant Kolmogorov–Smirnov test or highly skewed histogram by visual inspection), medians and interquartile ranges [IQR] (Q1–Q3) are displayed. The SF36 Physical Functioning scale is analyzed with means ± SD according to its manual [22]. To assess differences between ConHD diagnostic groups (simple versus moderate/ complex), t-tests or Mann–Whitney-U tests were utilized. Longitudinal comparison was assessed by paired sample t-tests or Wilcoxon signedrank tests.
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
Categorical variables are represented by percentages. When comparing categorical variables between ConHD diagnostic groups (simple versus moderate/complex ConHD), Chi-square tests or Fisher exact tests were used. Longitudinal (repeated measures) comparison of categorical variables was analyzed by the McNemar test for 2 × 2 paired tables. Univariate Cox regression was used to assess the association between death and ConHD, and to assess the association between death and practicing sports in 2001. Hazard ratios (HRs) with 95% confidence intervals (CIs) are reported. Univariate logistical regression analyses were applied to assess the association between arrhythmias and sports participation in 2011 (yes/no), adjusted for age, gender, complexity of ConHD, and systemic ventricular function in 2011. We report odds ratios (ORs) and corresponding 95% confidence intervals. Only variables that had a p-value of b0.1 were used in the multivariate regression. Multivariate logistical regression was applied to PVCs (127 cases) adjusting for complexity of congenital heart disease, systemic ventricular function and sports (yes/no), SVTs (69 cases) adjusting for age and gender and VTs (23 cases) adjusting for complexity of congenital heart disease, systemic ventricular function, exercise capacity and sport (yes/no). Two-tailed probability values of b 0.05 were considered statistically significant. The statistical program IBM SPSS Statistics for Mac, Version 20.0 (released 2011) was used for all statistics. Figures were made using GraphPad Prism version 6.0a for Mac, GraphPad Software (released July 18, 2012), La Jolla, California, USA.
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3. Results 3.1. Baseline characteristics in 2001 The baseline characteristics of the patients included in this analysis are shown in Table 1. Patients with simple ConHD who were not practicing sports were smoking more cigarettes per day compared with those who were practicing sports (hours) (20 [14–20] vs. 10 [4–15], p = 0.009), and showed a significantly lower exercise capacity (% predicted of norm) (89.6 ± 21.9 vs. 97.5 ± 15.2, p = 0.008). Patients with moderate/complex ConHD who were not practicing sports in 2001 were significantly older (years) (29.5 [24.2–35.0] vs. 26.7 [23.7–29.3], p = 0.036), had a significantly lower exercise capacity (% predicted of norm) (75.3 ± 17.2 vs. 84.1 ± 21.0, p = 0.047), had a higher average heart rate (beats per minute) (76.0 [72.0–80.0] vs. 71.0 [66.8–77.5], p = 0.021) and showed worse results on subjective physical functioning (QoL score) (95.0 [75.0–100.0] vs. 95.0 [90.0–100.0], p = 0.039) compared with patients who were practicing sports in 2001. 3.2. Mortality or arrhythmias Of the 362 patients evaluated in 2001, 10 patients died and 1 patient underwent heart transplantation over the follow-up period of 10 years. Clinical characteristics of these 11 patients are reported in Table 2. We found an association between the presence of moderate/complex ConHD and the occurrence of death (HR [95% CI]: 4.3 [1.1–16.6], p =
Table 1 Patient characteristics in 2001. Total
Age in 2001 (years) Age at first surgery Gender Male Female Smoking Yes Amount/day SYSVF Good Mild Moderate Bad Exercise capacity Holter data Average beats per minute SVT PVC N 10 complexes VT Medication Any medication Aspirin Calcium antagonist Betablocker Nitrate Anti-arrhythmics Digitalis Diuretics ACE inhibitor Cholesterol lowering Oral anticoagulation Physical Functioning
Simple ConHD
p
No sports
Sports
n = 245
n = 74
n = 91
29.7 [26.5–34.8] 4.6 [1.1–1.3]
31.6 [28.0–36.1] 5.5 [1.8–8.8]
30.1 [27.7–35.2] 5.7 [2.1–8.9]
0.161 0.814
52.2 (128) 47.8 (117)
45.9 (34) 54.1 (40)
51.6 (47) 48.4 (44)
22.6 (55) 10 [5–20]
27.0 (20) 20 [14–20]
80.8 (189) 9.8 (23) 6.0 (14) 3.4 (8) 79.1 ± 19.3
Moderate/complex ConHD
p
No sports
Sports
n = 45
n = 35
29.5 [24.2–35.0] 3.2 [0.7–6.5]
26.7 [23.7–29.3] 1.2 [0.5–3.1]
0.036 0.059
0.466
62.2 (28) 37.8 (17)
54.3 (19) 45.7 (16)
0.474
18.9 (17) 10 [4–15]
0.215 0.009
20.5 (9) 4 [3–12]
25.7 (9) 7 [3–15]
0.580 0.478
90.1 (64) 8.5 (6) 1.4 (1) 0.0 (0) 89.6 ± 21.9
94.1 (80) 4.7 (4) 1.2 (1) 0.0 (0) 97.5 ± 15.2
0.628
55.9 (19) 20.6 (7) 14.7 (5) 8.8 (3) 84.1 ± 21.0
0.866
0.008
59.1 (26) 13.6 (6) 15.9 (7) 11.4 (5) 75.3 ± 17.2
75.0 [69.0–81.0] 0.4 (1) 0.0 (0) 4.5 (11)
76.0 [70.0–82.2] 0.0 (0) 0.0 (0) 2.7 (2)
76.5 [69.0–81.8] 0.0 (0) 0.0 (0) 2.2 (2)
0.624 – – 1.000
76.0 [72.0–80.0] 2.2 (1) 0.0 (0) 8.9 (4)
71.0 [66.8–77.5] 0.0 (0) 0.0 (0) 8.6 (3)
0.021 1.000 – 1.000
4.9 (10) 0.5 (1) 0.0 (0) 1.0 (2) 0.0 (0) 0.5 (1) 2.0 (4) 0.0 (0) 2.5 (5) 0.0 (0) 0.5 (1) 95.0 [90.0–100.0]
96.8 (61) 1.6 (1) 0.0 (0) 0.0 (0) 0.0 (0) 0.0 (0) 1.6 (1) 0.0 (0) 1.6 (1) 0.0 (0) 0.0 (0) 95.0 [90.0–100.0]
97.4 (74) 0.0 (0) 0.0 (0) 1.3 (1) 0.0 (0) 0.0 (0) 0.0 (0) 0.0 (0) 1.3 (1) 0.0 (0) 0.0 (0) 100.0 [93.8–100.0]
1.000 0.453 – 1.000 – – 0.453 – 1.000 – – 0.152
94.1 (32) 0.0 (0) 0.0 (0) 0.0 (0) 0.0 (0) 2.9 (1) 5.9 (2) 0.0 (0) 2.9 (1) 0.0 (0) 2.9 (1) 95.0 [75.0–100.0]
87.1 (27) 0.0 (0) 0.0 (0) 3.2 (1) 0.0 (0) 0.0 (0) 3.2 (1) 0.0 (0) 6.5 (2) 0.0 (0) 0.0 (0) 95.0 [90.0–100.0]
0.413 – – 0.477 – 1.000 1.000 – 0.602 – 0.475 0.039
0.047
Abbreviations: Complex ConHD = Tetralogy of Fallot (ToF) & Transposition of the Great Arteries (TGA); ConHD = congenital heart disease; exercise capacity is expressed in % of expected workload; NYHA = New York Heart Association; PVC = premature ventricular complex; Simple ConHD = Atrial Septal Defect (ASD) & Ventricular Septal Defect (VSD) & Pulmonary Stenosis (PS); SVT = supraventricular tachycardia; SYSVF = systemic ventricular function; VT = ventricular tachycardia; data are expressed as proportion and (actual number). Holter data has been recorded for 24 h, and amount of SVT, PVC and VTs reported is per 24 h.
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Table 2 Data on patients that died between 2001 and 2011. Pulmonary valve
Arrhythmias
No regurgitation No regurgitation Severe regurgitation N/A
No regurgitation
Heart failure, cardiac arrest Untreatable VF by ICD Directly after jogging
Endocardial VVI since 1990 Bad (no ICD) SR Moderately reduced Epicardial VVI since 1996 Bad (ICD) Endocardial DDD since Mildly 1981 (no ICD) reduced
No regurgitation
Sick-sinus syndrome No
Moderate regurgitation Severe regurgitation
Sick-sinus syndrome Sick-sinus syndrome
Unknown
Unknown
SR
Good
No regurgitation
No
0–1
Tennis
During tennis
SR
0–1
Hiking, walking
Heart failure
27
No
0–1
Surfing
VF
N/A
No
1968
27
No
5+
Unknown
32
No
5+
Light regurgitation N/A
No
1980
Fitness, rowing, running Fitness, soccer
SR RBTB SR RBTB SR RBTB SR RBTB
Moderate regurgitation No regurgitation
No
No
Mildly reduced Good
Light regurgitation N/A
Surgery BMI Hypertension Hours of Type of sport sports/week
Cause of death
ECG + pacemaker
51
F
Fallot
1968
18
No
0
No
Heart failure
28
M
VSD
1980
18
No
0
No
41
M
1973
21
No
0
No
41
M
TGA (Mustard) Fallot
1988
32
No
1–5
Jogging
45
F
ASD
1972
25
Yes
1–5
33
M
1974
28
No
32
F
TGA (Mustard) Fallot
1974
19
28
M
Fallot
1978
52
M
VSD
32
M
Fallot
Shot to death
Systemic ventricular function
Good Good Mildly reduced
No regurgitation No regurgitation Light regurgitation Light regurgitation
Other
No pulmonary homograft because of high surgery risk
Mild baffle stenosis
No
No
Abbreviations: ASD = Atrial Septal Defect; AV-valve = Atrioventricular valve; BMI = Body Mass Index; ConHD = congenital heart disease; ECG = electrocardiogram; Fallot = Tetralogy of Fallot; ICD = Implantable Cardioverter Defibrillator; N/A = not available; RBTB = Right Ventricular Bundle Branch Block; TGA = Transposition of the Great Arteries (all Mustard surgery); VF = Ventricular Fibrillation; VSD = Ventricular Septal Defect. Hours of sports are expressed in hours per week.
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
Systemic AV-valve
Age Gender ConHD
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
0.036). We found no relationship between practicing sports and the occurrence of death (HR [95% CI]: 0.6 [0.2–2.3], p = 0.482). The changes in sports participation in patients in the period from 2001 to 2011 are shown in Fig. 2. We also did not find a relationship between sports participation and the occurrence of PVCs (OR [95% CI]: 0.3 [0.1–1.05], p = 0.06), SVTs (OR [95% CI]: 0.9 [0.5–1.5], p = 0.572) or the average heart rate (R2 = .008, F(1,224) = 1.8, p = 0.181). There was a negative association between sports participation and the occurrence of VTs (OR [95% CI]: 0.3 [0.1–0.7], p = 0.010); however, this association disappeared after adjustment for potential confounders (OR [95% CI]: 0.5 [0.2–1.4], p = 0.169).
3.3. Characteristics of sports participation in patients with ConHD in 2011 The types of sports participated by patients in 2011 are listed in Table 3. More than half of the patients were involved in high-dynamic sports (57.9%). Fig. 3 shows sports participation in patients with ConHD in 2011 compared with normative data derived from the general population. Specifically, patients with moderate/complex ConHD practiced fewer hours of sports in comparison with the normal population. Both simple and moderate/complex ConHD patients were underrepresented in the ‘Extensive’ sports category compared with the general Dutch population. Fig. 4 shows that patients who practiced sports had a significantly higher exercise capacity compared with patients who did not practice any sport.
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Table 4 shows that patients with simple ConHD who were not practicing sports had a worse exercise capacity than patients who were practicing sports (86.6 ± 18.2 vs. 96.8 ± 19.2, p = 0.001). The same influence was observed for moderate/complex patients (75.9 ± 19.3 vs. 90.1 ± 19.2, p = 0.003). The total sports group showed a lower incidence of ventricular tachycardia (14.5% vs. 4.7%, p = 0.008, not in the table) and fewer premature ventricular complexes (64.2% vs. 49.2%, p = 0.023, not in the table). In addition, patients with moderate/complex ConHD who were not practicing sports showed a worse exercise capacity compared with those who were practicing sports (90.0 [75.0–95.0] vs. 95.0 [89.7–100.0], p-value = 0.001). Patients with moderate/complex ConHD who were practicing sports showed a significantly higher Physical Functioning compared with patients who did not practice sports (95.0 [89.7–100.0] vs. 90.0 [75.0–95.0], p = 0.001). No differences were found on quality of life (according to the LAS instrument). 3.4. Clinical consequences of sports participation over time Over the period of 10 years, 22.5% of the patients showed deterioration in systemic ventricular function, while 69.6% remained stable. In 7.9% of the patients, an improvement in systemic ventricular function was observed. The patients who practiced sports, more often remained stable (77.0% vs. 61.0%, overall p-value = 0.031). When looking at subjective physical functioning over time, deterioration was seen in the group of patients that stopped practicing sports between 2001 and 2011 (95.0 [85.0–100.0] vs. 92.5 [75.0–100.0], p = 0.035). Systemic ventricular function did not deteriorate in patients who stopped practicing sports over time compared with patients who remained practicing sports (20.9% vs. 30.6%, p = 0.276). These influences were observed only when the data were analyzed for the overall patient's cohort, and were lost when looking separately at simple and moderate/complex ConHD. 3.5. Major complications between 2001 and 2011 Table 5 lists the major complications that occurred between 2001 and 2011. In total, 46 patients had only events before the first assessment in 2001. These patients were excluded from analysis. A total of 26 patients had one or more major cardiac events. There were no significant differences between patients who did or did not practice sports. 4. Discussion Our study shows that sports participation in adults with ConHD has a positive influence on exercise capacity, both in patients with simple and moderate/complex ConHD, without conveying major negative consequences such as death or arrhythmias. In the patients with moderate/ complex ConHD a positive influence on subjective physical function was also observed. 4.1. Sports participation in patients with ConHD
Fig. 2. Flowchart of sports participation between 2001 and 2011. Abbreviations: ConHD = congenital heart disease. The category “No sports” contains patients who never practiced any sports and patients who stopped practicing sports between 2001 and 2011. The category “Sports” contains patients who remained practicing sports between 2001 and 2011, and patients who started practicing sports between 2001 and 2011.
Almost half of the adults with ConHD included in the present analysis were engaged in sports. Compared with the general Dutch population, the patients with ConHD, and especially those with moderate/ complex ConHD, practice sports for fewer hours per week. Our findings are in line with two previous studies [24,25], and in contrast only with Immer et al. who unexpectedly reported a higher rate of sports participation in patients with simple and complex forms of ConHD compared with the general population [26]. Patients with more complex forms of ConHD are known to have a reduced exercise capacity [27]. This finding is the most logic explanation why complex patients (as shown in our study) are less often involved in sports. They are probably limited in
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Increasing static component
Table 3 Type of sports participation of ConHD patients in 2011.
High 30.9%
23.0%
1.3%
6.6%
Moderate 43.4%
4.6%
3.3%
35.5%
Low 25.7%
9.2%
0.7%
15.8%
Low 36.8%
Moderate 5.3%
High 57.9%
Increasing dynamic component Data is for total ConHD group. Table based on the study of Mitchell et al. [18].
their possibility to sport, based on their reduced physical capacities. However, it has been shown that children and adolescents with ConHD were able to improve their peak oxygen uptake with an exercise program, so the depressed exercise capacity may partially be explained by the absolute lack of exercise, mostly based on fear for complications or negative impact on cardiac function [6,24,25,27–30]. We found that practicing sports did increase exercise capacity in patients with ConHD, including those with the more complex lesions. Indeed, we found not only a positive influence on exercise capacity but also on subjective physical functioning, including the patients with more complex ConHD. Previous studies have shown ambiguous results, with some studies reporting that an active lifestyle in ConHD patients has a positive influence on exercise capacity or peak oxygen uptake and perceived physical functioning [6,25,31,32], while other studies found no relationship [29,33].
regarding the safety of sports participation and explains the implementation of strict regulations [34]. On the other hand, many patients with moderate/complex ConHD can and do participate in sports without major problems and can even reach the highest level of achievement. For instance, Shaun White, also operated for Tetralogy of Fallot won Olympic gold twice on snowboarding [35]. Proper studies on safety are lacking and are difficult to perform. In our prospective observational cohort, we found no significant relation between sports participation and sudden cardiac death or other adverse events such as ominous arrhythmias. Moreover, we found less ventricular ectopic beats and short VTs on Holter ECG monitoring in patients that were actively engaged in sports and a trend for a favorable influence on ventricular function. Of course, there may be a bias selection in sports, usually compromising the individuals in better conditions, but nevertheless these patients seem not to be at higher risk. We found high scores on subjective physical functioning in patients with ConHD, with the possible consequence of overestimating their physical capabilities. This might indeed imply that they might choose a sport that can potentially be harmful. In our study, over 50% was engaged in high-dynamic sports and a small subset of patients (6.6%) practiced sports both at the highest static and dynamic level. Despite participation in these demanding sports, we found no negative influence on ventricular function or clinical events. Our study supports the concept that sports participation in patients with Con HD can be approached with more confidence and may imply more beneficial consequences than believed in the past. Our findings open also the question of the appropriateness of the current restrictive guidelines regarding sports participation in these patients [36].
4.3. Recommendations
The results on safety in this article should be interpreted with caution due to the small sample size. The rare case of a young man with an operated Tetralogy of Fallot dying suddenly at the sports field is a dramatic and emotional event that raises a number of questions
Although our study shows no overall increased risk of cardiac complications and death, recommendation for practicing sports should take into account the unique medical history of the patient. Advice on the type of sport should be based on the personal situation and anticipated effort levels, and if needed can be supervised by a physician first [6,37,38]. In a recent publication, Budts et al. have published a 6-step evaluation in order to be able to advise patients for a high, moderate or low intensity sport [39]. Takken et al. have published guidelines for children with ConHD on physical activity, recreational sports and exercise training [36]. Solid programs of sports participation with medical supervision as described successfully for patients with heart failure may be necessary also for our (adult) patients with ConHD.
Fig. 3. Sports participation in 2011 versus normative data. Abbreviations: Extensive = 5 or more hours per week; Little/moderate = 1 to 5 hours per week; more than 5 h of sports per week; None = Up to 1 h or less of sports per week. Categories are based on normative data derived from the Dutch Central Bureau of statistics [15].
Fig. 4. Relationship between exercise capacity and hours of sports practiced. Abbreviations: Extensive = 5+ hours of sports per week; Little/Moderate = 1 to 5 h of sports per week; None = up to 1 h or less of sports per week. The X-axis represents the hours of sports practiced in 2011. On the Y-axis the exercise capacity is shown. Practicing N5 h/week sports per week or more results in a better exercise capacity.
4.2. Safety
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Table 4 Results in 2011. 2011 Total
Exercise capacity SYSVF Good Mild Moderate Bad Holter data Average beats per minute PVC N 10 complexes SVT VT Physical Functioning LAS
Simple ConHD
p
No sports
Sports
n = 245
n = 74
n = 91
89.2 ± 20.3
86.6 ± 18.2
96.8 ± 19.2
0.001
66.1 (156) 22.9 (54) 7.6 (18) 3.4 (8)
82.8 (53) 15.6 (10) 0.0 (0) 1.6 (1)
86.0 (80) 12.9 (12) 1.1 (1) 0.0 (0)
73.0 [68.0–79.0] 56.2 (127) 28.2 (69) 9.4 (23) 95.0 [85.0–100.0] 80.0 [75.0–88.0]
74.0 [69.0–79.3] 51.6 (32) 25.4 (18) 7.0 (5) 95.0 [80.0–100.0] 80.0 [74.0–85.0]
72.0 [68.0–79.0] 44.4 (40) 30.9 (29) 2.1 (2) 100.0 [90.0–100.0] 80.5 [75.0–90.0]
Moderate/complex ConHD
p
No sports
Sports
n = 45
n = 35
75.9 ± 19.3
90.1 ± 19.2
0.003
0.564
24.4 (11) 48.9 (22) 15.6 (7) 11.1 (5)
35.3 (12) 29.4 (10) 29.4 (10) 5.9 (2)
0.173
0.491 0.384 0.438 0.121 0.122 0.062
73.5 [69.3–80.8] 81.8 (36) 34.8 (16) 26.1 (12) 90.0 [75.0–95.0] 80.0 [74.3–85.0]
74.0 [65.0–78.0] 63.3 (19) 17.6 (6) 11.8 (4) 95.0 [89.7–100.0] 83.0 [75.0–89.0]
0.352 0.074 0.090 0.113 0.001 0.105
Abbreviations: Complex ConHD = Tetralogy of Fallot (ToF) & Transposition of the Great Arteries (TGA); ConHD = congenital heart disease; exercise capacity is expressed in % of expected workload; LAS = Linear Analogue Scale; PVC = premature ventricular complex; Simple ConHD = Atrial Septal Defect (ASD) & Ventricular Septal Defect (VSD) & Pulmonary Stenosis (PS); SVT = supra-ventricular tachycardia; SYSVF = systemic ventricular function; VT = ventricular tachycardia. Data are expressed as n (%). Holter data has been recorded for 24 h, and amount of SVT, PVC and VTs reported is per 24 h.
4.4. Limitations of the study Although this is the first report based on longitudinal data, no firm conclusions on safety can be drawn due to the relatively small size of the patient population. Larger series and longer follow-up are clearly warranted to consolidate our positive experiences regarding the low mortality and better ventricular function associated with sports participation. Our findings open also the discussion regarding less conservative rules regarding sports participation in these patients. The patients included in this study had one of the following diagnoses: ASD, VSD, PS, ToF or TGA, therefore, the obtained results may not be applicable to all forms of ConHD. The results of the safety of sports from the logistic regression (mortality, heart frequency and arrhythmias) should be drawn with caution since our data is based on an observational cohort and selection bias may exist. Patients participating in sports may have been in better health condition at baseline. We tried to avoid this bias by dividing the group into patients with simple and moderate/ complex heart disease, but still a bias cannot be ruled out. Also, the study design in 2001 did not anticipate these analyses on sports behavior in 2011. Therefore, patients were not instructed in 2001 to write down the dates in which they changed or stopped to practice sports, nor did they wear an accelerometer to objectify duration and intensity of sports participation. Therefore, in the “No sports” category patients
who did practice sports for a certain amount of hours prior to the assessment in 2011 could have been included. Lastly, a questionnaire on sports participation (without objective measurement) unfortunately does not always reflect reality. Furthermore, duration of sports does not equal intensity. Outcome on these questionnaires should be interpreted bearing this in mind. 5. Conclusion The results of this study suggest that sports participation in patients with congenital heart disease does not increase the risk of arrhythmias or sudden cardiac death. Patients with congenital heart disease who participate in sports have better subjective and objective (physical) functioning than those who do not. Funding This study was supported by the “van de Hoop” foundation, which is a non-profit organization that supports a wide scale of medical research. Conflict of interests No conflict of interests reported.
Table 5 Clinical events between 2001 and 2011. Major events between 2001 and 2011 Total
Major events Overall Arrhythmia Pacemaker implantation Surgical reintervention ICD implantation Heart failure
Simple ConHD
p
Moderate/complex ConHD
No sports
Sports
No sports
Sports
n = 245
n = 74
n = 91
n = 45
n = 35
10.6 (26) 6.5 (16) 2.9 (7) 2.4 (6) 1.6 (4) 1.2 (3)
8.5 (6) 5.6 (4) 2.8 (2) 2.8 (2) 1.4 (1) 0.0 (0)
5.3 (5) 4.3 (4) 0.0 (0) 0.0 (0) 1.1 (1) 0.0 (0)
19.6 (9) 10.9 (5) 8.7 (4) 4.3 (2) 4.3 (2) 6.5 (3)
17.6 (6) 8.8 (3) 2.9 (1) 5.9 (2) 0.0 (0) 0.0 (0)
0.425 0.726 0.184 0.184 1.000 –
p
0.828 1.000 0.388 1.000 0.505 0.258
Abbreviations: Complex ConHD = Tetralogy of Fallot (ToF) & Transposition of the Great Arteries (TGA); ConHD = congenital heart disease; ICD = Implantable Cardioverter Defibrillator; Simple ConHD = Atrial Septal Defect (ASD) & Ventricular Septal Defect (VSD) & Pulmonary Stenosis (PS). Data are expressed as n (%); one person can have multiple major complications.
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Acknowledgments The authors gratefully thank Bob Rigter, for the valuable assistance in the preparation of this manuscript.
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Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Sports participation in adults with congenital heart disease☆,☆☆ Petra Opić a,b, Elisabeth M.W.J. Utens b, Judith A.A.E. Cuypers a, Maarten Witsenburg a, Annemien van den Bosch a, Ron van Domburg a, Ad J.J.C. Bogers c, Eric Boersma a, Antonio Pelliccia d, Jolien W. Roos-Hesselink a,⁎ a
Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, The Netherlands d Institute of Sports Medicine and Science, Rome, Italy b c
a r t i c l e
i n f o
Article history: Received 9 November 2014 Received in revised form 4 February 2015 Accepted 7 March 2015 Available online 11 March 2015 Keywords: Sport Congenital heart disease Arrhythmia Safety Quality of life
a b s t r a c t Background: It is unclear whether sports participation in adults with repaired congenital heart disease is safe and has benefits. Methods: Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis, Tetralogy of Fallot or Transposition of the Great Arteries in our center between 1968 and 1980 were included, and participated in our longitudinal follow-up study with serial evaluations in 2001 and 2011. At both time points patients filled in questionnaires on sports participation, subjective physical functioning and quality of life. Exercise testing, echocardiogram and 24-hour continuous ambulatory ECG-monitoring were performed in both 2001 and 2011. All clinical events (re-intervention, arrhythmia, heart failure) were prospectively recorded. Results: No relationship was found between practicing sports and the occurrence of sudden death, PVCs or SVTs. Patients with moderate/complex forms of ConHD practiced fewer hours of sports compared with the general Dutch normative population. Patients with both simple and moderate/complex ConHD who practiced sports showed a higher exercise capacity. More favorable subjective physical functioning was found for moderate/ complex patients who practiced sports. Conclusions: Adults with repaired ConHD are less often involved in sports than the Dutch general population. The patients that were engaged in sports show a higher exercise capacity than those who did not. Sports participation in patients with ConHD was not associated with an increased incidence of adverse cardiac events. © 2015 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Since the first surgical techniques for patients with congenital heart disease (ConHD) became available some 55 years ago, virtually every area of medical care has evolved substantially. These improvements led to an increased survival for patients with ConHD, with over 90% of infants reaching adulthood nowadays [1]. Sports participation in adults with ConHD is a relatively new territory and many physicians are having difficulty in advising their patients.
Abbreviations: ASD, Atrial Septal Defect; CBS, Central Bureau of Statistics; CI, confidence interval; ConHD, congenital heart disease; LAS, Linear Analogue Scale; OR, odds ratio; PS, Pulmonary Stenosis; SVT, supraventricular tachycardia; TGA, Transposition of the Great Arteries; ToF, Tetralogy of Fallot; VSD, Ventricular Septal Defect; VT, ventricular tachycardia ☆ All authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation. ☆☆ Grant support: van de Hoop foundation, Dordrecht, The Netherlands. ⁎ Corresponding author at: Erasmus Medical Center, Department of Cardiology, Room Ba-583a, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands. E-mail address: [email protected] (J.W. Roos-Hesselink).
http://dx.doi.org/10.1016/j.ijcard.2015.03.107 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
The first concern is the safety of the patients, and the fear exists that exercise training and competitive sports participation may increase the risk of adverse events, including sudden death. In this field, however, there is a paucity of prospective data and controversial opinions still exist concerning the safety for these patients to be regularly engaged in sports [2,3]. Participation in sports can have beneficial effects on quality of life, as was recently shown in a multicenter randomized controlled trial for adolescents with ConHD, and also for ischemic heart disease and heart failure [4–6]. Moreover, adolescents overall do not achieve the 60 min of recommended daily moderate to vigorous physical activity [7]. The recent RCT showed that practicing sports reduced passive leisure time spending [4]. We therefore planned the present study to investigate the clinical consequences of sports participation in adults with repaired ConHD, with particular emphasis on the occurrence of adverse cardiac events, but also assessing the cardiovascular functional capacity, the patients' physical fitness and quality of life. To this scope, we took advantage of the long-standing follow-up program, which includes consecutive
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2. Methods
administered verbally for patients who had difficulty reading or understanding the written questions. The research protocol was approved by the institutional ethical committee of the Erasmus MC and all patients included in the present analysis provided written informed consent.
2.1. Inclusion criteria
2.3. Questionnaires and normative groups
The original cohort consisted of all consecutive patients who underwent surgical correction for Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD), Pulmonary Stenosis (PS), Tetralogy of Fallot (ToF) or Transposition of the Great Arteries (TGA) between 1968 and 1980 in the Erasmus MC, and were younger than 15 years at the time of surgery. This patient cohort has been investigated in 1991, in 2001, and again in 2011. The baseline characteristics and medical and psychosocial results of these investigations have been reported previously [8–13]. The target population of the third follow-up (conducted in 2011) consisted of the 362 patients who had previously participated in 2001. Of these patients, 10 died (i.e., 6 cardiac-related, 3 unknown, 1 accident), 1 underwent heart transplantation and 22 patients were lost to the follow-up. Of the remaining 329 eligible patients, 245 patients (already examined in 2001) agreed to participate and represent the study population of the current analysis in 2011. The flow-chart describing the plan of the study is shown in Fig. 1. No differences were found in baseline characteristics between the patients who participated again in 2011 and those who did not.
Sports participation was assessed using the same general questionnaire on leisure activities in 2001 and 2011 which is based on the Dutch Central Bureau of Statistics (CBS) [10,14,15]. In addition, in 2011, four items of the “Baecke questionnaire”, widely used for assessing habitual physical activity in epidemiological studies [16] were used. Sports participation was defined as any form of solitary or group physical activities outside of regular walking and cycling throughout the day. In order to make comparison with the general Dutch population possible, the types of sports were classified according to the CBS, which was also used to obtain normative values for the average Dutch population (data retrieved in 2012) [17]. Specifically, the categories “Extensive” (more than 5 h of sports per week), “Little/ Moderate” (in between 1 and 5 h of sports per week) and “None” (up to 1 h or less of sports per week) were used. The physiologic types of sports that patients in our study were practicing (listed in Table 3) were classified according to Mitchell et al. [18]. This classification shows the percentage of patients practicing dynamic and static sports in different intensities. According to the classification adopted at the American Heart Association Task Force on Adults with CHD [19], patients with repaired ASD, VSD and PS were classified as simple ConHD (unless they had complications such as severe ventricular dysfunction), while patients with ToF or TGA (all operated with a Mustard repair) were classified as moderate to complex ConHD. Clinical events were defined as surgical/transcatheter re-intervention, ICD implantation, pacemaker implantation, heart failure or symptomatic and clinically relevant arrhythmia. Arrhythmias (supraventricular tachycardia or ventricular tachycardia) were defined as clinically relevant if anti-arrhythmic medication was needed, cardioversion or catheter-based or surgical ablation had been applied, or hospitalization was necessary. Data on events were collected from patient records, and classified by the first and last authors. Maximal exercise capacity was assessed by bicycle ergometry and was compared with that of normal individuals corrected for age, gender, body height and weight [20]. Gradual increments of workload of 20 Watts per minute were used. Exercise capacity b 85% of the predicted value was considered to be decreased. VO2 max was also measured in the evaluation of 2011, but not in 2001. Subjective physical functioning [21] was assessed by the physical functioning scale of the SF-36. This scale measures the amount of limitation in physical activities due to health problems. Good reliability and validity for the Dutch version of the SF-36 have been reported [22]. Self-perceived quality of life was assessed by the Linear Analogue Scale (LAS) for Quality of Life. The LAS has been proven valid, reliable, and sensitive for the ConHD population [23]. This instrument was not used in 2001 and therefore no historical comparison can be made.
ConHD patients operated at the Erasmus Medical Center between 1968 and 1980, and extensively followed-up every 10 years.
2.2. Assessment procedure All patients were invited to the outpatient clinic for cardiac and psychological examination and were informed of the study project. During the outpatient visit, patients underwent a cardiac examination which consisted of 24-hour Holter monitoring, an electrocardiogram, an echocardiogram and exercise testing. The participation in exercise programs, sports and psychosocial characteristics were assessed by questionnaires, which are described in detail below. The questionnaires were
2.4. Statistical analyses
Fig. 1. Flow-chart of patient inclusion.
Continuous data are presented by means ± SD. In case of a skewed distribution (significant Kolmogorov–Smirnov test or highly skewed histogram by visual inspection), medians and interquartile ranges [IQR] (Q1–Q3) are displayed. The SF36 Physical Functioning scale is analyzed with means ± SD according to its manual [22]. To assess differences between ConHD diagnostic groups (simple versus moderate/ complex), t-tests or Mann–Whitney-U tests were utilized. Longitudinal comparison was assessed by paired sample t-tests or Wilcoxon signedrank tests.
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
Categorical variables are represented by percentages. When comparing categorical variables between ConHD diagnostic groups (simple versus moderate/complex ConHD), Chi-square tests or Fisher exact tests were used. Longitudinal (repeated measures) comparison of categorical variables was analyzed by the McNemar test for 2 × 2 paired tables. Univariate Cox regression was used to assess the association between death and ConHD, and to assess the association between death and practicing sports in 2001. Hazard ratios (HRs) with 95% confidence intervals (CIs) are reported. Univariate logistical regression analyses were applied to assess the association between arrhythmias and sports participation in 2011 (yes/no), adjusted for age, gender, complexity of ConHD, and systemic ventricular function in 2011. We report odds ratios (ORs) and corresponding 95% confidence intervals. Only variables that had a p-value of b0.1 were used in the multivariate regression. Multivariate logistical regression was applied to PVCs (127 cases) adjusting for complexity of congenital heart disease, systemic ventricular function and sports (yes/no), SVTs (69 cases) adjusting for age and gender and VTs (23 cases) adjusting for complexity of congenital heart disease, systemic ventricular function, exercise capacity and sport (yes/no). Two-tailed probability values of b 0.05 were considered statistically significant. The statistical program IBM SPSS Statistics for Mac, Version 20.0 (released 2011) was used for all statistics. Figures were made using GraphPad Prism version 6.0a for Mac, GraphPad Software (released July 18, 2012), La Jolla, California, USA.
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3. Results 3.1. Baseline characteristics in 2001 The baseline characteristics of the patients included in this analysis are shown in Table 1. Patients with simple ConHD who were not practicing sports were smoking more cigarettes per day compared with those who were practicing sports (hours) (20 [14–20] vs. 10 [4–15], p = 0.009), and showed a significantly lower exercise capacity (% predicted of norm) (89.6 ± 21.9 vs. 97.5 ± 15.2, p = 0.008). Patients with moderate/complex ConHD who were not practicing sports in 2001 were significantly older (years) (29.5 [24.2–35.0] vs. 26.7 [23.7–29.3], p = 0.036), had a significantly lower exercise capacity (% predicted of norm) (75.3 ± 17.2 vs. 84.1 ± 21.0, p = 0.047), had a higher average heart rate (beats per minute) (76.0 [72.0–80.0] vs. 71.0 [66.8–77.5], p = 0.021) and showed worse results on subjective physical functioning (QoL score) (95.0 [75.0–100.0] vs. 95.0 [90.0–100.0], p = 0.039) compared with patients who were practicing sports in 2001. 3.2. Mortality or arrhythmias Of the 362 patients evaluated in 2001, 10 patients died and 1 patient underwent heart transplantation over the follow-up period of 10 years. Clinical characteristics of these 11 patients are reported in Table 2. We found an association between the presence of moderate/complex ConHD and the occurrence of death (HR [95% CI]: 4.3 [1.1–16.6], p =
Table 1 Patient characteristics in 2001. Total
Age in 2001 (years) Age at first surgery Gender Male Female Smoking Yes Amount/day SYSVF Good Mild Moderate Bad Exercise capacity Holter data Average beats per minute SVT PVC N 10 complexes VT Medication Any medication Aspirin Calcium antagonist Betablocker Nitrate Anti-arrhythmics Digitalis Diuretics ACE inhibitor Cholesterol lowering Oral anticoagulation Physical Functioning
Simple ConHD
p
No sports
Sports
n = 245
n = 74
n = 91
29.7 [26.5–34.8] 4.6 [1.1–1.3]
31.6 [28.0–36.1] 5.5 [1.8–8.8]
30.1 [27.7–35.2] 5.7 [2.1–8.9]
0.161 0.814
52.2 (128) 47.8 (117)
45.9 (34) 54.1 (40)
51.6 (47) 48.4 (44)
22.6 (55) 10 [5–20]
27.0 (20) 20 [14–20]
80.8 (189) 9.8 (23) 6.0 (14) 3.4 (8) 79.1 ± 19.3
Moderate/complex ConHD
p
No sports
Sports
n = 45
n = 35
29.5 [24.2–35.0] 3.2 [0.7–6.5]
26.7 [23.7–29.3] 1.2 [0.5–3.1]
0.036 0.059
0.466
62.2 (28) 37.8 (17)
54.3 (19) 45.7 (16)
0.474
18.9 (17) 10 [4–15]
0.215 0.009
20.5 (9) 4 [3–12]
25.7 (9) 7 [3–15]
0.580 0.478
90.1 (64) 8.5 (6) 1.4 (1) 0.0 (0) 89.6 ± 21.9
94.1 (80) 4.7 (4) 1.2 (1) 0.0 (0) 97.5 ± 15.2
0.628
55.9 (19) 20.6 (7) 14.7 (5) 8.8 (3) 84.1 ± 21.0
0.866
0.008
59.1 (26) 13.6 (6) 15.9 (7) 11.4 (5) 75.3 ± 17.2
75.0 [69.0–81.0] 0.4 (1) 0.0 (0) 4.5 (11)
76.0 [70.0–82.2] 0.0 (0) 0.0 (0) 2.7 (2)
76.5 [69.0–81.8] 0.0 (0) 0.0 (0) 2.2 (2)
0.624 – – 1.000
76.0 [72.0–80.0] 2.2 (1) 0.0 (0) 8.9 (4)
71.0 [66.8–77.5] 0.0 (0) 0.0 (0) 8.6 (3)
0.021 1.000 – 1.000
4.9 (10) 0.5 (1) 0.0 (0) 1.0 (2) 0.0 (0) 0.5 (1) 2.0 (4) 0.0 (0) 2.5 (5) 0.0 (0) 0.5 (1) 95.0 [90.0–100.0]
96.8 (61) 1.6 (1) 0.0 (0) 0.0 (0) 0.0 (0) 0.0 (0) 1.6 (1) 0.0 (0) 1.6 (1) 0.0 (0) 0.0 (0) 95.0 [90.0–100.0]
97.4 (74) 0.0 (0) 0.0 (0) 1.3 (1) 0.0 (0) 0.0 (0) 0.0 (0) 0.0 (0) 1.3 (1) 0.0 (0) 0.0 (0) 100.0 [93.8–100.0]
1.000 0.453 – 1.000 – – 0.453 – 1.000 – – 0.152
94.1 (32) 0.0 (0) 0.0 (0) 0.0 (0) 0.0 (0) 2.9 (1) 5.9 (2) 0.0 (0) 2.9 (1) 0.0 (0) 2.9 (1) 95.0 [75.0–100.0]
87.1 (27) 0.0 (0) 0.0 (0) 3.2 (1) 0.0 (0) 0.0 (0) 3.2 (1) 0.0 (0) 6.5 (2) 0.0 (0) 0.0 (0) 95.0 [90.0–100.0]
0.413 – – 0.477 – 1.000 1.000 – 0.602 – 0.475 0.039
0.047
Abbreviations: Complex ConHD = Tetralogy of Fallot (ToF) & Transposition of the Great Arteries (TGA); ConHD = congenital heart disease; exercise capacity is expressed in % of expected workload; NYHA = New York Heart Association; PVC = premature ventricular complex; Simple ConHD = Atrial Septal Defect (ASD) & Ventricular Septal Defect (VSD) & Pulmonary Stenosis (PS); SVT = supraventricular tachycardia; SYSVF = systemic ventricular function; VT = ventricular tachycardia; data are expressed as proportion and (actual number). Holter data has been recorded for 24 h, and amount of SVT, PVC and VTs reported is per 24 h.
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Table 2 Data on patients that died between 2001 and 2011. Pulmonary valve
Arrhythmias
No regurgitation No regurgitation Severe regurgitation N/A
No regurgitation
Heart failure, cardiac arrest Untreatable VF by ICD Directly after jogging
Endocardial VVI since 1990 Bad (no ICD) SR Moderately reduced Epicardial VVI since 1996 Bad (ICD) Endocardial DDD since Mildly 1981 (no ICD) reduced
No regurgitation
Sick-sinus syndrome No
Moderate regurgitation Severe regurgitation
Sick-sinus syndrome Sick-sinus syndrome
Unknown
Unknown
SR
Good
No regurgitation
No
0–1
Tennis
During tennis
SR
0–1
Hiking, walking
Heart failure
27
No
0–1
Surfing
VF
N/A
No
1968
27
No
5+
Unknown
32
No
5+
Light regurgitation N/A
No
1980
Fitness, rowing, running Fitness, soccer
SR RBTB SR RBTB SR RBTB SR RBTB
Moderate regurgitation No regurgitation
No
No
Mildly reduced Good
Light regurgitation N/A
Surgery BMI Hypertension Hours of Type of sport sports/week
Cause of death
ECG + pacemaker
51
F
Fallot
1968
18
No
0
No
Heart failure
28
M
VSD
1980
18
No
0
No
41
M
1973
21
No
0
No
41
M
TGA (Mustard) Fallot
1988
32
No
1–5
Jogging
45
F
ASD
1972
25
Yes
1–5
33
M
1974
28
No
32
F
TGA (Mustard) Fallot
1974
19
28
M
Fallot
1978
52
M
VSD
32
M
Fallot
Shot to death
Systemic ventricular function
Good Good Mildly reduced
No regurgitation No regurgitation Light regurgitation Light regurgitation
Other
No pulmonary homograft because of high surgery risk
Mild baffle stenosis
No
No
Abbreviations: ASD = Atrial Septal Defect; AV-valve = Atrioventricular valve; BMI = Body Mass Index; ConHD = congenital heart disease; ECG = electrocardiogram; Fallot = Tetralogy of Fallot; ICD = Implantable Cardioverter Defibrillator; N/A = not available; RBTB = Right Ventricular Bundle Branch Block; TGA = Transposition of the Great Arteries (all Mustard surgery); VF = Ventricular Fibrillation; VSD = Ventricular Septal Defect. Hours of sports are expressed in hours per week.
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
Systemic AV-valve
Age Gender ConHD
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
0.036). We found no relationship between practicing sports and the occurrence of death (HR [95% CI]: 0.6 [0.2–2.3], p = 0.482). The changes in sports participation in patients in the period from 2001 to 2011 are shown in Fig. 2. We also did not find a relationship between sports participation and the occurrence of PVCs (OR [95% CI]: 0.3 [0.1–1.05], p = 0.06), SVTs (OR [95% CI]: 0.9 [0.5–1.5], p = 0.572) or the average heart rate (R2 = .008, F(1,224) = 1.8, p = 0.181). There was a negative association between sports participation and the occurrence of VTs (OR [95% CI]: 0.3 [0.1–0.7], p = 0.010); however, this association disappeared after adjustment for potential confounders (OR [95% CI]: 0.5 [0.2–1.4], p = 0.169).
3.3. Characteristics of sports participation in patients with ConHD in 2011 The types of sports participated by patients in 2011 are listed in Table 3. More than half of the patients were involved in high-dynamic sports (57.9%). Fig. 3 shows sports participation in patients with ConHD in 2011 compared with normative data derived from the general population. Specifically, patients with moderate/complex ConHD practiced fewer hours of sports in comparison with the normal population. Both simple and moderate/complex ConHD patients were underrepresented in the ‘Extensive’ sports category compared with the general Dutch population. Fig. 4 shows that patients who practiced sports had a significantly higher exercise capacity compared with patients who did not practice any sport.
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Table 4 shows that patients with simple ConHD who were not practicing sports had a worse exercise capacity than patients who were practicing sports (86.6 ± 18.2 vs. 96.8 ± 19.2, p = 0.001). The same influence was observed for moderate/complex patients (75.9 ± 19.3 vs. 90.1 ± 19.2, p = 0.003). The total sports group showed a lower incidence of ventricular tachycardia (14.5% vs. 4.7%, p = 0.008, not in the table) and fewer premature ventricular complexes (64.2% vs. 49.2%, p = 0.023, not in the table). In addition, patients with moderate/complex ConHD who were not practicing sports showed a worse exercise capacity compared with those who were practicing sports (90.0 [75.0–95.0] vs. 95.0 [89.7–100.0], p-value = 0.001). Patients with moderate/complex ConHD who were practicing sports showed a significantly higher Physical Functioning compared with patients who did not practice sports (95.0 [89.7–100.0] vs. 90.0 [75.0–95.0], p = 0.001). No differences were found on quality of life (according to the LAS instrument). 3.4. Clinical consequences of sports participation over time Over the period of 10 years, 22.5% of the patients showed deterioration in systemic ventricular function, while 69.6% remained stable. In 7.9% of the patients, an improvement in systemic ventricular function was observed. The patients who practiced sports, more often remained stable (77.0% vs. 61.0%, overall p-value = 0.031). When looking at subjective physical functioning over time, deterioration was seen in the group of patients that stopped practicing sports between 2001 and 2011 (95.0 [85.0–100.0] vs. 92.5 [75.0–100.0], p = 0.035). Systemic ventricular function did not deteriorate in patients who stopped practicing sports over time compared with patients who remained practicing sports (20.9% vs. 30.6%, p = 0.276). These influences were observed only when the data were analyzed for the overall patient's cohort, and were lost when looking separately at simple and moderate/complex ConHD. 3.5. Major complications between 2001 and 2011 Table 5 lists the major complications that occurred between 2001 and 2011. In total, 46 patients had only events before the first assessment in 2001. These patients were excluded from analysis. A total of 26 patients had one or more major cardiac events. There were no significant differences between patients who did or did not practice sports. 4. Discussion Our study shows that sports participation in adults with ConHD has a positive influence on exercise capacity, both in patients with simple and moderate/complex ConHD, without conveying major negative consequences such as death or arrhythmias. In the patients with moderate/ complex ConHD a positive influence on subjective physical function was also observed. 4.1. Sports participation in patients with ConHD
Fig. 2. Flowchart of sports participation between 2001 and 2011. Abbreviations: ConHD = congenital heart disease. The category “No sports” contains patients who never practiced any sports and patients who stopped practicing sports between 2001 and 2011. The category “Sports” contains patients who remained practicing sports between 2001 and 2011, and patients who started practicing sports between 2001 and 2011.
Almost half of the adults with ConHD included in the present analysis were engaged in sports. Compared with the general Dutch population, the patients with ConHD, and especially those with moderate/ complex ConHD, practice sports for fewer hours per week. Our findings are in line with two previous studies [24,25], and in contrast only with Immer et al. who unexpectedly reported a higher rate of sports participation in patients with simple and complex forms of ConHD compared with the general population [26]. Patients with more complex forms of ConHD are known to have a reduced exercise capacity [27]. This finding is the most logic explanation why complex patients (as shown in our study) are less often involved in sports. They are probably limited in
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Increasing static component
Table 3 Type of sports participation of ConHD patients in 2011.
High 30.9%
23.0%
1.3%
6.6%
Moderate 43.4%
4.6%
3.3%
35.5%
Low 25.7%
9.2%
0.7%
15.8%
Low 36.8%
Moderate 5.3%
High 57.9%
Increasing dynamic component Data is for total ConHD group. Table based on the study of Mitchell et al. [18].
their possibility to sport, based on their reduced physical capacities. However, it has been shown that children and adolescents with ConHD were able to improve their peak oxygen uptake with an exercise program, so the depressed exercise capacity may partially be explained by the absolute lack of exercise, mostly based on fear for complications or negative impact on cardiac function [6,24,25,27–30]. We found that practicing sports did increase exercise capacity in patients with ConHD, including those with the more complex lesions. Indeed, we found not only a positive influence on exercise capacity but also on subjective physical functioning, including the patients with more complex ConHD. Previous studies have shown ambiguous results, with some studies reporting that an active lifestyle in ConHD patients has a positive influence on exercise capacity or peak oxygen uptake and perceived physical functioning [6,25,31,32], while other studies found no relationship [29,33].
regarding the safety of sports participation and explains the implementation of strict regulations [34]. On the other hand, many patients with moderate/complex ConHD can and do participate in sports without major problems and can even reach the highest level of achievement. For instance, Shaun White, also operated for Tetralogy of Fallot won Olympic gold twice on snowboarding [35]. Proper studies on safety are lacking and are difficult to perform. In our prospective observational cohort, we found no significant relation between sports participation and sudden cardiac death or other adverse events such as ominous arrhythmias. Moreover, we found less ventricular ectopic beats and short VTs on Holter ECG monitoring in patients that were actively engaged in sports and a trend for a favorable influence on ventricular function. Of course, there may be a bias selection in sports, usually compromising the individuals in better conditions, but nevertheless these patients seem not to be at higher risk. We found high scores on subjective physical functioning in patients with ConHD, with the possible consequence of overestimating their physical capabilities. This might indeed imply that they might choose a sport that can potentially be harmful. In our study, over 50% was engaged in high-dynamic sports and a small subset of patients (6.6%) practiced sports both at the highest static and dynamic level. Despite participation in these demanding sports, we found no negative influence on ventricular function or clinical events. Our study supports the concept that sports participation in patients with Con HD can be approached with more confidence and may imply more beneficial consequences than believed in the past. Our findings open also the question of the appropriateness of the current restrictive guidelines regarding sports participation in these patients [36].
4.3. Recommendations
The results on safety in this article should be interpreted with caution due to the small sample size. The rare case of a young man with an operated Tetralogy of Fallot dying suddenly at the sports field is a dramatic and emotional event that raises a number of questions
Although our study shows no overall increased risk of cardiac complications and death, recommendation for practicing sports should take into account the unique medical history of the patient. Advice on the type of sport should be based on the personal situation and anticipated effort levels, and if needed can be supervised by a physician first [6,37,38]. In a recent publication, Budts et al. have published a 6-step evaluation in order to be able to advise patients for a high, moderate or low intensity sport [39]. Takken et al. have published guidelines for children with ConHD on physical activity, recreational sports and exercise training [36]. Solid programs of sports participation with medical supervision as described successfully for patients with heart failure may be necessary also for our (adult) patients with ConHD.
Fig. 3. Sports participation in 2011 versus normative data. Abbreviations: Extensive = 5 or more hours per week; Little/moderate = 1 to 5 hours per week; more than 5 h of sports per week; None = Up to 1 h or less of sports per week. Categories are based on normative data derived from the Dutch Central Bureau of statistics [15].
Fig. 4. Relationship between exercise capacity and hours of sports practiced. Abbreviations: Extensive = 5+ hours of sports per week; Little/Moderate = 1 to 5 h of sports per week; None = up to 1 h or less of sports per week. The X-axis represents the hours of sports practiced in 2011. On the Y-axis the exercise capacity is shown. Practicing N5 h/week sports per week or more results in a better exercise capacity.
4.2. Safety
P. Opić et al. / International Journal of Cardiology 187 (2015) 175–182
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Table 4 Results in 2011. 2011 Total
Exercise capacity SYSVF Good Mild Moderate Bad Holter data Average beats per minute PVC N 10 complexes SVT VT Physical Functioning LAS
Simple ConHD
p
No sports
Sports
n = 245
n = 74
n = 91
89.2 ± 20.3
86.6 ± 18.2
96.8 ± 19.2
0.001
66.1 (156) 22.9 (54) 7.6 (18) 3.4 (8)
82.8 (53) 15.6 (10) 0.0 (0) 1.6 (1)
86.0 (80) 12.9 (12) 1.1 (1) 0.0 (0)
73.0 [68.0–79.0] 56.2 (127) 28.2 (69) 9.4 (23) 95.0 [85.0–100.0] 80.0 [75.0–88.0]
74.0 [69.0–79.3] 51.6 (32) 25.4 (18) 7.0 (5) 95.0 [80.0–100.0] 80.0 [74.0–85.0]
72.0 [68.0–79.0] 44.4 (40) 30.9 (29) 2.1 (2) 100.0 [90.0–100.0] 80.5 [75.0–90.0]
Moderate/complex ConHD
p
No sports
Sports
n = 45
n = 35
75.9 ± 19.3
90.1 ± 19.2
0.003
0.564
24.4 (11) 48.9 (22) 15.6 (7) 11.1 (5)
35.3 (12) 29.4 (10) 29.4 (10) 5.9 (2)
0.173
0.491 0.384 0.438 0.121 0.122 0.062
73.5 [69.3–80.8] 81.8 (36) 34.8 (16) 26.1 (12) 90.0 [75.0–95.0] 80.0 [74.3–85.0]
74.0 [65.0–78.0] 63.3 (19) 17.6 (6) 11.8 (4) 95.0 [89.7–100.0] 83.0 [75.0–89.0]
0.352 0.074 0.090 0.113 0.001 0.105
Abbreviations: Complex ConHD = Tetralogy of Fallot (ToF) & Transposition of the Great Arteries (TGA); ConHD = congenital heart disease; exercise capacity is expressed in % of expected workload; LAS = Linear Analogue Scale; PVC = premature ventricular complex; Simple ConHD = Atrial Septal Defect (ASD) & Ventricular Septal Defect (VSD) & Pulmonary Stenosis (PS); SVT = supra-ventricular tachycardia; SYSVF = systemic ventricular function; VT = ventricular tachycardia. Data are expressed as n (%). Holter data has been recorded for 24 h, and amount of SVT, PVC and VTs reported is per 24 h.
4.4. Limitations of the study Although this is the first report based on longitudinal data, no firm conclusions on safety can be drawn due to the relatively small size of the patient population. Larger series and longer follow-up are clearly warranted to consolidate our positive experiences regarding the low mortality and better ventricular function associated with sports participation. Our findings open also the discussion regarding less conservative rules regarding sports participation in these patients. The patients included in this study had one of the following diagnoses: ASD, VSD, PS, ToF or TGA, therefore, the obtained results may not be applicable to all forms of ConHD. The results of the safety of sports from the logistic regression (mortality, heart frequency and arrhythmias) should be drawn with caution since our data is based on an observational cohort and selection bias may exist. Patients participating in sports may have been in better health condition at baseline. We tried to avoid this bias by dividing the group into patients with simple and moderate/ complex heart disease, but still a bias cannot be ruled out. Also, the study design in 2001 did not anticipate these analyses on sports behavior in 2011. Therefore, patients were not instructed in 2001 to write down the dates in which they changed or stopped to practice sports, nor did they wear an accelerometer to objectify duration and intensity of sports participation. Therefore, in the “No sports” category patients
who did practice sports for a certain amount of hours prior to the assessment in 2011 could have been included. Lastly, a questionnaire on sports participation (without objective measurement) unfortunately does not always reflect reality. Furthermore, duration of sports does not equal intensity. Outcome on these questionnaires should be interpreted bearing this in mind. 5. Conclusion The results of this study suggest that sports participation in patients with congenital heart disease does not increase the risk of arrhythmias or sudden cardiac death. Patients with congenital heart disease who participate in sports have better subjective and objective (physical) functioning than those who do not. Funding This study was supported by the “van de Hoop” foundation, which is a non-profit organization that supports a wide scale of medical research. Conflict of interests No conflict of interests reported.
Table 5 Clinical events between 2001 and 2011. Major events between 2001 and 2011 Total
Major events Overall Arrhythmia Pacemaker implantation Surgical reintervention ICD implantation Heart failure
Simple ConHD
p
Moderate/complex ConHD
No sports
Sports
No sports
Sports
n = 245
n = 74
n = 91
n = 45
n = 35
10.6 (26) 6.5 (16) 2.9 (7) 2.4 (6) 1.6 (4) 1.2 (3)
8.5 (6) 5.6 (4) 2.8 (2) 2.8 (2) 1.4 (1) 0.0 (0)
5.3 (5) 4.3 (4) 0.0 (0) 0.0 (0) 1.1 (1) 0.0 (0)
19.6 (9) 10.9 (5) 8.7 (4) 4.3 (2) 4.3 (2) 6.5 (3)
17.6 (6) 8.8 (3) 2.9 (1) 5.9 (2) 0.0 (0) 0.0 (0)
0.425 0.726 0.184 0.184 1.000 –
p
0.828 1.000 0.388 1.000 0.505 0.258
Abbreviations: Complex ConHD = Tetralogy of Fallot (ToF) & Transposition of the Great Arteries (TGA); ConHD = congenital heart disease; ICD = Implantable Cardioverter Defibrillator; Simple ConHD = Atrial Septal Defect (ASD) & Ventricular Septal Defect (VSD) & Pulmonary Stenosis (PS). Data are expressed as n (%); one person can have multiple major complications.
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Acknowledgments The authors gratefully thank Bob Rigter, for the valuable assistance in the preparation of this manuscript.
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