1. Barranco, Mariola Cortina MD, MSc
  2. Velasquez, Alessandra Bertha Castillo MD
  3. Supervia, Marta MD, PhD, MSc, CCRP
  4. Riano, Maria Olga Arroyo PhD
  5. Smith, Joshua R. PhD

Article Content

Congenital heart disease (CHD) is a common pathology at birth, with an incidence of 8/1000 live births. Over the past 30 yr, these patients have exhibited significantly longer life expectancies but not necessarily increased quality of life (QOL).1-3 Furthermore, these patients are often considered "fragile" due to their high rates of comorbidities and hospital readmissions.4,5


There is a broad consensus on the benefits of cardiac rehabilitation programs (CRP) for the pediatric population. However, most studies vary in design and focus only on clinical outcomes.6-8 Therefore, studies with a more global view and better methodological quality are needed to investigate CRP in the pediatric population.


The aim of this study was to characterize the CHD patients treated in our CRP and analyze the association between CRP attendance and potential modifications to QOL and other clinical variables, as well as the incidence of possible adverse events.



This was a prospective observational study that recruited pediatric patients with CHD who were referred to the Children's CRP of the General Hospital Gregorio Maranon University (Madrid, Spain) between April 2018 and December 2019. The study was approved by the Ethics Committee of the Gregorio Maranon Research Institute.


Data from 12 patients with different cardiologic diagnoses were collected. The inclusion criteria included the following: CHD diagnosis, written consent, age 6-18 yr (both inclusive), and ergospirometry performed in our hospital for a maximum of 6 mo prior to inclusion. The exclusion criteria included ergospirometry with negative tension response, oxygen saturation of <80%, clinical or electrocardiographic presentation of ischemia, pathology that may need surgery or if surgery had been performed during the year before the start of the program, nonstable cardiac pathology, cognitive impairment, severe psychiatric or neuromuscular pathology that makes participation difficult, and other acute pathologies.


An initial interview was conducted, and sociodemographic and clinical variables were collected. Then ergospirometry to measure peak oxygen uptake, maximal inspiratory and expiratory pressure measurements, and 6-min walk test were performed. In addition, the questionnaires of QOL (PedsQL cardiac version) and physical activity (International Physical Activity Questionnaire [IPAQ-C]), child or adolescent version) were completed.


The CRP duration was 3 mo consisting of two sessions/wk (hospital-based) for 1 hr in the afternoon and a home-based program with a daily progressive respiratory physiotherapy regimen. The sessions were supervised by a rehabilitation physician and a physiotherapist. At the beginning of each session, baseline variables were recorded, and telemetry was started (heart rate and electrocardiogram were monitored throughout the session), followed by 5 min of respiratory physiotherapy (eg, chest expansion movements, diaphragmatic breathing, use of incentivator and inspirometer set at 30% and gradually increasing to 10%/wk). Next, the patient performed 5-10 min of strength training, 20-30 min of aerobic exercise on a treadmill, and occasionally leisure activity (eg, dance, Wii). The goal of the aerobic training was to achieve near-the-ventilatory threshold 1 at the beginning of the program and progressively increase to ventilatory threshold 2 or a heart rate of 60-70% of peak heart rate. The session ended with 5 min of stretching and relaxation exercises.



Statistical significance was considered as a value of P < .05. Numerical variables are presented with their mean +/- SD, and categorical variables are presented as n (%). Normality was tested with the Kolmogorov-Smirnov test, and a two-way paired Student test was employed using SPSS Statistics for Windows, version 25 (IBM Corp).



We included 12 CHD patients of pediatric age in our CRP (10.4 +/- 2.8 yr), of which 58% (n = 7) were male. The most prevalent diagnosis and surgical procedures were dextrotransposition of the great arteries, valvuloplasties, and Fontan surgery, with 25% (n = 3) of cases in each (N = 9 in total). One patient underwent cardiac transplant, and two patients were on the cardiac transplant waiting list. Two patients dropped out of the CRP for family issues. Therefore, the pre- to post-CRP analysis was performed with data from 10 patients. The CRP consisted of 24 sessions; 90% (n = 9) of the patients completed >=23 sessions, while one patient attended 19 sessions (absences were for nonmedical reasons). No adverse events were reported such as arrhythmias during the CRP.


Pre- to post-CRP variables are shown in the Table. Maximal inspiratory pressure significantly increased from pre- to post-CRP (P < .001). There were no significant differences in other measured variables, although some variables showed positive changes such as maximum expiratory pressure (P = .095) and peak oxygen uptake (P = .156). We found a positive change, although not significant (P < .1) on QOL tests and the IPAQ-C questionnaire (pre: 1.88 +/- 0.74 vs post: 2.15 +/- 0.58). We found no relevant differences between the total score of PedsQL (cardiac version).

Table Variation of P... - Click to enlarge in new windowTable Variation of Pre- and Post-training Variables


In the present study, we found that maximal inspiratory pressure increased from pre- to post-CRP in CHD patients. These findings are in line with a previous study by Ferrer.8 In contrast, QOL was not altered from pre- to post-CRP herein. This was surprising and may be due to CRP duration. It may also be explained by improved disease consciousness, given that clinical variables did not worsen during this time frame.


The high adherence in the present study may be due, in part, to the patients not missing school, as the CRP sessions were conducted in the afternoon. Furthermore, patient trust in the program is encouraged by the absence of adverse events and positive change (albeit nonsignificant) in the IPAQ-C score. In contrast to other studies, maximal expiratory pressure, body mass index, and 6-min walk test were not improved from pre- to post-CRP in pediatric patients with CHD.9-11 It is possible that with a larger sample or control group, statistical differences may have been detected in these outcomes.


In conclusion, the CRP elicited improvements in inspiratory muscle strength in children with CHD without any adverse events reported. Future studies with larger sample sizes are necessary to investigate the impact of cardiac rehabilitation on other clinical outcomes.




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