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Vol. 73. Issue 7.
Pages 586-589 (July 2020)
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Vol. 73. Issue 7.
Pages 586-589 (July 2020)
Scientific letter
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Safety and clinical benefit of cardiopulmonary rehabilitation in complex congenital heart disease
Seguridad y beneficio de la rehabilitación cardiopulmonar en cardiopatías congénitas complejas
Luisa García-Cuenllas Álvareza,
Corresponding author

Corresponding author:
, Fernando del Campo Bujedob, Carmen Oreja Sánchezc, María Ángela Centeno Garridoc, Juan Ignacio Castillo Martínd, Pedro L. Sánchezb
a Servicio de Pediatría y Cardiología Pediátrica, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain
b Servicio de Cardiología, Complejo Asistencial Universitario de Salamanca-IBSAL, CIBERCV, Salamanca, Spain
c Servicio de Rehabilitación y Fisioterapia, Complejo Asistencial Universitario de Salamanca-IBSAL, Salamanca, Spain
d Servicio de Rehabilitación y Fisioterapia, Hospital Universitario 12 de Octubre-IIS i+12, Madrid, Spain
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Tables (2)
Table 1. Characteristics of patients undergoing the cardiac rehabilitation program
Table 2. Parameters assessed in forced spirometry, 6minute walk test, and ergospirometry, before and after the cardiopulmonary rehabilitation program
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To the Editor,

Cardiopulmonary rehabilitation (CR) in patients who have undergone surgery for congenital heart defects (CHDs) is rarely undertaken in Spain, despite its beneficial effects and the fact that physical activity is recommended for CHDs by the European scientific societies.1

An interventional, experimental, prospective, phase I study was conducted (with no randomization for rehabilitation program assignment) to evaluate program safety and functional improvement in 24 young patients (median age, 19 [range, 9-31] years) with complex CHDs that had been treated surgically. This phase 1 study was designed with safety as its primary endpoint and avoided the need to calculate the sample size. The intervention consisted of a 3-month program of twice-weekly CR sessions in groups of 4 or 5 individuals. Each 1-hour session included personalized exercise consisting of warmups, respiratory physiotherapy, aerobic exercise (treadmill, bicycle, and/or videogames), cooldowns, and stretches. Assessments and monitoring were performed in a session with a cardiologist, a physical therapist, a rehabilitation therapist, a psychologist, and a nurse. The program incorporated health instruction, nutritional support, and psychological orientation, with family participation. In addition to ultrasound and electrocardiography, patient assessment included forced spirometry, 6minute walk test, ergospirometry, and quality of life surveys2,3 before and after the program. Patients were not enrolled if they had syndromal CHDs or major comorbidities that could affect or influence the parameters assessed. All patients signed an informed consent form.

Categorical variables are shown as percentages, and continuous variables are shown as the median (range). Nonparametric tests were used to compare dependent paired proportions (McNemar) or ordinal variables (Wilcoxon). A P value <.005 was considered significant.

The patient sample is described in table 1. The number of scheduled sessions was 24, with a median adherence of 23.5 (range, 9-31). Patient #18 was treated by pulmonary valve replacement, whereas the others required no therapeutic or medical intervention of any kind. No adverse cardiovascular events or electrocardiographic or echocardiographic changes were reported before or after the program.

Table 1.

Characteristics of patients undergoing the cardiac rehabilitation program

Sex  Age, y  BMI  CHD  Surgery/Residual Lesions  Medication  Number of Sessions 
Male  17  23.35  PA-IVS  RV-PA conduit, mild PI  No  27 
Female  21  23.14  PA-IVS  Transannular patch, severe PI, moderate TR  No  17 
Female  19  21.56  PA-IVS  RV-PA conduit, moderate conduit stenosis, moderate TR  Aspirin  24 
Female  26  20.52  Tricuspid atresia  Glenn, Fontan, chronic Fontan failure  Aspirin, diuretics, BB  21 
Male  22  22.09  d-TGA  Arterial switch, VSD closure, mild PS  No  27 
Male  26  26.2  d-TGA  Mustard, closed baffle leak, sinus node syndrome  Aspirin  28 
Male  25  19.91  d-TGA  Mustard, systemic ventricular dysfunction  No  24 
Male  31  20.43  d-TGA  Mustard, systemic ventricular dysfunction, sinus node syndrome, pacemaker  Aspirin, BB  31 
Female  14  13.2  d-TGA  Arterial switch, VSD and ASD closure; arch, AoV, and pulmonary repair; PVR  ACEI  27 
Female  11  16.44  d-TGA  Arterial switch, moderate DPL, mild AoR  No  26 
Male  19  22.85  d-TGA  Mustard, sinus node syndrome  Aspirin, BB  14 
Male  13  24.6  d-TGA  Arterial switch, VSD closure, aortic arch dilatation, moderate DPL  No  24 
Male  27  19.63  Fallot  Complete correction, PVR  No  21 
Male  28  25.02  Fallot  Palliative fistula, complete correction, PVR  Aspirin  27 
Male  31  29.92  Fallot  Complete correction, restrictive VSD, mild PI  No  30 
Female  20  20.24  Fallot  Complete correction, severe PI  No  16 
Male  10  17.36  Fallot  Complete correction, mild RV dilatation, severe PI, mild PS  No  23 
Female  18.02  Fallot  Transannular patch, VSD closure, RV dilatation, moderate PI  No  16 
Female  23  25.15  Fallot  Complete correction with conduit, severe PI, RV dysfunction, CATCH 22  Aspirin  15 
Female  28  15.45  Type 1 truncus  RV-PA conduit, conduit expansion, DPL with severe PI  No  10 
Female  21.36  Type 1 truncus  Truncal valve repair, RV-PA conduit, severe valve regurgitation  No  25 
Male  16  24.66  Fallot-type DORV  VSD closure, infundibular resection, severe PI  No  12 
Male  19  18.49  Fallot-type DORV  VSD closure, infundibular resection, moderate PI  No 
Female  12  16.49  Fallot-type DORV  VSD closure, infundibular resection, moderate PS  No  21 

ACEI, angiotensin-converting enzyme inhibitor; AoR, aortic regurgitation; AoV, aortic valve; ASD, atrial septal defect; BB, beta-blockers; BMI, body mass index; CHD, congenital heart defect; DORV, double-outlet right ventricle; d-TGA, dextro-transposition of the great arteries; DPL, double pulmonary lesion; PA-IVS, pulmonary atresia with intact ventricular septum; PI, pulmonary insufficiency; PS, pulmonary stenosis; PVR, pulmonary valve replacement; RV, right ventricle; RV-PA, right ventricle-pulmonary artery; TR, tricuspid regurgitation; VSD, ventricular septal defect.

The course of the various parameters assessed before and after CR is shown in table 2. Upon completion of the program, the most significant cardiopulmonary changes were: a) increased inspiratory muscle strength and increased maximal inspiratory pressure; b) greater exertional capacity and tolerance to exercise, with increase in distance walked in the 6-minute walk test; longer exertion time (more than 1minute) and tendency toward better heart rate recovery in the first minute after exertion, as a possible improvement in autonomic nervous system regulation; c) improvement in maximal aerobic capacity, with a significant increase in peak O2 uptake (VO2, expressed as % theoretical); d) improvement in aerobic physical performance, considered a higher VO2 in the anaerobic threshold; e) improvement in cardiocirculatory response, as shown by the lower resting heart rate (with no drug-induced changes), increase in predicted maximal VO2 as an indirect estimator of cardiac output, and in predicted O2 pulse as a parameter to estimate systolic volume at maximal exertion; f) improvement in ventilatory efficiency in exercise, with a decrease in the slope of the plot line for ventilation per minute and CO2 production (VE/VCO2 slope), with a higher number of patients showing a ratio <30, considered normal for patient age and sex. Furthermore, these improvements were achieved in the absence of other changes in ventilatory efficiency and ventricular function variables, as shown by similar values for respiratory equivalents (VE/VCO2, VE/VO2), end-tidal partial pressure of CO2, slope of VO2 efficiency, ventilatory reserve, and echocardiographic measurements of ventricular function before and after the program. These data were consistent with subjective assessments of the New York Heart Association functional class, which reported 18 patients in class I (75%) and 6 in class II (25%) at baseline. By completion of the program, functional class had improved in 4 patients and worsened in 2, for a total of 20 patients in class I (83.3%) and 4 (16.7%) in class II. Last, quality of life questionnaire scores were normal, regardless of the grade of CHD complexity, with no differences between baseline status and the end of the program. The usefulness of the program was highly rated by patients and their families.

Table 2.

Parameters assessed in forced spirometry, 6minute walk test, and ergospirometry, before and after the cardiopulmonary rehabilitation program

  Before CR  After CR  P 
Forced spirometry parameters
Patients, n  24  23   
FVC, % theoretical  84 (48-110)  86 (60-120)  .106 
Patients with FVC> 80% theoretical  13 (54.2)  13 (54.2)  1.000 
FEV1, % theoretical  87.5 (45-112)  84 (59-117)  .795 
Patients with FEV1> 80% theoretical  17 (70.8)  14 (58.3)  .125 
FEV1/FVC  105.9 (78.3-121.1)  104.1 (76.7-119.3)  .128 
Patients with FEV1/FVC> 70% theoretical  24 (100)  23 (100)  1.000 
FVC, % theoretical  84 (48-110)  86 (60-120)  .106 
6minute walk test parameters
Patients, n  24  22   
Distance walked, m  524.5 (415-735)  640 (475-840)  <.001 
Ergospirometry parameters
Patients, n  24  24   
Exercise time, min  10.1 (6.1-12.3)  11.3 (6.4-13.2)  .002 
Direct METs, VO2/3.5 mL/kg/min  8.1 (4.1-12.4)  8.9 (3.9-11.2)  .094 
Resting HR, bpm  92.5 (60-122)  86.5 (60-116)  .068 
Maximum HR, bpm  177 (143-197)  179 (158-202)  .721 
Maximum HR, % theoretical  87.3 (73.8-98.3)  89.1 (78.7-96.9)  .648 
Reserve HR, bpm  86.5 (54-107)  92.5 (58-113)  .069 
Patients with HR decrease>12 bpm in 1st minute  24 (100)  24 (100)  1.000 
Resting SBP, mmHg  115 (90-130)  107 (90-125)  .052 
Resting DBP, mmHg  70 (45-90)  61.5 (50-90)  .819 
Maximum SBP, mmHg  150 (100-180)  143.5 (105-185)  .896 
Maximum DBP, mmHg  80 (50-90)  80 (60-100)  .955 
Double product  25 500 (18 700-33 300)  25 570 (17 490-33 670)  .670 
VO2max, mL/kg/min  28.2 (14.3-43.4)  31 (13.8-39.3)  .091 
VO2máx, % theoretical  69.2 (45.5-99.5)  71.5 (50-103.3)  .042 
AT, mL/kg/min  17.1 (9.2-24.6)  18.1 (10.6-25.5)  .045 
AT, % theoretical  60.5 (30.5-77)  67.2 (42-83)  .050 
Patients with AT> 60% (normal)  12 (50)  18 (75)  .031 
AT HR, bpm  123 (73-156)  125.5 (90-153)  .077 
RER> 1.10  23 (95.8)  23 (95.8)  1.000 
PO2max*, mL/beat  7.9 (5-16.4)  8.1 (4.9-16.4)  .182 
PO2max*, % theoretical  76 (48.2-124)  76 (58-118)  .039 
VE/VCO2slope*  30 (22.3-38.8)  28.3 (19-37.2)  .021 
Patients with VE/VCO2slope* <30% (normal)  11 (47.8)  14 (60.9)  .375 
Equivalent for CO2 (VE/VCO229.2 (23-42.4)  29.5 (20.9-40.5)  .764 
Equivalent for O2 (VE/VO236.5 (28-51.9)  37 (29.1-54.8)  .449 
PetCO2resting, mmHg  31 (21-40)  32 (22-37)  .503 
PetCO2max, mmHg  33 (24-42)  33 (23-47)  .612 
OUES  1.4 (0.4-3.4)  1.3 (0.6-3.3)  .617 
OUES, % theoretical  62 (18.4-92.6)  56.4 (31-97)  .693 
VR  42.5 (0-69)  37.5 (6-58)  .853 
Patients with VR> 20% (normal)  17 (70.8)  21 (87.5)  .219 
Quality of life
Number of PedsQL questionnaires, child self-report   
PedsQL score, child self-report  1775 (1300-1850)  1700 (1550-1950)  .225 
Number of PedsQL questionnaires, parent-proxy   
PedsQL score, parent-proxy  1700 (1550-1900)  1775 (1175-2075)  .144 
Number of NSS-36 questionnaires, young adults  14  15   
SF-36 score, young adults  103 (94-110)  103 (87-115)  .779 

AT, anaerobic threshold; CR, cardiopulmonary rehabilitation; DBP, diastolic blood pressure; FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; HR, heart rate; O2P, oxygen pulse; OUES, oxygen uptake efficiency slope; PedsQL, Pediatric Quality of Life Inventory Cardiac Module, version 4.0 used in our study for pediatric patients (age 8 to 18 years) and their parents; PetCO2, end-tidal partial pressure of CO2; SBP, systolic blood pressure; SF-36, Short Form Health Survey; VCO2, carbon dioxide production; VE, minute ventilation; VO2, oxygen uptake; VR, ventilatory reserve.


Patient #9 was excluded from this analysis due to Fontan circulation (this parameter is interpreted differently between cyanotic and noncyanotic patients).

Due to medical and surgical advances, it is estimated that more than 85% of children with CHDs in Spain will reach adulthood.4 However, CHD patients who have undergone surgery have lower progressive functional capacity, which increases their morbidity and mortality. In this context, efficient resources for improvement, such as CR, have been implemented; however, they are not widely used in Spain, and there is only 1 published report on experience with 8 patients who had CHDs and pulmonary hypertension,5 with increased functional class and exercise capacity in the 6minute walk test and no adverse events.

The importance of our study is that it is the first to demonstrate the benefits of a CR program in Spain for young people with complex CHDs treated by surgery and that it includes a thorough assessment with ergospirometry. The main limitations of the study are the small, heterogeneous sample and the lack of a control group. Implementation of the program was a challenge, as difficulties were encountered for administration to understand that CR should focus on comprehensive prevention units open to all heart diseases, rather than only coronary patients. We show that, despite these difficulties, CR could be a cost-effective tool capable of improving functional capacity and quality of life in complex CHDs. In our experience, CR has helped to support our patients and their families and enabled them to understand their limits and to encourage improvements in their functional capacity.


Biomedicine, health management, and social and health care research project funded by the Regional Health Agency of Castilla y León (G 1369/A/16) and the CIBERCV, Carlos III Health Institute, Ministry of Science, Innovation, and Universities.

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Copyright © 2020. Sociedad Española de Cardiología
Revista Española de Cardiología (English Edition)

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