Comprehensive medical history: underlying heart disease, surgical interventions and catheterizations, noncardiac comorbidities, medication, and signs and symptoms triggered by physical exercise (table 5).

The important tests for risk stratification include the following (recommendation class IIa, level of evidence C):

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  Resting ECG and 24-hour Holter monitoring for arrhythmias.

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  Transthoracic echocardiography study.

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  6-minute walk test. This is a simple, accessible, low-cost, submaximal evaluation that is well tolerated and does not require complex equipment: measuring tape, 2 cones, a 30-meter track, a heart rate (HR) monitor, SaO2, and a standardized protocol. It is preferably administered in children less than 8 years of age, along with a subjective effort scale (RPE, rating of perceived exertion) for the initial assessment and subsequent monitoring.

• •

  Conventional exercise testing or cardiopulmonary exercise testing (CPET) with analysis of gases. These tests are used to evaluate the cardiovascular response to physical activity in CHD patients. Conventional exercise tests monitor blood pressure, HR, and SaO2 during exertion and can detect arrhythmias and other ECG abnormalities. CPET, however, is the reference technique, delivering data on peak and maximum VO2, respiratory efficiency, respiratory reserve, anaerobic threshold, oxygen pulse, and ventilation-perfusion ratio, among numerous other parameters. The drawbacks of this test include its high cost and limited availability. CPET is not recommended for prescribing exercise regimens in children younger than 8 years due to inadequate quality (recommendation class IIa, level of evidence C).15

Risk stratification should be carried out (figure 2). Patients are assigned to 1 of the 3 training intensity levels described by Budts et al.,15 and subsequently incorporated into the CRRP (recommendation class IIa, level of evidence C) (figure 3 and figure 4).

Figure 2.

Cardiovascular factors to assess for risk stratification. AoCo, aortic coarctation; AF, atrial fibrillation; CPET, cardiopulmonary exercise testing; HR, heart rate; LL, lower limbs; LV, left ventricle; LVEF, left ventricular ejection fraction; LVOT, left ventricular outflow tract; MET, metabolic equivalent of task; PAP, pulmonary arterial pressure; RV, right ventricle; RVOT, right ventricular outflow tract; TR, tricuspid regurgitation; UL, upper limbs; VT, ventricular tachycardia; VO2, oxygen consumption.

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Figure 3.

Expected aerobic capacity in children and adolescents with CHD up to 16 years old. Compiled using data from Amedro et al.16 and Ten Harkel et al.17 ASD, atrial septal defect; AoCo, aortic coarctation; AoS, aortic stenosis; ccTGA, congenitally corrected transposition of great arteries; PS, pulmonary stenosis; TGA, transposition of great arteries; ToF, tetralogy of Fallot; VO2, oxygen consumption; VSD, ventricular septal defect.

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Figure 4.

Expected aerobic capacity in children with CHD older than 17 years and adults. Compiled using data from Abella et al.,18 Rhodes et al.,19 and Kempny et al.20 AoCo, aortic coarctation; AoS, aortic stenosis; ASD, atrial septal defect; ccTGA, congenitally corrected transposition of great arteries; PS, pulmonary stenosis; TGA, transposition of great arteries; ToF, tetralogy of Fallot; VO2, oxygen consumption; VSD, ventricular septal defect.

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Use of the FORCE21 tool is recommended, an acronym for the following analysis: F, ventricular function; O, O2 saturation; R, heart rhythm; C, complexity of the coronary anatomy, E, elevated pressure or elevated ventricular volume.

Currently, hybrid programs are being implemented: initially, patients and guardians undergo training and education in the hospital, and thereafter, they continue activities at home. This approach fosters acceptance, adherence, and favorable results.22 Patients with values in red must undergo fully monitored hospital-based CRR due to the associated risk, while those at low to moderate risk may transition to home-based CRR, as further explained below (recommendation class IIa, level of evidence C).

At the start, a meeting will be held with the patient and guardians to explain the assessment and training schedule. A local informed consent form detailing potential adverse effects during CRR is then signed. A record of the planned activities should be maintained for monitoring and adjustment purposes.

Use of the FITT acronym (frequency, intensity, time, and type of exercise) is recommended for designing the exercise regimen (recommendation class IIa, level of evidence C) (table 6).23

The sessions will include groups of 2 to 6 children, 2 to 3 times per week for 12 weeks, with each session lasting 45 to 60minutes, all under the supervision of a professional. Groups will be organized based on pubertal development and familiarity with the exercises performed, rather than age, sex, or body size (recommendation class IIa, level of evidence C).

A kinesiologist, physical education specialist, or sports medicine physician will conduct the functional assessment of strength, flexibility, and coordination. All exercises should be supervised to ensure proper technique and prevent injuries (recommendation class IIa, level of evidence C).

Each CRR session consists of 4 stages: a) respiratory physiotherapy; b) muscle warm-up and strengthening exercises; c) aerobic training; and d) cooling down and stretching (table 7).

Inspiratory muscle strength should be assessed24 by measuring the maximum inspiratory pressure25 or maximum nasal inspiratory pressure (SNIP, sniff nasal inspiratory pressure)26 (recommendation class IIa, level of evidence C) (table 8). There is a good correlation between these 2 parameters, but the correlation decreases in the presence of airway obstruction.26

The inspiratory muscles should be trained using inspiratory valves, such as the Threshold IMT (Philips Respironics, United States) or POWERbreathe (POWERbreathe Intl Ltd, United Kingdom). To work on muscle strength, pressures should be increased with fewer repetitions per session. These exercises improve maximum inspiratory pressure, vital capacity and total lung capacity, fatigue resistance, and peak VO2 in heart failure patients.27 In addition, they enhance diaphragm thickness,28 promote greater systemic venous return during inspiration,29 and improve ventilatory pump function (recommendation class IIa, level of evidence C).30 Furthermore, when combined with abdominal or abdominal plus diaphragmatic breathing, they help to reduce dyspnea and enhance HR on exertion, and improve QoL.31

It is important to train the major muscle groups, such as the deltoids, biceps, triceps, core muscles, quadriceps, hamstrings, and calf muscles. These exercises improve and enhance the peripheral muscle pump, leading to increased heart rate due to greater venous return and cardiac output, both at rest and during exercise (recommendation class IIa, level of evidence C).

Training for prepubescent patients focuses on promoting greater agility, balance, coordination, flexibility, and strength (recommendation class IIa, level of evidence C).15,25

Aerobic training can be done on a treadmill, stationary bicycle, rowing machine, or arm bicycle. Moderate-intensity continuous training (MICT) and high-intensity interval training (HIIT) are both effective for improving FC.32 MICT is preferable for older patients with pulmonary or cardiac limitations, as it is better tolerated, although it demands more time. HIIT is more appealing to prepubescent patients and induces greater metabolic stress compared with MICT. It also requires better cardiorespiratory adaptation, because peak effort is rapidly reached. Patients with CHD can undertake HIIT, but only after a period of low- to moderate-intensity training to gradually build tolerance.33 Risk stratification is particularly important for these high-intensity activities. In postpubescent individuals, programs can be structured similarly to those for adults, focusing on aerobic and muscle strength training (recommendation class IIa, level of evidence C).

Exercise intensity can be adjusted through: a) HR reserve; b) maximum HR, determined by conventional exercise testing or estimated using Tanaka's (208 – [0.7 × age]) or Karvonen's (220 – age) formula; c) maximum or peak VO2; d) subjective effort scale; and e) 1-repetition maximum test (recommendation class IIa, level of evidence C) (table 9).

The subjective effort scale, SaO2, blood pressure, and HR should be monitored before, during, and after exercise, while always being attentive to signs and symptoms of alarm (recommendation class IIa, level of evidence C).

These are low-intensity exercises that should last from 5 to 10minutes. Omitting the cooling down stage could result in a temporary decrease in venous return and coronary flow when HR and myocardial oxygen consumption are still high, leading to hypotension, angina, ST-segment changes, and ventricular arrhythmias.34

Periodically (weekly or monthly) perform a reassessment of the type of training, the technique, and the workload, making adjustments based on any changes (recommendation class IIa, level of evidence C).

Upon completion of CRRP, follow-up is conducted to evaluate whether the benefits achieved are sustained. It is recommended to monitor patients at 3, 6, and 12 months following completion (recommendation class IIa, level of evidence C).

A physiotherapist should be present throughout all phases of the CRRP. Their involvement starts in the immediate postoperative period, focusing on weaning patients off mechanical ventilation, providing respiratory physiotherapy, initiating early mobilization, and introducing light exercise, based on the patient's clinical and hemodynamic status. The objective is to prevent respiratory and thromboembolic complications, reduce the duration of mechanical ventilation, intensive care stay and hospitalization, ensure patients are discharged in the best physical condition, and provide educational guidance for prompt CRR initiation (recommendation class IIa, level of evidence C).39

After the initial physical evaluation, the physiotherapist will indicate the training program and identify any neuromuscular deficits that could hinder achievement of goals or aerobic training (recommendation class IIa, level of evidence C).

In children with CHD, the prevalence of overweight ranges from 9.5% to 31.5% and that of obesity from 9.5% to 26%.40 Therefore, it is essential to encourage a healthy diet to prevent poor nutrition due to excess, decrease blood pressure, improve peak VO2, lengthen endurance time,41 and avert early cardiovascular disease. Evaluation of nutritional status (table 10) should be continuous, personalized, and involve the family (recommendation class IIa, level of evidence C).

Active and passive exposure to tobacco and electronic cigarettes, as well as alcohol consumption, should be avoided (recommendation class IIa, level of evidence C).42

Psychological interventions alleviate distress and improve blood pressure and cholesterol levels.43 Effective communication with health care staff is a key component in CRRPs. An approach to mental health care that promotes patient and family acceptance and commitment is important to motivate healthy behavior patterns (recommendation class IIa, level of evidence C).10

QoL questionnaires, such as PedsQL44 or ConQol,45 should be administered at the beginning and end of CRRP (recommendation class IIa, level of evidence B; table 11).

The necessary equipment needed will depend on the type of patient, local infrastructure, and whether the approach is in-person or remote.

The optimal framework involves a multidisciplinary team. However, at the very least, a physician is required for risk assessment, together with a qualified professional for prescribing and conducting training.

The setting can be a closed, climate-controlled environment or an open space, such as a plaza or sports field.48 Closed environments should have an area of at least 24 m2, be well-ventilated and illuminated, with a ceiling height of >2.5 m, a temperature between 22°C and 25°C, and nonslip flooring (recommendation class IIa, level of evidence C).48

In a closed environment, the following should be available: a) a treadmill with front and side support, a safety lock, and adjustable speed and incline, or a cycle ergometer with cadence or speed readings and power in watts10,49; b) suspended ropes or bands, dumbbells, weights, or elastic bands with various resistance levels for muscle strengthening48; and c) additional material, such as balls, trampolines, and recreational items (recommendation class IIa, level of evidence C).

At a basic level, the following items should be available: a) a stethoscope, digital oximeter, sphygmomanometer, and anthropometry equipment48; b) subjective effort scale, such as EPInfant-Ex50 (figure 5), OMNI, or Borg (recommendation class IIa, level of evidence C); c) continuous ECG and saturation monitoring for patients at high risk48; d) smart watch for RCRR (recommendation class IIa, level of evidence C); and e) platform for communication between the attending center and patients and guardians for RCRR. The platform should be user-friendly, enabling video calls to answer exercise-related questions, oversee proper execution, and provide periodic encouragement (recommendation class IIa, level of evidence B).48,49

Figure 5.

Subjective effort scale, EPInfant-Ex. PE, physical exercise; HR, heart rate. Reproduced with permission from Rodríguez-Núñez.50

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Given that any patient can experience a serious adverse event, it is essential to have an emergency protocol in place. Both the guardians and health care team should be trained in basic and advanced cardiopulmonary resuscitation and use of an automated external defibrillator with a full crash cart and supply of supplemental oxygen therapy (recommendation class IIa, level of evidence C).48