To unmask an eventual Brugada syndrome, the patient received 100 mg Tambocor. Although there were no arguments for Brugada syndrome, the test revealed frequent PVB’s with multifocal couplets and triplets (Fig. 11.2).

Electrophysiological examination reproduced the symptoms, where echography revealed no suspicion for arrhythmogenic right ventricular dysplasia (ARVD), nor were late potentials found. Treatment with amiodarone (200 mg; 2/day) was then started. After 6 weeks of pharmacological treatment, a control examination showed that the patient still experienced the same symptoms together with high nausea and photo sensibility indicating a low tolerability for the medication. Electrophysiological examination still induced symptoms and arrhythmias in spite of the use of amiodarone and indicated a right ventricular origin. Therefore, the option of ICD implantation (implantable cardioverter defibrillator) became plausible. Patient-specific characteristics can be found in Table 11.1.

Four months after the first cardiac examination, a single chamber ICD was implanted with one lead in the right ventricular apex. Four weeks after implantation, this patient was included in a cardiovascular rehabilitation programme where a baseline symptom-limited exercise test was undertaken. Results from this test are presented in Table 11.2.

When testing or training an ICD patient, the level of exercise that could cause a defibrillation shock or anti-tachycardia pacing intervention should be avoided. The design of an exercise programme should always be preceded by a maximal or symptom-limited exercise test. Despite the fear of patients with an ICD and the risk of harmful and threatening symptoms, the exercise test has a key role in the evaluation of arrhythmias, the ICD device, peak heart rate and exercise tolerance and medical therapy (Fig. 11.3).

The testing protocol should consist of a standardized graded exercise tolerance test on a motor-driven treadmill or bicycle ergometer with assessment of ECG, blood pressure and oxygen uptake. The golden standard for the assessment of the functional capacity is peak oxygen uptake [1]. A submaximal test (terminating the test at a given percentage of predicted maximum heart rate) is not recommended. Firstly because medications affect the age-predicted maximum of the heart rate, as a result of which it would only give an estimate of the actual exercise tolerance. Secondly it would not give the opportunity to evaluate the reactions of cardiac rhythm and the ICD on maximal exercise. The participant reaches a maximum cardiorespiratory response by continuing the exercise test till exhaustion or fatigue. In some studies, the point where the patient reached a heart rate threshold of cut-off point minus 10–30 beats was one of the endpoints of the test, in order to avoid discharge of the ICD. [2–5]. However, Lampman and colleague stated that when the cut-off point is situated below the age-predicted maximum (220 minus age), the ICD should temporarily be switched off during the exercise test [6]. This way, the patient could reach his or her true maximum without being at risk for inappropriate shocks. A similar strategy was used in the study from Belardinelli et al., where the minimal firing rate of the ICD device was set 20 beats above the peak heart rate achieved during maximal exercise testing [5]. However, it seems more logical to perform a maximal exercise test with the ICD activated, because that way you can gather information about the reaction of the cardiac rhythm and the ICD to exercise. Also the result of the exercise test can give confidence to the patient that exercise at a predetermined level is safe and can be performed in the controlled environment of a cardiac rehabilitation centre. A recent study evaluated the safety of symptom-limited exercise testing in 400 ICD patients [7]. They performed exercise testing until symptoms or until the achievement of a heart rate 20 beats below the VT zone while remaining on pharmacological treatment. Most of the patients stopped exercise because of exhaustion (±70 %) or dyspnoea (±25 %). Signs of ischaemia were very rare, no ICD shock or antitachycardia pacing occurred and only in 16 out of the 400 patients exercise testing had to be stopped prematurely because of reaching the HR threshold without being exhausted. These results show that maximal or symptom-limited exercise testing in ICD patients with optimal pharmacological treatment is safe and feasible, but should only be performed in a professional and medical environment with continuous emphasis on safety measures. To ensure this safety precaution, information concerning the device settings should be available, and (Table 11.3) during testing and training, a (donut) magnet needs to be in the immediate vicinity of the patient to be able to interrupt eventual inappropriate interventions of the ICD.

In the beginning of a training programme, ECG monitoring during exercise is advisable to be able to document eventual exercise induced arrhythmias. The rehabilitation team should be well instructed about emergency measures in this particular patient population. They also have to know that they do not incur a risk by touching a patient while his ICD discharges, to avoid reactions of fear from the team members in case of an emergency.

Except from general safety recommendations when working with ICD patients, like thorough knowledge of the patient and the implanted device, the proximity of specialized ICD care and of course the active knowledge of the emergency procedure, some specific recommendations when training ICD patients are important. For the ICD lead to well grow in, a time interval of 4 weeks is mandatory before initiating any form of physical training and especially exercises which include movement of the left arm, as the ICD device is mostly implanted in the left pectoral muscle region. And although one would expect the ICD lead to have grown in, left arm hyperextension, arm ergometry and upper body strength exercises are to be postponed for at least 6 weeks after implantation. When exercises would include this left arm, low mobilization range and low intensity is mandatory. The patient needs correct information from the rehabilitation team to know which movements are acceptable.

Apart from complications related to surgery, most post-operative stress is caused by the possibility of experiencing an electrical shock and the lack of treatment of the underlying cause for implantation. It is logical to think that the implantation of a lifesaving device would make the patient confident of the improved life expectancy and relieve the fear of sudden death. But living with the possibility of receiving a defibrillating shock at any time can be emotionally devastating. Compared to the general population, quality of life and psychosocial adjustment are poor in patients with an ICD [8–10]. According to Sears et al. [9], ICD-specific fears and symptoms of anxiety are the most common symptoms experienced by patients with ICD. Moreover, 13–38 % of these patients experience diagnosable levels of anxiety. ICD-specific fears include fear of shock, fear of device malfunction, fear of death and fear of embarrassment. The health-related quality of life is also negatively associated with fear of exercise [8]. When comparing two groups of ICD patients according to the experience of a defibrillating shock, Jacq et al. concluded that exposure to shocks may lead to an increased risk of anxiety and depressive symptoms [11].

Social and working life can also be negatively influenced as there is a limitation in physical activity, due to the fear that stress or emotions might alert the device. Others may worry about their body image or avoid exercise and sexual activity because of fears of arrhythmias and discharge of the ICD. In some countries, driving is even, at least temporarily, prohibited. Also partners of ICD patients report feelings of helplessness and uncertainty about what to do if, or when, the ICD discharges. They worry about the reliability of the ICD and about their own position if their partner should die. This may commonly result in overprotection of the ICD patient, and partners often restrict or restrain them from doing physical activities. The importance of involving, educating and equipping partners with the relevant information and skills so that they can empower and support the patient to reach informed decisions should not be underestimated [12, 13]. In the absence of such interventions, the potential for misconceptions, misguided beliefs and marital conflicts can increase, perpetuating further uncertainty, fear and loss of control as well as precipitating physical symptoms. Various studies report on the psychological benefits for patients with an ICD after psychological intervention or comprehensive cardiac rehabilitation. Kohn et al. [14] studied cognitive behavioural therapy in patients with an ICD in a randomized controlled trial. They concluded that cognitive behavioural therapy was associated with decreased levels of depression and anxiety, and increased adjustment, particularly among those patients who received a shock [14]. Fitchet et al. reported decreased anxiety scores after 12 weeks of comprehensive cardiac rehabilitation (CR), including psychosocial counselling, in a randomized controlled trial [4]. These data demonstrate the importance of planning and organizing psychosocial support for patients with an ICD in comprehensive cardiac rehabilitation. However, a recent review concluded that more randomized controlled studies are warranted to better highlight the impact of CR and exercise training on anxiety, depression and quality of life [15]. But although more and more patients are treated with an ICD device, the referral from hospitals to cardiac rehabilitation centres is still negatively influenced by the fear of inappropriate shock delivery during exercise. The beneficial effects of cardiac rehabilitation in terms of secondary prevention and on physiological and psychosocial functioning of cardiac patients in general are well established [16].

In many countries, the law forbids patients with an ICD to drive trucks or transport people as a profession. This item needs to be addressed already in the pre-implantation period and from the beginning of the ambulatory rehabilitation programme. Reorientation or retraining for other professions can help the patient in finding a new job, which is a prerequisite for many to return to a “normal” life. Also counselling concerning sports participation is needed. Competition is to be avoided, but low level exercise such as doubles tennis, cycling, etc. can be performed [17].