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 Table of Contents  
REVIEW ARTICLE
Year : 2018  |  Volume : 2  |  Issue : 3  |  Page : 65-69

Cardiac rehabilitation in patients with thoracic aortic disease: Review of the literature and design of a program


Department of Epidemiology, Cardiovascular Disease Prevention and Health Promotion, Institute of Cardiology, Warsaw, Poland

Date of Submission08-Aug-2019
Date of Decision27-Aug-2019
Date of Acceptance28-Aug-2019
Date of Web Publication27-Sep-2019

Correspondence Address:
Lukasz A Malek
Department of Epidemiology, Cardiovascular Disease Prevention and Health Promotion, Institute of Cardiology, Niemodlinska Street 33, 04-635 Warsaw
Poland
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/hm.hm_25_19

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  Abstract 

The pathology of aortic dilatation is still not fully understood. Increased aortic dimensions are found in patients with hypertension, bicuspid aortic valve or various elastopathies, most commonly with Marfan syndrome. In general, there is only a mild adaptation of the aortic root to physical activity. Although there is no clear evidence that physical activity significantly influences aortic root size, individuals with thoracic aortic disease (TAD) are generally discouraged from engaging in sports activities. Most of them resign from sports and moves toward sedentary life with all of its consequences such as obesity, hypertension, and worsening lipid profile. There are also many sedentary individuals with TAD who had never even started any physical activity for the same reasons. Athletes who survived aortic dissection may also be stressed about regaining sports activity. On the other hand, there is mounting data on the potentially beneficial influence of mild-to-moderate exercise in patients with TAD. Therefore, a design of a comprehensive, monitored, cardiac rehabilitation program for patients with TAD, presented in the current manuscript, is highly warranted. It is based on an available data and should help both doctors and patients with TAD to overcome restrictions regarding the safety of sports activity. If proven successful, it may be used in the future for exercise prescription in patients with TAD in the community setting.

Keywords: Aorta, dilatation, Marfan syndrome, rehabilitation


How to cite this article:
Malek LA. Cardiac rehabilitation in patients with thoracic aortic disease: Review of the literature and design of a program. Heart Mind 2018;2:65-9

How to cite this URL:
Malek LA. Cardiac rehabilitation in patients with thoracic aortic disease: Review of the literature and design of a program. Heart Mind [serial online] 2018 [cited 2022 Oct 2];2:65-9. Available from: http://www.heartmindjournal.org/text.asp?2018/2/3/65/268092


  Introduction Top


Thoracic aortic disease (TAD) is a unified term used to define enlargement of the aortic root and/or ascending aorta due to various diseases such as hypertension, bicuspid aortic valve, Marfan syndrome and other rare elasticities (Ehlers–Danlos, Loeys–Dietz, or familial aortic aneurysm).[1] Ascending aorta dilatation may lead to sudden cardiac death (SCD) in sedentary individuals and athletes (1%–5% of all SCD in athletes) due to aortic dissection and rupture, but also due to other forms of acute aortic diseases such as intramural hematoma or penetrating atherosclerotic ulcer.[2],[3],[4]

It has been established that regular and particularly intensive physical activity causes a mild increase of aortic dimensions. Other factors, beside genetic predisposition and hypertension, influencing thoracic aorta size include height, gender, and age.[5] Largest aortic dimensions are found in tall, male athletes, but they only rarely exceed 40 mm in males and 34 mm in females as demonstrated in a large cohort of 2317 Italian athletes age 25 ± 6 years.[6] These values form a 99th upper percentile with larger aortic dimensions found in only 1.3% of male athletes and 0.9% of female athletes. Another study, published recently, on thoracic aortic dimensions in 3781 British athletes age 19 ± 6 years has demonstrated similar upper limit for males (0.17% of cases above 40 mm), but significantly higher for women (38 mm with 0.4% of cases exceeding this upper cutoff value).[7] Importantly, however, hereditary forms of TAD were excluded from that study. Only one study on volleyball players, including per group definition tallest athletes, reported a higher prevalence of thoracic aorta dilatation (>40 mm in 8% of male athletes and >34 mm in 6% of female athletes).[8]

All individuals, including athletes, with aortic dimensions above the upper limit, should be thoroughly examined for the presence of the conditions, which might have caused the enlargement. The size of the aorta should be confirmed with more precise methods than transthoracic echocardiogram such as computed tomography (CT) or magnetic resonance imaging. The work-up should include family history and risk factors assessment, the use of Marfan syndrome scores and often genetic testing.[4],[9],[10] Any co-existing conditions, such as aortic valve disease and its severity as well as left ventricular size and systolic function, should be also taken into consideration.

For a long time, it has been believed that regular physical activity, in particular, strength training, in patients with TAD should be avoided, because of the risk of further aortic size expansion. It was hypothesized that bursts of high systolic blood pressure occurring during weight-lifting, might especially increase the risk of aortic dissection.[11] This is in line with the Laplace's law, where wall tension is proportional to transmural pressure and radius and inversely proportional to the wall thickness.[5]

In accordance with this current eligibility and disqualification recommendations for competitive athletes state that:[9]

  • Athletes with mildly increased aortic root size (40–42 mm in men and 36–39 mm in women may still engage in Class I (low static/dynamic) and Class II exercises (moderate static/dynamic, only moderate static not dynamic for Marfan syndrome), however without the risk of bodily collision and under strict echocardiographic or other imaging method surveillance every 12 months
  • Athletes with aortic root size exceeding 42 mm in men and 40 mm in women physical should limit activity to Class I exercises (such as bowling, cricket, curling, golf, riflery, and yoga).


When aortic root size exceeds 45 mm in patients with Marfan syndrome, 50 mm in case of the bicuspid aortic valve or 55 mm in the remaining population, surgical correction should be considered.[10]

Nevertheless, studies on athletes did not show large differences in thoracic aorta size among different sports or even suggested that sports does not have a strong influence on aortic root size.[12] The questionnaire study on aortic dissection survivors revealed that most of the patients did not engage in strenuous sports activities on a regular basis prior to dissection. They reported walking and sexual relations as most frequent activities with only low frequency of running (4%), cycling (12%), and weight-lifting (15%) similar to golf (11%) and yard-working or cleaning (9%).[13] In a multicenter forensic study aortic dissection in only 8% of cases occurred during exercise and much more frequently (60% of cases) during sleep or rest suggesting that sleep apnea and/or resting blood pressure could play a stronger role in dissection rather than bouts of increased blood pressure during exercise.[14] However, regular physical activity status of the deceased was not reported in that study. Following on that, a study on Italian athletes with aortic size >40 mm who were allowed to compete demonstrated that over the course of 8 ± 5 years there was only a mild increase of aortic size (from 40.9 ± 1.3 to 42.9 ± 3.6) without any case of aortic dissection.[15] Similar findings were reported in nonhereditary forms of TAD in a recent study by Gati et al., who showed that after a mean follow-up of 5 ± 1.5 years none of the studied athletes demonstrated a significant increase of the aortic root size.[7] There were no aortic events reported in the entire population from that study. Sports activity also did not seem to affect aortic root size in athletes with bicuspid aortic valve.[16]

A larger expansion of aortic size is usually observed only after cessation of sports career in the late-adulthood, which should also be taken into consideration.[15] In fact, as mentioned earlier age was shown to influence aortic size with a predicted increase of 0.89 mm in men and 0.68 mm in women for every decade of life beginning in mid-adulthood.[17] Therefore, it may be speculated that it is rather one of the preexisting conditions than sports itself, which leads to aortic dilatation. There is one study on former American football players, which raises concern in that matter. In that study, Gentry et al. reported a much higher prevalence of increased aortic size in 207 former athletes age 57 ± 10 years with 30% of cases exceeding 40 mm. Almost 9% of that group had aortic dilatation equal to or above 45 mm.[18] It is hypothesized that late aortic dilatation in that group might have been caused by injuries to the aortic wall during active career in this based on the body collisions sports.

To add more to that picture, a recent data on a mouse-model have shown that mild to moderate dynamic physical activity leads to a reduction in aortic diameter growth rate in comparison to sedentary mice.[19],[20] Apart from that, aortic wall became stronger and less prone to rapture under mechanical stress. The optimal intensity of exercise was achieved at 55%–65% of maximum oxygen uptake (VO2 max).

Considering the effects on some forms of exercise on resting sympathetic tone, blood pressure, renin-angiotensin-aldosterone, it seems plausible that the proposed program would prevent progression of aortic disease.


  Rationale for the Development of New Service Top


Studies presented above clearly demonstrate why current guidelines on limitation of sports activity in patients with TAD are based on a relatively low level of evidence.[9] Sports activity is known to cause beneficial changes in the human body extending beyond cardiovascular system by lowering the risk of neoplastic diseases, depression, and dementia and all-together leading to longer survival.[21] Sports activity was shown to decrease hypertension, in mild forms to the similar level as pharmacotherapy.[22],[23] A French study demonstrated that patients with TAD often have worse risk factors profile (including blood pressure control and lipid profile) in comparison to another secondary prevention group in a cardiac rehabilitation program.[24] Another study demonstrated that obesity in Marfan syndrome is related to a higher risk of aortic complications.[25]

Even less is known on patients after corrective surgical procedures for TAD. As mentioned already, they are also discouraged from participation in other than mild physical activities due to the risk of distal aorta disease.[9]

Unfortunately, many athletes found to have TAD are told to completely withhold sports activity, which cause high stress and fear of uncertainty about the future and strongly influences their quality of life.[26] Most of them stop training at all, while others dismiss check-ups and continue sports activities, putting themselves at risk of unmonitored disease progression. Both situations are not good.

Therefore, it seems reasonable to design a dedicated, comprehensive, monitored, cardiac rehabilitation, and surveillance program for patients with TAD. The program should offer detailed diagnostic work-up, sports cardiology counseling, lifestyle modification, exercise prescription, and implementation as well as long-term monitoring.


  the Design of the Program Top


The program will be based on:

  • The preliminary study of cardiac rehabilitation in patients with Marfan syndrome [27]
  • The cross-sectional survey mailed to survivors of aortic dissection along with accompanying commentary [13],[28]
  • The aneurysm CaRe trial design assessing cardiac rehabilitation versus. standard of care after aortic aneurysm repair [29]
  • The activity recommendations for postaortic dissection patients.[30]


The completed rehabilitation program in patients with TAD included two groups of 8 and 10 middle-aged patients of both sexes with Marfan syndrome.[26] Both groups underwent a 3 weeks' program with 1-year follow-up and with emphasis on endurance training. Exercise intensity was defined by target systolic blood pressure ≤160 mmHg as a stop criterium with at least 3 exercise units per day. This pilot study was safe and helped patients with Marfan syndrome in terms of physical fitness, health-related quality of life, and in terms of psychological well-being.

As shown above, even less is known in terms of cardiac rehabilitation in patients after aortic repair, where similar training program can be applied.

Therefore, I have decided to design a comprehensive program including a broad range of patients with TAD.

Principles of the program

Cardiac rehabilitation participants

  • Adult males and females between 18 and 65 years of age
  • Sedentary individuals and ex-athletes
  • TAD of different origin: Hereditary forms (bicuspid artic disease, familial, Marfan syndrome, and other elasticities), hypertension caused and idiopathic forms
  • Patients diagnosed with TAD under close monitoring and those who underwent thoracic aortic repair, but are currently stable and out of hospital setting and finished phases I-II of cardiac rehabilitation
  • Thoracic aortic dimensions 40–44 mm in males and 38–44 in females
  • Stable clinical condition (lack of: Unstable heart failure defined as left ventricular ejection fraction <50% and/or diastolic dysfunction Grade III or above, moderate or severe valvular disease, severe arrhythmias, coronary ischemia on noninvasive diagnostic tests or coronary stenosis of more than 50% on standard coronary angiography or moderate/severe stenosis on CT coronary angiography, untreated or poorly controlled hypertension, anemia, and overt hypothyroidism).


Exclusion criteria

  • Children
  • Thoracic aortic dimensions ≥45 mm
  • Unstable clinical conditions (as defined above)
  • Orthopedic difficulties/restriction
  • Lack of will to participate in the cardiac rehabilitation program.


Group size per program

Ten patients.

Setting of rehabilitation

Outpatient program (and for those patients after aortic repair who finished phase II rehabilitation program) with a planned self-monitored, long-term extension period.

Cardiac rehabilitation team

It includes sports cardiologist, cardiac rehabilitation specialist, clinical geneticist, physiotherapist, physical fitness trainer, dietician, psychologist, nurse, and secretary.

Duration of the program

Twelve weeks with planned long-term annual follow-up (by E-mail and in-person).

Assessment

At baseline and the end including medical tests with initial risk assessment, psychological assessment with quality of life questionnaires, physical fitness, and laboratory assessment.

Frequency of intervention

Three times/week.

Intensity

Light-to-moderate intensity (defined with the either of the two cutoff values: 55%–65% of maximal heart rate and systolic blood pressure ≤160 mmHg).

Time

20–60 min sessions.

Type

Aerobic endurance or light strength training of choice – preferably mixed (running, cycling, fitness training, and gymnastics).

Monitoring

Heart rate monitoring with polar watches.

Outcome measures

(1) Clinical (aortic root size and adverse events); (2) physical fitness assessment (exercise stress test on a treadmill or ergometer); (3) quality-of life measures (HeartQOL and Short-Form 36 (SF-36) questionnaires); and (4) life-style measures (body mass index [BMI], blood pressure control, and lipid profile).

Details of the program

The program is meant to be as comprehensive as possible, so after initial experience, it can be divided into several programs tailored to different subgroups of patients with TAD (former athletes, sedentary individuals, and recreational athletes). It should allow gaining initial experience on the status, needs and possibilities of that group of patients in terms of cardiac rehabilitation. It is presumed that most of the group will consist of sedentary individuals with low fitness level, but in terms of risk – a relatively low-to-moderate risk group.

The introduction to the program will be voluntary and advertised through TAD societies, sports clinics, and hospital units treating patients with TAD in the region.

The initial visit will be performed by a sports cardiologist and a cardiac rehabilitation specialist. It will include demographic and anthropometric data collection, medical history and family history collection, medical examination, and initial physical activity questionnaire set to determine: subjective level of fitness, preferred physical activities, exercise goals expected by the patient, occupation, functionality, depression/anxiety, sexual activity, and effect a TAD diagnosis patient's on life. It will also address any fears or stress issues related to participation in the program.

The initial diagnostic testing led by cardiologists will include electrocardiogram, transthoracic echocardiography, and blood pressure measurement (ambulatory blood pressure monitoring in cases of high normal blood pressure or suspicion of hypertension).

In case of suspicion of genetic forms of TAD patients will be referred to clinical geneticist specializing in aortopathies for further testing.

Uncontrolled hypertension or other issues related to medical therapy in TAD will be addressed before the initiation of the cardiac rehabilitation program.

Those who initially qualify and sign the agreement for participation in the program will undergo physical fitness assessment by exercise testing on a treadmill or ergometer to determine: objective physical fitness level, target heart rate values, blood pressure response during exercise, and safety of the exercise prescription. At this time, a final qualification for the program will be made.

This will be followed by education sessions (approximately 2 h) on pathogenesis of TAD, risks, and benefits of physical activity in TAD, avoidance of triggers leading to aortic enlargement (trauma, unaccustomed exertion, lifting heavy objects, emotional stress, scuba diving, isometric activities, and anaerobic contact sports), necessity of general lifestyle changes, and details of the cardiac rehabilitation program with questions and answers part.

Subsequently, patients will undergo other initial tests: blood draw for lipid profile, body composition assessment, blood pressure measurement, and a set of psychological tests led by psychologist with possible 1 h of individual consultation. Those will include a cardiac QOL questionnaire – HeartQOL (polish version) and a more general SF-36 questionnaire (polish version). There will be also a 30-min dietician consultation available.

After that, patients will be introduced (by physiotherapist and physical fitness trainer under supervision of sports cardiologist) to the details of the training program. Each patient will receive an individual exercise prescription based on established physical fitness level, preferred types of exercise (mixed), and individual goals.

From this point on patients will start a rehabilitation program with initially three sessions of 30 min of exercise, extended by 10 min every 2 weeks until 60 min and with intensity increased adequately to established targets (55%–65% of maximal heart rate and/or systolic blood pressure ≤160 mmHg). Blood pressure will be measured before and at the end of each session. Adequate heart rate will be ascertained with wearable monitors available on-site (polar watches).

A dedicated gym will be booked for training sessions with a treadmill, cycle ergometers, fitness equipment, and gymnastics pads. Trainings will be led and supervised by the trainer, physiotherapist, and one other person from the team (nurse, sports cardiologist, or cardiac rehabilitation specialist) – 1:3.3 ratio.

The final assessment (after 12 weeks) will include: (1) clinical measures (echocardiography with measurement of the aortic size, adverse events summary); (2) physical fitness assessment (exercise stress test on treadmill/ergometer as at baseline); (3) quality of life measures (Heart QOL and SF-36 questionnaires); (4) life-style measures (BMI, blood pressure control, and lipid profile).

After completion of the rehabilitation program patients will be encouraged to continue regular physical activities in the introduced form, but in the community setting with self-monitoring. They will have a possibility to contact the cardiac rehabilitation team or otherwise will be contacted bi-annually by mail and will be encouraged to report any adverse events and to undergo transthoracic echocardiography every 12 months with aortic size assessment.


  Conclusions Top


The study presented background and rationale for the development of the cardiac rehabilitation program in patients with TAD. Following that, the detailed program design has been proposed. Its implementation should increase physical activity in this group of patients with all of the benefits of healthy, active lifestyle while maintaining safety and close monitoring. However, before prescription, the presented program needs to be clinically validated.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
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