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In carriers of PLN p.(Arg14del), an exercise history of mild-moderate exercise prior to clinical presentation does not predict: (1) development of ventricular arrhythmias or heart failure (time/severity) or (2) a diagnosis of cardiomyopathy.
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There is no reason to limit mild-moderate exercise in PLN p.(Arg14del) individuals without signs or symptoms.
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As malignant ventricular arrhythmias occur in nearly 50% of these individuals while being active, a clinical cardiomyopathy diagnosis or the presence of arrhythmia-related risk factors (decreased left ventricular ejection fraction, prior sustained or non-sustained ventricular tachycardia, low-voltage electrocardiogram, number of leads with negative T waves or premature ventricular contraction count) warrant discussion of exercise restrictions.
Introduction
Methods
Study population
Genotype and phenotype
Clinical variables
Exercise interviews
Analysis of exercise history
Statistical analysis
Results
Study population
Characteristic | Total (N = 207) | Active (n = 104)a | Inactive (n = 103)b | P-value |
---|---|---|---|---|
Male | 81 (39.1) | 47 (45.2) | 34 (33.0) | 0.064 |
Proband | 48 (23.2) | 24 (23.1) | 24 (23.3) | 0.608 |
Caucasian | 207 (100) | 104 (100) | 103 (100) | 1 |
Age at interview, years | 53 ± 15 | 53 ± 13 | 52 ± 16 | 0.707 |
Mean MET-hours per year until presentation | 1661 (852–3199) | 3199 (1662–5623) | 852 (381–1190) | < 0.001 |
Presentation | ||||
Age at presentation, years | 45 ± 15 | 45 ± 14 | 44 ± 16 | 0.576 |
Type of presentation: | ||||
– Resuscitated SCD | 4 (1.9) | 4 (3.8) | 0 | 0.121 |
– Symptomatic | 48 (23.2) | 23 (22.1) | 25 (24.3) | 0.713 |
– Sustained VA | 13 (6.3) | 8 (7.7) | 5 (4.9) | 0.241 |
– Positive family history | 136 (65.7) | 66 (63.5) | 70 (68.0) | 0.495 |
At last follow-up visit | ||||
Age at last clinical follow-up, years | 52 ± 15 | 52 ± 13 | 51 ± 16 | 0.716 |
Task Force Criteria met | 19 (9.2) | 10 (9.6) | 9 (8.7) | 0.259 |
Fulfilment DCM diagnosis | 52 (25.1) | 28 (26.9) | 24 (23.3) | 0.193 |
ICD implantation | 72 (34.8) | 38 (36.5) | 34 (33.0) | 0.594 |
Sustained VA: | 38 (18.4) | 19 (18.3) | 19 (18.4) | 0.974 |
– Sustained VT/VF | 18 (8.7) | 11 (10.6) | 7 (6.8) | 0.334 |
– Appropriate ICD shock | 15 (7.2) | 7 (6.7) | 8 (7.8) | 0.774 |
Hospitalisation for heart failure | 19 (9.2) | 10 (9.6) | 9 (8.7) | 0.827 |
RVEF < 45% | 39 (30.2) | 24 (34.3) | 15 (25.4) | 0.275 |
LVEF < 45% | 49 (23.7) | 28 (35) | 21 (29.2) | 0.442 |
Cardiac transplantation | 11 (5.3) | 7 (6.7) | 4 (3.9) | 0.834 |
Death | 2 (1) | 0 | 2 (1.9) | 0.224 |
Follow-up duration, years | 5.9 (2.8–9.1) | 6 (3.1–8.8) | 5.6 (2.3–9) | 0.692 |
Exercise history
Time spent on activity | Before presentation | After presentation | P-value |
---|---|---|---|
Mean MET-hours/year spent on any activitya | 1661 (852–3200) | 1152 (199–2880) | < 0.001 |
– Leisure activity | 490 (168–1148) | 282 (0–720) | < 0.001 |
– Work activity | 607 (0–2239) | 0 (0–2095) | 0.004 |
– Transportation activity | 0 (0–203) | 0 | < 0.001 |
Mean hours/year spent on any activity | 367 (147–1038) | 180 (36–840) | < 0.001 |
– Leisure activity | 79 (28–162) | 45 (0–137) | 0.002 |
– Work activity | 178 (0–850) | 0 (0–720) | 0.028 |
– Transportation activity | 0 (0–49) | 0 | < 0.001 |
Assigned METs of activities performed | 4.1 (3.1–5.2) | 3.4 (2.6–5.0) | < 0.001 |
Association of exercise prior to presentation with clinical outcomes
Association of exercise prior to presentation with clinical outcomes during follow-up
Characteristic | Total (N = 198) | Active (n = 99) | Inactive (n = 99) | P-value |
---|---|---|---|---|
Male | 79 (39.9) | 46 (46.5) | 33 (33.3) | 0.059 |
Age at presentation, years | 44 | 43 | 45 | 0.228 |
Non-sustained VT | 33 (16.7) | 17 (17.2) | 16 (16.2) | 0.849 |
Negative T wave in precordial leads (V1–V6) | 33/159 (20.8) | 17/79 (21.5) | 16/80 (22.2) | 0.813 |
Negative T wave in inferior leads (II, III, aVF) | 18/159 (11.3) | 11/79 (13.9) | 7/80 (8.8) | 0.303 |
Microvoltages on ECG | 34/161 (21.1) | 16/80 (20) | 18/81 (22.2) | 0.730 |
RVEF < 45% | 23/138 (16.7) | 16/69 (27.1) | 7/69 (10.1) | 0.07 |
LVEF < 45% | 33/140 (23.6) | 19/70 (27.1) | 14/70 (20) | 0.412 |
PVC count > 500 | 27/76 (35.5) | 14/35 (40) | 13/41 (31.7) | 0.446 |