Avramidou V, Hatziagorou E, Kampouras A, Hebestreit H, Kourouki E,
Kirvassilis F, Tsanakas J

Pediatr Pulmonol. 2018 Jan;53(1):81-87. doi: 10.1002/ppul.23833. Epub 2017 Sep
26.

INTRODUCTION: FEV1 is often considered the gold standard to monitor lung disease
in cystic fibrosis (CF). Recently, there has been increasing interest in multiple
breath washout (MBW) and cardiopulmonary exercise testing (CPET) as alternative
or even more sensitive techniques. However, limited data exist on associations
among the above methods.
AIM: To evaluate the correlations between outcome measures of MBW and CPET and to
examine if ventilation inhomogeneity can predict exercise intolerance.
SUBJECTS AND METHODS: Ninety-seven children and adults with CF (47 males, mean
[range] age 14.9 (6.6; 26.7) years, mean FEV1 : 90.8% predicted, mean lung
clearance index [LCI]: 11.4, and mean peak oxygen uptake [VO2 peak]: 82.4%
predicted) performed spirometry, MBW, and CPET on the same day during their
admission or outpatient visit.
RESULTS: LCI, m1 /m0 , and m2 /m0 (P < 0.001) as well as VO2 peak%, breathing
reserve (BR), minute ventilation (VE)/VO2 (P < 0.001), and VE/carbon dioxide
release (VCO2 ) (P = 0.006) correlated significantly with FEV1 %. LCI, m1 /m0 ,
and m2 /m0 correlated with VO2 peak (P ≤ 0.001), VE (L/min) (P < 0.05), BR
(P < 0.01), VE/VO2 (P < 0.001), and VE/VCO2 (P < 0.01). Multiple regression
analysis showed that LCI could predict BR% (P < 0.001, r2 :0.272) and VE/VO2
(P < 0.001, r2 : 0.207) while LCI and FRC could predict VO2 peak% P < 0.001, r2 :
0.216) and VE/VCO2 (P < 0.001, r2 : 0.226).
CONCLUSION: Ventilation inhomogeneity as indicated by increased LCI is associated
with less efficient ventilation during strenuous exercise and negatively impacts
exercise capacity in CF.

Williams CA, Tomlinson OW, Chubbock LV, Stevens D, Saynor
ZL, Oades PJ, Barker AR

Pediatr Pulmonol. 2018 Jan;53(1):36-42. doi: 10.1002/ppul.23896. Epub 2017 Oct
24.

BACKGROUND: Maximal cardiopulmonary exercise testing is recommended on an annual
basis for children with cystic fibrosis (CF), due to clinically useful prognostic
information provided by maximal oxygen uptake (V̇ O2max ). However, not all
patients are able, or willing, to reach V̇O2max , and therefore submaximal
alternatives are required. This study explored the validity of the oxygen uptake
efficiency slope (OUES) as a submaximal measure of V̇O2max in children and
adolescents with CF.
METHODS: Data were collated from 72 cardiopulmonary exercise tests (36 CF, 36
controls), with OUES determined relative to maximal and submaximal parameters of
exercise intensity, time, and individual metabolic thresholds. Pearson’s
correlation coefficients, independent t-tests, and factorial ANOVAs were used to
determine validity.
RESULTS: Significant (P < 0.05) correlations with V̇O2max were observed for most
expressions of OUES, but were consistently weaker in CF (r = 0.30-0.47) when
compared to CON (r = 0.58-0.89). Mean differences for all OUES parameters between
groups were not significant (P > 0.05). When split by V̇O2max tertiles, minimal
significant differences were found between, and within, groups for OUES,
indicating poor discrimination of V̇O2max .
CONCLUSIONS: The OUES is not a valid (sub) maximal measure of V̇O2max in children
and adolescents with mild-to-moderate CF. Clinicians should continue to use
maximal markers (ie, V̇O2max ) of exercise capacity.

Aguiar Rosa S; Agapito A; Soares RM; Sousa L; Oliveira JA; Abreu A; Silva AS; Alves S; Aidos H; Pinto FF; Ferreira RC;

Revista Portuguesa De Cardiologia: Orgao Oficial Da Sociedade Portuguesa De Cardiologia = Portuguese Journal Of Cardiology: An Official Journal Of The Portuguese Society Of Cardiology [Rev Port Cardiol] 2018 May 15. Date of Electronic Publication: 2018 May 15.

Aim: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET).
Methods: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction.
Results: We analyzed 154 CPET cases. There were significant differences between groups, with the lowest peak oxygen consumption (VO2) values seen in patients with cardiac shunts (39% with Eisenmenger physiology) (17.2±7.1ml/kg/min, compared to 26.2±7.0ml/kg/min in tetralogy of Fallot patients; p<0.001), the lowest percentage of predicted peak VO2 in complex heart defects (50.1±13.0%) and the highest minute ventilation/carbon dioxide production slope in cardiac shunts (38.4±13.4). Chronotropism was impaired in patients with complex defects. Eisenmenger syndrome (n=17) was associated with the lowest peak VO2 (16.9±4.8 vs. 23.6±7.8ml/kg/min; p=0.001) and the highest minute ventilation/carbon dioxide production slope (44.8±14.7 vs. 31.0± 8.5; p=0.002). Age, cyanosis, CPET duration, peak systolic blood pressure, time to anaerobic threshold and heart rate at anaerobic threshold were predictors of the combined outcome of all-cause mortality and hospitalization for cardiac cause.
Conclusion: Across the spectrum of CHD, cardiac shunts (particularly in those with Eisenmenger syndrome) and complex defects were associated with lower functional capacity and attenuated chronotropic response to exercise.

D’Andrea A; Radmilovic J; Caselli S; Carbone A; Scarafile R; Sperlongano S; Tocci G; Formisano T; Martone F; Liccardo B; D’Alto M; Bossone E; Galderisi M; Golino P;

The International Journal Of Cardiovascular Imaging [Int J Cardiovasc Imaging] 2018 May 22. Date of Electronic Publication: 2018 May 22.

Anabolic-androgenic steroids (AAS) are used by power athletes to improve performance. However, the real effects of the chronic consumption of AAS on cardiovascular structures are subjects of intense debate. To detect by speckle tracking echocardiography (STE) underlying left atrial (LA) dysfunction in athletes abusing AAS and assess possible correlation between LA myocardial function and exercise capacity during cardiopulmonary stress test. 65 top-level competitive bodybuilders were selected (45 males), including 35 athletes misusing AAS for at least 5 years (users), 30 anabolic-free bodybuilders (non-users), compared to 40 age- and sex-matched healthy sedentary controls. Standard Doppler echocardiography, STE analysis and bicycle ergometric test were performed to assess LA myocardial function and exercise capacity. Athletes showed increased left ventricular (LV) mass index, wall thickness and stroke volume compared with controls, whereas LV ejection fraction, LV end-diastolic diameter and transmitral Doppler indexes were comparable between the three groups. Conversely, LA volume index, LV and LA strain and LV E/Em were significantly increased in AAS users. By multivariate analyses, LV E/Em (beta = - 0.30, p < 0.01), LA volume index (- 0.42, p < 0.001) and number of weeks of AAS use per year (- 0.54, p < 0.001) emerged as the only independent determinants of LA lateral wall peak STE. In addition, a close association between LA myocardial function and VO2 peak during cardiopulmonary exercise testing was evidenced (p < 0.001), showing a powerful incremental value with respect to clinical and standard echocardiographic data. STE represents a promising technique to assess LA myocardial function in athletes abusing steroids. AAS users showed a more impaired LA deformation, associated with reduced functional capacity during physical effort.

Reeves T; Bates S; Sharp T; Richardson K; Bali S; Plumb J; Anderson H; Prentis J; Swart M; Levett DZH

Perioperative Medicine (London, England) [Perioper Med (Lond)], ISSN: 2047-0525, 2018 May 04; Vol. 7, pp. 8

Erratum for: Perioper Med (Lond). 2018 Jan 26;7:2. (PMID: 29423173)

No abstract available

Tsuboi Y, Tanaka H, Nishio R, Sawa T, Terashita D, Nakayama K,
Satomi-Kobayashi S, Sakai Y, Emoto N, Hirata KI.

J Cardiopulm Rehabil Prev. 2017 Sep;37(5):341-346

PURPOSE: Pulmonary arterial hypertension (PAH) and chronic thromboembolic
pulmonary hypertension (CTEPH) are the main subgroups of pulmonary hypertension
(PH). Despite differences in their etiologies, both diseases are characterized by
vascular remodeling, resulting in progressive right heart failure. Noninvasive
periodic evaluation of exercise tolerance has become increasingly important.
Cardiopulmonary exercise testing (CPET) and a 6-minute walk test (6MWT) are now
both recommended for evaluating exercise tolerance, but there is insufficient
knowledge about possible differences in the associations of exercise tolerance
with right heart catheterization (RHC) data for patients with PAH and CTEPH.
METHODS: A retrospective study was performed with 57 patients with PH (24 with
PAH and 33 with CTEPH) all of whom underwent echocardiography, CPET, 6MWT, and
RHC.
RESULTS: For both patients with PAH and CTEPH, peak heart rate during CPET was
significantly higher than that from 6MWT, whereas minimum peripheral oxygen
saturation during CPET and 6MWT was similar. For patients with PAH, significant
correlations were observed between peak (Equation is included in full-text
article.)O2 and cardiac index (CI) (r = 0.59; P = .002) and between (Equation is
included in full-text article.)E/(Equation is included in full-text article.)CO2
slopes and CI (r =-0.46, P = .02), as well as a nonsignificant correlation
tendency for peak (Equation is included in full-text article.)O2 and pulmonary
vascular resistance (PVR) and for (Equation is included in full-text
article.)E/(Equation is included in full-text article.)CO2 and PVR (r =-0.39; P =
.05; and r = 0.39; P = .06, respectively). For patients with CTEPH, however, a
significant correlation was observed only between (Equation is included in
full-text article.)E/(Equation is included in full-text article.)CO2 slopes and
CI (r =-0.38; P = .02).
CONCLUSION: PH etiology should be considered when assessing exercise tolerance,
whereas CPET can be effective in addition to hemodynamic assessment by means of
RHC for periodic evaluation during followup.

Moneghetti KJ, Kobayashi Y, Christle JW, Ariyama M, Vrtovec
B, Kouznetsova T, Wilson A, Ashley E, Wheeler MT,
Myers J, Haddad F

Echocardiography. 2017 Aug;34(8):1179-1186. doi: 10.1111/echo.13623. Epub 2017
Jul 6

BACKGROUND: Left ventricular (LV) contractile reserve assessed using imaging and
cardiopulmonary exercise testing (CPX) has been shown to predict outcome in
patients with dilated cardiomyopathy (DCM). Few clinical studies have, however,
analyzed the relationship between them.
METHODS: A cohort of 75 ambulatory patients with DCM underwent stress treadmill
echocardiography with CPX. LV contractile reserve was calculated as absolute
change (ΔLVEF=LVEFpeak -LVEFrest ) and percent change (%LVEF=[(LVEFpeak -LVEFrest
)/LVEFpeak) ]×100) in LVEF, circumferential and longitudinal strain (LS).
Exercise capacity was measured as peak oxygen uptake (peak VO2 ) and ventilatory
efficiency as the slope of minute ventilation to CO2 production (VE/VCO2 slope).
Values of contractile reserve were compared to matched controls. We also explored
which metric of ventricular response (absolute or percent change) was less
dependent on baseline LV function.
RESULTS: Patients with DCM had a mean age, rest and peak LVEF of 44±10 years,
42±10% and 50±12%, respectively. Among parameters of contractile reserve, peak
cardiac output was the strongest parameter associated with peak VO2 (r=.63,
P<.001). Along with age, sex, and BMI, it explained more than 70% of the variance
in peak VO2 . In contrast, LVEF and LS were only weakly related to peak VO2 .
With regard to ventilatory efficiency, the strongest parameter that emerged was
right atrial volume index (r=.36, P<.001). Percent change in LVEF was more
independent of baseline function than absolute change.
CONCLUSION: Echocardiographic contractile reserve and CPX provide complementary
information. Percent change in contractile reserve was most independent of
baseline function, therefore may be preferred when analyzing the ventricular
response to exercise.

Kunutsor SK, Kurl S, Khan H, Zaccardi F, Rauramaa R, Laukkanen
JA.

Ann Med. 2017 Dec;49(8):698-709. doi: 10.1080/07853890.2017.1367958. Epub 2017
Aug 22

PURPOSE: We aimed to assess the associations of oxygen uptake at aerobic
threshold (VO2 at AT) with cardiovascular and all-cause mortality.
DESIGN: VO2 at AT was assessed in 1663 middle-aged men in a cohort study. Hazard
ratios (HRs) were calculated for sudden cardiac death (SCD), fatal coronary heart
disease (CHD) and cardiovascular disease (CVD) and all-cause mortality.
RESULTS: During a median follow-up of 25.6 years, 138 SCDs, 209 fatal CHDs, 333
fatal CVDs and 719 all-cause mortality events occurred. On adjustment for
established risk factors, the HRs (95% CIs) for SCD, fatal CHD, fatal CVD and
all-cause mortality were 0.48 (0.28-0.82), 0.48 (0.31-0.74), 0.57 (0.41-0.79) and
0.66 (0.53-0.82), respectively comparing extreme quartiles of VO2 at AT. On
further adjustment for peak VO2, the HRs were 0.87 (0.48-1.56), 0.83 (0.52-1.34),
0.91 (0.63-1.30) and 0.88 (0.69-1.12), respectively. Addition of VO2 at AT to a
standard CVD mortality risk prediction model was associated with a C-index change
of 0.0085 (95% CI: -0.0002-0.0172; p = .05) at 25 years.
CONCLUSIONS: VO2 at AT is inversely associated with cardiovascular and all-cause
mortality events, but the associations are partly dependent on peak VO2. VO2 at
AT may improve the prediction of the long-term risk for CVD mortality. KEY
MESSAGES Oxygen uptake at aerobic threshold (VO2 at AT), a cardiopulmonary
exercise testing parameter, may be a useful prognostic tool for adverse clinical
outcomes in the general population. In a population-based prospective cohort
study of men, VO2 at AT was inversely associated with cardiovascular and
all-cause mortality events and improved the prediction of cardiovascular
mortality. In populations who cannot achieve maximal VO2, VO2 at AT may serve as
a useful prognostic tool; however, further studies are warranted.

Takken T, Bongers BC, van Brussel M, Haapala EA, Hulzebos EHJ

Ann Am Thorac Soc. 2017 Jul;14(Supplement_1):S123-S128. doi:
10.1513/AnnalsATS.201611-912FR.

Aerobic fitness is an important determinant of overall health. Higher aerobic
fitness has been associated with many health benefits. Because myocardial
ischemia is rare in children, indications for exercise testing differ in children
compared with adults. Pediatric exercise testing is imperative to unravel the
physiological mechanisms of reduced aerobic fitness and to evaluate intervention
effects in children and adolescents with a chronic disease or disability.
Cardiopulmonary exercise testing includes the measurement of respiratory gas
exchange and is the gold standard for determining aerobic fitness, as well as for
examining the integrated physiological responses to exercise in pediatric
medicine. As the physiological responses to exercise change during growth and
development, appropriate pediatric reference values are essential for an adequate
interpretation of the cardiopulmonary exercise test.

Laohachai K, Winlaw D, Selvadurai H, Gnanappa GK, d’Udekem
Y, Celermajer D, Ayer J

J Am Heart Assoc. 2017 Aug 21;6(8). pii: e005750. doi: 10.1161/JAHA.117.005750.

BACKGROUND: Patients with a Fontan circulation have reduced exercise capacity and
respiratory muscle strength. Inspiratory muscle training (IMT) improves exercise
capacity and quality of life in adults with heart failure. We assessed whether
6 weeks of a home-based program of IMT improves inspiratory muscle strength and
the ventilatory efficiency of exercise in adolescent patients with a Fontan
circulation.
METHODS AND RESULTS: Twenty-three adolescent participants (aged 16±2 years) with
a Fontan circulation underwent 6 weeks of IMT for 30 minutes daily. Respiratory
muscle strength (maximal inspiratory pressure and expiratory pressure), lung
function, and exercise capacity (cardiopulmonary exercise testing) were assessed.
Fourteen of 23 participants also underwent exercise cardiac magnetic resonance
imaging to examine the effects of IMT on cardiac output and systemic and
pulmonary blood flow. Six weeks of IMT improved maximal inspiratory pressure by
36±24 cm H2O (61±46%) with no change in maximal expiratory pressure. Ventilatory
efficiency of exercise improved after 6 weeks of IMT (from 34.2±7.8 to 32.2±5.6,
P=0.04). In those who underwent exercise cardiac magnetic resonance imaging, IMT
increased resting cardiac output (from 4.2±1.2 to 4.5±1.0 L/min, P=0.03) and
ejection fraction (from 50.1±4.3 to 52.8±6.1%, P=0.03).
CONCLUSIONS: Six weeks of IMT is associated with improved inspiratory muscle
strength, ventilatory efficiency of exercise, and resting cardiac output in young
Fontan patients. IMT may be a simple beneficial addition to the current
management of Fontan patients, potentially reducing exercise intolerance and
long-term morbidity and mortality.