Hansen K; Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA.;
Curran T; Reynolds L; Cameron C; Pymm J; O’Neill JA; Losi R; Sherman C;Ackermans E; Yin S; Singh T; Alexander ME; Gauvreau K; Gauthier N;

International journal of cardiology. Congenital heart disease [Int J Cardiol Congenit Heart Dis] 2024 Aug 27; Vol. 18, pp. 100540.
Date of Electronic Publication: 2024 Aug 27 (Print Publication: 2024).

Background: Change in the oxygen consumption (VO ₂ ) at the ventilatory anaerobic threshold (VAT) is an important outcome in research studies of children with congenital heart disease (CHD). The range of values reported by different raters for any given VAT is needed to contextualize a change in VAT in intervention studies.
Methods: Sixty maximal cardiopulmonary exercise tests (CPET) for CHD patients 8-21 years old were independently reviewed by six exercise physiologists and four pediatric cardiologists. For each of the unique rater pairs for the 60 CPETs, the absolute difference in VAT was calculated and displayed on a histogram to demonstrate the distribution of inter-rater variability. This method was repeated for subgroups of test modality (cycle/treadmill), patient factors (diagnoses, exercise capacity), and rater factors (cardiologist/physiologist, years of experience).
Results: Rater agreement was good with an intraclass correlation coefficient of 0.79-0.91 but the distribution of differences was broad. The median difference was 2.7 % predicted peak VO ₂ (60 mL/min, 1.0 mL/kg/min), the 75th percentile was 6.4 % (140 mL/min, 2.5 mL/kg/min), and the 95th percentile was 16.3 % (421 mL/min, 6.5 mL/kg/min). Distributions were similar for CPET modality and years of rater experience, but differed for other factors.
Conclusions: The baseline distribution of reported VAT is relatively broad, varied by units, and was not explained by differences in rater experience or test modality, but varies by patient factors. When evaluating clinical relevance, a change in the VO ₂ at VAT in response to an intervention of <6.5 % predicted falls within the majority (75th percentile) of expected variability and should be interpreted with caution.

FitzMaurice TS; Liverpool Centre for Cardiovascular Science, University of Liverpool, Liverpool, UK.;
Hawkes S; Liao Y; Cullington D; Bryan A; Redfern J; Ashrafi R;

International journal of cardiology. Congenital heart disease [Int J Cardiol Congenit Heart Dis] 2024 Sep 29; Vol. 18, pp. 100546.
Date of Electronic Publication: 2024 Sep 29 (Print Publication: 2024).

Introduction: Assessment of exercise capacity by cardiopulmonary exercise testing (CPET) in adults with congenital heart disease (CHD) is important for prognostication and preoperative assessment. Peak oxygen uptake (PVO ₂ ) is used commonly, but can be challenging due to the difficulties of undertaking maximal CPET testing in this population. We explored whether oxygen uptake efficiency slope (OUES) at ventilatory anaerobic threshold (VAT), the point during CPET at which OUES becomes strongly correlated with PVO ₂ , and is more reliably available from submaximal CPET, can predict PVO ₂ in adults with CHD.
Methods: We assessed consecutive individuals who completed maximal CPET at our cardiorespiratory centre, as part of routine service review, between March 2019 and August 2021, recording data such as PVO ₂ , VAT and OUES at various proportions of a maximal test (75 %, 90 %, 100 %, and VAT). We employed linear regression modelling to analyse the association between PVO ₂ and OUES at VAT, and subsequently create an equation to predict PVO ₂ from OUES at VAT. Parametric data are presented using Pearson’s correlation coefficient and non-parametric data using Spearman’s rho.
Results: We analysed 391 individuals (177 female, age 32 ± 11 years). Mean ± SD PVO ₂ was 23.3 ± 6.86 ml/min/kg or 1724 ± 540 ml/min, peak VE 86.7 ± 25.4 l/min. The point of VAT as a percentage of PVO ₂ achieved was 66.5 ± 9.4 %, and VAT as a percentage of predicted PVO ₂ 46.9 ± 11.4 %. PVO ₂ was correlated with OUES at 100 % (R = 0.91, P < .001), 90 % (R = 0.91, P < .001), 75 % (R = 0.89, P < .001) of maximum, and VAT (R = 0.83, P < .001). PVO ₂ (ml/min) could be predicted by: (OUES at VAT)∗685.245 + (BMI [kg/m ² ])∗5.045 + (FEV ₁ [l])∗223.620 – 153.205 .
Conclusions: OUES at VAT can be used to calculate PVO ₂ . To our knowledge, this is the first equation using OUES at VAT to predict PVO ₂ in adults with CHD. In a population who may find maximal CPET difficult, this may be a useful submaximal measurement of cardiovascular fitness, and to calculate PVO ₂ , which is commonly used in guideline-based decision making in CHD.

Stiefel M; Department of Cardiology, University Heart Center, University Hospital Zurich, 8091 Zurich, Switzerland.
Brito da Silva H; Schmied CM; NiederseerD;

Journal of cardiovascular development and disease [J Cardiovasc Dev Dis] 2024 Nov 27; Vol. 11 (12).
Date of Electronic Publication: 2024 Nov 27.

Introduction: Balancing the well-documented benefits of regular exercise, particularly its positive impact on cardiovascular risk factors like hypertension, with the potential risks for patients with aortic aneurysms presents a significant challenge. This narrative review aims to summarize the current evidence and guidelines to assist clinicians in making informed exercise and sports recommendations for patients with aortic aneurysms or post-aortic repair.
Methods: Nine clinical trials on the effect of exercise on abdominal aortic aneurysms (AAAs) were identified, including one study on cardiopulmonary exercise testing (CPET) in AAA patients. As no clinical trials on exercise in thoracic aortic aneurysms (TAAs) were found, we extrapolated data from other studies on exercise in aortic diseases, including data from patients who have had an aortic dissection, as well as three studies on cardiac rehabilitation (CR) and one study on CPET after proximal aortic repair. Review articles and guidelines were also incorporated to ensure a comprehensive overview of the topic.
Results: Currently, no clear correlation exists between intense sports activities and the development of aortic aneurysms or dissections.
Conclusions: Light to moderate physical activity appears safe and beneficial for patients with aortic aneurysms and post-aortic repair. Given the lack of evidence linking athletic activity to aortic complications, caution is warranted in restricting such activities for athletes, underscoring the importance of shared decision-making. Regular follow-up and optimal management of cardiovascular risk factors are essential.

Ahn HB; Department of Cardiology, Seongnam, Gyeonggi-do, Republic of Korea.;
Park J; Choi HJ; Choi HM; Hwang IC; Yoon YE; Cho GY;

American journal of preventive cardiology [Am J Prev Cardiol] 2024 Dec 06; Vol. 21, pp. 100913.
Date of Electronic Publication: 2024 Dec 06 (Print Publication: 2025).

Background: Given the high prevalence of stage A or B heart failure (HF), comprehensive screening for new-onset HF is cost-prohibitive. Therefore, further risk stratification is warranted to identify at-risk patients. This study aimed to evaluate the prognostic utility of cardiopulmonary exercise test (CPET) with bicycle stress echocardiography (BSE) in patients with stage A or B HF.
Methods: Among 687 consecutive patients who underwent CPET-BSE, 410 with stage A or B HF were analyzed. The association between the CPET-BSE parameters and adverse cardiac events (hospitalization for HF or cardiac-related death) was analyzed using the Cox proportional hazard model under univariate and multivariate analyses.
Results: After a median 9 years of follow-up, 47 (11.5 %) of the 410 patients had events. In the univariable analysis, age, diuretics, BUN, creatinine, peak oxygen uptake (VO2), ventilatory efficiency (VE/VCO ₂ ), time to VT and peak exercise, left atrial volume index, rest and exercise E/e’, and tricuspid regurgitation velocity demonstrated significant parameters. In multivariate analysis, VE/VCO ₂ (hazard ratio [HR] 1.205, 95 % CI 1.095-1.327) and VO ₂ at peak exercise (HR 1.164, 95 % CI 1.022-1.325), time to VT (HR 0.993, 95 % CI 0.989-0.997), and exercise E/e’ (HR 1.582, 95 % CI 1.199-2.087) were only independent predictors for events.
Conclusions: In patients with stage A or B HF, four parameters of CPET-BSE were good predictors of future development of HF or cardiac death. If patients are unable to perform complete exercise, the time to VT may serve as a sufficiently predictive parameter for clinical events.

Santana EJ; Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA, and other USA Universities
Cauwenberghs N; Celestin BE; Kuznetsova T; Gardner C; Arena R; Kaminsky LA; Harber MP; Ashley E;Christle JW; Myers J; Haddad F

Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2024 Dec 10; Vol. 11, pp. 1393363.
Date of Electronic Publication: 2024 Dec 10 (Print Publication: 2024).

Background: Indexing peak oxygen uptake (VO ₂ peak) to total body mass can underestimate cardiorespiratory fitness (CRF) in women, older adults, and individuals with obesity. The primary objective of this multicenter study was to derive and validate a body size-independent scaling metric for VO ₂ peak. This metric was termed exercise body mass (EBM).
Method: In a cohort of apparently healthy individuals from the Fitness Registry and the Importance of Exercise National Database, we derived EBM using multivariable log-normal regression analysis. Subsequently, we developed a novel workload (WL) equation based on speed (Sp), fractional grade (fGr), and heart rate reserve (HRR). The generalized equation for VO ₂ peak can be expressed as VO ₂ peak = Cst × EBM × WL, where Cst is a constant representing the VO ₂ peak equivalent of one metabolic equivalent of task. This generalized equation was externally validated using the Stanford exercise testing (SET) dataset.
Results: A total of 5,618 apparently healthy individuals with a respiratory exchange ratio >1.0 (57% men, mean age 44 ± 13 years) were included. The EBM was expressed as Mass (kg) ^0.63  × Height (m) ^0.53  × 1.16 (if a man) × exp (-0.39 × 10 ^-4  × age ² ), which was also approximated using simple sex-specific additive equations. Unlike total body mass, EBM provided body size-independent scaling across both sexes and WL categories. The generalized VO ₂ peak equation was expressed as 11 × EBM × [2 + Sp (in mph) × (1.06 + 5.22 × fGr) + 0.019 × HRR] and had an R ² of 0.83, p  < 0.001. This generalized equation mitigated bias in VO ₂ peak estimations across age, sex, and body mass index subgroups and was validated in the SET registry, achieving an R ² of 0.84 ( p  < 0.001).
Conclusions: We derived a generalized equation for measuring VO ₂ peak during treadmill exercise testing using a novel body size-independent scaling metric. This approach significantly reduced biases in CRF estimates across age, sex, and body composition.

Baral T; Department of Pharmacy Practice, Manipal College of Pharmaceutical Sciences, Manipal Academy of Higher Education, Manipal, KA, India.
Malakapogu P; Shyma Z; Kurian SJ;Benson R; Manu MK; Bagchi D; Miraj SS

Nutrition and health [Nutr Health] 2024 Dec 23, pp. 2601060241303814.
Date of Electronic Publication: 2024 Dec 23.

Aim: The systematic review summarizes the current evidence on the efficacy and safety of iron replacement in patients with pulmonary hypertension (PH). Methods: A systematic literature search was conducted in electronic databases like PubMed, Scopus, Web of Science, and Embase up to April 2024. Eligible studies investigating iron replacement therapy in pulmonary hypertension patients were included in the review. Quality assessment of included studies was performed using standardized risk of bias tools.
Results: Five studies met the study-eligible criteria and were included for review. Out of all final selected five studies, one was a randomized control trial (RCT), two were non-RCT, and two were observational studies. We observed an improvement in the six-minute walk distance (6MWD) test, iron indices, peak oxygen intake, and anaerobic threshold after the iron replacement. In all included studies, the iron replacement was tolerated well with no serious adverse events.
Conclusion: Regardless of the variation in the study design, positive effects were observed on multiple outcome measures like the 6MWD test, cardiopulmonary exercise test parameter, and iron indices upon iron replacement in PH patients with iron deficiency. Further controlled trials are needed to enable better treatment group comparisons. Exploring long-term impacts on comorbidities, mortality, and disease progression would provide valuable insights for managing pulmonary hypertension.

Morgan C; UK Service for Pulmonary Hypertension in Children, Great Ormond Street Hospital for Children London UK.
Idris N; Elterefi K; Di Ienno L; Constantine A;Quyam S; Bini R; Moledina S;

Pulmonary circulation [Pulm Circ] 2024 Dec 26; Vol. 14 (4), pp. e70011.
Date of Electronic Publication: 2024 Dec 26 (Print Publication: 2024).

The aim of this single-centre retrospective observational study was to evaluate the safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension. Children aged over 5 years who were established on sildenafil plus bosentan were offered to undergo a therapy switch from May 2014 to May 2021 and, if remaining in the service, followed up to May 2024. Children with Eisenmenger syndrome, open intra or extra-cardiac shunt, or with pulmonary hypertension-associated lung disease were excluded. As part of a structured clinical program children were assessed via walk test, echocardiography, cardiac magnetic resonance imaging (CMRI), cardiopulmonary exercise testing, and serum biomarkers. Fifty-two children were included, 33 in the switch group and 19 in the control group. Clinical characteristics at diagnosis and baseline assessments did not differ between groups. All children tolerated the medication switch. Over a median 13.0 [12.0,13.7] week follow-up in the switch group there was a significant improvement in World Health Organization functional class (WHO FC, p  < 0.001); reduction in estimated right ventricular systolic pressure by echocardiography of 7 mmHg ( p  = 0.03) and a 2% increase ( p  = 0.03) in right ventricular ejection fraction on CMRI. There was a sustained improvement in WHO FC ( p  < 0.01) in the switch group at medium-term follow-up of 40.9 [35.2,49.3] weeks. Long-term outcome of transplant- or Potts shunt-free survival was comparable between the two groups.
Competing Interests: AC has received an educational grant, payment for lectures/educational events, and nonfinancial support from Janssen‐Cilag Ltd. SM has acted as a consultant for Janssen‐Cilag Ltd and GSK. CM, NI, KE, LDI, SQ and RB declare no conflicts of interest.

Zeineddine RM; Department of Cardiovascular and Thoracic Surgery, Mayo Clinic, Phoenix, Ariz.
Farina JM; Shawwaf KA; Botros M; Saleeb A;Lackey JJ; D’Cunha J;Jaroszewski DE;

JTCVS techniques [JTCVS Tech] 2024 Sep 25; Vol. 28, pp. 194-202.
Date of Electronic Publication: 2024 Sep 25 (Print Publication: 2024).

Objective: Pectus arcuatum is a rare variant of pectus deformities that can cause varying degrees of cardiac compression. A review of the evaluation, surgical repair, and outcomes of pectus arcuatum is presented.
Methods: A retrospective review of all patients undergoing surgical treatment of pectus arcuatum at a single institution was conducted between January 1, 2010, and May 31, 2024. Descriptive statistics and surgical techniques are presented.
Results: Twenty patients underwent pectus arcuatum repair (median age, 22.9 years; 55.0% males, median Haller index 2.8 [interquartile range {IQR}, 2.2, 3.6]) during the study period. Preoperatively, all patients were bothered by their chest appearance and symptomatic, with the most common symptoms being exercise intolerance (95.0%), chest pain (90.0%), and shortness of breath (90.0%). Preoperatively, cardiopulmonary exercise testing was performed in almost half the patients with abnormal findings (median maximum oxygen consumption, 67.0% of predicted). A hybrid approach with sternal osteotomy and minimally invasive pectus excavatum repair was utilized in 19 out of 20 cases, with 1 case requiring sternal osteotomy only. Single wedge osteotomy was sufficient in most cases (70.0%). Median intraoperative time was 3.5 hours (IQR, 3.1, 4.2 hours). The adoption of cryoablation in 2018 significantly reduced hospital stays, from 5.0 days (IQR, 4.5, 6.0 days) to 3.0 days (IQR, 2.8, 5.0 days) ( P  < .001). At follow-up, all reported cosmetic satisfaction and most reported symptom improvement.
Conclusions: Pectus arcuatum can be successfully repaired with a hybrid surgical approach involving sternal osteotomy and Nuss bar placement. Symptomatic patients should be considered for surgery, with postoperative improvement expected.
Competing Interests: Dr Jaroszewski has collected consulting and IP/royalties through Mayo Clinic Ventures with Zimmer Biomet Inc, and is a speaker with AtriCure Inc. All other authors reported no conflict of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflict of interest.

Zhu WW; Department of Echocardiography, Beijing Chao Yang Hospital, Capital Medical University, Beijing, China.
Tian RY; Guo DC; Lin MM; Cai QZ; Qin YY; Ding XY; Lv XZ

Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2024 Nov 28; Vol. 11, pp. 1442263.
Date of Electronic Publication: 2024 Nov 28 (Print Publication: 2024).

Aims: This study applied exercise stress echocardiography (ESE) to identify risk factors associated with exercise intolerance in patients with chronic coronary syndrome (CCS).
Methods and Results: 90 CCS patients underwent a cardiopulmonary exercise test and ESE, assessing exercise capacity, left ventricular systolic and diastolic function, and systolic reserve. The patients were divided into two groups according to the percentage of predicted oxygen consumption (VO ₂ ) at peak (≥85%, normal exercise tolerance group; <85%, exercise intolerant group). The left ventricular ejection fraction, average mitral valve S’, and left ventricular global longitudinal strain were lower in the exercise intolerant group than in the normal group, but no significant differences were observed in myocardial work parameters at rest. The average mitral valve E/e’, EDVi/E/e’, and proportion of abnormal diastolic function at the peak were higher in the exercise intolerant group than in the normal group. Moreover, the Δ SVi and flow reserve were lower, but the Δ average mitral valve E/e’ was higher in the exercise-intolerant group. From univariate and multivariate logistic regression analysis, only peak EDVi/E/e’ and Δ SVi correlated independently with exercise intolerance in CCS patients. With cutoff values of 8.64 ml/m ² for peak EDVi/E/e’ and 12.17 ml/m ² for Δ SVi, the combination of these factors had an area under the receiver operating characteristic curve of 0.906 (95% confidence interval, 0.820-0.960) for the prediction of exercise intolerance in CCS patients.
Conclusion: Hemodynamic changes during exercise in CCS patients were effectively evaluated using ESE. An elevated peak EDVi/E/e’ and a decreased Δ SVi are independent risk factors for exercise intolerance in patients with CCS.