Why right is never left: the systemic right ventricle in transposition of the great arteries

Waterhouse, Benjamin R.; Bera, Katarzyna D.

doi:10.1113/jp271483

Cited by 4 publications

(2 citation statements)

References 5 publications

(9 reference statements)

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“…Thus, RV failure is caused by chronic microvascular ischemia and high wall stress, which lead to diffuse myocardial fibrosis and ongoing functional decline. 8 Roest and colleagues, 9 who used cardiovascular magnetic resonance to analyze ventricular function at rest and during exercise, found that RV ejection fraction did not increase during exercise and that RV functional reserve decreased. This is caused by morphologic changes of the SRV over time.…”

Section: Discussionmentioning

confidence: 99%

Clinical Evaluation of Exercise Capacity in Adults with Systemic Right Ventricle

Róg¹,

Sałapa²,

Okólska³

et al. 2019

Texas Heart Institute Journal

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The right ventricle provides systemic circulation in individuals with congenitally corrected transposition of the great arteries (CCTGA) and in those with complete transposition who have had an atrial switch repair (DTGA). The aim of this study was to evaluate how the systemic right ventricle adapts to increased workload and oxygen demand during exercise. From November 2005 through December 2015, 3,358 adult patients with congenital heart disease were treated at our institution; we identified 48 (26 females, 22 males; median age, 25.4 ± 8.1 yr) who met the study criteria; 37 had DTGA and atrial switch repair, and 11 had CCTGA. We studied their echocardiographic and cardiopulmonary exercise test results. A control group consisted of 29 healthy sex- and age-matched volunteers. On exercise testing, oxygen uptake at anaerobic threshold, peak oxygen uptake, peak heart rate, and percentage of maximal heart rate were significantly lower in the group with systemic right ventricle than in the control group (all P <0.001); in contrast, the peak ventilatory equivalent for carbon dioxide was higher in the study group (P=0.013). Impaired systemic right ventricular function reduced peak oxygen uptake. The peak heart rate was lower in the CCTGA group than in the DTGA group. Our results indicate that reduced exercise capacity is related to impaired systemic right ventricular function, severe tricuspid valve regurgitation, and chronotropic incompetence. There was no correlation between cardiopulmonary exercise test results and time after surgery. Chronotropic efficiency is lower in individuals with CCTGA than in those with DTGA.

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Section: Discussionmentioning

confidence: 99%

Clinical Evaluation of Exercise Capacity in Adults with Systemic Right Ventricle

Róg¹,

Sałapa²,

Okólska³

et al. 2019

Texas Heart Institute Journal

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“…The latter patients are now adults, and lingering concerns about long-term sustainability of the right ventricle acting as the systemic ventricle are being addressed by exercise testing [ 29 , 30 ]. While much work still needs to be done, exercise testing—particularly with the measurement of stroke volume—permits identification of those who benefit from exercise training, and by default, those in whom pharmacologic therapy may be indicated [ 31 , 32 ].…”

Section: Discussionmentioning

confidence: 99%

Pediatric Exercise Testing: Value and Implications of Peak Oxygen Uptake

et al. 2017

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Peak oxygen uptake (peakV˙O2) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. PeakV˙O2 was shown to predict longevity in patients aged 7–35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peakV˙O2 as an endpoint that likewise delivered clinically relevant data. Cardiopulmonary exercise testing provides clinicians with biomarkers and clinical outcomes, and researchers with novel insights into fundamental biological mechanisms reflecting an integrated physiological response hidden at rest. Momentum from these pioneering observations in multiple disease states should impel clinicians to employ similar methods in other patient populations; e.g., sickle cell disease. Advances in pediatric exercise science will elucidate new pathways that may identify novel biomarkers. Our initial aim of this essay is to highlight the clinical relevance of exercise testing to determine peakV˙O2, and thereby convince clinicians of its merit, stimulating future clinical investigators to broaden the application of exercise testing in pediatrics.

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