Cardiopulmonary exercise testing in adult congenital heart disease

Valentina Mantegazza, Anna Apostolo, Alfred Hager

Research output: Contribution to journalArticlepeer-review


Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries, and new issues are emerging: the evaluation of their capacity to cope with physical activity and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool, because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, and/or pulmonary hypertension), and help prescribe an individualized rehabilitation program when needed. The common findings on cardiopulmonary exercise testing in patients with congenital heart diseases are a reduced peak V O2, an early anaerobic threshold, a blunted heart rate response, a reduced increase of VT, and an increased V E/V CO2. All these measures suggest common pathophysiological abnormalities: (1) a compromised exercise capacity from anomalies affecting the heart, vessels, lungs, or muscles; (2) chronotropic incompetence secondary to cardiac autonomic dysfunction or b-blockers and antiarrhythmic therapy; and (3) ventilatory inefficiency caused by left-heart failurewith pulmonary congestion, pulmonary hypertension, pulmonary obstructive vascular disease, or cachexia. Most of these variables also have prognostic significance. For these patients, cardiopulmonary exercise testing allows evaluation and decisions affecting lifestyle and therapeutic interventions.

Original languageEnglish
Pages (from-to)S93-S101
JournalAnnals of the American Thoracic Society
Publication statusPublished - Jul 1 2017


  • Congenital heart defects
  • Effort intolerance
  • Functional capacity

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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