Exercise physiology in pulmonary hypertension patients with and without congenital heart disease

Background Cardiopulmonary exercise testing allows the assessment of integrative cardiopulmonary response to exercise. Aims The aim of the study was to better understand the exercise physiology in pulmonary arterial hypertension related to adult congenital heart disease compared to non-adult congenital heart disease patients by means of cardiopulmonary exercise testing parameters. Methods The present is a multicentre retrospective study which includes pulmonary hypertension group 1 and group 4 patients. All subjects underwent full clinical and instrumental evaluation, including cardiopulmonary exercise testing and right heart catheterization. Results One hundred and sixty-seven pulmonary hypertension patients (93 women and 74 men, 57 adult congenital heart disease and 110 non-adult congenital heart disease) were enrolled. Adult congenital heart disease patients had higher pulmonary pressure (mean pulmonary arterial pressure: 59.8 ± 19.5 mmHg vs 44.6 ± 16.5 mmHg, p < 0.001) and lower pulmonary blood flow (pulmonary blood flow: 3.3 (2.1–4.3) l/min vs 4.5 (3.8–5.4) l/min, p < 0.001). At cardiopulmonary exercise testing they had lower peak oxygen uptake/kg (12.8 ± 3.8 ml/kg/min vs 15.5 ± 4.2 ml/kg/min, p < 0.001) and higher ventilation/carbon dioxide elimination slope (53.2 (43.3–64.8) vs 44.0 (34.6–51.6), p < 0.001). When patients were paired for gender and peak oxygen uptake ( ± 1 ml/kg/min), obtaining 44 pairs, adult congenital heart disease patients had higher pulmonary pressure (mean pulmonary arterial pressure: 58.4 ± 20.2 mmHg vs 42.8 ± 16.8 mmHg, p < 0.001) and ventilation/carbon dioxide elimination slope (51.2 (43.4–63.6) vs 44.9 (35.4–55.1), p = 0.033). Conclusions In pulmonary arterial hypertension-adult congenital heart disease patients, pulmonary pressure and ventilation/carbon dioxide elimination slope are higher compared to non-adult congenital heart disease pulmonary hypertension patients, while pulmonary blood flow and peak oxygen uptake are lower. After matching patients for gender and peak oxygen uptake, pulmonary pressure and ventilation/carbon dioxide elimination remain higher in adult congenital heart disease patients suggesting that the long-term adaptation to high pulmonary pressure, hypoxia and low pulmonary blood flow, as well as a persisting shunt has, at least partially, preserved exercise performance of pulmonary arterial hypertension-adult congenital heart disease patients.

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Cardiopulmonary Exercise Testing—A Valuable Tool, Not Gatekeeper When Referring Patients With Adult Congenital Heart Disease for Transplant Evaluation

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118825263 ◽

2019 ◽

Vol 10 (3) ◽

pp. 286-291 ◽

Cited By ~ 5

Author(s):

Jonathan N. Menachem ◽

Nosheen Reza ◽

Jeremy A. Mazurek ◽

Danielle Burstein ◽

Edo Y. Birati ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Exercise Testing ◽

Congenital Heart ◽

Adult Congenital Heart Disease ◽

Cardiopulmonary Exercise Testing ◽

Peak Oxygen Consumption ◽

Cardiopulmonary Exercise ◽

Advanced Therapies ◽

Adult Congenital

Introduction: Treatment of patients with adult congenital heart disease (ACHD) with advanced therapies including heart transplant (HT) is often delayed due to paucity of objective prognostic markers for the severity of heart failure (HF). While the utility of Cardiopulmonary Exercise Testing (CPET) in non-ACHD patients has been well-defined as it relates to prognosis, CPET for this purpose in ACHD is still under investigation. Methods: We performed a retrospective cohort study of 20 consecutive patients with ACHD who underwent HT between March 2010 and February 2016. Only 12 of 20 patients underwent CPET prior to transplantation. Demographics, standard measures of CPET interpretation, and 30-day and 1-year post transplantation outcomes were collected. Results: Patient Characteristics. Twenty patients with ACHD were transplanted at a median of 40 years of age (range: 23-57 years). Of the 12 patients who underwent CPET, 4 had undergone Fontan procedures, 4 had tetralogy of Fallot, 3 had d-transposition of the great arteries, and 1 had Ebstein anomaly. Thirty-day and one-year survival was 100%. All tests included in the analysis had a peak respiratory quotient _1.0. The median peak oxygen consumption per unit time (_VO2) for all diagnoses was 18.2 mL/kg/min (46% predicted), ranging from 12.2 to 22.6. Conclusion: There is a paucity of data to support best practices for patients with ACHD requiring transplantation. While it cannot be proven based on available data, it could be inferred that outcomes would have been worse or perhaps life sustaining options unavailable if providers delayed referral because of the lack of attainment of CPET-specific thresholds.

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