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Cardiopulmonary exercise testing in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis.

Abstract

OBJECTIVES

The DETECT algorithm has been developed to identify SSc patients at risk for pulmonary arterial hypertension (PAH) yielding high sensitivity but low specificity, and positive predictive value. We tested whether cardiopulmonary exercise testing (CPET) could improve the performance of the DETECT screening strategy.

METHODS

Consecutive SSc patients over a 30-month period were screened with the DETECT algorithm and positive subjects were referred for CPET before the execution of right-heart catheterization. The predictive performance of CPET on top of DETECT was evaluated and internally validated via bootstrap replicates.

RESULTS

Out of 314 patients, 96 satisfied the DETECT application criteria and 54 were positive. PAH was ascertained in 17 (31.5%) and pre-capillary pulmonary hypertension in 23 (42.6%) patients. Within CPET variables, the slope of the minute ventilation to carbon dioxide production relationship (VE/VCO2 slope) had the best performance to predict PAH at right-heart catheterization [median (interquartile range) of specificity 0.778 (0.714-0.846), positive predictive value 0.636 (0.556-0.750)]; exploratory analysis on pre-capillary yielded a specificity of 0.714 (0.636-0.8) and positive predictive value of 0.714 (0.636-0.8).

CONCLUSION

In association with the DETECT algorithm, CPET may be considered as a useful tool in the workup of SSc-related pulmonary hypertension. The sequential determination of the VE/VCO2 slope in DETECT-positive subjects may reduce the number of unnecessary invasive procedures without any loss in the capability to capture PAH. This strategy had also a remarkable performance in highlighting the presence of pre-capillary pulmonary hypertension.

Authors+Show Affiliations

Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases.Cardiovascular Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan.Cardiovascular Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan. Department of Clinical Sciences and Community Health, University of Milan, Milan.Cardiovascular Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan.Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases.Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Italy.Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases.Cardiovascular Diseases Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan. Department of Clinical Sciences and Community Health, University of Milan, Milan.Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31637433

Citation

Santaniello, Alessandro, et al. "Cardiopulmonary Exercise Testing in a Combined Screening Approach to Individuate Pulmonary Arterial Hypertension in Systemic Sclerosis." Rheumatology (Oxford, England), 2019.
Santaniello A, Casella R, Vicenzi M, et al. Cardiopulmonary exercise testing in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford). 2019.
Santaniello, A., Casella, R., Vicenzi, M., Rota, I., Montanelli, G., Santis, M., ... Beretta, L. (2019). Cardiopulmonary exercise testing in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford, England), doi:10.1093/rheumatology/kez473.
Santaniello A, et al. Cardiopulmonary Exercise Testing in a Combined Screening Approach to Individuate Pulmonary Arterial Hypertension in Systemic Sclerosis. Rheumatology (Oxford). 2019 Oct 21; PubMed PMID: 31637433.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cardiopulmonary exercise testing in a combined screening approach to individuate pulmonary arterial hypertension in systemic sclerosis. AU - Santaniello,Alessandro, AU - Casella,Rosa, AU - Vicenzi,Marco, AU - Rota,Irene, AU - Montanelli,Gaia, AU - Santis,Maria De, AU - Bellocchi,Chiara, AU - Lombardi,Federico, AU - Beretta,Lorenzo, Y1 - 2019/10/21/ PY - 2019/03/26/received PY - 2019/09/11/revised PY - 2019/10/23/entrez KW - cardiopulmonary exercise testing KW - pulmonary arterial hypertension KW - screening KW - systemic sclerosis JF - Rheumatology (Oxford, England) JO - Rheumatology (Oxford) N2 - OBJECTIVES: The DETECT algorithm has been developed to identify SSc patients at risk for pulmonary arterial hypertension (PAH) yielding high sensitivity but low specificity, and positive predictive value. We tested whether cardiopulmonary exercise testing (CPET) could improve the performance of the DETECT screening strategy. METHODS: Consecutive SSc patients over a 30-month period were screened with the DETECT algorithm and positive subjects were referred for CPET before the execution of right-heart catheterization. The predictive performance of CPET on top of DETECT was evaluated and internally validated via bootstrap replicates. RESULTS: Out of 314 patients, 96 satisfied the DETECT application criteria and 54 were positive. PAH was ascertained in 17 (31.5%) and pre-capillary pulmonary hypertension in 23 (42.6%) patients. Within CPET variables, the slope of the minute ventilation to carbon dioxide production relationship (VE/VCO2 slope) had the best performance to predict PAH at right-heart catheterization [median (interquartile range) of specificity 0.778 (0.714-0.846), positive predictive value 0.636 (0.556-0.750)]; exploratory analysis on pre-capillary yielded a specificity of 0.714 (0.636-0.8) and positive predictive value of 0.714 (0.636-0.8). CONCLUSION: In association with the DETECT algorithm, CPET may be considered as a useful tool in the workup of SSc-related pulmonary hypertension. The sequential determination of the VE/VCO2 slope in DETECT-positive subjects may reduce the number of unnecessary invasive procedures without any loss in the capability to capture PAH. This strategy had also a remarkable performance in highlighting the presence of pre-capillary pulmonary hypertension. SN - 1462-0332 UR - https://www.unboundmedicine.com/medline/citation/31637433/Cardiopulmonary_exercise_testing_in_a_combined_screening_approach_to_individuate_pulmonary_arterial_hypertension_in_systemic_sclerosis L2 - https://academic.oup.com/rheumatology/article-lookup/doi/10.1093/rheumatology/kez473 DB - PRIME DP - Unbound Medicine ER -