Cardiopulmonary Exercise Testing Is an Accurate Tool for the Diagnosis of Pulmonary Arterial Hypertension in Scleroderma Related Diseases.

Mattia Bellan,Daniele Sola,Mario Pirisi,Giuseppe Patti,Roberta Pedrazzoli,Ailia Giubertoni,Debora Cromi,Ileana Gagliardi,Pier Paolo Sainaghi,Cristina Piccinino,Filippo Patrucco,Elisa Calzaducca,Mariachiara Buffa,Erika Zecca

doi:10.3390/ph14040342

Abstract

The early diagnosis of pulmonary arterial hypertension (PAH) is a major determinant of prognosis in patients affected by connective tissue diseases (CTDs) complicated by PAH. In the present paper we investigated the diagnostic accuracy of cardiopulmonary exercise testing (CPET) in this specific setting. We recorded clinical and laboratory data of 131 patients who underwent a CPET at a pulmonary hypertension clinic. Out of them, 112 (85.5%) had a diagnosis of CTDs; 8 (6.1%) received a diagnosis of CTDs-PAH and 11 (8.4%) were affected PH of different etiology. Among CPET parameters the following parameters showed the best diagnostic performance for PAH: peak volume of oxygen uptake (VO2; AUC: 0.845, CI95% 0.767–0.904), ratio between ventilation and volume of exhaled carbon dioxide (VE/VCO2 slope; AUC: 0.888, CI95%: 0.817–0.938) and end-tidal partial pressures (PetCO2; AUC: 0.792, CI95%: 0.709–0.861). These parameters were comparable among CTDs-PAH and PH of different etiology. The diagnostic performance was even improved by creating a composite score which included all the three parameters identified. In conclusion, CPET is a very promising tool for the stratification of risk of PAH among CTDs patients; the use of composite measures may improve diagnostic performance.

Highlights

Pulmonary arterial hypertension (PAH) is a progressive disease affecting the precapillary pulmonary vascular bed, leading to an increase in pulmonary vascular resistance and right ventricular failure, burdened by a high mortality rate [1]
8 (6.1%) received a diagnosis of connective tissue diseases (CTDs)-pulmonary arterial hypertension (PAH): 6 were affected by systemic sclerosis (SSc), 1 by overlap syndrome and 1 by mixed connective tissue diseases (MCTD); 6 patients were on endothelin receptor antagonists, 5 patients were receiving phosphodiesterare 5 inhibitors
11 (8.4%) patients received a diagnosis of PH of different etiology (5 PAH, 4 chronic thromboembolism, 1 unknown)

Summary

Introduction

Pulmonary arterial hypertension (PAH) is a progressive disease affecting the precapillary pulmonary vascular bed, leading to an increase in pulmonary vascular resistance and right ventricular failure, burdened by a high mortality rate [1]. The early diagnosis of SSc-PAH is difficult, since PAH is initially minimally symptomatic or asymptomatic, but absolutely pivotal: the early initiation of an effective treatment is the most relevant prognostic factor in patients affected by PAH [3]. This is why patients diagnosed with CTDs are commonly followed-up and screened for the development of PAH; the two-step algorithm DETECT is the most commonly used screening tool [4]. The. DETECT algorithm includes a first step in which patients are indicated to echocardiography according to a composite score derived from the following variables: forced vital. The detect score well performs in this setting, showing a very high sensitivity (96%), as required by any screening tool; the specificity is low (48%)

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