Abstract
BackgroundPulmonary arterial hypertension (PAH) is one of the most relevant causes of death in systemic sclerosis. The aims of this study were to analyse the recently published DETECT algorithm comparing it with European Society of Cardiology/European Respiratory Society (ESC/ERS) 2009 guidelines: as screening of PAH; (2) identifying median pulmonary arterial pressure (mPAP) ≥21 mmHg; and (3) determining any group of pulmonary hypertension (PH).MethodsEighty-three patients fulfilling LeRoy’s systemic sclerosis diagnostic criteria with at least right heart catheterization were studied retrospectively. Clinical data, serological biomarkers, echocardiographic and hemodynamic features were collected. SPSS 20.0 was used for statistical analysis.ResultsAccording to right heart catheterization findings, 35 patients with PAH and 28 with no PH met the standards for DETECT algorithm analysis: 27.0% of patients presented with functional class III/IV. Applying DETECT, the sensitivity was 100%, specificity 42.9%, the positive predictive value 68.6% and the negative predictive value 100%, whereas employing the ESC/ERS guidelines these were 91.4%, 85.7%, 88.9% and 89.3%, respectively. There were no missed diagnoses of PAH using DETECT compared with three patients missed (8.5%) using ESC/ERS guidelines. The DETECT algorithm also showed greater sensitivity and negative predictive value to identify patients with mPAP ≥21 mmHg or with any type of PH.ConclusionsThe DETECT algorithm is confirmed as an excellent screening method due to its high sensitivity and negative predictive value, minimizing missed diagnosis of PAH. DETECT would be accurate either for early diagnosis of borderline mPAP or any group of PH.
Highlights
Pulmonary arterial hypertension (PAH) is one of the most relevant causes of death in systemic sclerosis
Baseline characteristics Comparing the indications for right heart catheterization (RHC) in 83 patients, the proportions of patients diagnosed with pulmonary hypertension (PH) were 24/35 patients (68.5%) with right ventricular systolic pressure (RVSP) >36 mmHg plus forced vital capacity (FVC)/Diffusing capacity for carbon monoxide (DLCO) ratio >1.6, 23/34 patients (67.6%) with RVSP >36 mmHg, 4/8 patients (50.0%) with FVC/DLCO ratio >1.6 and 1/6 patients (16.6%) with progressive unexplained dyspnoea
There were no statistical differences in positivity to antinuclear antibodies or the observation of a nucleolar pattern in the indirect immunofluorescence, patients with PAH more often had Anticentromere antibodies (ACA) (n = 23, 65.7% vs n = 11, 39.3%, p = 0.03), but lower positivity against antitopoisomerase I antibodies (n = 3, 8.6% vs n = 9, 32.1%, p = 0.01)
Summary
Pulmonary arterial hypertension (PAH) is one of the most relevant causes of death in systemic sclerosis. Pulmonary arterial hypertension (PAH) is one of the most severe complications in patients with SSc, in whom the prevalence is 10% as diagnosed by right heart catheterization (RHC) [3, 4]. PAH has a major negative impact on survival and has become one of the leading causes of SSc-related deaths [5,6,7]. The outcome of this disease is poor, with a 51% survival rate at 5 years of diagnosis even after the introduction of potent vasodilatator agents [8]. RHC was carried out if the tricuspid regurgitant velocity (TRV) was ≥3.4 m/s, TRV between 2.8-3.4 m/s with symptoms or TRV ≤2.8 m/s with symptoms plus additional echocardiographic variables suggestive of PH
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