الفهرس 
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Abstract
SUMMARY
P
ulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest as assessed by right heart catheterization(5).
Pulmonary arterial hypertension (PAH) affects 0.5–15% of patients with connective tissue diseases (CTDs) and is one of the leading causes of mortality in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). Despite increasing recognition of PAH in CTDs, the diagnosis is often delayed, which may lead to unfavorable outcomes in these patients. (3)
RHC is required to confirm the diagnosis of PAH to assess the severity of haemodynamic impairment. When performed at expert centres, these procedures have low morbidity (1.1%) and mortality (0.055%) rates(5).
The aim of this work was to study CTD associated PAH guided by the novel ’’DETECT” algorithm.
This cross-sectional study was conducted upon 30 patients with connective tissue disease including 16 cases with SSc, 9 cases with SLE and 5 cases with RA.
The mean age for the study group was found to be 53.8 ± 5.57 years. The majority of cases were females (25 cases) while males were 5 cases.
A two-step composite score proposed in the novel evidence-based ”DETECT” algorithm(6) was applied to the included patients.
All patients included in the study underwent RHC to determine the diagnostic performance of the two steps of the algorithm and corresponding risk point cut-offs.
The following results were obtained from the study:
• All patients enrolled in the study underwent RHC and PAH was confirmed in 16 patients (53.3%) versus 14 (46.7%) non-PH patients.
• 21 patients had positive score (>300 risk points) by step 1 non-echocardiographic variables and were eligible for step 2 evaluation.
• 18 patients had positive score (>35risk points) by step 2 echocardiographic variables and were eligible for RHC.
• Sensitivity, specificity, PPV and NPV of step 1 of the algorithm to detect PAH were 87.5%, 50%, 66.7% and 77.7%, respectively.
• Sensitivity, specificity, PPV and NPV of step 2 of the algorithm to detect PAH were 92.8%, 28.5%, 72.2% and 66.7%, respectively.
• Estimated total sensitivity and specificity of step 1 and step 2 in diagnosis of PAH among all cases were 80% and 64%, respectively.
• New predefined sensitivity cut-off of 89% (corresponding to >298 risk points) of step 1 total prediction score was established.
• New predefined specificity cut-off of 50% (corresponding to >37.5 risk points) of step 2 total prediction score was established.
• Step 1 had 80% and 83.3% sensitivity and specificity; respectively in diagnosis of PAH among Ssc patients. PPV was 88.8% while NPV was 71.4%
• Step 2 had 100% sensitivity and didn’t show true negative or false negative cases of PAH among Ssc patients.
• Step 1 had 100% and 33.3% sensitivity and specificity; respectively in diagnosis of PAH among SLE patients. PPV was 75% while NPV was 100%.
• Step 2 had 83.3% and 50% sensitivity and specificity; respectively in diagnosis of PAH among SLE patients. PPV was 83.3% and NPV of 50%.
• Step 1 showed no true positive cases and 20% specificity in diagnosis of PAH among RA patients.
• Step 2 shows no true positive cases or false positive cases in diagnosis of PAH among RA patients.
• There was significant positive statistical correlation between mean PAP and FVC% predicted / DLco% predicted among the study group on the other hand there was significant negative statistical correlation between mean PAP and DLco% predicted