Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality
BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients. METHODS: Retrospective chart revie...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2019-01-01
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| Series: | Annals of Thoracic Medicine |
| Subjects: | |
| Online Access: | http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2019;volume=14;issue=1;spage=83;epage=89;aulast=Al |
| _version_ | 1818550598435340288 |
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| author | Hadil AK Al Otair Majdy M Idrees Sarfraz A Saleemi Ahmed M Eltoukhy Ali A Alhijji Waleed A Al Habeeb Mohammed A Omair |
| author_facet | Hadil AK Al Otair Majdy M Idrees Sarfraz A Saleemi Ahmed M Eltoukhy Ali A Alhijji Waleed A Al Habeeb Mohammed A Omair |
| author_sort | Hadil AK Al Otair |
| collection | DOAJ |
| description | BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients.
METHODS: Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics.
RESULTS: Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m2. The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73–633.10) and 6MWD (OR 1.02: 95% CI; 1.01–1.03) were associated with the development of PAH.
CONCLUSION: Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH. |
| first_indexed | 2024-12-12T08:48:36Z |
| format | Article |
| id | doaj.art-2e0dfc3f34624e4f835331b0ce365668 |
| institution | Directory Open Access Journal |
| issn | 1817-1737 1998-3557 |
| language | English |
| last_indexed | 2024-12-12T08:48:36Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Annals of Thoracic Medicine |
| spelling | doaj.art-2e0dfc3f34624e4f835331b0ce3656682022-12-22T00:30:21ZengWolters Kluwer Medknow PublicationsAnnals of Thoracic Medicine1817-17371998-35572019-01-01141838910.4103/atm.ATM_33_18Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortalityHadil AK Al OtairMajdy M IdreesSarfraz A SaleemiAhmed M EltoukhyAli A AlhijjiWaleed A Al HabeebMohammed A OmairBACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients. METHODS: Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics. RESULTS: Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m2. The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73–633.10) and 6MWD (OR 1.02: 95% CI; 1.01–1.03) were associated with the development of PAH. CONCLUSION: Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH.http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2019;volume=14;issue=1;spage=83;epage=89;aulast=AlMortalitypulmonary arterial hypertensionSaudi Arabiasystemic sclerosis |
| spellingShingle | Hadil AK Al Otair Majdy M Idrees Sarfraz A Saleemi Ahmed M Eltoukhy Ali A Alhijji Waleed A Al Habeeb Mohammed A Omair Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality Annals of Thoracic Medicine Mortality pulmonary arterial hypertension Saudi Arabia systemic sclerosis |
| title | Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality |
| title_full | Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality |
| title_fullStr | Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality |
| title_full_unstemmed | Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality |
| title_short | Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality |
| title_sort | pulmonary arterial hypertension in saudi patients with systemic sclerosis clinical and hemodynamic characteristics and mortality |
| topic | Mortality pulmonary arterial hypertension Saudi Arabia systemic sclerosis |
| url | http://www.thoracicmedicine.org/article.asp?issn=1817-1737;year=2019;volume=14;issue=1;spage=83;epage=89;aulast=Al |
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