| LUNG (PULMONARY): MAIN MENU |
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| Pulmonary Hypertension Diagnosis |
| This page was written by Shelley Ensz, and has not been medically edited. Systemic sclerosis (scleroderma) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer |
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| Overview of Diagnosis of PH/PAH in Systemic Sclerosis (Scleroderma) |
| All scleroderma patients should be screened for PAH and PH on a regular basis. |
| Prevalence of pulmonary arterial hypertension (PAH) in an Australian scleroderma population: screening allows for earlier diagnosis. Screening may result in earlier diagnosis of PAH with, in general more mild disease. This is important, given that early treatment for PAH while patients are less symptomatic is associated with improved exercise tolerance and pulmonary haemodynamics: indices indicative of disease progression and clinical worsening. S. Phung. Internal Medicine Journal October 2009. |
| Early, Simple and Reliable Detection of Pulmonary Arterial Hypertension (PAH) in Systemic Sclerosis (SSc) (DETECT). A two-stage prospective observational cohort study in scleroderma patients to evaluate screening tests and the incidence of pulmonary arterial hypertension and pulmonary hypertension. This study is currently recruiting participants. Verified by Actelion, October 2008. ClinicalTrials.gov. (Also see: Clinical Trials) |
| Assessment of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis: Comparison of Noninvasive Tests with Results of Right-Heart Catheterization (RHC). Echo appeared to be the most useful among the noninvasive tests. However, due to the low sensitivity of noninvasive testing, RHC should remain the gold standard. (PubMed) J Rheumatol. 2008 Jan 15. |
| “PH cannot be diagnosed without right heart catheterization.” |
How is PH/PAH Diagnosed? Pulmonary function tests can be helpful. Blood pressure in the lung can be estimated and the size and function of the right ventricle assessed by Doppler echocardiography. However, Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. James R. Seibold, MD, International Scleroderma Network Newsletter. 09-30-07. |
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| How is Pulmonary Arterial Hypertension diagnosed? Diagnosis can be delayed for months or even years while other possible causes are investigated and as a result, Pulmonary Arterial Hypertension (PAH) is frequently only identified when it is already quite advanced. Actelion Pharmaceuticals. |
| Diagnostic Algorithm for Pulmonary Hypertension with Scleroderma |
| Pulmonary Diagnostic Algorithm for Patients With Scleroderma The Journal of Rheumatology. |
| DLCO in Pulmonary Hypertension |
| Carbon Monoxide Diffusing Capacity (DLCO) Correlates With Functional Pulmonary Vascular Surface Area In Patients With Pulmonary Arterial Hypertension (PAH) From Connective Tissue Disease (CTD). This is the first demonstration that the reduction in DLCO in PAH-CTD is at least in part related to loss of functional capillary surface area, and is proportional to its severity. D. Langleben THU0285 EULAR 2007. (Also see: Connective Tissue Disease) |
| Echocardiograms for Pulmonary Hypertension |
| Accuracy of Doppler Echocardiography in the Hemodynamic Assessment of Pulmonary Hypertension. Transthoracic Doppler echocardiography is recommended for screening for the presence of pulmonary hypertension. However, some recent studies have suggested that Doppler echocardiographicpulmonary artery pressure estimates may frequently be inaccurate. M. R. Fisher. Am. J. Respir. Crit. Care Med. January 22, 2009. |
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| Exercise Echocardiography of Pulmonary Hypertension |
| Noninvasive detection of early pulmonary vascular dysfunction in scleroderma. Non-invasive assessment of pulmonary circulation during physical exercise and/or hypoxia enables screening for pulmonary vascular dysfunction in scleroderma. Reichenberger F. (PubMed) Respir Med. 2009 Jun 2. |
Using a bicycle ergometer
after physical activity,
and doing an echocardiogram
may help identify PAH early,
before it becomes irreversible. |
A new method allows for the early diagnosis of pulmonary hypertension. Using the bicycle ergometer to measure pulmonary artery systolic pressure of the patient after physical activity and performing an echocardiogram are very reliable methods, since they may help to identify scleroderma earlier. This confirms the importance of performing an echocardiogram on patients suffering from scleroderma, since this fatal complication may be indentified before it becomes irreversible. EurekAlert! 11/13/07. |
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| Functional Classification of Pulmonary Hypertension |
| Functional class of patients. Patients with pulmonary hypertension are categorised into four functional classes. Imperial College Healthcare. 2010. |
| The Classification of Pulmonary Arterial Hypertension (PAH). The functional classification is the measure of the limits imposed on a patient by a disease. It is a critical element of the assessment of patients with pulmonary arterial hypertension (PAH). Functional classification is strongly predictive of mortality, and is an important factor in the choice of PAH therapy. Ask the Experts. Medscape Cardiology. 09/11/06. |
| Implementing the ESC/ERS pulmonary hypertension guidelines: real-life cases from a national referral centre. Pulmonary hypertension (PH) comprises a heterogeneous group of disorders characterised by increased pulmonary vascular resistance that results in progressive right ventricular failure. In order to translate current evidence into routine clinical practice, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) have recently jointly proposed evidence-based guidelines for the optimal management of different PH patient groups. D. Montani. EUROPEAN RESPIRATORY REVIEW, 2009;18: 272-290. |
| High Resolution Chest CT for Pulmonary Hypertension is Not Helpful for Diagnosing PH in Patients with Advanced Pulmonary Fibrosis |
| High-Resolution Chest CT Findings Do Not Predict the Presence of Pulmonary Hypertension in Advanced Idiopathic Pulmonary Fibrosis. Chest CT-determined fibrosis score, ground-glass opacity score, honeycombing score, total profusion score, diameter of the main pulmonary artery, and the ratio of the pulmonary artery to aorta diameter did not differ between those with and without PH . There was no significant correlation between mean pulmonary artery pressure and any of the chest CT-determined measures. Chest. 2007; 132:773-779. (Also see: Pulmonary Fibrosis) |
| CT assessment of main pulmonary artery diameter. Right cardiac catheterization is considered to be a gold standard for measuring pulmonary artery pressure (PAP). However, this is an invasive procedure and carries a risk of mortality and morbidity (1, 2). Therefore, researchers have carried out several studies seeking a reliable and reproducible diagnostic imaging method for the assessment of the pulmonary artery diameter in order to predict the PAP. Diagn Interv Radiol 2008; 14:72-74. |
| Markers for Pulmonary Hypertension in Systemic Sclerosis (Scleroderma) |
| Telangiectases in Scleroderma: A Potential Clinical Marker of Pulmonary Arterial Hypertension (PAH). Increased numbers of telangiectases strongly associate with the presence of pulmonary vascular disease. Telangiectases may be a clinical marker of more widespread aberrant microvascular disease in scleroderma. Shah AA. J Rheumatol. 2009 Dec 1. (Also see: Telangiectasia) |
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| Ventricular mass index (VMI) correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH). The VMI, as demonstrated by magnetic resonant imaging (MRI), correlates well with mean pulmonary artery pressure in patients with SSc and may have a role in non-invasively excluding clinically significant PAH in breathless SSc patients in whom echocardiographic screening has failed. Dan Hagger. Rheumatology. July 14, 2009. (Also see: Cardiac MRI) |
| Serum Vascular Endothelial Growth Factor (VEGF) levels are related to the presence of pulmonary arterial hypertension (PAH) in systemic sclerosis. Serum VEGF levels are increased in systemic sclerosis patients with systolic pulmonary artery pressure(sPAP) ≥ 35 mmHg. The correlation between VEGF levels and sPAP may suggest a possible role of VEGF in the pathogenesis of PAH in systemic sclerosis. Andriana I Papaioannou. BMC Pulmonary Medicine 2009, 9:18. (Also see: VEGF) |
| Potential biomarkers for detecting pulmonary arterial hypertension (PAH) in patients with systemic sclerosis. The elevation of the serum levels of endoglin and endothelin-1 could represent a useful tool as PAH biomarkers. Nevertheless, the diagnostic value of these markers needs to be determined by prospective studies. P. Coral-Alvarado. (SpringerLink) Rheumatology International 01/07/2009. |
| Identification of Target Antigens of Antifibroblast Antibodies(AFAs) in Pulmonary Arterial Hypertension (PAH). AFAs detected in patients with PAH recognize cellular targets playing key roles in cell biology and maintenance of homeostasis. B. Terrier. American Journal of Respiratory and Critical Care Medicine Vol 177. pp. 1128-1134, (2008). (Also see: Antibodies) |
| Antiphospholipid antibody syndrome and autoimmune diseases. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases. (PubMed) Hematol Oncol Clin North Am. 2008 Feb; 22(1):53-65. (Also see: Antibodies, Lupus, and Cardiac) |
| Anticardiolipin antibodies (aCL) are significantly associated with pulmonary arterial hypertension in systemic sclerosis (scleroderma) patients. |
Anticardiolipin antibodies (aCL) are significantly associated with pulmonary arterial hypertension in systemic sclerosis (scleroderma) patients. (Also see: What is Scleroderma?, Types of Scleroderma, and Antiphospholipid Syndrome) |
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| P-Wave Amplitude |
| P-wave amplitude and pulmonary artery pressure in scleroderma. P-wave amplitude analysis on the ECG may be helpful in the assessment of pulmonary hypertension in patients with scleroderma. PubMed. J Electrocardiol. 2006 Aug 17. |
| Right Heart Catheterization (RHC) for Pulmonary Hypertension |
| “PH cannot be diagnosed without right heart catheterization.” |
How is PH/PAH Diagnosed? Pulmonary function tests can be helpful. Blood pressure in the lung can be estimated and the size and function of the right ventricle assessed by Doppler echocardiography. However, Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. James R. Seibold, MD, International Scleroderma Network Newsletter. 09-30-07. |
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| Risk Factors for Pulmonary Hypertension |
| Determinants of Pulmonary Arterial Hypertension (PAH) in Scleroderma. The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. PubMed. Semin Arthritis Rheum. 2007 Jan 2. |
| Six-Minute Walk for Pulmonary Hypertension in Scleroderma Patients |
| Lack of Specificity of the 6-Minute Walk Test as an Outcome Measure for Patients with Systemic Sclerosis. The 6MWD relates to broad factors in SSc and these results raise doubts about the specificity of the 6MWD in this systemic disease, and its relevance to monitoring therapy. Yoland Schoindre. The Journal of Rheumatology. vol. 36 no. 7 1481-1485. July 2009. (Also see: Pulmonary Fibrosis) |
| Limitations to the 6-Minute Walk Test in Interstitial Lung Disease and Pulmonary Hypertension in Scleroderma. Pain may cause failure to reach a dyspnea limitation during 6MWT, especially in SSc patients without both ILD and PH. 6 MWT interpretation should include consideration of vascular, pulmonary, and musculoskeletal exercise limitations. M.C. Garin. Journal of Rheumatology February 1, 2009 vol. 36 no. 2 330-336 . (Also see: Pulmonary Fibrosis) |
| TAPSE as Predictor of Patient Survival |
| A simple, noninvasive test measures survival time in adult pulmonary hypertension. A test called tricuspid annular plane systolic excursion (TAPSE) measures echocardiographic abnormalities in the tricuspid valve. "Our results suggest that TAPSE is a robust measure of right ventricular function and a powerful predictor of patient survival in pulmonary hypertension." EurekAlert! 11/01/06. |
