Enlarged right-sided dimensions and fibrosis of the right ventricular insertion point on cardiovascular magnetic resonance imaging is seen early in patients with pulmonary arterial hypertension associated with connective tissue disease
- 5 October 2010
- journal article
- research article
- Published by Taylor & Francis Ltd in Scandinavian Journal of Rheumatology
- Vol. 40 (2), 133-138
- https://doi.org/10.3109/03009742.2010.507217
Abstract
Objectives: To describe the findings of cardiovascular magnetic resonance (CMR) imaging in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) and in consecutive patients with systemic sclerosis (SSc) without PAH. Methods: The study comprised nine consecutive patients who were admitted for right heart catheterization (RHC) under a suspicion of CTD-PAH and 25 consecutive patients who were admitted for evaluation because of a clinical suspicion of SSc. In addition to the regular assessment, they also underwent examination by CMR. Results: CMR measurements of right ventricular (RV) volumes and function showed severe pathology in patients with CTD-PAH. Patients with SSc without PAH had similar but much less severe findings. Right ventricular end-diastolic volume (RVEDV) and right ventricular ejection fraction (RVEF) were abnormal in all patients with CTD-PAH. In eight out of nine patients with CTD-PAH, fibrosis was seen in the RV insertion point, probably caused by increased tension, but only in one of the consecutive SSc patients. This patient was diagnosed with CTD-PAH 20 months later. Conclusions: In CTD-PAH, CMR shows severe changes in RV volumes and function, but also fibrosis in the RV insertion point. Similar abnormalities, although much less severe, may be seen at diagnosis of SSc. Further evaluation is warranted to determine whether these findings are of value in screening for early signs of PAH in SScKeywords
This publication has 19 references indexed in Scilit:
- Cardiac Magnetic Resonance Imaging with Pharmacological Stress Perfusion and Delayed Enhancement in Asymptomatic Patients with Systemic SclerosisThe Journal of Rheumatology, 2009
- Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patientsAnnals Of The Rheumatic Diseases, 2008
- Detection of Myocardial Fibrosis in Systemic Sclerosis by Contrast-Enhanced Magnetic Resonance ImagingRöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, 2008
- Pattern and distribution of myocardial fibrosis in systemic sclerosis: A delayed enhanced magnetic resonance imaging studyArthritis & Rheumatism, 2007
- Changes in causes of death in systemic sclerosis, 1972-2002Annals Of The Rheumatic Diseases, 2007
- Magnetic Resonance Imaging for the Detection of Myocardial Fibrosis in SclerodermaThe New England Journal of Medicine, 2006
- Contrast enhanced-cardiovascular magnetic resonance imaging in patients with pulmonary hypertensionEuropean Heart Journal, 2005
- Clinical classification of pulmonary hypertensionJournal of the American College of Cardiology, 2004
- SURVIVAL FOLLOWING THE ONSET OF SCLERODERMA: RESULTS FROM A RETROSPECTIVE INCEPTION COHORT STUDY OF THE UK PATIENT POPULATIONRheumatology, 1995
- Preliminary criteria for the classification of systemic sclerosis (scleroderma)Arthritis & Rheumatism, 1980