Bi-ventricular interplay in patients with systemic sclerosis-associated pulmonary arterial hypertension: Detection by cardiac magnetic resonance.
OBJECTIVES
Pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) has a poor prognosis compared to PAH associated with other connective tissue diseases (CTD). The objective of this study was to examine the difference in hemodynamic state between SSc-PAH and other CTD-PAH by performing cardiac magnetic resonance (CMR) imaging.


METHODS
A single center retrospective analysis was conducted comprising 40 consecutive CTD patients who underwent right heart catheterization and CMR at the same period from January 2010 to October 2015.


RESULTS
Thirty-two patients had pre-capillary pulmonary hypertension. Of these, 15 had SSc and 17 had other CTD. CMR measurements, particularly the ratio of right to left end-diastolic volume (RVEDV/LVEDV), correlated well with mean pulmonary arterial pressure (mPAP). Conversely, RVEDV/LVEDV and mPAP correlated differently in SSc and non-SSc patients. In SSc patients, the ratio of RVEDV/LVEDV to mPAP was significantly higher compared to non-SSc patients. In the follow-up study, 2 SSc patients exhibited increased RVEDV/LVEDV in spite of decreased mPAP following treatment. Kaplan-Meier analysis revealed poor prognosis of patients with increased RVEDV/LVEDV following treatment.


CONCLUSIONS
Our data indicated that altered bi-ventricular interplay detected at CMR may represent SSc-related cardiac involvement and reflect poor prognosis of SSc-PAH.
['Noguchi|Atsushi|A|', 'Kato|Masaru|M|', 'Kono|Michihito|M|', 'Ohmura|Kazumasa|K|', 'Ohira|Hiroshi|H|', 'Tsujino|Ichizo|I|', 'Oyama-Manabe|Noriko|N|', 'Oku|Kenji|K|', 'Bohgaki|Toshiyuki|T|', 'Horita|Tetsuya|T|', 'Yasuda|Shinsuke|S|', 'Nishimura|Masaharu|M|', 'Atsumi|Tatsuya|T|']
D000328:Adult D000368:Aged D005260:Female D005500:Follow-Up Studies D006352:Heart Ventricles D006801:Humans D006976:Hypertension, Pulmonary D008279:Magnetic Resonance Imaging D008297:Male D008875:Middle Aged D012189:Retrospective Studies D012595:Scleroderma, Systemic
Pulmonary arterial hypertension Cardiac magnetic resonance Systemic sclerosis Prognosis Connective tissue diseases