Left heart abnormalities in connective tissue disease patients with pre-capillary pulmonary hypertension as well as borderline mean pulmonary arterial pressure.
OBJECTIVE
To describe co-existence of left heart abnormalities among case series of connective tissue disease (CTD) patients who showed pre-capillary pulmonary hypertension (PH) as well as borderline mean pulmonary arterial pressure (mPAP).


METHODS
From 2010 through 2012, 49 CTD patients suspected to have PH by exercise Doppler echocardiography underwent right heart catheterization. We retrospectively searched for left heart diseases from the available data on Doppler echocardiography, cardiac magnetic resonance imaging (MRI), scintigraphy, and endomyocardial biopsy.


RESULTS
Among 49 patients, 11 and 2 had pre- and post-capillary PH, respectively, and another 10 had borderline mPAP. Six of 11 patients with pre-capillary PH showed low pulmonary vascular resistance (PVR) (≤ 240 dynes•sec•cm(- 5)) and low diastolic pulmonary gradient (< 7 mmHg). Seven of 10 patients with borderline mPAP had normal PVR (< 160) suggesting the presence of left heart abnormalities. Other abnormal findings included increased left atrial volume index and E/E' on Doppler echocardiography, delayed contrast enhancement by MRI, patchy area of hypoperfusion on thallium scintigraphy, and fibrosis in endomyocardial biopsy.


CONCLUSIONS
The present case series suggested some contribution of left heart abnormalities to the increase in mPAP among CTD patients with pre-capillary PH as well as borderline mPAP.
a Division of Rheumatology and Allergology, Department of Internal Medicine , St. Marianna University School of Medicine , Miyamae-ku, Kawasaki , Japan.
Kosei
K
Tsuchida
Hidehiro
H
Yamada
Yoshioki
Y
Yamasaki
Kengo
K
Suzuki
Ken
K
Kongoji
Yoshihiro
Y
Akashi
Shoichi
S
Ozaki
D000328:Adult; D000368:Aged; D062186:Arterial Pressure; D006328:Cardiac Catheterization; D003240:Connective Tissue Diseases / Q000150:complications* / Q000175:diagnosis; D015150:Echocardiography, Doppler; D005260:Female; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014655:Vascular Resistance / Q000502:physiology; D018487:Ventricular Dysfunction, Left / Q000150:complications / Q000151:congenital* / Q000175:diagnosis; D016277:Ventricular Function, Left / Q000502:physiology
Borderline mPAP; Connective tissue disease; Diastolic dysfunction; Left heart abnormalities; Pre-capillary pulmonary hypertension