Predictors of exercise-induced pulmonary hypertension in patients with connective tissue disease

2019 ◽  
Vol 34 (9) ◽  
pp. 1509-1518
Author(s):  
Sunao Miyanaga ◽  
Kayoko Kubota ◽  
Noriko Iwatani ◽  
Kenjuro Higo ◽  
Masaaki Miyata ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Exercise Induced

scholarly journals Association between lung ultrasound B‐lines and exercise‐induced pulmonary hypertension in patients with connective tissue disease

2021 ◽  
Author(s):  
Kazuki Kagami ◽  
Tomonari Harada ◽  
Koichi Yamaguchi ◽  
Shunichi Kouno ◽  
Takahiro Ikoma ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lung Ultrasound ◽  
Exercise Induced ◽  
B Lines

scholarly journals Significant Clinical Indexes of Exercise-Induced Pulmonary Hypertension in Patients With Connective Tissue Disease

2019 ◽  
Vol 1 (12) ◽  
pp. 610-616
Author(s):  
Satoko Ojima ◽  
Takuro Kubozono ◽  
Keishi Saihara ◽  
Takahiro Miyauchi ◽  
Shin Kawasoe ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Exercise Induced

scholarly journals Association between Lung Ultrasound B-lines and Exercise-Induced Pulmonary Hypertension in Patients with Connective Tissue Disease

2021 ◽  
Author(s):  
Kazuki Kagami ◽  
Tomonari Harada ◽  
Koichi Yamaguchi ◽  
Shunichi Kouno ◽  
Takahiro Ikoma ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Pulmonary Artery ◽  
Connective Tissue Disease ◽  
Lung Ultrasound ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Peak Exercise ◽  
Exercise Induced ◽  
B Lines

Background: Identification of elevation in pulmonary pressures during exercise may provide prognostic and therapeutic implications in patients with connective tissue disease (CTD). Interstitial lung disease (ILD) is common in CTD patients and subtle interstitial abnormalities detected by lung ultrasound could predict exercise-induced pulmonary hypertension (PH). Methods and Results: Echocardiography and lung ultrasound were performed at rest and bicycle exercise in CTD patients (n=41) and control subjects without CTD (n=24). Ultrasound B-lines were quantified by scanning four intercostal spaces in the right hemithorax. We examined the association between total B-lines at rest and the development of exercise-induced PH during ergometry exercise. Compared to controls, the number of total B-lines at rest was higher in CTD patients (0 [0, 0] vs. 2 [0, 9], p<0.0001) and was correlated with radiological severity of ILD assessed by computed tomography (fibrosis score, r=0.70, p<0.0001). Pulmonary artery systolic pressure (PASP) was increased with ergometry exercise in CTD compared to controls (48±14 vs. 35±13 mmHg, p=0.0006). The number of total B-lines at rest was highly correlated with higher PASP (r=0.52, p<0.0001) and poor right ventricular pulmonary artery coupling (tricuspid annular plane systolic excursion/PASP ratio, r=-0.31, p=0.01) during peak exercise. The number of resting B-lines predicted the development of exercise-induced PH with an area under the curve 0.79 (p=0.0003). Conclusions: These data may suggest the value of a simple resting assessment of lung ultrasound as a potential tool for assessing the risk of exercise-induced PH in CTD patients.

Classification tree criteria of pulmonary hypertension in mixed connective tissue disease

1997 ◽  
Vol 7 (4) ◽  
pp. 293-303
Author(s):  
Lisa Kotajima ◽  
Shinichi Aotsuka ◽  
Tomoe Nishimaki ◽  
Heihachiro Kashiwagi ◽  
Takeyoshi Kunieda ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Classification Tree

scholarly journals Fatal Pulmonary Hypertension in a Patient with Mixed Connective Tissue Disease: Report of an Autopsy Case.

1992 ◽  
Vol 31 (1) ◽  
pp. 74-77 ◽  
Author(s):  
Makoto SUZUKI ◽  
Mareomi HAMADA ◽  
Michihito SEKIYA ◽  
Yuji SHIGEMATSU ◽  
Soei GO ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Autopsy Case

scholarly journals Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

2010 ◽  
Vol 17 (6) ◽  
pp. 282-286 ◽  
Author(s):  
Shikha Mittoo ◽  
Thomas Jacob ◽  
Andrea Craig ◽  
Zoheir Bshouty
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Term Treatment ◽  
Kaplan Meier ◽  
Long Term Treatment ◽  
Survival Differences

BACKGROUND: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.OBJECTIVE: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.METHODS: A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.RESULTS: Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.CONCLUSION: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.

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