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Lung Function, Inflammation, and Endothelin-1 in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

Lung Function, Inflammation, and Endothelin-1 in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.
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Low A, George S, Howard L, Bell N, Millar A, Tulloh RMR,


Low A, George S, Howard L, Bell N, Millar A, Tulloh RMR, (click to view)

Low A, George S, Howard L, Bell N, Millar A, Tulloh RMR,

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Journal of the American Heart Association 2018 02 147(4) pii 10.1161/JAHA.117.007249

Abstract
BACKGROUND
Breathlessness is the most common symptom in people with pulmonary arterial hypertension and congenital heart disease (CHD-APAH), previously thought to be caused by worsening PAH, but perhaps also by inflammation and abnormalities of lung function. We studied lung function and airway inflammation in patients with CHD-APAH and compared the results with controls.

METHODS AND RESULTS
Sixty people were recruited into the study: 20 CHD-APAH, 20 CHD controls, and 20 healthy controls. Spirometry, gas transfer, whole body plethysmography and lung clearance index, 6-minute walk distance, and medical research council dyspnea scoring were performed. Inflammatory markers and endothelin-1 levels were determined in blood and induced sputum. The CHD-APAH group had abnormal lung function with lung restriction, airway obstruction, and ventilation heterogeneity. Inverse correlations were shown for CHD-APAH between medical research council dyspnea score and percent predicted peak expiratory flow (=-0.5383,=0.0174), percent predicted forced expiratory flow rate at 50% of forced vital capacity (=-0.5316,=0.0192), as well as for percent predicted forced expiratory volume in 1 s (=-0.6662,=0.0018) and percent predicted forced vital capacity (=-0.5536,=0.0186). The CHD-APAH patients were more breathless with lower 6-minute walk distance (360 m versus 558 m versus 622 m,=0.00001). Endothelin-1, interleukin (IL)-β, IL-6, IL-8, tumor necrosis factor α, and vascular endothelial growth factor were significantly higher in CHD-APAH than controls. Serum endothelin-1 for CHD-APAH correlated with airflow obstruction with significant negative correlations with percent predicted forced expiratory flow rate at 75% of forced vital capacity (=-0.5858,=0.0135).

CONCLUSIONS
Raised biomarkers for inflammation were found in CHD-APAH. Significant abnormalities in airway physiology may contribute to the dyspnea but are not driven by inflammation as assessed by circulating and sputum cytokines. A relationship between increased serum endothelin-1 and airway dysfunction may relate to its bronchoconstrictive properties.

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