Changes in main pulmonary artery diameter during follow-up have prognostic implications in pulmonary arterial hypertension

Respirology. 2017 Nov;22(8):1649-1655. doi: 10.1111/resp.13073. Epub 2017 May 17.

Abstract

Background and objective: A dilated pulmonary artery (PA) is a common finding in patients with pulmonary arterial hypertension (PAH). Little is known on the variations in PA size over time and whether these changes track with disease severity and/or predict long-term survival.

Methods: We included patients with PAH who had at least two computed tomography (CT) scans of the chest done on different visits. Both scans matched the use of i.v. contrast.

Results: Pairs of CT scans were compared in 113 PAH patients. During a median (interquartile range (IQR)) time difference between scans of 8 (IQR: 3.5-20.0) months, we noted an increase in main PA diameter of 0.5 ± 1.8 mm (mean ± SD) (P = 0.008). When CT scans were performed >12 months apart (n = 47), the main PA diameter increased or decreased by >1 mm in 40% and 13% of the patients, respectively. An increase in main PA diameter was associated with lower PA compliance, higher right ventricular (RV) systolic pressure, worse RV function and a decline in 6-min walk distance. During a median (IQR) follow-up of 33 (IQR: 4.5-47) months, 53 (46.9%) patients died. The change in PA diameter was a significant predictor of mortality (hazard ratio (HR) per mm increase: 1.33 (95% CI: 1.11-1.61), P = 0.002) when adjusted for difference in time and slice thickness between CT scans, age, gender, PAH aetiology and pulmonary vascular resistance.

Conclusion: In PAH patients, an increase in CT-derived main PA diameter over time is associated with progression in pulmonary pressures, RV dysfunction, a decline in functional capacity and higher mortality.

Keywords: outcomes; pulmonary arterial hypertension; pulmonary artery diameter; survival.

MeSH terms

  • Adult
  • Aged
  • Disease Progression
  • Familial Primary Pulmonary Hypertension / physiopathology
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Pulmonary* / diagnosis
  • Hypertension, Pulmonary* / mortality
  • Hypertension, Pulmonary* / physiopathology
  • Male
  • Middle Aged
  • Organ Size
  • Predictive Value of Tests
  • Prognosis
  • Proportional Hazards Models
  • Pulmonary Artery* / diagnostic imaging
  • Pulmonary Artery* / pathology
  • Tomography, X-Ray Computed / methods
  • United States / epidemiology
  • Vascular Resistance / physiology
  • Ventricular Dysfunction, Right / physiopathology
  • Ventricular Function, Right / physiology