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Changes in right heart haemodynamics and echocardiographic function in an advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis
  1. Rajeev Saggar1,
  2. Dinesh Khanna2,
  3. Anjali Vaidya3,
  4. Ariss Derhovanessian4,
  5. Paul Maranian2,
  6. Erin Duffy5,
  7. John A Belperio4,
  8. Sam S Weigt4,
  9. Shiv Dua6,
  10. Shelley S Shapiro4,
  11. Jonathan G Goldin7,
  12. Fereidoun Abtin7,
  13. Joseph P Lynch III4,
  14. David J Ross4,
  15. Paul R Forfia8,
  16. Rajan Saggar4
  1. 1Thoracic Transplantation, Heart-Lung Institute, St Joseph Hospital & Medical Center, Phoenix, Arizona, USA
  2. 2Division of Rheumatology, Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA
  3. 3Cardiovascular Division, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA
  4. 4Division of Pulmonary and Critical Care Medicine, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  5. 5Department of Medicine Statistics Core, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  6. 6George Washington University, School of Medicine and Health Sciences, Washington, DC, USA
  7. 7Division of Radiology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  8. 8Cardiovascular Division, Temple University School of Medicine, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Rajan Saggar, David Geffen School of Medicine, University of California Los Angeles, 10833 Le Conte Ave. CHS 37-131, Los Angeles, CA 90095, USA; rsaggar{at}


Background Pulmonary hypertension (PH)-targeted therapy in the setting of pulmonary fibrosis (PF) is controversial; the main clinical concern is worsening of systemic hypoxaemia. We sought to determine the effects of gentle initiation and chronic administration of parenteral treprostinil on right heart function in patients with PF associated with an advanced PH phenotype.

Methods Open-label, prospective analysis of patients with PF-PH referred for lung transplantation (LT). Advanced PH was defined as mean pulmonary artery pressure (mPAP) ≥35 mm Hg. We compared haemodynamics, Doppler echocardiography (DE), oxygenation, dyspnoea and quality of life indices, and 6 min walk distance (6MWD) before and 12 weeks after parenteral treprostinil.

Results 15 patients were recruited in the study. After therapy, there were significant improvements in right heart haemodynamics (right atrial pressure (9.5 ± 3.4 vs 6.0 ± 3.7); mPAP (47 ± 8 vs 38.9 ± 13.4); CI (2.3 ± 0.5 vs 2.7 ± 0.6); pulmonary vascular resistance (698 ± 278 vs 496 ± 229); transpulmonary gradient (34.7 ± 8.7 vs 28.5 ± 10.3); mvO2 (65 ± 7.2 vs 70.9 ± 7.4); and stroke volume index (29.2 ± 6.7 vs 33 ± 7.3)) and DE parameters reflecting right heart function (right ventricular (RV) end diastolic area (36.4 ± 5.2 vs 30.9 ± 8.2 cm2), left ventricular eccentricity index (1.7 ± 0.6 vs 1.3 ± 0.5), tricuspid annular planar systolic excursion (1.6 ± 0.5 vs 1.9 ± 0.2 cm)). These changes occurred without significant alteration in systemic oxygenation, heart rate, or mean systemic arterial pressure. In addition, improvements were seen in 6MWD (171 ± 93 vs 230 ± 114), 36-Item Short Form Health Survey Mental Component Summary aggregate (38 ± 11 vs 44.2 ± 10.7), University of California, San Diego Shortness of Breath Questionnaire (87 ± 17.1 vs 73.1 ± 21), and brain natriuretic peptide (558 ± 859 vs 228 ± 340).

Conclusions PH-targeted therapy may improve right heart haemodynamics and echocardiographic function without affecting systemic oxygen saturation in an advanced PH phenotype associated with RV dysfunction in the setting of PF.

  • Primary Pulmonary Hypertension
  • Lung Transplantation
  • Interstitial Fibrosis

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