Thorax 62:617-622 doi:10.1136/thx.2006.070680
  • Pulmonary hypertension

Demographic features, BMPR2 status and outcomes in distal chronic thromboembolic pulmonary hypertension

  1. Jay Suntharalingam1,
  2. Rajiv D Machado2,
  3. Linda D Sharples3,
  4. Mark R Toshner1,
  5. Karen K Sheares1,
  6. Rodney J Hughes1,
  7. David P Jenkins1,
  8. Richard C Trembath2,
  9. Nicholas W Morrell4,
  10. Joanna Pepke-Zaba1
  1. 1Papworth Hospital NHS Trust, Papworth Everard, Cambridgeshire, UK
  2. 2Division of Genetics and Molecular Medicine (Guy’s Campus), King’s College, London, UK
  3. 3MRC Biostatistics Unit, Robinson Way, Cambridge, UK
  4. 4University of Cambridge School of Clinical Medicine, Addenbrooke’s Hospital, Cambridge, UK
  1. Correspondence to:
    Dr Joanna Pepke-Zaba
    Pulmonary Vascular Diseases Unit, Papworth Hospital NHS Trust, Papworth Everard, Cambridgeshire CB3 8RE, UK; Joanna.pepkezaba{at}
  • Received 22 August 2006
  • Accepted 1 January 2007
  • Published Online First 7 February 2007


Background: Although pulmonary endarterectomy (PEA) is potentially curative in chronic thromboembolic pulmonary hypertension (CTEPH), some patients have distally distributed disease that is not amenable to surgery. The aetiology and characteristics of this patient group are currently not well understood.

Objectives: This study compares the baseline demographic features and outcomes in subjects with distal CTEPH, those with proximal CTEPH and those with idiopathic pulmonary arterial hypertension (IPAH) to determine whether these conditions represent separate entities or whether they exist along the same spectrum of disease.

Methods: The medical history, clinical characteristics, bone morphogenetic protein receptor type II (BMPR2) mutation status and outcomes of 96 subjects with IPAH, 35 with distal CTEPH and 68 with proximal CTEPH referred to a single specialist centre between 1994 and 2005 were reviewed.

Results: There were significant differences between the distal CTEPH, proximal CTEPH and IPAH groups in age (55.9 years vs 54.8 years vs 46.2 years, p<0.001), proportion who were male (43% vs 69% vs 29%, p<0.001), previous deep vein thrombosis (28.6% vs 30.9% vs 3.1%, p<0.001), positive BMPR2 status (0% vs 0% vs 15%, p = 0.018), mean pulmonary artery pressure (47.3 mm Hg vs 45.4 mm Hg vs 54.8 mm Hg, p<0.001) and total pulmonary resistance (12.9 WU vs 12.4 WU vs 18.1 WU, p<0.001). Patients with distal CTEPH and those with IPAH were managed similarly and had comparable survival characteristics (1 year survival 77% vs 86%; 3 year survival 53% vs 60%; p = 0.68).

Conclusions: Patients with distal CTEPH share certain demographic features with patients with proximal CTEPH that not only indicate a common aetiology but also help to differentiate them from patients with IPAH. Despite more favourable haemodynamic parameters in those with distal CTEPH, patients in this group had a poor long-term outcome which was similar to that of patients with IPAH.


  • Published Online First 7 February 2007

  • This study had no financial support from external sources.

  • Competing interests: JS has received travel grants from Actelion Pharmaceuticals, Encysive, GlaxoSmithKline Beecham and Pfizer Ltd. RH has received travel grants, consulting fees and speaker’s fees from Actelion Pharmaceuticals. KS has received travel grants from Actelion Pharmaceuticals. NM has received speaker’s fees from Actelion Pharmaceuticals and received consulting fees from Novartis. JP-Z has received consulting fees from Actelion Pharmaceuticals, Pfizer Ltd and Encysive and research support from Actelion Pharmaceuticals and Pfizer Ltd. RM, LS, MT, DJ and RT have no relevant financial disclosures.