J Gerry Coghlan

Publications (5)41.56 Total impact

• Article: Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United kingdom and ireland.

  Yi Ling, Martin K Johnson, David G Kiely, Robin Condliffe, Charlie A Elliot, J Simon R Gibbs, Luke S Howard, Joanna Pepke-Zaba, Karen K K Sheares, Paul A Corris, Andrew J Fisher, James L Lordan, Sean Gaine, J Gerry Coghlan, S John Wort, Michael A Gatzoulis, Andrew J Peacock
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  ABSTRACT: Rationale: Incident pulmonary arterial hypertension was underrepresented in most pulmonary hypertension registries and may have a different disease profile to prevalent disease. Objectives: To determine the characteristics and outcome of a purely incident, treatment-naive cohort of idiopathic, heritable, and anorexigen-associated pulmonary arterial hypertension and to determine the changes in presentations and survival over the past decade in the United Kingdom and Ireland. Methods: All consecutive newly diagnosed patients from 2001 to 2009 were identified prospectively. Measurements and Main Results: A total of 482 patients (93% idiopathic, 5% heritable, and 2% anorexigen-associated pulmonary arterial hypertension) were diagnosed, giving rise to an estimated incidence of 1.1 cases per million per year and prevalence of 6.6 cases per million in 2009. Younger patients (age ≤ 50 yrs) had shorter duration of symptoms, fewer comorbidities, better functional and exercise capacity, higher percent diffusing capacity of carbon monoxide, more severe hemodynamic impairment, but better survival compared with older patients. In comparison with the earlier cohorts, patients diagnosed in 2007-2009 were older, more obese, had lower percent diffusing capacity of carbon monoxide,(,) and more comorbidities, but better survival. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) equation, REVEAL risk score, and Pulmonary Hypertension Connection Registry survival equation accurately predicted survival of our incident cohort at 1 year. Conclusions: This study highlights the influence of age on phenotypes of incident pulmonary arterial hypertension and has shown the changes in demographics and epidemiology over the past decade in a national setting. The results suggest that there may be two subtypes of patients: the younger subtype with more severe hemodynamic impairment but better survival, compared with the older subtype who has more comorbidities.
  American Journal of Respiratory and Critical Care Medicine 07/2012; 186(8):790-6. · 11.08 Impact Factor
• Article: Survival in pulmonary hypertension registries: the importance of incident cases.

  Robin Condliffe, David G Kiely, J Gerry Coghlan, J Simon R Gibbs, S John Wort, Paul A Corris, Andrew J Peacock, Joanna Pepke-Zaba
  Chest 06/2011; 139(6):1547-8; author reply 1548-9. · 5.25 Impact Factor
• Article: Assessment of endpoints in pulmonary arterial hypertension associated with connective tissue disease.

  J Gerry Coghlan, Janet Pope, Christopher P Denton
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  ABSTRACT: Connective tissue disease-associated pulmonary arterial hypertension (PAH) is mostly related to systemic sclerosis, overlap syndromes with features of systemic sclerosis, mixed connective tissue disease, and systemic lupus erythematosus. It is an important cause of mortality in these conditions and represents up to one third of patients seen in most specialist pulmonary hypertension centers. Patients with PAH associated with connective tissue disease may have a worse outcome than those with other forms of PAH. Most randomized clinical trials of PAH therapies have included patients with connective tissue disease as part of a mixed population. Overall, analyses suggest that treatment responses parallel those seen in idiopathic PAH, though subgroup analyses must be interpreted with caution. There may also be significant comorbidity from other pulmonary complications such as interstitial fibrosis, aspiration, or chest wall restriction. In addition, the majority of clinical trials have used 6-minute walk distance as the primary endpoint; the clinical relevance of this is questionable in connective tissue disease patients, who often have multiple comorbidities, other than PAH, which adversely affect exercise tolerance.
  Current opinion in pulmonary medicine 05/2010; 16 Suppl 1:S27-34. · 3.08 Impact Factor
• Article: Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era.

  Robin Condliffe, David G Kiely, Andrew J Peacock, Paul A Corris, J Simon R Gibbs, Florenc Vrapi, Clare Das, Charlie A Elliot, Martin Johnson, Julia DeSoyza, Chantal Torpy, Kim Goldsmith, Denise Hodgkins, Rodney J Hughes, Joanna Pepke-Zaba, J Gerry Coghlan
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  ABSTRACT: Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, with a 1-year survival rate among patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) of 45%. However, more therapies have become available. To investigate the survival and characteristics of all patients diagnosed with CTD-PAH in the U.K. pulmonary hypertension service. National registry of all incident cases of CTD-PAH diagnosed consecutively between January 2001 and June 2006. Patients with CTD-PAH (429; 73% SSc-PAH) were diagnosed by a catheter-based approach. One- and 3-year survival rates were 78 and 47% for patients with isolated SSc-PAH. Survival was worse for those with respiratory disease-associated SSc-PAH (3-yr survival, 28%; P = 0.005) whereas survival among patients with exercise-induced SSc-PAH was superior (3-yr survival, 86%; P = < 0.001). Age, sex, mixed venous oxygen saturation, and World Health Organization functional class were independent predictors of survival in isolated SSc-PAH. Nineteen percent of patients with exercise-induced SSc-PAH and 39% of patients with isolated SSc-PAH who were in functional classes I and II had evidence of disease progression. The prevalence of diagnosed SSc-PAH is 2.93 per 1 million. The 3-year survival rate of 75% for those with pulmonary arterial hypertension associated with systemic lupus erythematosus (SLE-PAH) was significantly better than that for patients with SSc-PAH (P = 0.01). Survival of patients with SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival of patients with respiratory disease-associated pulmonary hypertension is inferior. SLE-PAH has a better prognosis than SSc-PAH.
  American Journal of Respiratory and Critical Care Medicine 10/2008; 179(2):151-7. · 11.08 Impact Factor
• Article: Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension.

  Robin Condliffe, David G Kiely, J Simon R Gibbs, Paul A Corris, Andrew J Peacock, David P Jenkins, Denise Hodgkins, Kim Goldsmith, Rodney J Hughes, Karen Sheares, Steven S L Tsui, Iain J Armstrong, Chantal Torpy, Rachel Crackett, Christopher M Carlin, Clare Das, J Gerry Coghlan, Joanna Pepke-Zaba
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  ABSTRACT: The management of chronic thromboembolic pulmonary hypertension (CTEPH) has changed over recent years with the growth of pulmonary endarterectomy surgery and the availability of disease-modifying therapies. To investigate the prognosis of CTEPH in a national setting during recent years. All incident cases diagnosed in one of the five pulmonary hypertension centers in the United Kingdom between January 2001 and June 2006 were identified prospectively. Information regarding baseline characteristics, treatment, and follow-up was subsequently collected from hospital records. A total of 469 patients received a diagnosis, of whom 148 (32%) had distal, nonsurgical disease. One- and three-year survival from diagnosis was 82 and 70% for patients with nonsurgical disease and 88 and 76% for those treated surgically (P = 0.023). Initial functional improvement in patients with nonsurgical disease was noted but did not persist at 2 years. Significant functional and hemodynamic improvements were seen in surgically treated patients with an increase in six-minute-walk distance of 105 m (P < 0.001) at 3 months. Five-year survival from surgery in the 35% of patients who survived to 3 months but had persistent pulmonary hypertension was 94%. The prognosis in nonsurgical disease has improved. We have confirmed the previously described good outcome in surgically treated disease. However, we have also demonstrated that the long-term prognosis for patients who have persistent pulmonary hypertension at 3 months after surgery is good. The observed improvements in outcome during the modern treatment era reinforce the importance of identifying patients with this increasingly treatable condition.
  American Journal of Respiratory and Critical Care Medicine 05/2008; 177(10):1122-7. · 11.08 Impact Factor