Robert Tulloh1, Kostas Dimopoulos2, Robin Condliffe3, Paul Clift4. 1. Department of Congenital Heart Disease, University Hospitals Bristol NHS Foundation Trust, Bristol, UK. Electronic address: Robert.Tulloh@bristol.ac.uk. 2. Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College London, London, UK. 3. Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK. 4. Department of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK.
Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH) is a well-recognised complication of adult congenital heart disease (CHD). However, management is not currently standardised between centres and specific guidelines are lacking. In order to identify and understand the unmet needs related to PAH associated with CHD (PAH-CHD), a survey of physicians was performed. METHODS: An electronic survey was sent to two physician groups: (1) cardiologists registered in a UK cardiology directory; (2) specialist pulmonary hypertension (PH) physicians known to manage patients with adult PAH-CHD. The questions related to referral pathways, screening, therapy and palliative care. RESULTS: 821 surveys were distributed and 106 were returned. Respondents included a broad mix of specialist physicians with many patients along with general cardiologists managing only a small number of PAH-CHD patients. Although 97% of respondents have access to a specialist PH centre, patients are still being managed in non-specialist settings. Shared care arrangements are widespread but only 41% have formal shared care protocols. Palliative care services are limited and general cardiologists rarely perform 6-minute walk tests (6MWT) or quality of life assessments. People with PAH-CHD are often undertreated, with 39% of respondents reporting that fewer than 25% of these patients were receiving PAH-specific therapies. CONCLUSIONS: The survey revealed gaps and inconsistencies in the management of patients with PAH-CHD therefore patient-specific guidance is needed for many of these aspects.
BACKGROUND:Pulmonary arterial hypertension (PAH) is a well-recognised complication of adult congenital heart disease (CHD). However, management is not currently standardised between centres and specific guidelines are lacking. In order to identify and understand the unmet needs related to PAH associated with CHD (PAH-CHD), a survey of physicians was performed. METHODS: An electronic survey was sent to two physician groups: (1) cardiologists registered in a UK cardiology directory; (2) specialist pulmonary hypertension (PH) physicians known to manage patients with adult PAH-CHD. The questions related to referral pathways, screening, therapy and palliative care. RESULTS: 821 surveys were distributed and 106 were returned. Respondents included a broad mix of specialist physicians with many patients along with general cardiologists managing only a small number of PAH-CHD patients. Although 97% of respondents have access to a specialist PH centre, patients are still being managed in non-specialist settings. Shared care arrangements are widespread but only 41% have formal shared care protocols. Palliative care services are limited and general cardiologists rarely perform 6-minute walk tests (6MWT) or quality of life assessments. People with PAH-CHD are often undertreated, with 39% of respondents reporting that fewer than 25% of these patients were receiving PAH-specific therapies. CONCLUSIONS: The survey revealed gaps and inconsistencies in the management of patients with PAH-CHD therefore patient-specific guidance is needed for many of these aspects.
Authors: Harald Kaemmerer; Matthias Gorenflo; Dörte Huscher; David Pittrow; Christian Apitz; Helmut Baumgartner; Felix Berger; Leonhard Bruch; Eva Brunnemer; Werner Budts; Martin Claussen; Gerry Coghlan; Ingo Dähnert; Michele D'Alto; Marion Delcroix; Oliver Distler; Sven Dittrich; Daniel Dumitrescu; Ralf Ewert; Martin Faehling; Ingo Germund; Hossein Ardeschir Ghofrani; Christian Grohé; Karsten Grossekreymborg; Michael Halank; Georg Hansmann; Dominik Harzheim; Attila Nemes; Kalman Havasi; Matthias Held; Marius M Hoeper; Michael Hofbeck; Wolfgang Hohenfrost-Schmidt; Elena Jurevičienė; Lina Gumbienè; Hans-Joachim Kabitz; Hans Klose; Thomas Köhler; Stavros Konstantinides; Martin Köestenberger; Rainer Kozlik-Feldmann; Hans-Heiner Kramer; Cornelia Kropf-Sanchen; Astrid Lammers; Tobias Lange; Philipp Meyn; Oliver Miera; Katrin Milger-Kneidinger; Rhoia Neidenbach; Claus Neurohr; Christian Opitz; Christian Perings; Bjoern Andrew Remppis; Gabriele Riemekasten; Laura Scelsi; Werner Scholtz; Iveta Simkova; Dirk Skowasch; Andris Skride; Gerd Stähler; Brigitte Stiller; Iraklis Tsangaris; Carmine Dario Vizza; Anton Vonk Noordegraaf; Heinrike Wilkens; Hubert Wirtz; Gerhard-Paul Diller; Ekkehard Grünig; Stephan Rosenkranz Journal: J Clin Med Date: 2020-05-13 Impact factor: 4.241