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Corresponding Author: Johannes Menno Douwes
Menno obtained his medical degree at the University of Groningen, The Netherlands. During his study he participated in a research project at the Dutch Asthma Center in Davos, and in a research project focused on pediatric pulmonary arterial hypertension at the University Medical Center Groningen. Currently, Menno is coordinator of the Dutch Network for Pediatric Pulmonary Hypertension and PhD-student at the University Medical Center Groningen. His PhD study focuses on pulmonary arterial hemodynamics in pediatric pulmonary arterial hypertension, and is supported by Professor R.M.F. Berger.
Abstract: Pulmonary arterial stroke volume: a new and strong prognostic factor in pediatric pulmonary arterial hypertension
JM Douwes, MTR Roofthooft, M Talsma, HL Hillege, RMF Berger,
In the current era of evolving treatment options, there is a need for reliable prognostic parameters in pediatric pulmonary arterial hypertension (PAH). The purpose of this study was to determine the prognostic value of pulmonary stroke volume (PSV) and pulmonary arterial compliance (stroke volume /pulse pressure; PAC) in pediatric PAH.
Cardiac catheterization data of 50 consecutive children with idiopathic/hereditary PAH (iPAH/HPAH; n=30) or PAH associated with congenital heart disease (PAH-CHD; n=20) were retrospectively reviewed. PSVi and PACi (both indexed for body surface area) were determined at baseline and during acute vasodilator response tests (AVR). Survival analyses were performed using Kaplan Meier curves and (multivariate) Cox Regression analyses, using death or lung transplantation as endpoints.
PSVi (38.4 25.4 ml/m2) and PACi (0.97 0.83 ml/mmHg/m2) were both age-independent and did not differ between iPAH/HPAH and PAH-CHD. During AVR, PSVi increased to 45.6 25.4 ml/m2 (p<0.01) and PACi to 1.80 2.87 ml/mmHg/m2 (p<0.01). In univariate analysis, higher baseline PSVi and PACi were associated with improved outcome (figure). In multivariate analysis, corrected for diagnosis, systemic to pulmonary shunt, gender, age, drug-treatment, mean right atrial pressure, cardiac index and pulmonary vascular resistance index, baseline PSVi independently predicted prognosis (HR 0.25 [95% CI 0.07-0.88] per SD p=0.03), in contrast PACi did not predict prognosis. PSVi and PACi during AVR did not have additional prognostic value.
Baseline PSVi is a strong independent predictor for prognosis in pediatric PAH and may be used to identify higher risk patients and guide therapy.
Barbara is a genetic counsellor working at the Antoine Béclère Hospital - Clamart, France since 2006. She offers genetic counselling and genetic testing to all patients followed in our centre with idiopathic and heritable pulmonary arterial hypertension (PAH) and their high risk relatives.
Barbara obtained her PhD in 2010 from Université Paris-sud 11, with Pr Marc Humbert acting as her thesis supervisor. Her research interests include the study of the genotype/phenotype relationship as it pertains to PAH.
Abstract: Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension
Barbara Girerd, David Montani, Mélanie Eyries, Azzedine Yaici, Benjamin Sztrymf, Florence Coulet, Olivier Sitbon, Gérald Simonneau, Florent Soubrier and Marc Humbert
Purpose: Previous studies indicate that patients with pulmonary arterial hypertension (PAH) carrying a mutation in the bone morphogenetic protein receptor type 2 (BMPR2) gene, develop the disease 10 years earlier than non-carriers, and have a more severe hemodynamic compromise at diagnosis. A recent report has suggested that this may only be the case for females and that patients with missense mutations in BMPR2 gene have more severe disease than patients with truncating mutations.
Methods: We reviewed data from all patients with PAH considered as idiopathic and patients with a family history of PAH, who underwent genetic counselling in the French PAH network between January, 1st 2004 and April, 1st 2010. We compared clinical, functional, and hemodynamic characteristics between carriers and non-carriers of a BMPR2 mutation, according to gender or BMPR2 mutation type.
Results: PAH patients carrying a BMPR2 mutation (n=115) were significantly younger at diagnosis than non-carriers (n=267) (35.8±15.4 and 47.5±16.2 respectively, p<0.0001). The presence of a BMPR2 mutation was associated with a younger age at diagnosis in females (36.4±14.9 in BMPR2 mutation carriers and 47.4±15.8 in non-carriers, p<0.0001), and males (34.6±16.8 in BMPR2 mutation carriers and 47.8±17.1 in non-carriers, p<0.0001). BMPR2 mutation carriers had a more severe hemodynamic compromise at diagnosis, but this was not influenced by gender. No differences in survival and time to death or lung transplantation were found in male and female PAH patients carrying a BMPR2 mutation. No differences were observed in clinical outcomes according to the type of BMPR2 mutations (missense, truncating, large rearrangement or splice defect).
Conclusion: When compared to non-carriers, BMPR2 mutation carriers from the French PAH network are younger at diagnosis and present with a more severe hemodynamic compromise, irrespective of gender. Moreover, BMPR2 mutation type had no influence on clinical phenotypes in our patient population.
Corresponding author: Enri Leci
Abstract: ESC-ERS 2009 pulmonary hypertension guidelines: an evaluation study in 9 European countries (the guide to PH study)
E. Leci (Institute of Cardiology, University of Bologna, Bologna /Italy), M. Palazzini (Institute of Cardiology, University of Bologna, Bologna /Italy), F. Sgro' (Institute of Cardiology, University of Bologna, Bologna /Italy), A. Manes (Institute of Cardiology, University of Bologna, Bologna /Italy), E. Conficoni (Institute of Cardiology, University of Bologna, Bologna /Italy), E. Beciani (Institute of Cardiology, University of Bologna, Bologna /Italy), C. Bachetti (Institute of Cardiology, University of Bologna, Bologna /Italy), E. Gotti (Institute of Cardiology, University of Bologna, Bologna /Italy), A. Branzi (Institute of Cardiology, University of Bologna, Bologna /Italy), N. Galie' (Institute of Cardiology, University of Bologna, Bologna /Italy)
Purpose: The evaluation of practice guidelines application in the European medical community presents multiple challenges including the selection of appropriate methodologies to be applied uniformly in heterogeneous countries. We utilized a multiple choice questionnaire administered with an internet-based, online, interview system. The 33 multiple-choice questions translated in different languages were related to 15 sections of the 2009 European Society of Cardiology and European Respiratory Society guidelines on pulmonary hypertension (PH guidelines).
Methods: 191 physicians from 9 European countries (30 Germany, 30 Italy, 30 Spain, 30 United Kingdom, 26 France, 15 Austria, 15 Switzerland, 8 Netherlands, 7 Belgium) and of 4 different specialties (94 pulmonologists, 63 cardiologists, 24 rheumatologists and 10 internists) were selected according to a minimum number of patients with pulmonary arterial hypertension (PAH) they were actively following and according to a minimal period of time of their interest in this clinical condition. The online interviews were performed from 1st to 31st November 2009.
Results: 88% of physicians acknowledged the value of the PH guidelines and declared to apply them "usually" (75%) or "always" (13%) in their clinical practice. The average number of correct answers to the questionnaire was 21 out of 33 questions (65%). The correct answers ranged from 45% to 79% between countries and from 58% to 70% between medical specialties. There was heterogeneity also according to the different topic explored: the highest number of correct answers were observed in drug therapy (80%) and the lowest in the clinical classification (33%), the hemodynamic definitions (52%), the pediatric PH (52%), the associated PAH conditions (53%) and the drug-drug interactions (54%).
Conclusions: While the acknowledgment of the value of the PH guidelines is uniformly high in all included countries and medical specialties, the appropriate knowledge and application of them is heterogeneous between specialties, between countries and between topics. These data may indicate country and/or specialty and/or topic-specific guidelines implementation programs. Sequential test repetition may help to understand the efficacy of the implementation activities. The internet-based online interview methodology can be easily repeated and could be utilized to evaluate the knowledge and application of other practice guidelines.
Abstract: Survival in patients with pulmonary arterial hypertension associated with connective tissue disease over the past decade
C.J. Valerio (Royal Free & University College London Medical School, London /United Kingdom), B.E. Schreiber (Royal Free & University College London Medical School, London /United Kingdom), C. Handler (Royal Free & University College London Medical School, London /United Kingdom), C.P. Denton (Royal Free & University College London Medical School, London /United Kingdom), J.G. Coghlan (Royal Free & University College London Medical School, London /United Kingdom)
Purpose: To determine historic and contemporary survival rates of patients treated for pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) over the past decade at our hospital.
Methods: Patients with right heart catheterisation confirmed PAH-CTD were identified from the hospital database and grouped by year of diagnosis: ≤2002; 2003–5; 2006–7; 2008–10. Before 2002 only iv prostacyclin therapy was available, between 2002–5 oral endothelin receptor antagonists were used as first line therapy, from 2006 oral phosphodiesterase type-5 inhibitors were available and used in combination regimens but from 2008 onwards, combination therapy was driven by a goal-oriented algorithm. Survival was analysed via Kaplan-Meier methods, log-rank tests and Cox proportional hazards (PH) models.
Results: For the 544 patients included, mean±SD age at diagnosis was 58.9±12.7 years, 82.7% were female and most had limited (61.2%) or diffuse (17.3%) systemic sclerosis. 1.3%, 14.3%, 59.9% and 24.4% were in WHO functional class (FC) I, II, III and IV, respectively. Mean±SD pulmonary artery pressure (mPAP; mmHg) was 39.8±12.2, 39.9±12.2, 39.8±12.9 and 37.6±10.4 and pulmonary vascular resistance (PVR; dynes/s/cm5) was 641±476, 649±405; 597±430 and 515±339 by diagnostic year ≤2002, 2003–5, 2006–7, 2008–10, respectively. 1-year survival rates (95% CI) were 75% (67–81%), 79% (72–85%), 88% (80–93%), and 86% (79–92%), respectively (p=0.009, log rank test for trend). This difference persisted when age, sex and baseline WHO FC, mPAP and PVR were adjusted for (p=0.003, Cox PH model) (Figure).
Conclusions: Outcomes in CTD-PAH at our centre have sequentially improved over the past decade as our treatment strategy has altered, even after adjustment for baseline prognostic factors.
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