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Gérald Simonneau - One of the best experts on this subject based on the ideXlab platform.
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updated clinical classification of Pulmonary Hypertension
Türk Kardiyoloji Derneği arşivi : Türk Kardiyoloji Derneğinin yayın organıdır, 2014Co-Authors: Gérald Simonneau, Miguel Angel Gomez Sanchez, Ardeschir Ghofrani, Ian Adatia, Christopher P. Denton, David Celermajer, Michael A Gatzoulis, Krishna R Kumar, Michael J Landzberg, Roberto F MachadoAbstract:In 1998, a clinical classification of Pulmonary Hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (Pulmonary arterial Hypertension [PAH]) were proposed. The main change was to withdraw persistent Pulmonary Hypertension of the newborn (YPPH) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1". Pulmonary Hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric Pulmonary Hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental Pulmonary Hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4. (J Am Coll Cardiol 2013;62:D34-41) a 2013 by the American College of Cardiology Foundation.
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updated clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2009Co-Authors: Gérald Simonneau, Ardeschir Ghofrani, Ian Adatia, Christopher P. Denton, David Celermajer, Michael A Gatzoulis, Miguel Angel Gomez SanchezAbstract:In 1998, a clinical classification of Pulmonary Hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (Pulmonary arterial Hypertension [PAH]) were proposed. The main change was to withdraw persistent Pulmonary Hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1. Pulmonary Hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric Pulmonary Hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental Pulmonary Hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4.
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splenectomy and chronic thromboembolic Pulmonary Hypertension
Thorax, 2005Co-Authors: Xavier Jais, Gérald Simonneau, Philippe Dartevelle, Olivier Sitbon, V Ioos, Carlos Jardim, Florence Parent, A Hamid, Elie Fadel, Marc HumbertAbstract:Background: An increased prevalence of splenectomy has been reported in patients with idiopathic Pulmonary arterial Hypertension. Examination of small Pulmonary arteries from these subjects has revealed multiple thrombotic lesions, suggesting that thrombosis may contribute to this condition. Based on these findings, we hypothesised that splenectomy could be a risk factor for chronic thromboembolic Pulmonary Hypertension (CTEPH), a condition defined by the absence of thrombus resolution after acute Pulmonary embolism that causes sustained obstruction of the Pulmonary arteries and subsequent Pulmonary Hypertension. Methods: The medical history, clinical characteristics, thrombotic risk factors and haemodynamics of 257 patients referred for CTEPH between 1989 and 1999 were reviewed. In a case-control study the prevalence of splenectomy in patients with CTEPH was compared with that of patients evaluated during the same period for idiopathic Pulmonary Hypertension (n = 276) or for lung transplantation in other chronic Pulmonary conditions (n = 180). Results: In patients with CTEPH, 8.6% (95% CI 5.2 to 12.0) had a history of splenectomy compared with 2.5% (95% CI 0.7 to 4.4) and 0.56% (95% CI 0 to 1.6) in cases of idiopathic Pulmonary arterial Hypertension and other chronic Pulmonary conditions, respectively (p<0.01). Conclusion: Splenectomy may be a risk factor for chronic thromboembolic Pulmonary Hypertension.
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clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2004Co-Authors: Gérald Simonneau, Simon Gibbs, Didier Lebrec, David Langleben, Guido Domenighetti, Lewis J Rubin, Rudolf Speich, Nazzareno Galiè, Werner Seeger, Maurice BeghettiAbstract:In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary Pulmonary Hypertension" and to replace it with "idiopathic Pulmonary Hypertension"; to reclassify Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for Pulmonary arterial Hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-Pulmonary shunts.
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clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2004Co-Authors: Gérald Simonneau, Simon Gibbs, Didier Lebrec, David Langleben, Guido Domenighetti, Lewis J Rubin, Rudolf Speich, Nazzareno Galiè, Werner Seeger, Maurice BeghettiAbstract:In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary Pulmonary Hypertension" and to replace it with "idiopathic Pulmonary Hypertension"; to reclassify Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for Pulmonary arterial Hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-Pulmonary shunts.
Maurice Beghetti - One of the best experts on this subject based on the ideXlab platform.
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clinical features of paediatric Pulmonary Hypertension a registry study
The Lancet, 2012Co-Authors: R Berger, Maurice Beghetti, Zhicheng Jing, Tilman Humpl, Gary E Raskob, Dunbar D Ivy, Damien Bonnet, Ingram Schulzeneick, Robyn J BarstAbstract:Summary Background Paediatric Pulmonary Hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric Pulmonary Hypertension. Methods Consecutive patients aged 18 years or younger at diagnosis with Pulmonary Hypertension and increased Pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, Pulmonary Hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs. Findings 362 of 456 consecutive patients had confirmed Pulmonary Hypertension (defined as mean Pulmonary artery pressure ≥25 mm Hg, Pulmonary capillary wedge pressure ≤12 mm Hg, and Pulmonary vascular resistance index ≥3 WU/m −2 ). 317 (88%) patients had Pulmonary arterial Hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had Pulmonary Hypertension associated with respiratory disease or hypoxaemia, with bronchoPulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic Pulmonary Hypertension or miscellaneous causes of Pulmonary Hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3–12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-Pulmonary shunts had syncope. Despite severe Pulmonary Hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function. Interpretation TOPP identifies important clinical features specific to the care of paediatric Pulmonary Hypertension, which draw attention to the need for paediatric data rather than extrapolation from adult studies. Funding Actelion Pharmaceuticals.
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clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2004Co-Authors: Gérald Simonneau, Simon Gibbs, Didier Lebrec, David Langleben, Guido Domenighetti, Lewis J Rubin, Rudolf Speich, Nazzareno Galiè, Werner Seeger, Maurice BeghettiAbstract:In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary Pulmonary Hypertension" and to replace it with "idiopathic Pulmonary Hypertension"; to reclassify Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for Pulmonary arterial Hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-Pulmonary shunts.
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clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2004Co-Authors: Gérald Simonneau, Simon Gibbs, Didier Lebrec, David Langleben, Guido Domenighetti, Lewis J Rubin, Rudolf Speich, Nazzareno Galiè, Werner Seeger, Maurice BeghettiAbstract:In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary Pulmonary Hypertension" and to replace it with "idiopathic Pulmonary Hypertension"; to reclassify Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for Pulmonary arterial Hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-Pulmonary shunts.
Lewis J Rubin - One of the best experts on this subject based on the ideXlab platform.
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clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2004Co-Authors: Gérald Simonneau, Simon Gibbs, Didier Lebrec, David Langleben, Guido Domenighetti, Lewis J Rubin, Rudolf Speich, Nazzareno Galiè, Werner Seeger, Maurice BeghettiAbstract:In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary Pulmonary Hypertension" and to replace it with "idiopathic Pulmonary Hypertension"; to reclassify Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for Pulmonary arterial Hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-Pulmonary shunts.
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clinical classification of Pulmonary Hypertension
Journal of the American College of Cardiology, 2004Co-Authors: Gérald Simonneau, Simon Gibbs, Didier Lebrec, David Langleben, Guido Domenighetti, Lewis J Rubin, Rudolf Speich, Nazzareno Galiè, Werner Seeger, Maurice BeghettiAbstract:In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary Pulmonary Hypertension" and to replace it with "idiopathic Pulmonary Hypertension"; to reclassify Pulmonary veno-occlusive disease and Pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for Pulmonary arterial Hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-Pulmonary shunts.
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primary Pulmonary Hypertension
The New England Journal of Medicine, 1997Co-Authors: Lewis J RubinAbstract:Primary Pulmonary Hypertension (PPH) is a rare disease of unknown aetiology which typically results in right heart failure and death within several years of the onset of symptoms. While there is no cure for PPH, several pharmacological and surgical approaches to treatment have been developed over the past decade which have proved useful in a significant proportion of patients. In particular, vasodilator therapy may produce sustained haemodynamic and symptomatic improvement in up to approximately two-thirds of patients; in the remaining patients, vasodilators may either produce no benefit or result in deterioration. The calcium channel blocking agents are the most widely used oral vasodilators; continuous intravenous infusions of epoprostenol (prostacyclin; prostaglandin I2) have been used in some patients who are refractory to oral therapy, particularly as a bridge to transplantation. While combined heart-lung transplantation has been considered the surgical procedure of choice for severe Pulmonary Hypertension, single lung transplantation has been performed successfully in a small number of patients, and may be the preferred approach in patients with reasonably preserved right heart function.
Einar Stefánsson - One of the best experts on this subject based on the ideXlab platform.
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Exudative retinal detachment in familial Pulmonary Hypertension.
Acta ophthalmologica, 2009Co-Authors: Ingimundur Gíslason, Fridbert Jonasson, Einar StefánssonAbstract:In a family with Pulmonary Hypertension, one woman developed bilateral exudative retinal detachments and her daughter developed a unilateral exudative retinal detachment during pregnancy. Treatment with Acetazolamide provided quick recovery in the bilateral case, whereas the unilateral exudative detachment recovered without treatment after delivery. Exudative retinal detachments have not previously been associated with Pulmonary Hypertension.
Rachel K Hopper - One of the best experts on this subject based on the ideXlab platform.
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treprostinil improves persistent Pulmonary Hypertension associated with congenital diaphragmatic hernia
The Journal of Pediatrics, 2018Co-Authors: Kendall M Lawrence, Rachel K Hopper, Holly L Hedrick, Heather M Monk, Lisa M Herkert, Lindsay N Waqar, Brian D Hanna, William H Peranteau, Natalie E RintoulAbstract:Objective To evaluate the effect of continuous treprostinil in infants with severe Pulmonary Hypertension associated with congenital diaphragmatic hernia (CDH) on specific markers of Pulmonary Hypertension severity and to report the safety and tolerability of treprostinil. Study design We conducted a retrospective cohort study of infants with CDH-associated Pulmonary Hypertension treated with treprostinil from January 2011 to September 2016. Severity of Pulmonary Hypertension was assessed by echocardiogram and serum B-type natriuretic peptide (BNP) by using time points before initiation and 24 hours, 1 week, and 1 month after treprostinil initiation. Fisher exact tests, Wilcoxon-rank sum tests, and mixed-effects models were used for analysis. Results Seventeen patients were treated with treprostinil for a median of 54.5 days (IQR 44.3-110 days). Compared with the concurrent CDH population (n = 147), infants treated with treprostinil were more likely to require extracorporeal support (76.5% vs 25.2%, P P P P P = .01). Severity of Pulmonary Hypertension by echocardiogram improved at 1 month (OR 0.14, CI 95% 0.04-0.48, P = .002). Despite these improvements, overall mortality remained high (35%). There were no adverse events related to treprostinil, including no hypotension, hypoxia, or thrombocytopenia. Conclusions In this cohort, treprostinil use was associated with improved severity of Pulmonary Hypertension assessed by echocardiogram and decreased BNP, with no significant side effects.
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persistent challenges in pediatric Pulmonary Hypertension
Chest, 2016Co-Authors: Rachel K Hopper, Steven H AbmanAbstract:Pulmonary Hypertension and related Pulmonary vascular diseases cause significant morbidities and high mortality and present many unique challenges toward improving outcomes in neonates, infants, and children. Differences between pediatric and adult disease are reflected in controversies regarding etiologies, classification, epidemiology, diagnostic evaluations, and therapeutic interventions. This brief review highlights several key topics reflecting recent advances in the field and identifies persistent gaps in our understanding of clinical pediatric Pulmonary Hypertension.
