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Respir Med Res ; 76: 48-53, 2019 Nov.
Article En | MEDLINE | ID: mdl-31557688

Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by cutaneous capillary malformations, soft tissue and bone hypertrophy, and multiple capillary, venous or lymphatic malformations. KTS is associated with recurrent thromboembolic events. We reported herein five cases of chronic thromboembolic pulmonary hypertension (CTEPH) associated with KTS (age minimum-maximum 26-50 years old, 3 males/2 females). Hemodynamics showed severe pulmonary hypertension (PH) with pulmonary vascular resistance ranging from 5.6 to 18.3 Wood units (WU), associated with marked clinical impairment (NYHA functional class III or IV in 4 patients). Computed tomography (CT) of the chest and pulmonary angiography confirmed proximal CTEPH accessible to surgical intervention in one patient and distal forms of CTEPH in 4 patients. Evolution after pulmonary endarterectomy showed hemodynamic normalization, while the patients with distal CTEPH had severe outcomes with 2 early deaths after PH diagnosis (44 and 35 months respectively). One patient with distal CTEPH was still alive 16 years after diagnosis on specific PH therapy and one was transplanted after 15 years because of right heart failure (death after 12 months). Histological analysis of the lung explants showed typical chronic thromboembolic material specific for CTEPH. In conclusion, KTS may be complicated by severe CTEPH requiring careful anticoagulation and multidisciplinary follow-up in expert centers to screen for disease potentially accessible to endarterectomy. In the modern management era of CTEPH, balloon pulmonary angioplasty will certainly be an interesting option in patients with inoperable disease.

Hypertension, Pulmonary/etiology , Klippel-Trenaunay-Weber Syndrome/complications , Pulmonary Embolism/etiology , Adult , Chronic Disease , Female , Humans , Hypertension, Pulmonary/diagnosis , Klippel-Trenaunay-Weber Syndrome/diagnosis , Male , Middle Aged , Pulmonary Embolism/diagnosis , Thromboembolism/diagnosis , Thromboembolism/etiology
Pulmonology ; 25(4): 248-251, 2019.
Article En | MEDLINE | ID: mdl-31080042

BACKGROUND: Cardiac magnetic resonance (CMR) imaging has gained importance in pulmonary hypertension (PH) and studies have demonstrated its use as a surrogate marker and in following treatment of these patients. The pathophysiology of PH differs between pulmonary arterial hypertension (PAH, group 1) and chronic thromboembolic PH (CTEPH, group 4). OBJECTIVES: The present study tested the hypothesis that PAH and CTEPH display different characteristics on CMR imaging. METHODS: 46 patients were evaluated for pulmonary vascular disease in the French National Reference Center for PH (23 PAH and 23 CTEPH matched for age and gender). All patients had the right heart catheterization (RHC) and CMR imaging performed within 48h. CMR imaging was performed on a 1.5 T scanner. RESULTS: PAH and CTEPH had similar body surface area and similar invasive hemodynamics, including mean pulmonary arterial pressure, cardiac index, pulmonary vascular resistance and right atrial pressure. PAH and CTEPH had similar CMR data. Right ventricular (RV) morphology and function and pulmonary artery (PA) data were also similar. CONCLUSION: Age- and sex-matched PAH and CTEPH patients displayed similar values of the CMR indices of RV and PA morphology and function, suggesting that the RV-PA responses are similar in both groups, mostly related to the overall increase in after load.

Heart Ventricles/diagnostic imaging , Magnetic Resonance Imaging/methods , Pulmonary Arterial Hypertension/diagnostic imaging , Pulmonary Embolism/diagnostic imaging , Adult , Aged , Cardiac Catheterization/methods , Chronic Disease , Female , France/epidemiology , Heart Atria/physiopathology , Heart Ventricles/physiopathology , Hemodynamics , Humans , Hypertension, Pulmonary/physiopathology , Lung/blood supply , Lung/pathology , Lung/physiopathology , Male , Middle Aged , Pulmonary Arterial Hypertension/physiopathology , Pulmonary Artery/pathology , Pulmonary Artery/physiopathology , Pulmonary Embolism/physiopathology , Pulmonary Wedge Pressure/physiology , Vascular Diseases/physiopathology , Vascular Resistance , Ventricular Function, Right/physiology
Rev Mal Respir ; 35(2): 160-170, 2018 Feb.
Article Fr | MEDLINE | ID: mdl-29501213

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding. A noninvasive diagnostic approach, including oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest, is used to support a diagnosis of PVOD. PVOD prognosis is worse than other forms of PAH. There is no evidence-based medical therapy for PVOD and life-threatening pulmonary edema may occur following PAH targeted therapy in PVOD. Lung transplantation remains the preferred definitive therapy for eligible patients.

Pulmonary Veno-Occlusive Disease , Animals , Diagnostic Imaging/methods , Disease Models, Animal , Humans , Pulmonary Veno-Occlusive Disease/diagnosis , Pulmonary Veno-Occlusive Disease/epidemiology , Pulmonary Veno-Occlusive Disease/therapy , Rare Diseases/diagnosis , Rare Diseases/epidemiology , Rare Diseases/therapy , Respiratory Function Tests/methods , Risk Factors
Thromb Haemost ; 112(3): 598-605, 2014 Sep 02.
Article En | MEDLINE | ID: mdl-24898545

Chronic thromboembolic pulmonary hypertension (CTEPH) has been estimated to occur in 0.1-0.5% of patients who survive a pulmonary embolism (PE), but more recent prospective studies suggest that its incidence may be much higher. The absence of initial haemodynamic evaluation at the time of PE should explain this discrepancy. We performed a prospective multicentre study including patients with PE in order to assess the prevalence and to describe risk factors of CTEPH. Follow-up every year included an evaluation of dyspnea and echocardiography using a predefined algorithm. In case of suspected CTEPH, the diagnosis was confirmed using right heart catheterisation (RHC). Signs of CTEPH were searched on the multidetector computed tomography (CT) and echocardiography performed at the time of PE. Of the 146 patients analysed, eight patients (5.4%) had suspected CTEPH during a median follow-up of 26 months. CTEPH was confirmed using RHC in seven cases (4.8%; 95%CI, 2.3 - 9.6) and ruled-out in one. Patients with CTEPH were older, had more frequently previous venous thromboembolic events and more proximal PE than those without CTEPH. At the time of PE diagnosis, patients with CTEPH had a higher systolic pulmonary artery pressure and at least two signs of CTEPH on the initial CT. After acute PE, the prevalence of CTEPH appears high. However, initial echocardiography and CT data at the time of the index PE suggest that a majority of patients with CTEPH had previously unknown pulmonary hypertension, indicating that a first clinical presentation of CTEPH may mimic acute PE.

Hypertension, Pulmonary/epidemiology , Pulmonary Embolism/epidemiology , Acute Disease , Adult , Age Factors , Aged , Chronic Disease , Echocardiography , Follow-Up Studies , France , Humans , Incidence , Middle Aged , Multidetector Computed Tomography , Prevalence , Prospective Studies , Pulmonary Embolism/diagnosis , Risk Factors
Rev Mal Respir ; 29(4): 491-500, 2012 Apr.
Article Fr | MEDLINE | ID: mdl-22542407

Pulmonary arterial hypertension (PAH) is a rare but potentially fatal complication of human immunodeficiency virus (HIV). It may occur in HIV-1 or 2 infection, irrespective of the route of transmission or the degree of immunosuppression. The improved survival of patients infected with HIV in the era of highly active antiretroviral therapy (HAART) justifies systematic screening for PAH according to an algorithm in patients with unexplained dyspnea. In all cases, right heart catheterization must be performed to establish the definitive diagnosis of pulmonary hypertension. The prevalence of PAH is about 0.5% in patients with HIV infection. A beneficial effect of HAART on the course of HIV-related PAH has not been clearly established. In contrast, PAH-specific therapies such as epoprostenol and bosentan have been demonstrated to be efficacious for short- and long-term outcomes in this context. Notably, some patients pulmonary hemodynamics and functional class normalized or near normalized with these treatments. Other PAH-specific therapies remain to be evaluated. The advent of HAART associated with the development of PAH-specific therapies has improved the prognosis of patients HIV-related PAH, with a survival rate of about 70% at 3 years.

HIV Infections/complications , HIV-1/physiology , Hypertension, Pulmonary/etiology , Diagnostic Techniques, Cardiovascular , Diagnostic Techniques, Respiratory System , HIV Infections/diagnosis , HIV Infections/epidemiology , HIV Infections/therapy , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/therapy , Models, Biological
Arthritis Rheum ; 64(9): 2995-3005, 2012 Sep.
Article En | MEDLINE | ID: mdl-22549387

OBJECTIVE: Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) characterized by obstruction of small pulmonary veins. Pulmonary venous involvement has been reported in pathologic assessment of patients with systemic sclerosis (SSc) presenting with precapillary PH. High-resolution computed tomography (HRCT) of the chest is a noninvasive diagnostic tool used to screen for PVOD. No HRCT data are available on SSc patients with precapillary PH. We undertook this study to evaluate the frequency and effect on prognosis of HRCT signs of PVOD in SSc patients with precapillary PH. METHODS: We reviewed chest HRCT data from 26 SSc patients with precapillary PH and 28 SSc patients without pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD). RESULTS: The radiographic triad of HRCT signs of PVOD (lymph node enlargement [57.7% versus 3.6%], centrilobular ground-glass opacities [46.2% versus 10.7%], and septal lines [88.5% versus 7.1%]) was significantly more frequent in SSc patients with precapillary PH than in SSc patients without PAH or ILD (all P < 0.005). Indeed, 61.5% of SSc patients with precapillary PH had ≥ 2 of these signs. Cardiomegaly (P < 0.0001), pulmonary artery enlargement (P < 0.0001), and pericardial effusion (P < 0.0005) were also significantly more frequent in SSc patients with precapillary PH. Pulmonary venous involvement was histologically confirmed in 2 patients with radiographic signs of PVOD. The presence of ≥ 2 radiographic signs of PVOD was associated with the occurrence of pulmonary edema after initiation of PAH-specific therapy (in 8 of 16 patients) and with more rapid progression from diagnosis of PH to death. CONCLUSION: HRCT signs of PVOD are frequently observed in SSc patients with precapillary PH, correlated with histologic assessment, and were associated with a high risk of pulmonary edema.

Hypertension, Pulmonary/diagnostic imaging , Pulmonary Veno-Occlusive Disease/diagnostic imaging , Scleroderma, Systemic/diagnostic imaging , Adult , Aged , Aged, 80 and over , Female , Humans , Hypertension, Pulmonary/complications , Male , Middle Aged , Prognosis , Pulmonary Veno-Occlusive Disease/complications , Radiography , Scleroderma, Systemic/complications
Eur Respir J ; 39(2): 313-8, 2012 Feb.
Article En | MEDLINE | ID: mdl-21737562

The mean pulmonary artery pressure (P(pa)) achieved on mild-to-moderate exercise is age related and its haemodynamic correlates remain to be documented in patients free of pulmonary hypertension (PH). Our retrospective study involved patients free of PH investigated in our centre for possible pulmonary vascular disease between January 1, 2007 and October 31, 2009 who underwent right heart catheterisation at rest and during supine exercise up to 60 W. The 38 out of 99 patients aged <50 yrs were included and a P(pa) of 30 mmHg was considered the upper limit of normal on exercise. The 24 subjects who developed P(pa)>30 mmHg on exercise had higher resting P(pa) (19±3 versus 15±4 mmHg) and indexed pulmonary vascular resistance (PVRi; 3.4±1.5 versus 2.2±1.1 WU·m(2); p<0.05) than the remaining 14 subjects. Resting P(pa) >15 mmHg predicted exercise P(pa) >30 mmHg with 88% sensitivity and 57% specificity. The eight patients with resting P(pa) 22-24 mmHg all had exercise P(pa) >30 mmHg. In subjects aged <50 yrs investigated for possible pulmonary vascular disease and free of PH, patients with mild-to-moderate exercise P(pa) >30 mmHg had higher resting PVRi and higher resting P(pa), although there was no resting P(pa) threshold value that could predict normal response on mild-to-moderate exercise. The clinical relevance of such findings deserves further long-term follow-up studies.

Cardiac Output/physiology , Exercise/physiology , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/physiopathology , Pulmonary Wedge Pressure/physiology , Rest/physiology , Adult , Cardiac Catheterization , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Pulmonary Artery/physiology , ROC Curve , Retrospective Studies , Sensitivity and Specificity , Supine Position
Eur Respir Rev ; 20(122): 254-61, 2011 Dec.
Article En | MEDLINE | ID: mdl-22130818

Over the past decade, awareness among the medical profession of pulmonary arterial hypertension (PAH) being a treatable disease has increased. Despite this, approximately one-fifth of newly diagnosed patients are classified as being in the most severely compromised functional class (i.e. New York Health Association/World Health Organization functional class (NYHA/WHO FC) IV). The prognosis for patients in NYHA/WHO FC IV is poor, with 3-yr survival being around 40%, even with treatment. Poor prognosis coupled with severe functional impairment means it is vital that these patients receive optimal treatment. There are also subgroups of patients, who, although classified as NYHA/WHO FC III, may actually be severely haemodynamically compromised and at risk of rapid deterioration. Such subgroups include patients with PAH associated with systemic sclerosis or certain heritable mutations. These patients should be considered as being at the more severe end of the disease spectrum. In this article we will discuss the optimal management of patients with severe PAH. This includes newly emerging evidence from small-scale, open-label studies that use upfront combination therapy with intravenous epoprostenol plus oral PAH-specific drugs. We also review treatment strategies that may offer clinical benefits to patients with more severe PAH.

Disease Management , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Severity of Illness Index , Antihypertensive Agents/therapeutic use , Drug Therapy, Combination , Epoprostenol/therapeutic use , Familial Primary Pulmonary Hypertension , Humans , Lung Transplantation , Prognosis
Rev Mal Respir ; 28(2): 139-51, 2011 Feb.
Article Fr | MEDLINE | ID: mdl-21402229

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a severe disease that has undergone a dramatic improvement in therapeutic management over the past 20 years. Among the new therapeutic options, surgery has the potential to dramatically improve or, in some cases, cure PAH. BACKGROUND: Surgical treatment of PAH includes pulmonary endarterectomy which can cure PAH when the cause is obstruction of the pulmonary arteries by fibrous tissue resulting from pulmonary embolism, by tumours as angiosarcomas, and echinococcus cysts. Transplantation is required in end-stage PAH after failure of medical treatment. Atrial septostomy and Potts procedure are palliative surgical procedures that can delay transplantation. VIEWPOINT: Extracorporeal cardiopulmonary support is the latest surgical improvement, not only as a bridge to transplantation in end-stage PAH but also during recovery after transplantation or pulmonary endarterectomy. CONCLUSIONS: Surgery is part of the therapeutic management of PAH. Dialogue between physicians and surgeons is a prerequisite for any reasoned therapeutic decision.

Hypertension, Pulmonary , Endarterectomy , Extracorporeal Circulation , Familial Primary Pulmonary Hypertension , Heart Transplantation , Humans , Hypertension, Pulmonary/surgery , Lung Transplantation , Patient Selection , Postoperative Care
Eur Respir J ; 37(4): 813-22, 2011 Apr.
Article En | MEDLINE | ID: mdl-20693255

Pulmonary arterial hypertension (PAH) is associated with dysregulated bone morphogenetic protein receptor (BMPR)-II signaling and pulmonary vascular inflammation. We evaluated the effects of dexamethasone on monocrotaline (MCT)-induced PAH in rats for potential reversal of PAH at late time-points. Saline-treated control, MCT-exposed, MCT-exposed and dexamethasone-treated rats (5 mg·kg⁻¹·day⁻¹, 1.25 mg·kg⁻¹ and 2.5 mg·kg⁻¹·48 h⁻¹) were evaluated at day 28 and day 35 following MCT for haemodynamic parameters, right ventricular hypertrophy, morphometry, immunohistochemistry, and IL6 and BMPR2 expression. Dexamethasone improved haemodynamics and pulmonary vascular remodelling, preventing PAH development at early (day 1-14 and 1-28) and reversing PAH at late (day 14-28 and 21-35) time-points following MCT, as well as improving survival in MCT-exposed rats compared with controls. Both MCT-induced pulmonary IL6 overexpression and interleukin (IL)-6-expressing adventitial inflammatory cell infiltration were reduced with dexamethasone. This was associated with pulmonary BMPR2 downregulation following MCT, which was increased with dexamethasone, in whole lung and control pulmonary artery smooth muscle cells. Dexamethasone also reduced proliferation of rat pulmonary artery smooth muscle cells in vitro. Experimental PAH can be prevented and reversed by dexamethasone, and survival is improved. In this model, mechanisms may involve reduction of IL-6-expressing inflammatory cells, restoration of pulmonary BMPR2 expression and reduced proliferation of vascular smooth muscle cells.

Dexamethasone/pharmacology , Lung/drug effects , Monocrotaline/pharmacology , Muscle, Smooth/drug effects , Animals , Anti-Inflammatory Agents/pharmacology , Bone Morphogenetic Protein Receptors/metabolism , Cell Proliferation , Familial Primary Pulmonary Hypertension , Hemodynamics , Hypertension, Pulmonary/drug therapy , Immunohistochemistry/methods , Interleukin-6/metabolism , Male , Rats , Rats, Wistar , Treatment Outcome
Int J Clin Pract Suppl ; (169): 11-8, 2011 Jan.
Article En | MEDLINE | ID: mdl-21176011

Portopulmonary hypertension (PoPH) is a rare but life-threatening complication of portal hypertension that is characterised by proliferative changes in the pulmonary microvasculature indistinguishable from other forms of pulmonary arterial hypertension (PAH). Although PoPH is most commonly observed in the setting of cirrhosis, patients with non-cirrhotic portal hypertension are also at risk of developing the disorder. A definitive diagnosis requires invasive haemodynamic confirmation by right heart catheterisation and screening for PoPH should be routinely performed in all patients being considered for liver transplantation. Although severe PoPH is considered a contraindication to liver transplantation, there is now compelling data supporting the use of PAH-specific therapies with the aim of improving pulmonary haemodynamics to allow transplantation to be successfully performed. This review explores possible relevant aetiological factors and summarises current diagnostic and therapeutic approaches for PoPH patients.

Hypertension, Portal/complications , Hypertension, Pulmonary/complications , Diuretics/therapeutic use , Endothelin Receptor Antagonists , Hemodynamics/physiology , Humans , Hypertension, Portal/diagnosis , Hypertension, Portal/drug therapy , Hypertension, Portal/physiopathology , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/physiopathology , Phosphodiesterase 5 Inhibitors/therapeutic use , Pulmonary Circulation/physiology
Eur Respir J ; 37(6): 1392-9, 2011 Jun.
Article En | MEDLINE | ID: mdl-20884740

Haematopoietic c-kit+ progenitor cells may contribute to pulmonary vascular remodelling and pulmonary hypertension (PH). Stromal derived factor-1 (SDF-1/CXCL12) and its receptors CXCR4 and CXCR7 have been shown to be critical for homing and mobilisation of haematopoietic c-kit+ progenitor cells in the perivascular niche. We administered AMD3100, a CXCR4 antagonist, and CCX771, a CXCR7 antagonist, to chronic hypoxia exposed mice in order to study the role of c-kit+ progenitor cells in PH. CXCL12, CXCR4 and CXCR7 protein expression, haemodynamic parameters, right ventricular mass, extent of vascular remodelling and perivascular progenitor cell accumulation were studied. Chronic hypoxia-exposed mice showed increased total lung tissue expression of CXCR4, CXCR7 and CXCL12 after development of PH. This was associated with significantly increased right ventricular systolic pressure and evidence of right ventricular hypertrophy, vascular remodelling and perivascular c-kit+/sca-1+ progenitor cell accumulation. CCX771 administration did not abrogate these effects. In contrast, administration of AMD3100, whether alone or combined with CCX771, prevented vascular remodelling, PH and perivascular accumulation of c-kit+/sca-1+ progenitor cells, with a synergistic effect of these agents. This study offers important pathophysiological insights into the role of haematopoietic c-kit+ progenitors in hypoxia-induced vascular remodelling and may have therapeutic implications for PH.

Hematopoietic Stem Cells/drug effects , Heterocyclic Compounds/administration & dosage , Hypertension, Pulmonary/drug therapy , Hypoxia/drug therapy , Proto-Oncogene Proteins c-kit/physiology , Animals , Antigens, Ly/metabolism , Chemokine CXCL12/biosynthesis , Hematopoietic Stem Cells/metabolism , Hematopoietic Stem Cells/physiology , Hypertension, Pulmonary/metabolism , Hypertension, Pulmonary/physiopathology , Hypertrophy, Right Ventricular/drug therapy , Hypoxia/metabolism , Hypoxia/physiopathology , Lung/blood supply , Male , Membrane Proteins/metabolism , Mice , Mice, Inbred C57BL , Receptors, CXCR/antagonists & inhibitors , Receptors, CXCR/biosynthesis , Receptors, CXCR4/antagonists & inhibitors , Receptors, CXCR4/biosynthesis
Eur Respir Rev ; 19(118): 279-87, 2010 Dec.
Article En | MEDLINE | ID: mdl-21119186

Pulmonary arterial hypertension (PAH) is a severe, progressive condition of the small pulmonary vessels that leads to increased pulmonary vascular resistance, right ventricular failure and death. Patients in World Health Organization functional class (WHO FC) IV are the most severely affected in terms of disease severity, symptomatic impairment, exercise capacity and haemodynamics, with a very poor prognosis and low survival rate. Recent developments in PAH-specific therapies have conferred significant prognostic improvements upon PAH patients, especially when coupled with management strategies such as goal-oriented therapy and combination treatment. Despite these important developments, the outlook for WHO FC IV PAH patients remains poor. This article examines the recommendations for WHO FC IV patients that appear in current PAH treatment guidelines and the research underpinning this guidance, and discusses possible future directions for treatment of this severely unwell patient population.

Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Severity of Illness Index , Drug Therapy, Combination , Humans , Hypertension, Pulmonary/classification , Lung Transplantation , Practice Guidelines as Topic , Prognosis , World Health Organization
Eur Respir J ; 36(3): 549-55, 2010 Sep.
Article En | MEDLINE | ID: mdl-20562126

Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. We studied 674 consecutive adult patients who were prospectively enrolled in the French PAH registry (121 incident and 553 prevalent cases). Two survival analyses were performed. First, the cohort of 674 patients was followed for 3 yrs after study entry and survival rates described. Then, we focused on the subset with incident idiopathic, familial and anorexigen-associated PAH (n = 56) combined with prevalent patients who were diagnosed <3 yrs prior to study entry (n = 134). In the cohort of 674 patients, 1-, 2-, and 3-yr survival rates were 87% (95% CI 84-90), 76% (95% CI 73-80), and 67% (95% CI 63-71), respectively. In prevalent idiopathic, familial and anorexigen-associated PAH, 1-, 2-, and 3-yr survival rates were higher than in incident patients (p = 0.037). In the combined cohort of patients with idiopathic, familial and anorexigen-associated PAH, multivariable analysis showed that survival could be estimated by means of a novel risk-prediction equation using patient sex, 6-min walk distance, and cardiac output at diagnosis. This study highlights survivor bias in prevalent cohorts of PAH patients. Survival of idiopathic, familial and anorexigen-associated PAH can be characterised by means of a novel risk-prediction equation using patients' characteristics at diagnosis.

Hypertension, Pulmonary , Aged , Cohort Studies , Familial Primary Pulmonary Hypertension , Female , Humans , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/mortality , Male , Middle Aged , Multivariate Analysis , Prevalence , Pulmonary Medicine/methods , Risk Factors , Treatment Outcome
Rev Mal Respir ; 27(2): 141-50, 2010 Feb.
Article Fr | MEDLINE | ID: mdl-20206062

INTRODUCTION: A joint Task Force of the ESC and of the ERS has developed guidelines on the diagnosis and treatment of pulmonary hypertension (PH) to provide updated information on the management of patients with this condition. STATE OF THE ART: The term pulmonary hypertension (PH) describes a group of devastating and life-limiting diseases, defined by mean pulmonary artery pressure >25 mmHg at rest. The diagnosis of PH requires a series of investigations intended to confirm the diagnosis, clarify the clinical group and the specific aetiology and an algorithm for this is proposed. Several drugs are currently approved to try to correct endothelial dysfunction. They lead to a significant improvement in the prognosis of patients who are in NYHA functional class II, III or IV. The evaluation of the severity of PH has a pivotal role in the choice of initial treatment and evaluation of the response to therapy in individual patients. PERSPECTIVE: These guidelines should be widely disseminated and implemented in order to improve the management of patients with PH. CONCLUSION: These guidelines summarise recent advances in the understanding and management of PH.

Antihypertensive Agents/therapeutic use , Evidence-Based Medicine , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/drug therapy , Algorithms , Antihypertensive Agents/adverse effects , Bosentan , Carbolines/adverse effects , Carbolines/therapeutic use , Drug Therapy, Combination , Endothelin Receptor Antagonists , Europe , Humans , Hypertension, Pulmonary/classification , Hypertension, Pulmonary/etiology , Piperazines/adverse effects , Piperazines/therapeutic use , Practice Guidelines as Topic , Purines/adverse effects , Purines/therapeutic use , Sildenafil Citrate , Sulfonamides/adverse effects , Sulfonamides/therapeutic use , Sulfones/adverse effects , Sulfones/therapeutic use , Tadalafil , Vasodilator Agents/adverse effects , Vasodilator Agents/therapeutic use
Eur Respir J ; 35(1): 105-11, 2010 Jan.
Article En | MEDLINE | ID: mdl-19643948

This study aims to describe the haemodynamic and survival characteristics of patients with pulmonary hypertension in the recently individualised syndrome of combined pulmonary fibrosis and emphysema. A retrospective multicentre study was conducted in 40 patients (38 males; age 68+/-9 yrs; 39 smokers) with combined pulmonary fibrosis and emphysema, and pulmonary hypertension at right heart catheterisation. Dyspnoea was functional class II in 15%, III in 55% and IV in 30%. 6-min walk distance was 244+/-126 m. Forced vital capacity was 86+/-18%, forced expiratory volume in 1 s 78+/-19%, and carbon monoxide diffusion transfer coefficient 28+/-16% of predicted. Room air arterial oxygen tension was 7.5+/-1.6 kPa (56+/-12 mmHg). Mean pulmonary artery pressure was 40+/-9 mmHg, cardiac index 2.5+/-0.7 L x min(-1) x m(-2) and pulmonary vascular resistance 521+/-205 dyn x s x cm(-5). 1-yr survival was 60%. Higher pulmonary vascular resistance, higher heart rate, lower cardiac index and lower carbon monoxide diffusion transfer were associated with shorter survival. Patients with combined pulmonary fibrosis and emphysema syndrome and pulmonary hypertension confirmed by right heart catheterisation have a dismal prognosis despite moderately altered lung volumes and flows and moderately severe haemodynamic parameters.

Hypertension, Pulmonary/etiology , Pulmonary Emphysema/complications , Pulmonary Fibrosis/complications , Aged , Aged, 80 and over , Female , Hemodynamics , Humans , Hypertension, Pulmonary/mortality , Hypertension, Pulmonary/physiopathology , Male , Middle Aged , Retrospective Studies , Survival Rate , Syndrome