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Angiostatic Factors in the Pulmonary Endarterectomy Material from Chronic Thromboembolic Pulmonary Hypertension Patients Cause Endothelial Dysfunction  [PDF]
Diana Zabini, Chandran Nagaraj, Elvira Stacher, Irene M. Lang, Patrick Nierlich, Walter Klepetko, Akos Heinemann, Horst Olschewski, Zoltán Bálint, Andrea Olschewski
PLOS ONE , 2012, DOI: 10.1371/journal.pone.0043793
Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease with persistent thrombotic occlusion or stenosis of the large pulmonary arteries resulting in pulmonary hypertension. Surgical removal of the neointimal layer of these vessels together with the non-resolved thrombus consisting of organized collagen-rich fibrotic areas with partly recanalized regions is the treatment of choice (pulmonary endarterectomy, PEA). The present study investigates endothelial cells isolated from such material as well as factors present in the surgical PEA material, which may contribute to impairment of recanalization and thrombus non-resolution. We observed muscularized vessels and non-muscularized vessels in the PEA material. The isolated endothelial cells from the PEA material showed significantly different calcium homeostasis as compared to pulmonary artery endothelial cells (hPAECs) from normal controls. In the supernatant (ELISA) as well as on the tissue level (histochemical staining) of the PEA material, platelet factor 4 (PF4), collagen type I and interferon-gamma-inducible 10 kD protein (IP-10) were detected. CXCR3, the receptor for PF4 and IP-10, was particularly elevated in the distal parts of the PEA material as compared to human control lung (RT-PCR). PF4, collagen type I and IP-10 caused significant changes in calcium homeostasis and affected the cell proliferation, migration and vessel formation in hPAECs. The presence of angiostatic factors like PF4, collagen type I and IP-10, as recovered from the surgical PEA material from CTEPH patients, may lead to changes in calcium homeostasis and endothelial dysfunction.
Chronic Thromboembolic Pulmonary Hypertension: Treat the Patient Not the Haemodynamics
Ben Dunne,Annika van den Broek,Vaughan Williams,Gregory Smith,Tamas Revesz,Mark Edwards,Eli Gabbay
Case Reports in Pulmonology , 2012, DOI: 10.1155/2012/108672
Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a disabling condition that is being increasingly recognised. It is unique as a cause of pulmonary hypertension in that it is surgically curable. We wish to highlight the importance of recognition and early referral of any patient who may have CTEPH even in the absence of resting pulmonary hypertension as excellent results can be achieved by restoring pulmonary vascular anatomy, reducing exercise-induced pulmonary hypertension, and reducing dead-space ventilation. We present a case that illustrates these points and discuss our experience as a referral centre for CTEPH.
Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: A case report and review of the literature
Lina Gumbien , , , Taida Ivanauskien , Mindaugas Mata iūnas , Rolandas Zablockis , Raimondas irmenis , Karolis Urbonas , Rimantas Karalius
Seminars in Cardiovascular Medicine , 2012, DOI: 10.2478/v10287-012-0004-2
Abstract: Chronic thromboembolic pulmonary hypertension is a progressive and disabling disease with high morbidity and mortality. Pulmonary endarterectomy has been approved as a first choice treatment for chronic thromboembolic pulmonary hypertension with obstruction of proximal pulmonary arteries. The aim of the article is to describe our first experience in this kind of treatment. Diagnosis of chronic thromboembolic pulmonary hypertension, criteria for the surgery, operative technique and results, perioperative management are discussed.
Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor?
Terra-Filho, Mario;Mello, Marcos Figueiredo;Lapa, M?nica Silveira;Teixeira, Ricardo Henrique Oliveira Braga;Jatene, Fábio Biscegli;
Clinics , 2010, DOI: 10.1590/S1807-59322010001100018
Abstract: introduction: chronic thromboembolic pulmonary hypertension is a disease affecting approximately 4,000 people per year in the united states. the incidence rate in brazil, however, is unknown. the estimated survival for patients with chronic thromboembolic pulmonary hypertension without treatment is approximately three years. pulmonary thromboendarterectomy for select patients is a potentially curative procedure when correctly applied. in brazil, the clinical and hemodynamic profiles of chronic thromboembolic pulmonary hypertension patients have yet to be described. objectives: to evaluate the clinical and hemodynamic characteristics of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy in a referral center for chronic thromboembolic pulmonary hypertension treatment in brazil. methods: from december 2006 to november 2009, patients were evaluated and scheduled for pulmonary thromboendarterectomy. the subjects were classified according to gender, age and functional class and were tested for thrombofilia and brain natriuretic peptide levels. results: thirty-five consecutive chronic thromboembolic pulmonary hypertension patients were evaluated. two patients tested positive for schistosomiasis, and 31 were enrolled in the study (19 female, 12 male). the majority of patients were categorized in functional classes iii and iv. hemodynamic data showed a mean pulmonary vascular resistance (pvr) of 970.8 ± 494.36 dynas·s·cm-5 and a low cardiac output of 3.378 ± 1.13 l/min. linear regression revealed a direct relation between cardiac output and pulmonary vascular resistance. paradoxical septal movement was strongly correlated with pulmonary vascular resistance and cardiac output (p=0.001). brain natriuretic peptide serum levels were elevated in 19 of 27 patients. conclusions: in a referral center for pulmonary hypertension in brazil, chronic thromboembolic pulmonary hypertension patients evaluated for pulmonary thromboendarterect
Cardiovocal Syndrome (Ortner's Syndrome) Associated with Chronic Thromboembolic Pulmonary Hypertension and Giant Pulmonary Artery Aneurysm: Case Report and Review of the Literature  [PDF]
Jaakko Heikkinen,Katrin Milger,Enrique Alejandre-Lafont,Christian Woitzik,Detlef Litzlbauer,Julia-Franziska Vogt,Jens Peter Klu?mann,Ardeschir Ghofrani,Gabriele A. Krombach,Henning Tiede
Case Reports in Medicine , 2012, DOI: 10.1155/2012/230736
Abstract: Cardiovocal syndrome or Ortner's syndrome is hoarseness due to left recurrent laryngeal nerve palsy caused by mechanical affection of the nerve from enlarged cardiovascular structures. Chronic thromboembolic pulmonary hypertension is extremely rarely found to cause this syndrome. We describe a case of a 56-year-old patient with sudden onset of hoarseness. The patient had known long standing severe pulmonary hypertension. Fiberoptic laryngoscopy showed left vocal cord palsy. Computed tomography of the neck and chest revealed extensive enlargement of the pulmonary arteries and excluded a malignant tumor. The diagnosis of cardiovocal syndrome was retained. It is important for the radiologist to be aware of this possible etiology causing left recurrent laryngeal nerve palsy and to understand its mechanism. 1. Introduction Hoarseness of voice is a very common condition and underlying causes vary from reversible benign causes to life-threatening malignancies. Unilateral recurrent laryngeal nerve injury is most commonly caused by surgical trauma or a malignant tumor [1]. Cardiovocal syndrome or Ortner’s syndrome is hoarseness due to left recurrent laryngeal nerve palsy caused mainly by mechanical affection of the nerve from enlarged cardiovascular structures. Cardiovocal syndrome is a rare condition and to our knowledge only 40 patients have been presented in the literature as a thorough research in PubMed showed. It is also described to be more common in men because possibly of a higher incidence of cardiovascular conditions than in females [2]. Cardiovocal syndrome has a peak incidence in older age, but can occur in any age group, and it has been described even in infants [3]. We present a case report of Ortner’s syndrome of uncommon etiology that illustrates the underlying pathoanatomical mechanisms. 2. Case Report A 56-year-old patient with a fifteen-year history of known severe chronic thromboembolic pulmonary hypertension (CTEPH) and chronic obstructive pulmonary disease (COPD) developed sudden hoarseness of voice. He had a history of smoking (over 25 pack years). Fiberoptic laryngoscopy revealed left vocal cord palsy (Figure 1), while the rest of the otolaryngologic exam was normal. Chest radiograph was relatively unchanged from previous studies obtained years ago and demonstrated massive dilatation of the pulmonary arteries, and tapering of peripheral pulmonary vessels (Figure 2). Contrast-enhanced computed tomography (CT) of the neck and chest was performed to evaluate the patient for a possible malignant process causing the symptoms. The CT
Risk factors for chronic thromboembolic pulmonary hypertension  [cached]
N.H. Kim,I.M. Lang
European Respiratory Review , 2012,
Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by obstruction and vascular remodelling of pulmonary arteries following pulmonary embolism. Risk factors that predispose patients to CTEPH include the size of the initial thrombus and numerous associated host or medical conditions. Haemostatic risk factors include elevated levels of factor VIII and phospholipid antibodies or intrinsic abnormalities in fibrinogen. Medical conditions that are associated with an increased risk of CTEPH include a history of splenectomy, cancer, ventriculoatrial shunt, chronic inflammatory disease, antiphospholipid antibodies and hypothyroidism. Although CTEPH is potentially curable by pulmonary endarterectomy (PEA), up to 40% of patients evaluated for PEA may be denied surgery depending on the level of surgical experience and disease accessibility after pre-operative assessment. Furthermore, an estimated 10–15% of patients are at risk for residual pulmonary hypertension following PEA surgery, due to significant concomitant small-vessel disease. However, pre-operative identification of small-vessel involvement remains a challenge. The current medications effective in the treatment of pulmonary arterial hypertension have not demonstrated efficacy in CTEPH. Accordingly, identification of CTEPH, followed by early referral for evaluation and treatment by an experienced PEA centre, is recommended.
Riociguat: an upcoming therapy in chronic thromboembolic pulmonary hypertension?
N. H. Kim
European Respiratory Review , 2010,
Abstract: Although pulmonary endarterectomy remains the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH), not all patients will benefit from or receive this highly specialised surgery. Patients whose CTEPH is deemed inoperable by an experienced centre and patients with persistent pulmonary hypertension after surgery are candidates for trial of pulmonary arterial hypertension (PAH) specific pharmacotherapies. However, the currently available PAH specific pharmacotherapies have not demonstrated a clear benefit in either of these patient groups. Accordingly, PAH therapies remain off-label for use in CTEPH. Riociguat (BAY 63-2521) is a stimulator of soluble guanylate cyclase, and may represent a novel agent in the treatment of select patients with CTEPH. Pre-clinical and human phase II studies with riociguat have reported promising results, and a multinational, randomised, controlled, double-blinded phase III study is currently underway to investigate the effect of riociguat in patients with inoperable CTEPH and those with persistent or recurrent pulmonary hypertension following pulmonary endarterectomy.
Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options
I. M. Lang
European Respiratory Review , 2009,
Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition in which organised thrombi obstruct the pulmonary vessels, causing increased pulmonary vascular resistance, progressive pulmonary hypertension (PH) and right heart failure. The treatment of choice is pulmonary endarterectomy, which restores pulmonary haemodynamics with acceptable periprocedural mortality rates in the majority of suitable patients. However, CTEPH may be inoperable owing to surgically inaccessible thrombi or comorbid diseases that confer an unacceptably high risk. Pharmacotherapies, although not yet approved, may be useful in this situation or for treating residual or recurrent PH following surgery. Vasodilator drugs for PH are attracting growing interest as potential treatments for CTEPH because this disease has recently been labelled as a "dual" pulmonary vascular disorder: major vessel obstruction and remodelling is combined with a small vessel arteriopathy that is histologically indistinguishable from the classical pulmonary arteriopathy observed in pulmonary arterial hypertension. Of three completed randomised controlled trials in patients with CTEPH, only one was powered to detect a treatment effect. The BENEFIT trial employed the dual endothelin-receptor antagonist bosentan. Although haemodynamics improved significantly, the second component of the primary end-point, exercise capacity, was not met. More evidence is required to resolve whether vasodilator treatments are beneficial for inoperable chronic thromboembolic pulmonary hypertension.
Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
M. Humbert
European Respiratory Review , 2010,
Abstract: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis), and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy.
Chronic Thromboembolic Pulmonary Hypertension: Treat the Patient Not the Haemodynamics  [PDF]
Ben Dunne,Annika van den Broek,Vaughan Williams,Gregory Smith,Tamas Revesz,Mark Edwards,Eli Gabbay
Case Reports in Pulmonology , 2012, DOI: 10.1155/2012/108672
Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a disabling condition that is being increasingly recognised. It is unique as a cause of pulmonary hypertension in that it is surgically curable. We wish to highlight the importance of recognition and early referral of any patient who may have CTEPH even in the absence of resting pulmonary hypertension as excellent results can be achieved by restoring pulmonary vascular anatomy, reducing exercise-induced pulmonary hypertension, and reducing dead-space ventilation. We present a case that illustrates these points and discuss our experience as a referral centre for CTEPH. 1. Introduction We report the case of a 15-year-old boy, heterozygous for the antithrombin mutation, who developed chronic thromboembolic pulmonary hypertension (CTEPH) secondary to recurrent pulmonary emboli and was successfully treated with pulmonary thromboendarterectomy. This case highlights a number of points relevant to both primary care physicians and those working in specialist centres. In particular, we wish to emphasise that significantly symptomatic CTEPH can present with only borderline abnormal resting pulmonary haemodynamics and that it probably remains significantly underdiagnosed. Recognition of this complex condition allows the consideration of curative surgery and a return to normal life for the patient. 2. Case Report Our patient presented at the age of 15 to the ED of an Adelaide hospital with a several-month history of breathlessness, nausea, and dull bilateral chest pain, which worsened over 7 days. He had presented on several occasions in the preceding few months to primary care doctors without an apparent diagnosis. By the time of presentation to the ED, he was noted to be hypoxaemic and had clinical signs of right heart dysfunction (tender hepatomegaly and elevated jugular venous pressure) without dependent oedema. He was known to have antithrombin deficiency due to a heterozygous mutation, being diagnosed at the age of 7 after screening in view of a strong family history on his maternal side. His AT III levels were recorded at 38% predicted, which is lower than the generally reported range of 40–60% associated with heterozygosity [1]. He had no other known risk factors for venous thromboembolism. CTPA demonstrated evidence of acute bilateral submassive pulmonary emboli extending from both left and right pulmonary arteries to the subsegmental level involving all lobes of the lungs. Further, there was evidence of chronic thromboembolism with webs and cutoffs in both segmental and subsegmental arteries
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