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Treating the cardio-oncology patient
Published in Susan F. Dent, Practical Cardio-Oncology, 2019
Pulmonary hypertension has several causes in cancer patients. It may be a sign of cancer progression, such as a progressive myeloproliferative disease (57). It may also be caused by cancer therapy (58). For instance, dasatinib therapy, used to treat chronic myelogenous leukemia and acute lymphoblastic leukemia, has been associated with pulmonary hypertension; dasatinib-related pulmonary hypertension can be treated with sildenafil (59,60). The veno-occlusive form of pulmonary hypertension may be associated with alkylating agents, but medical management of this form of pulmonary hypertension is usually unsatisfactory (61). Chronic thromboembolic pulmonary hypertension may be observed in cancer patients and must be distinguished from pulmonary hypertension associated with HF or lung diseases (62).
Clinical Diagnosis of Pulmonary Vascular Disease
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, Diagnostic Pulmonary Pathology, 2008
An assessment of pulmonary function is appropriate in the evaluation of a patient with evidence of pulmonary vascular disease to exclude significant airway or parenchymal lung disease. In patients with chronic airflow obstruction, the presence and severity of pulmonary hypertension correlates with the degree of airflow obstruction. Most patients with a forced expiratory volume in one second (FEV1) below 1 L will have pulmonary hypertension, although the degree of hemodynamic abnormality is usually modest compared with conditions that primarily affect the pulmonary circulation (IPAH, chronic thromboembolic disease, connective tissue disease) (6). Patients with interstitial lung disease develop secondary pulmonary hypertension once both a significant impairment in intrapulmonary gas exchange [diffusing capacity of lung for carbon monoxide (DLCO) less than 50% of predicted] and restriction in lung volume (forced vital capacity less than 50% predicted) are present. For all forms of parenchymal lung disease, significant hypoxemia correlates with the presence and severity of pulmonary hypertension, consistent with the known effects of hypoxia on pulmonary vasoreactivity and remodeling. Pulmonary function studies may be mildly abnormal in patients with PPH or chronic thromboembolic pulmonary hypertension (CTEPH). A mild restrictive ventilatory defect may be observed and the DLCO may be moderately to severely reduced. The latter abnormality is because of the reduction in cross-sectional vascular surface area and the diminution in pulmonary blood volume that are characteristic of these conditions.
Pulmonary hypertension: Hemodynamic assessment and response to vasodilators
Published in Debabrata Mukherjee, Eric R. Bates, Marco Roffi, Richard A. Lange, David J. Moliterno, Nadia M. Whitehead, Cardiovascular Catheterization and Intervention, 2017
Myung H. Park, Vallerie V. Mclaughlin
Riociguat is the first agent within the soluble guanylate cyclase stimulator pathway approved for Group 1 PAH. It is also the first and only therapy approved for treatment of chronic thromboembolic pulmonary hypertension (CTEPH: Group 4 PAH). Riociguat works with dual mechanisms to increase the soluble guanylate cyclase stimulator. It directly stimulates the soluble guanylate cyclase stimulator, independent of nitric oxide, and enhances the sensitivity of soluble guanylate cyclase stimulator to nitric oxide. The pivotal Phase III clinical trial Pulmonary Arterial Hypertension Soluble Guanylate Cyclase-Stimulator Trial 1 (PATENT-1) enrolled 443 patients with PAH in an 12-week trial. Most of the patients had idiopathic PAH (61%) and an NYHA FC of II or III (95%). The majority of the patients were on background therapy (44% previously treated with ERA and 6% with inhaled prostanoids). Riociguat was given in doses of 0.5-2.5 mg three times daily, and the primary outcome was the placebo-corrected change from baseline in 6MWD. The results showed a significant increase in 6MWD from a baseline of 35.8 m with riociguat versus placebo (95% CI 20.1-51.5 m, P < 0.0001) and the results were similar between treatment-naïve patients versus patients on background therapy. Significant improvements were also seen in PVR (P < 0.0001) and clinical deterioration (P = 0.0046). The most common reported side effects included headache, gastritis/reflux, dizziness, and hypotension. Cases of hemoptysis were also reported. Concomitant use of riociguat and PDE-5 inhibitors is contraindicated due to hypotension.
Development and validation of a claims-based model to identify patients at risk of chronic thromboembolic pulmonary hypertension following acute pulmonary embolism
Published in Current Medical Research and Opinion, 2021
Manreet K. Kanwar, Michele Cole, Marjolaine Gauthier-Loiselle, Ameur M. Manceur, Yuen Tsang, Patrick Lefebvre, Sumeet Panjabi, Raymond L. Benza
Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the most significant long-term complications of acute pulmonary embolism that ensues from unresolved thrombi in the pulmonary tree and results in pulmonary hypertension1. Patients with CTEPH can present with non-specific symptoms, which contributes to the challenge of assessing the true incidence of CTEPH following pulmonary embolism. Hence, there is a wide variation in reported estimates of CTEPH incidence following pulmonary embolism (0.46–6.2%)2. Furthermore, 25–63% of patients with CTEPH do not have a documented history of venous thromboembolism or pulmonary embolism3–6. In the general population, the prevalence of CTEPH was also found to be variable, ranging from 47 to 1007 cases per million individuals7,8.
Chronic Thromboembolic Disease: Epidemiology, Assessment with Invasive Cardiopulmonary Exercise Testing, and Options for Management
Published in Structural Heart, 2021
W. Cameron McGuire, Mona Alotaibi, Timothy A. Morris, Nick H. Kim, Timothy M. Fernandes
The clinical significance of these perfusion defects varies from patient to patient. Some may have no symptoms even with exertion, while others have chronic thromboembolic pulmonary hypertension. Many of these patients will have complaints of dyspnea on exertion with exercise limitation, but will not have overt pulmonary hypertension evident on transthoracic echocardiogram performed at rest. It is in these patients with persistent perfusion defects, complaints of dyspnea on exertion, and no evidence of resting pulmonary hypertension where the diagnosis of chronic thromboembolic disease (CTED) is entertained.3 In the absence of both symptoms and abnormal hemodynamics, we do not feel residual perfusion defects alone constitute a disease condition. However, the presence or absence of symptoms is sometimes quite challenging for the clinician to ascertain. The World Symposium on Pulmonary Hypertension guidelines acknowledged the important role that cardiopulmonary exercise testing (CPET) plays in the evaluation of these patients.4
Long term use of anticoagulant therapy for patients with pulmonary embolism
Published in Expert Review of Hematology, 2020
Cecilia Becattini, Ludovica Anna Cimini
About 30% of patients treated for acute PE will complain of persistent dyspnea (mostly mild) or functional limitation in the two-three years after index PE with right ventricle overload [77]. Whether these symptoms may be due to residual pulmonary arteries obstruction is unknown. In fact, the prevalence of residual pulmonary arteries obstruction after anticoagulant treatment for acute PE varies between 15 to over 65% [56]. However, only a minority of patients will develop signs of pulmonary hypertension and only about 4% of patients will receive a diagnosis of chronic thromboembolic pulmonary hypertension (CTPH) within 2 years after the index PE (reported rates vary between 0.1% and 9.1%) [3]. It is conceivable that thrombi organization in fibrotic tissue can lead to chronic obstruction of the pulmonary artery tree, then to small-vessel arteriopathy (with high pulmonary vascular resistance), and finally to a progressive right heart failure [78].