scholarly journals An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management

2016 ◽  
Vol 6 (4) ◽  
pp. 472-482 ◽  
Author(s):  
Henning Gall ◽  
Ioana R. Preston ◽  
Barbara Hinzmann ◽  
Sabina Heinz ◽  
David Jenkins ◽  
...  
Keyword(s):  
United States ◽  
Pulmonary Hypertension ◽  
Treatment Guidelines ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The United States ◽  
Patient Record ◽  
Heart Catheterization ◽  
Referral Rates ◽  
Record Data ◽  
Thromboembolic Pulmonary Hypertension

We conducted an international study to evaluate practices in the diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) globally across different regions. Between August and October 2012, CTEPH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 2–5 most recent patients with CTEPH. Overall, 496 physicians (Europe: 260; United States: 152; Argentina: 52; Japan: 32) completed the questionnaire and provided patient record data for 1,748 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension (PH) center ranged from 38% in France and Italy to 83% in the United States. A large proportion of patients did not undergo ventilation/perfusion scanning (46%–67%) or right heart catheterization (24%–57%) for the diagnosis of CTEPH. Referral rates for pulmonary endarterectomy evaluation ranged from 25% in Japan to 44% in Europe, with higher referral rates in PH centers; the main reasons for lack of referral were that surgery was not considered unless medical treatment was failing and patient refusal. Other variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of combination treatment in the United States than in Europe. Physicians' perceptions of their treatment strategy were generally consistent with patient record data. Results from this study, which includes a global aspect of CTEPH care, demonstrate not only regional differences in CTEPH management but, more importantly, considerable nonadherence to the diagnosis and treatment guidelines for CTEPH, even in PH centers.

scholarly journals United States Chronic Thromboembolic Pulmonary Hypertension Registry: Protocol Design (Preprint)

2020 ◽  
Author(s):  
Kim M. Kerr ◽  
C. Greg Elliott ◽  
Raymond L. Benza ◽  
Richard N. Channick ◽  
Kelly Chin ◽  
...  
Keyword(s):  
Quality Of Life ◽  
United States ◽  
Risk Factors ◽  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The United States ◽  
Thromboembolic Pulmonary Hypertension ◽  
Prospective Longitudinal ◽  
The U.S

BACKGROUND Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare sequela of acute pulmonary embolism that is treatable when recognized. Awareness of this disease has increased with recent advancements in therapeutic options, but delays in diagnosis remain common and diagnostic and treatment guidelines are often not followed. Data gathered from international registries has improved our understanding of CTEPH, but this data may not be applicable to the U.S. population due to differences in demographics and medical practice patterns. OBJECTIVE The U.S. CTEPH Registry (US-CTEPH-R) was developed to provide essential information to better understand the demographics, risk factors, evaluation, and treatment of CTEPH in the United States, as well as short and long-term outcomes of surgical and non-surgical therapies in the modern treatment era. METHODS Thirty sites throughout the United States enrolled 750 subjects in this prospective, longitudinal, observational registry of newly diagnosed CTEPH patients. Enrollment criteria included a mean pulmonary artery pressure > 25 mmHg by right heart catheterization and radiologic confirmation of CTEPH by a multi-disciplinary adjudication committee. Following enrollment, subjects were followed bi-annually until the conclusion of the study. Quality of life surveys were administered at enrollment and biannually, all other testing was at the discretion of the treating clinician. Details regarding surgical therapy, balloon pulmonary angioplasty and medical therapy were collected at enrollment and at follow-up as well as information related to health care utilization and survival. RESULTS Data from this registry will improve the understanding of the demographics, risk factors, and treatment patterns of CTEPH patients and the longitudinal impact of therapies on quality of life, healthcare utilization, and survival. CONCLUSIONS This manuscript details the methodology and design of the first large, prospective, longitudinal, registry of CTEPH patients in the U.S. CLINICALTRIAL ClinicalTrials.gov ID: NCT02429284

scholarly journals Validation of a claims-based algorithm to identify patients with chronic thromboembolic pulmonary hypertension using electronic health record data

2018 ◽  
Vol 9 (1) ◽  
pp. 204589401881477 ◽  
Author(s):  
Simon Teal ◽  
William R. Auger ◽  
Rodney J. Hughes ◽  
Dena Rosen Ramey ◽  
Kelly S. Lewis ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Gold Standard ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Administrative Claims ◽  
Electronic Health Record Data ◽  
Health Records ◽  
Record Data ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
Electronic Health

This study aimed to validate an algorithm developed to identify chronic thromboembolic pulmonary hypertension (CTEPH) among patients with a history of pulmonary embolism. Validation was halted because too few patients had gold-standard evidence of CTEPH in the administrative claims/electronic health records database, suggesting that CTEPH is underdiagnosed.

scholarly journals Interventricular septal curvature as an additional echocardiographic parameter for evaluating chronic thromboembolic pulmonary hypertension: A single-center retrospective study

2021 ◽  
Author(s):  
Akane Matsumura ◽  
Ayako Shigeta ◽  
Hajime Kasai ◽  
Hajime Yokota ◽  
Jiro Terada ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pressure Gradient ◽  
Interventricular Septum ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Left Ventricular ◽  
Accurate Estimation ◽  
Heart Catheterization ◽  
Systolic Pulmonary Artery Pressure ◽  
Additional Tool ◽  
Thromboembolic Pulmonary Hypertension

Abstract Background: Noninvasive estimation of the actual systolic pulmonary artery pressure measured via right-sided heart catheterization (sPAPRHC) is important for the management of pulmonary hypertension, including chronic thromboembolic pulmonary hypertension (CTEPH). Evaluation related to the interventricular septum (IVS) is generally performed with only visual assessment and has been rarely assessed quantitatively in the field of echocardiography. Thus, this study aimed to investigate the utility of echocardiographic IVS curvature to estimate sPAPRHC in patients with CTEPH. Methods: Data of 72 patients with CTEPH were studied retrospectively. We estimated sPAPRHC using echocardiographic IVS curvature (esPAPcurv) and left ventricular eccentricity index (esPAPLVEI), and compared their ability to predict sPAPRHC with estimated sPAPRHC using tricuspid regurgitant pressure gradient (esPAPTRPG). Results: IVS curvature and LVEI were significantly correlated with sPAPRHC (r = - 0.52 and r = 0.49, respectively). Moreover, the IVS curvature was effective in estimating the sPAPRHC of patients with trivial tricuspid regurgitation (r = - 0.56) and in determining patients with sPAPRHC ≥70 mmHg with higher sensitivity (77.0%) compared to those with esPAPTRPG and esPAPLVEI. Conclusion: Our results indicate that the echocardiographic IVS curvature could be a useful additional tool for estimating sPAPRHC in CTEPH patients in whom accurate estimation of sPAPRHC using tricuspid regurgitant pressure gradient is difficult.

Risk Factors and Epidemiology of Pulmonary Embolism

2020 ◽  
Author(s):  
Aaron B Waxman ◽  
Aaron W Aday
Keyword(s):  
United States ◽  
Risk Factors ◽  
Pulmonary Embolism ◽  
Recurrent Events ◽  
Hormone Replacement ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The United States ◽  
Vein Thrombosis ◽  
Thromboembolic Pulmonary Hypertension ◽  
Deep Vein

More than 200,000 individuals are hospitalized with an acute pulmonary embolism in the United States annually. Despite advances in diagnosis and treatment, pulmonary embolism accounts for nearly 1% of all cardiovascular-related deaths each year in the United States alone. Those who survive an acute episode remain at a risk of recurrent events as well as ongoing dyspnea, reduced quality of life, and chronic thromboembolic pulmonary hypertension. Recognized risk factors for pulmonary embolism include advanced age, obesity, smoking, malignancy, immobilization from any cause, pregnancy and the postpartum period, oral contraceptives, and hormone replacement therapy. Numerous heritable and acquired thrombophilias increase the risk of pulmonary embolism. Additionally, inflammation and autoimmune disorders are increasingly recognized as potent risk factors for pulmonary embolism. This review contains 3 figures, 6 tables, 54 references. Key Words: anticoagulation, deep vein thrombosis, epidemiology, genetics, inflammation, malignancy, pulmonary embolism, thrombosis, venous thromboembolism

scholarly journals Successful right heart remodelling and subsequent pregnancy in a patient with chronic thromboembolic pulmonary hypertension undergoing balloon pulmonary angioplasty: a case report

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Nobutaka Ikeda ◽  
Masaru Hatano ◽  
Takeshi Nagamatsu ◽  
Masato Nakamura
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Subsequent Pregnancy ◽  
Heart Catheterization ◽  
Right Heart ◽  
Promising Alternative ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

AbstractBackgroundTo date, the management of pregnancy in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and the associated risk of maternal mortality have not been established. Although balloon pulmonary angioplasty (BPA) in patients with CTEPH is still an emerging procedure, this approach represents a promising alternative to pulmonary endarterectomy (PEA), especially in patients with inoperable CTEPH.Case summaryWe present a case of a 34-year-old woman with CTEPH who desired to have a child. Right heart catheterization showed a mean pulmonary artery pressure of 54 mmHg. Since the lesions were observed in the distal part of subsegmental pulmonary arteries, there was no indication for PEA. After improvement in her haemodynamic status by BPA, she became pregnant. At 40 weeks of gestation, a normal baby was delivered vaginally. Both mother and baby have made satisfactory progress.DiscussionIn cases in which the haemodynamic status is improved by effective BPA, pregnancy and childbirth may be possible, even in patients with CTEPH.

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