Lung perfusion characteristics in pulmonary arterial hypertension (PAH) and peripheral forms of chronic thromboembolic pulmonary hypertension (pCTEPH): Dual-energy CT experience in 31 patients

2016 ◽  
Vol 27 (4) ◽  
pp. 1631-1639 ◽  
Author(s):  
Jessica Giordano ◽  
Suonita Khung ◽  
Alain Duhamel ◽  
Claude Hossein-Foucher ◽  
Dimitri Bellèvre ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Dual Energy ◽  
Lung Perfusion ◽  
Dual Energy Ct ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

scholarly journals Microvasculopathy evaluated by dual-energy computed tomography in chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Miwa ◽  
Y Taniguchi ◽  
K Sumimoto ◽  
Y Matsuoka ◽  
Y Izawa ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Hounsfield Unit ◽  
Dual Energy ◽  
Dual Energy Computed Tomography ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Abstract Background It has been previously reported that poor subpleural perfusion (PSP) in dual-energy computed tomography (DE-CT) might suggest the microvasculopathy in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, it remains unclear whether pathological findings of microvasculopathy in CTEPH and pulmonary arterial hypertension (PAH) are equivalent. The aim is to evaluate the microvasculopathy in CTEPH and PAH by using clinical parameters and DE-CT. Methods We retrospectively reviewed PSP (defined as subpleural spaces either not or minimally perfused in all segments) of consecutive treatment-naïve 89 CTEPH patients and 20 PAH patients who underwent DECT from Feb. 2015 to Dec. 2019. We also evaluated hemodynamic parameters and DE-CT parameters including quantitative evaluation of pulmonary blood volume (PBV) which was calculated as the average of entire lung iodine density. Results PSP was observed in 49.4% of patients in CTEPH group versus 5.0% in PAH group (p<0.01). There were no significant differences in hemodynamics and lung PBV between CTEPH group and PAH group (mean pulmonary arterial pressure; 36.4±10.4mmHg vs 38.3±8.5mmHg p=0.464, pulmonary vascular resistance; 700±388dyne*sec/cm5 vs 805±440 dyne*sec/cm5 p=0.288, lung PBV; 24.9±6.4 Hounsfield Unit vs 22.0±6.6 Hounsfield Unit p=0.06, respectively), however diffusing capacity for carbon monoxide (%DLCO/VA) was significantly lower (69.5±16.8% vs 45.7±23.7% p<0.01) in PAH group. Conclusion PSP in DE-CT, which was observed more frequently in patients with CTEPH, might suggest the different mechanism of microvasculopathy from PAH in patients with CTEPH. Microvasculopathy in CTEPH would be diffuse very distal thrombosis. DE-CT is effective modality to detect microvasculopathy of diffuse distal thrombosis in patients with CTEPH. DECT, Poor subpleural perfusion Funding Acknowledgement Type of funding source: None

scholarly journals Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with chronic thromboembolic pulmonary hypertension (CTEPH): a qualitative interview study

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Brooke Currie ◽  
Evan Davies ◽  
Amélie Beaudet ◽  
Larissa Stassek ◽  
Leah Kleinman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Shortness Of Breath ◽  
Response Options ◽  
Qualitative Interview Study ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Impact

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients’ lives is needed to optimally assess the impact of the disease and treatment. Objectives This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients’ lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™‘s instructions, items, and response options. Results Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an “extremely important” symptom, and seven participants rated fatigue as “extremely important.” The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH.

scholarly journals Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 9 (1) ◽  
pp. 204589401983489 ◽  
Author(s):  
Meghan M. Cirulis ◽  
John J. Ryan ◽  
Stephen L. Archer
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Molecular Mechanisms ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Outcome Data ◽  
Current Evidence ◽  
Clinical Aspects ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Arrhythmias are increasingly recognized as serious, end-stage complications of pre-capillary pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Although arrhythmias contribute to symptoms, morbidity, in-hospital mortality, and possibly sudden death in PAH/CTEPH, there remains a paucity of epidemiologic, pathophysiologic, and outcome data to guide management of these patients. This review summarizes the most current evidence on the topic: from the molecular mechanisms driving arrhythmia in the hypertrophied or failing right heart, to the clinical aspects of epidemiology, diagnosis, and management.

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