Chronic Thromboembolic Pulmonary Hypertension in a Pediatric Patient with Exertional Dyspnea

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension caused by persistent thromboembolism of the pulmonary arteries. In clinical practice, CTEPH patients often show obstructive ventilatory impairment, even in the absence of a smoking history. Recent reports imply a tendency for CTEPH patients to have a lower FEV1.0; however, the mechanism underlying obstructive impairment remains unknown. Methods We retrospectively analyzed CTEPH patients who underwent a pulmonary function test and respiratory impedance test to evaluate their exertional dyspnea during admission for right heart catheterization from January 2000 to December 2019. We excluded patients with a smoking history to rule out the effect of smoking on obstructive impairment. Results A total of 135 CTEPH patients were analyzed. The median FEV1.0/FVC was 76.0%, %FEV 1.0 had a negative correlation with the mean pulmonary artery pressure and pulmonary vascular resistance and the CT Angiogram (CTA) obstruction score. A multivariate regression analysis revealed that the CTA obstruction score was an independent factor of a lower %FEV1.0. In the 54 patients who underwent pulmonary endarterectomy, %FEV1.0 was improved in some cases and was not in some. Mean PAP largely decreased after PEA in the better %FEV1.0 improved cases, suggesting that vascular involvement in CTEPH could be associated with spirometry obstructive impairment. Conclusion %FEV1.0 had a significant correlation with the CTA obstruction score. Obstructive impairment might have an etiological relationship with vascular involvement. Further investigations could shed new light on the etiology of CTEPH.

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Vascular involvement in chronic thromboembolic pulmonary hypertension is associated with spirometry obstructive impairment

10.21203/rs.3.rs-871334/v1 ◽

2021 ◽

Author(s):

Asako Yanagisawa ◽

Akira Naito ◽

Takayuki-Jujo Sanada ◽

Nobuhiro Tanabe ◽

Keiichi Ishida ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Multivariate Regression Analysis ◽

Smoking History ◽

Vascular Involvement ◽

Heart Catheterization ◽

Exertional Dyspnea ◽

Ct Angiogram ◽

Thromboembolic Pulmonary Hypertension

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension caused by persistent thromboembolism of the pulmonary arteries. In clinical practice, CTEPH patients often show obstructive ventilatory impairment, even in the absence of a smoking history. Recent reports imply a tendency for CTEPH patients to have a lower FEV1.0; however, the mechanism underlying obstructive impairment remains unknown. Methods We retrospectively analyzed CTEPH patients who underwent a pulmonary function test (PFT) and respiratory impedance test to evaluate their exertional dyspnea during admission for right heart catheterization from January 2000 to December 2019. We excluded patients with a smoking history to rule out the effect of smoking on obstructive impairment. Results A total of 135 CTEPH patients were analyzed. The median FEV1.0/FVC was 76.0%, %FEV 1.0 had a negative correlation with the mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) and the CT Angiogram (CTA) obstruction score. A multivariate regression analysis revealed that the CTA obstruction score was an independent factor of a lower %FEV1.0. In the 54 patients who underwent pulmonary endarterectomy, %FEV1.0 was improved in some cases and was not in some. Mean PAP largely decreased after PEA in the better %FEV1.0 improved cases, suggesting that vascular involvement in CTEPH could be associated with spirometry obstructive impairment. Conclusion %FEV1.0 had a significant correlation with the CTA obstruction score. Obstructive impairment might have an etiological relationship with vascular involvement. Further investigations could shed new light on the etiology of CTEPH.

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Improvement after bariatric surgery in chronic thromboembolic pulmonary hypertension: a novel treatment target

BMJ Case Reports ◽

10.1136/bcr-2018-228358 ◽

2021 ◽

Vol 14 (3) ◽

pp. e228358

Author(s):

Anika Vaidy ◽

Paul Forfia ◽

Jeremy Mazurek ◽

Anjali Vaidya

Keyword(s):

Bariatric Surgery ◽

Pulmonary Hypertension ◽

Medical Therapy ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Walk Distance ◽

Right Heart ◽

Exertional Dyspnea ◽

Heart Catheterisation ◽

Right Heart Catheterisation ◽

Thromboembolic Pulmonary Hypertension

A 66-year-old man with a medical history significant for multiple pulmonary embolism and morbid obesity was evaluated for chronic thromboembolic pulmonary hypertension. Echocardiogram and right heart catheterisation were significant for severe pulmonary hypertension. Therefore, he was started on pulmonary hypertension medical therapy with riociguat and ambrisentan, in addition to anticoagulation. He experienced a dramatic clinical response to medical therapy. Despite haemodynamic improvement, the patient remained symptomatic with significant fatigue, exertional dyspnea and poor functional status as highlighted by a 6 min walk distance of only 128 m. Patient was referred for bariatric surgery with a gastric sleeve, after which he successfully lost 95 lbs in 6 months. Postoperative right heart catheterisation demonstrated normal pulmonary vascular resistance and cardiac output. His echocardiogram revealed normal right ventricular size and function. His 6 min walk distance also nearly quadrupled from 128 to 512 m, consistent with WHO Functional Class I.

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