Chronic Thromboembolic Pulmonary Hypertension: When to Suspect It, When to Refer for Surgery

Chronic thromboembolic obstruction of the major pulmonary arteries is an underrecognized sequela of acute pulmonary embolism. Depending on the burden and location of thrombus, as well as on the duration of vessel obstruction, chronic thromboembolic disease may lead to pulmonary hypertension and cor pulmonale. Chronic thromboembolic disease affects an estimated 500 to 2500 patients each year in the United States, roughly 0.1 to 0.5 percent of patients who survive acute pulmonary embolism. Consequently, while this disease is uncommon, chronic thromboembolic pulmonary hypertension (CTEPH) is not rare, and should be considered in patients with unexplained dyspnea, as it is potentially correctible with pulmonary thromboendarterectomy.1

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P2771A novel doppler index for the diagnosis of post pulmonary embolism impairment syndrome

European Heart Journal ◽

10.1093/eurheartj/ehz748.1088 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

O Dzikowska-Diduch ◽

M Kostrubiec ◽

K Brodka ◽

A Wyzgal-Chojecka ◽

P Pacho ◽

...

Keyword(s):

Heart Failure ◽

Pulmonary Embolism ◽

Pulmonary Hypertension ◽

Ejection Fraction ◽

Functional Limitation ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Thromboembolic Disease ◽

Preserved Ejection Fraction ◽

Thromboembolic Pulmonary Hypertension ◽

Chronic Thromboembolic Disease

Abstract Introduction Follow-up studies demonstrated that after an episode of acute pulmonary embolism (PE), half of patients report functional limitation. Chronic thromboembolic pulmonary hypertension (CTEPH) is diagnosed in approximately 4%, while others are supposed to have heart failure with preserved ejection fraction (HFpEF) or chronic thromboembolic disease (CTED). Echo can not only assess tricuspidal regurgitation pressure gradient (TRPG) indicating pulmonary hypertension but also with tissue Doppler, E/e' ratio can diagnose left ventricular diastolic dysfunction. We tried to asses if a novel echo index: TRPG to E/e' could be useful in differentiation between CTEPH, CTED and HFpEF. Material and methods We analyzed data of consecutive 535 PE survivors (313 F, aged 61±17 yrs). 342/535 (64%) reported significant functional impairment after at least 6 months anticoagulation. All symptomatic subjects underwent detailed diagnostic workup which included standardized echocardiography, lung scintigraphy, pulmonary functional tests, and chest CT, RHC and coronary angiography when appropriate. Results Eventually, out of 342 symptomatic PE survivors (220 F, aged 65±15 yrs) CTEPH was diagnosed In 17 cases, CTED in 8 pts and HFpEF in 174 pts and in the remaining other causes were found (i.e. coronary artery disease, anemia, pulmonary disease). Doppler echocardiography showed that TRPG/E/e' was significantly increased in CTEPH and CTED patients when compared to subjects with HFpEF (Table 1). Echocardiographic doppler assessment HFpEF p CTEPH p CTED p n=174 HFpEF vs CTEPH n=17 CTEPH vs CTED n=8 HFpEF vs CTED TRPG (mmHg) 26.7±8.9 <0.01 59.5±32.0 0.013 34.4±15.1 ns E/e' 11.1±2.9 ns 10.1±32.0 ns 8.9±2.0 ns TRPG / E/e' 2.5±1.0 <0.001 6.0±2.4 <0.01 3.8±1.1 0.013 TRPG, Tricuspidal regurgitation pressure gradient; CTEPH, Chronic thromboembolic pulmonary hypertension; CTED, Chronic thromboembolic disease; HFpEF, Heart failure with preserved ejection fraction. Conclusion Our data indicate that echocardiographic index TRPG/(E/e') may be helpful in the differentiation of functional limitation in patients after pulmonary embolism.

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Recurrent chronic thromboembolic disease despite optimal anticoagulation in setting of recent COVID-19 infection

BMJ Case Reports ◽

10.1136/bcr-2020-238733 ◽

2021 ◽

Vol 14 (1) ◽

pp. e238733 ◽

Cited By ~ 1

Author(s):

Vittorio Romeo Terrigno ◽

Daniel Anthony Ricketti ◽

Pranav Patel ◽

Satyajeet Roy

Keyword(s):

Pulmonary Embolism ◽

Acute Pulmonary Embolism ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

International Normalized Ratio ◽

Thromboembolic Disease ◽

Anticoagulation Treatment ◽

Therapeutic Anticoagulation ◽

Thromboembolic Pulmonary Hypertension ◽

History Of ◽

Chronic Thromboembolic Disease

We present a case of a 38-year-old man with a history of chronic thromboembolic pulmonary hypertension on therapeutic anticoagulation and recent hospitalisation for COVID-19 disease who was hospitalised for recurrent acute pulmonary embolism despite therapeutic anticoagulation with warfarin (International Normalized Ratio (INR) of 3.0). Our case highlights the hypercoagulable state associated with COVID-19 disease and the absence of standardised approaches to anticoagulation treatment for this population.

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Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-12.4.186 ◽

2014 ◽

Vol 12 (4) ◽

pp. 186-192 ◽

Cited By ~ 1

Author(s):

David Poch ◽

Victor Pretorius

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Pulmonary Artery Pressure ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Pulmonary Artery Wedge Pressure ◽

Pulmonary Endarterectomy ◽

Pulmonary Thromboendarterectomy ◽

Thromboembolic Pulmonary Hypertension ◽

Wedge Pressure

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

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Fibrinogen AAlpha-Thr312ala Polymorphism Is Comparably Associated With Acute Pulmonary Embolism And With Chronic Thromboembolic Pulmonary Hypertension

10.1164/ajrccm-conference.2011.183.1_meetingabstracts.a2418 ◽

2011 ◽

Author(s):

Cynthia W. Baffi ◽

Timothy A. Morris

Keyword(s):

Pulmonary Embolism ◽

Pulmonary Hypertension ◽

Acute Pulmonary Embolism ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Thromboembolic Pulmonary Hypertension

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The soluble guanylate cyclase stimulator riociguat is the first-line therapy for chronic thromboembolic pulmonary hypertension patients

Kardiologiia ◽

10.18087/cardio.2020.8.n1198 ◽

2020 ◽

Vol 60 (8) ◽

pp. 115-123

Author(s):

Z. S. Valieva ◽

S. E. Gratsianskaya ◽

T. V. Martynyuk

Keyword(s):

Pulmonary Hypertension ◽

Guanylate Cyclase ◽

Soluble Guanylate Cyclase ◽

Pulmonary Arteries ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

First Line ◽

Pulmonary Thromboendarterectomy ◽

First Line Therapy ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Microcirculation

Chronic thromboembolic pulmonary hypertension (CTEPH) is a precapillary type of pulmonary hypertension with chronic obstruction of large and medium branches of pulmonary arteries along with secondary alterations in pulmonary microcirculation, which cause progressive increases in pulmonary vascular resistance and pulmonary arterial pressure and ensuing severe right heart dysfunction and heart failure. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for CTEPH; however, this procedure is available not for all patients. Although the surgery performed in the conditions of centers with advanced experience generally shows good results, up to 40% of patients are technically inoperable or PTE is associated with a high risk of complications. At present, riociguat, the only officially approved drug from the class of soluble guanylate cyclase stimulators, is considered as a first-line treatment for inoperable and residual forms of STEPH. Introduction of riociguat to clinical practice can be called a real breakthrough in the treatment of patients with STEPH who cannot undergo PTE or those with relapse or persistent STEPH after the surgery.

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