Chronic Thromboembolic Pulmonary Hypertension (CTEPH) ...
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Description

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Pulmonary Thromboendarterectomy

Chronic Thromboembolic Pulmonary Hypertension

Definition: mean pulmonary-artery pressure > 25mmHg persisting 6 months after pulmonary embolism

Pathophysiology:

Small-vessel arteriopathy with

 • Medial hypertrophy

 • Intimal proliferation

 • Microvascular thrombosis

 • Plexiform-lesion formation

Clinical presentation

 • Exercise intolerance, dyspnea, fatigue

 • Decreasing right heart function (JVD, fixed splitting of S2, S3, tricuspid regurgitation)

Diagnosis

 • TTE with Doppler 6 months after PE — sensitive but not specific

 • Chest CTA showing thromboembolic material, mosaic parenchymal pattern

 • Ventilation-perfusion lung scanning showing multiple bilateral defects

 • Confirm diagnosis with right heart catheterization and pulmonary angiography

Pulmonary Thromboendarterectomy - Potentially curative procedure:

 • Median Sternotomy → Cardiopulmonary Bypass Started →  Patient Cooling to 20 degrees C → Right Pulmonary Vessels Thrombi Removal → Left Pulmonary Vessels Thrombi Removal → Patient Warming (90-120m) → Removal of Cardiopulmonary Bypass

Other Treatment Options

Balloon Pulmonary-Artery Angioplasty

 • Used to reduce pulmonary-artery pressure in patients with CTEPH who have inoperable disease

 • Rarely performed

Anticoagulation

 • Goal: prevent pulmonary-artery thrombosis and recurrent VTE

 • RCT data lacking for use in CTEPH

Bosentan

 • Endothelin-receptor antagonist

 • BENEFiT Trial: significant improvements compared to placebo in hemodynamic variables, dyspnea, pro-BNP levels

 • Risk of hepatotoxicity

Sildenafil - Phosphodiesterase inhibitor

 • Improvements in hemodynamic variables, WHO functional class

Epoprostenol / Treprostinil - Prostacyclin analogues

 • Improvements in BNP, hemodynamic variables, WHO functional class

 • Promising inhaled and oral forms



- Rebecca Steinberg @RebeccaSteinb17



#CTEPH #PHTN #Pulmonary #Hypertension #Thromboendarterectomy #diagnosis #management
Contributed by

Dr. Gerald Diaz
@GeraldMD
Board Certified Internal Medicine Hospitalist, GrepMed Editor in Chief 🇵🇭 🇺🇸 - Sign up for an account to like, bookmark and upload images to contribute to our community platform. Follow us on IG:  https://www.instagram.com/grepmed/ | Twitter: https://twitter.com/grepmeded/
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