Treatment of Chronic Thromboembolic Pulmonary Hypertension: The Role of Medical Therapy and Balloon Pulmonary Angioplasty

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable disease when treated with pulmonary thromboendarterectomy (PTE). However, even at experienced surgical centers, nearly one-third of patients with CTEPH will be deemed inoperable for reasons including distal disease, comorbidities, or out-of-proportion pulmonary hypertension. It is in these patients with inoperable CTEPH that pulmonary hypertension (PH)-targeted medical therapy and balloon pulmonary angioplasty have potential therapeutic value.Previous unblinded cohort trials have assessed PH-targeted medical therapy in various subpopulations of CTEPH patients using epoprostenol, treprostinil, sildenafil, bosentan, and iloprost, each demonstrating measurable pulmonary hemodynamic effects. However, riociguat, a soluble guanylate cyclase stimulator, is the first FDA-approved therapy for inoperable CTEPH to demonstrate both an improvement in functional capabilities (6-minute walk time) as well as significant gains in secondary pulmonary hemodynamic end points in a large placebo-controlled trial.Balloon pulmonary angioplasty is an interventional procedure using telescoping catheters placed in the pulmonary arteries, through which wires and balloons are used to mechanically disrupt chronic clot material and relieve pulmonary vascular obstruction. Contemporary case series from multiple centers worldwide have demonstrated pulmonary hemodynamic improvement with this approach.As a result of these advances, patients with inoperable CTEPH who had few options as recently as 5 years ago now have alternatives with emerging evidence of therapeutic efficacy.

Long‐term changes of resting and exercise right ventricular systolic performance in patients with chronic thromboembolic pulmonary hypertension following pulmonary thromboendarterectomy – A two‐dimensional and three‐dimensional echocardiographic study

“…The observed decline in TAPSE is in accordance with findings from other studies investigating the effect of PEA, and it is also found in patients undergoing general open heart surgery . Studies have suggested that the mechanism explaining the general reduction in TAPSE after open heart surgery may be associated with pericardiotomy, sternotomy itself, cardiopulmonary bypass, cardioplegia, or myocardial hypothermia . We also examined RV function by pulsed tissue Doppler of the lateral free wall using RV‐S′ which is another recognized parameter of RV systolic function.…”

“…Hemodynamic post‐PEA evaluation is often performed to assess the degree of normalization of pulmonary pressure and RV function to inform further treatment decisions, for example, specific vasoactive PH medication, renewed evaluation for second PEA, or balloon pulmonary angioplasty (BPA) treatment . Overall, our study demonstrated no major changes of RV systolic function from 3 to 12 months, indicating that post‐PEA evaluation can be performed already after 3 months.…”

Balloon pulmonary angioplasty vs riociguat in patients with inoperable chronic thromboembolic pulmonary hypertension: A systematic review and meta‐analysis

“…BPA relies on the use of telescoping catheters placed in a central vein, through which wires and balloons are guided to mechanically disrupt chronic clot material and relieve pulmonary vascular obstruction. Recently, additional groups have reported that BPA improved symptoms and hemodynamic parameters in patients with peripheral‐type CTEPH . In addition, repeated PEA is not a feasible way due to high perioperative risk.…”

Chronic thromboembolic pulmonary hypertension: modern view on the problem

“…1 указан список исследований, посвященных изучению эффективности патогенетической терапии при ХТЭЛГ [42]. Современные методы лечения ХТЭЛГ С начала 60-х годов ХХ века и по сегодняшний день «золотым стандартом» лечения ХТЭЛГ остается операция двусторонней ТЭЭ, которая при успешном выполнении, помимо нормализации гемодинамических параметров и улучшения функционального статуса, позволяет обеспечить 5-летнюю выживаемость на уровне 74-89% [42,43]. По данным J. Lewczuk и соавт., 2-летняя выживаемость больных с ХТЭЛГ при срДЛА более 30 мм рт.ст., при отсутствии хирургического лечения составляет 12% [44].…”