Chronic thromboembolic pulmonary hypertension

Abstract

Pulmonary arterial hypertension is a severe disease that has been ignored for a long time. However, over the past 20 yrs chest physicians, cardiologists and thoracic surgeons have shown increasing interest in this disease because of the development of new therapies, that have improved both the outcome and quality of life of patients, including pulmonary transplantation and prostacyclin therapy.Chronic thromboembolic pulmonary arterial hypertension (CTEPH) can be cured surgically through a complex surgical procedure: the pulmonary thromboendarterectomy. Pulmonary thromboendarterectomy is performed under hypothermia and total circulatory arrest.Due to clinically evident acute-pulmonary embolism episodes being absent in w50% of patients, the diagnosis of CTEPH can be difficult. Lung scintiscan showing segmental mismatched perfusion defects is the best diagnostic tool to detect CTEPH.Pulmonary angiography confirms the diagnosis and determines the feasability of endarterectomy according to the location of the disease, proximal versus distal. The technique of angiography must be perfect with the whole arterial tree captured on the same picture for each lung. The lesions must start at the level of the pulmonary artery trunk, or at the level of the lobar arteries, in order to find a plan for the endarterectomy.When the haemodynamic gravity corresponds to the degree of obliteration, pulmonary thromboendarterectomy can be performed with minimal perioperative mortality, providing definitive, excellent functional results in almost all cases. Pulmonary arterial hypertension is a severe disease that has been historically neglected. Over the past 20 yrs, chest physicians, cardiologists and thoracic surgeons have shown increasing interest in this disease because of the development of new therapies that have improved the outcome and quality of life of patients. Available options now include prostacyclin therapy, pulmonary endarterectomy and pulmonary transplantation.Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by obstruction of the large pulmonary arteries by acute and recurrent pulmonary emboli, and organisation of these blood clots. This disease, initially considered to be rare, is being diagnosed more and more frequently. A possible reason for this is the availability of successful medical and surgical treatment. The development of centres specialising in the diagnosis and treatment of pulmonary hypertension, and more consistent follow-up procedures for patients presenting with acute pulmonary emboli may also contribute to the ongoing increase in the number of patients diagnosed and treated for CTEPH. Pathogenesis or natural history of pulmonary emboliHaemodynamic failure and death occurs in 20-40% of patients within 1 h of acute pulmonary emboli [1]. Among survivors, the natural evolution, in most cases, is resorption of blood clots by local fibrinolysis with complete restoration of

Risk factors for chronic thromboembolic pulmonary hypertension

“…Previously, large patient series have been derived from medical [15][16][17] and surgical databases [18,19], with the most recent representing Data are presented as n or n (%). CTEPH: chronic thromboemolic pulmonary hypertension; PEA: pulmonary endarterectomy; PAH: pulmonary arterial hypertension; CVD:…”

Chronic thromboembolic pulmonary hypertension

“…Often, it is permanently curative, as long as patients remain on lifelong anticoagulation, and may result in a substantial improvement in cardiopulmonary hemodynamics [8,12,[26][27][28]. Significant improvements in New York Heart Association functional class, dyspnea scores, gas exchange/exercise capacity, and long-term survival have been reported [25•, 29-31].…”

“…However, medical therapies directed at treating PH have been investigated and increasingly used in subcategories of CTEPH patients [9,10]: 1) patients deemed inoperable because of extensive distal precapillary vasculopathy not amenable to surgery or those who have significant comorbidities that contraindicate surgery [11]; 2) the 10% to 15% of patients who have residual PH following thromboendarterectomy surgery [12]; and 3) patients with severe hemodynamic compromise, as a bridge to surgery. Xa inhibitors (fondaparinux) may be considered.…”

Red cell distribution width

“…1,2 Although the incidence of CTEPH is usually estimated to be <1% among patients with acute PE, [2][3][4] several studies have proposed that this low incidence was an underestimation and showed approximately 4%, a relatively high incidence of CTEPH after PE. [5][6][7] In addition, it has not been shown why some PE patients develop CTEPH and others do not.…”