Chronic thromboembolic pulmonary hypertension

• Published in: The European respiratory journal Vol. 23; no. 4; pp. 637 - 648
• Main Authors: Dartevelle, P, Fadel, E, Mussot, S, Chapelier, A, Herve, P, de Perrot, M, Cerrina, J, Ladurie, F.L, Lehouerou, D, Humbert, M, Sitbon, O, Simonneau, G
• Format: Journal Article
• Language: English
• Published: Leeds Eur Respiratory Soc 01-04-2004; Maney
• Subjects: Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Chronic Disease; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Endarterectomy; Heart Arrest, Induced; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - surgery; Hyperthermia, Induced; Medical sciences; Pneumology; Pulmonary Artery - surgery; Pulmonary Embolism - complications; Pulmonary Embolism - diagnosis; Pulmonary Embolism - surgery; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Quality of Life; Treatment Outcome; Vascular disease; Chronic; Pulmonary arterial hypertension pulmonary embolism pulmonary thromboendarterectomy; Respiratory disease; Cardiovascular disease; Thromboembolism; Pulmonary hypertension; Pneumology
• ISSN: 0903-1936; 1399-3003
• DOI: 10.1183/09031936.04.00079704

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 > 50% 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 feasibility 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.