[Chronic thromboembolic pulmonary hypertension (CTEPH)]

The patients with chronic thromboembolic pulmonary hypertension (CTEPH) usually shows impaired physical activity and poor prognosis, proportional to the degree of pulmonary hypertension. Effort dyspnea and fatigue, the major symptoms, are quite nonspecific, and the physical findings of pulmonary hypertension are easily overlooked until they are far advanced. The most important clue to the diagnosis is perfusion and ventilation lung scan which demonstrates more segmental sized or larger perfusion defects that are normally ventilated. The presence of precapillary pulmonary hypertension should be confirmed by right heart catheterization. Pulmonary angiography is essential not only for diagnosing CTEPH but also for defining thrombus extent and location. The current criteria for considering for surgery include: 1) significant exertional limitation defined as Hugh-Jones > or = III, or NYHA > or = III, 2) pulmonary hypertension defined as PAm > or = 30 mmHg, 3) thrombi located at least as proximally as the lobar level, 4) the absence of significant co-morbid disease, 5) a willingness of the patient and his family to accept the risks of surgery. Although the hemodynamic and symptomatic improvement associated with pulmonary thromboendarterectomy is apparent, the operative mortality is still high, especially in patients with severe hemodynamic disease. The accurate diagnosis and selection criteria for surgery are crucial.