Chronic Thromboembolic Pulmonary Hypertension
Section snippets
CTEPH: epidemiology and predisposing factors
The incidence of CTEPH following an acute pulmonary embolic event (or events) has not been adequately defined. Early characterization of patients with CTEPH resulted in the speculation that 0.1% to 0.5% of acute embolic survivors might develop this disease.1 However, more recent data suggest that this estimate may be a significant understatement of how common CTEPH might be worldwide.2 A recent prospective longitudinal study by Pengo and colleagues3 reported a 2-year, cumulative incidence of
Natural history of chronic thromboembolic disease and CTEPH
The pathophysiologic mechanism whereby an acute pulmonary embolus evolves into the thromboembolic residua that becomes incorporated into the wall of the pulmonary vessel remains poorly understood. In most circumstances, with antithrombotic therapy, resolution of an acute pulmonary embolic burden is to a degree that normal pulmonary hemodynamics, gas exchange, and exercise tolerance are restored. However, complete anatomic recovery after acute pulmonary embolism may not occur, and the basis for
Clinical presentation
Establishing the diagnosis of CTEPH can be difficult. The presenting signs and symptoms can be difficult to distinguish from other common cardiopulmonary conditions. Box 1 lists the common presenting symptoms in CTEPH. Because of the nonspecificity of these symptoms, patients with CTEPH are often misdiagnosed, resulting in misdirected therapies and significant delays in seeking appropriate medical and surgical treatment.1 These symptoms are caused by a combination of hemodynamic and ventilatory
Preoperative evaluation
The possibility of CTEPH should be considered in all patients with PH, regardless of age, gender, or comorbidities. This is particularly important if the cause of PH seems to be isolated to the pulmonary vasculature and common secondary causes have been excluded.35 The presence of even 1 persistent segmental defect on perfusion scan should raise a suspicion for chronic thromboembolic disease.
The preoperative evaluation of patients with suspected chronic thromboembolic disease serves 4 important
PTE surgery and postoperative outcomes
PTE surgery, or what is increasingly referred to as PEA surgery,49 is the preferred treatment of selected patients with chronic thromboembolic disease. The presence of surgically accessible chronic thromboembolic lesions, as judged by the diagnostic studies discussed earlier, is the principal criterion in determining whether a patient is a candidate for surgery. The experience and capabilities of the surgical team also determine what will be considered accessible lesions. Beyond this technical
Small vessel disease in CTEPH
The concern regarding concomitant small vessel disease in CTEPH stems from 3 timely issues: (1) not all patients benefit from PTE as a result of significant concomitant small vessel disease, (2) advances in medical therapies for the treatment of PAH are focusing on treating other secondary types of PH including CTEPH, and (3) defining the small vessel contribution to CTEPH may improve the approach to establishing operability and help formulate a preoperative classification system.
Although most
Medical therapies for CTEPH
Whenever possible, the definitive, and potentially life-saving, therapy for CTEPH is surgical PTE. Attempts at medical PAH-specific therapy for this disease should never be used in an attempt to avoid or delay evaluation for surgically operable disease.1 It is of concern that use of PAH-specific therapy before referral to the UCSD center has steadily increased in recent years,34 and that this practice did not result in any discernible advantage in preoperative or postoperative hemodynamics.
Summary
CTEPH develops in at least 1% of acute pulmonary emboli events and is likely underdiagnosed. Because evidence of an acute pulmonary embolus event is lacking in 42% to 63% of patients with CTEPH, it is likely that the number of CTEPH cases is even greater than is projected from known acute thromboembolic events. In the absence of appropriate treatment, the prognosis is poor, with survivorship dependent on the severity of right heart failure at the time of diagnosis. Risk factors for the
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Update in the management of chronic thrombo-embolic pulmonary hypertension
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2015, International Journal of CardiologyCitation Excerpt :Chronic thromboembolic pulmonary hypertension (CTEPH) treated with conventional medical therapy, such as ambulatory oxygen therapy and anticoagulation, carries a poor prognosis [1–6].