Pulmonary endarterectomy (PEA) is a complex surgical intervention in selected operable patients. Treatment may be either surgical or medical therapy. Then, upon completing the evaluation, CTEPH is confirmed, and the clinician can make clinical decisions regarding treatment strategy. After this period, the diagnosis of CTEPH is considered to distinguish it from the subacute episode of PE. During this period, the disease can regress, and significant hemodynamic improvement or even full resolution of pulmonary hypertension may occur. All patients with suspected CTEPH should start on anticoagulation for at least three months. Patients with CTEPH should be evaluated and treated in an experienced center by a specialized team of experts. The combination of different diagnostic tools can provide additional information and assist with differential diagnosis, planning treatment strategies, and assessing response to treatment. Novel techniques such as dual-energy CT, dynamic contrast-enhanced MRI, and optical coherence tomography (OCT) are also valuable diagnostic tools and assist in the evaluation of patients with CTEPH. Right heart catheterization with conventional pulmonary angiography is the gold standard in diagnosing CTEPH. Diagnostic criteria for CTEPH include mean pulmonary arterial pressure over 20 mm of Hg, pulmonary arterial wedge pressure under 15 mm of Hg, elevated pulmonary vascular resistance greater than 3 Woods units, and evidence of chronic pulmonary embolism on CT or V/Q scan. V/Q scan has high sensitivity (90 to 100%) and specificity (94 to 100%) and is a first-line screening tool for diagnosing CTEPH. Transthoracic echocardiography is a primary diagnostic tool for the diagnosis of pulmonary hypertension, but it is not specific, as it cannot differentiate between acute and chronic PE. In later stages, Chest X-ray shows signs of pulmonary hypertension, cardiac chambers enlargement, and segmental oligemia with pleuroparenchymal scarring. Initial evaluation of a patient often includes Chest X-ray, which may be normal in the early stages of the disease. Confirmation of the diagnosis requires a combination of imaging techniques such as echocardiography, Ventilation/perfusion scan (V/Q) scan, and CT pulmonary angiography. This finding gets described as subtle bruits, high pitched, blowing quality, accentuated on inspiration, and usually heard on holding breath.Įarly diagnosis of chronic pulmonary thromboembolism is a challenging task due to nonspecific symptoms and delayed manifestation of the disease. The presence of pulmonary flow murmurs signaling turbulent flow across narrowed segments of large pulmonary arteries over lung fields is a unique finding in CTEPH. With the progression of pulmonary hypertension, a tricuspid regurgitation murmur, pedal edema, jugular venous distention, and other symptoms of right ventricular failure appear.Ĭlassic signs of pulmonary hypertension that are present in CTEPH are an S4 gallop, a tricuspid regurgitant murmur, and a precordial right ventricular heave. In most cases, the diagnosis of CTEPH is established only in the later stages of the disease, when peripheral edema, dyspnea during exercise, dizziness, or syncopal episodes have already appeared.ĭuring the early stages of CTEPH, physical examination of the patient may not reveal any notable findings. Other infrequent symptoms are cough, episodic hemoptysis, atypical chest pain, and palpitations. The most common presenting symptoms are exertional dyspnea and a decline in exercise tolerance. CTEPH comes under group four of the World Health Organization classification of pulmonary hypertension. Chronic thromboembolism differs from CTEPH by the presence of resting pulmonary hypertension, a situation not seen in chronic thromboembolic disease. CTEPH will eventually lead to a significant limitation of functional capacity secondary to worsening hemodynamics and ultimately cause premature death if the disease is left to run its natural course without intervening. Thus, a low threshold is necessary for these patients to monitor for developing CTEPH. Chronic thromboembolism often results in CTEPH and overt right heart failure. Acute pulmonary embolism in a few cases in combination with other risk factors like prothrombotic tendencies, recurrent thromboemboli, genetic, and intrinsic factors may progress to chronic thromboembolic disease. However, advances in medical and surgical treatments have markedly improved the outcomes. Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially life-threatening condition and is associated with high morbidity and mortality.
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