The axial image of the chest CT angiogram demonstrated a constellation of findings suggestive of chronic thromboembolic pulmonary hypertension (CTEPH); the flattened eccentric thrombus in the right pulmonary artery caused the contour of the intimal surface to become irregular, and the abrupt vessel narrowing resulted from recanalization of the organized thrombus. These findings are direct signs of chronic thromboembolic disease. The CT image also showed an enlarged pulmonary trunk and a ratio of the diameter of the main pulmonary artery to the diameter of the aorta that was greater than 1. This finding, together with the fixed, split S2 and prominent P2 on cardiac examination and the ECG changes (suggestive of right ventricular hypertrophy [RVH] and right heart strain) pointed toward a diagnosis of chronic pulmonary hypertension. The combination of the CT signs of chronic thromboembolic disease and clinical signs of chronic pulmonary hypertension made CTEPH the most likely diagnosis.
Further testing in this patient included a duplex ultrasound scan of the lower extremities, which revealed a bilateral calf deep vein thrombosis; a ventilation/perfusion scan, which showed several mismatched perfusion defects of the posterior segment of the right and anterior segments of the left upper lobes (read as high probability for pulmonary embolism); and an elevated factor VIII assay. Echocardiography showed a high pulmonary systolic pressure of 82 mm Hg, right ventricular hypertrophy, and reduced right ventricular function. A subsequent right heart catheterization confirmed the diagnosis of pulmonary hypertension, with an elevated mean pulmonary artery pressure of 52 mm Hg and pulmonary vascular resistance of 12.3 Wood units.
CTEPH is the result of obstruction of the pulmonary arteries by acute and recurrent PE, with subsequent organization of the thrombi. In most patients with acute PE, the thrombus resolves with minimal or no residua. The incidence of CTEPH is unclear. In a study that enrolled 223 patients with PE, the cumulative incidence of symptomatic CTEPH was 1% of cases that occurred 6 months after acute episodes of PE, 3.1% at 1 year, and 3.8% at 2 years. Elevated factor VIII and antiphospholipid antibodies have been seen in patients with CTEPH. Other factors that have been shown to place patients at greater risk for CTEPH include a younger age at presentation, larger perfusion defects at diagnosis, idiopathic pulmonary embolic disease, pulmonary artery systolic pressures > 50 mm Hg at presentation, and the size of the initial thrombus burden.[2,4] Additionally, certain medical conditions, such as prior splenectomy, the presence of a ventriculoatrial shunt to treat hydrocephalus, and chronic inflammatory disorders (such as osteomyelitis and inflammatory bowel disease), have all been associated with an increased risk for CTEPH.
A patient may provide a history that is consistent with acute PE, but nearly two thirds of patients with CTEPH may not have a history of acute PE, which may result in diagnostic delays. Patients who have CTEPH usually complain of exertional dyspnea and a gradual decrease in exercise tolerance over months to years. Other symptoms that may be reported include a nonproductive cough, hemoptysis, pleuritic chest pain, and presyncope/syncope. Findings on physical examination in patients with CTEPH reflect the degree of pulmonary vascular disease on presentation. Physical signs attributable to CTEPH may be subtle or absent in the absence of right-sided heart failure. Signs of pulmonary hypertension include right parasternal heave, fixed splitting of S2 with accentuated pulmonic component (P2), right ventricular fourth heart sound gallop, tricuspid regurgitation, and/or pulmonary insufficiency murmur. As the disease progresses and if left untreated, right ventricular dysfunction and right heart failure occur; significant findings at this time include pedal edema, elevated jugular venous pressure, and hepatomegaly.
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