Chronic Thromboembolic Pulmonary Hypertension

By Peter F. Fedullo, MD

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Clinical Presentation

As in other forms of pulmonary arterial hypertension, the complaint common to all patients with CTEPH is exertional dyspnea, which is related to increased dead space ventilation and limitation in cardiac output. Patients accustomed to higher levels of activity on a daily basis recognize the decline in exercise capacity at an earlier point than those who lead a sedentary lifestyle. In certain patients, tolerable dyspnea at sea level is amplified by ascent to altitude. As the disease progresses, symptoms of lightheadedness or presyncope may occur with exertion, with coughing, or when bending at the waist, the latter symptom perhaps related to a transient decrease in venous return and cardiac output. Late in the course of the disease, as right coronary artery perfusion and right ventricular function become incapable of responding to increased demands, syncopal events and exertional chest pain may develop.

Early in the course of the disease, physical examination findings may be subtle and limited to an accentuation of the pulmonic component of the second heart sound. As the pulmonary hypertension progresses, findings consistent with pulmonary hypertension develop: a right ventricular lift, a closely split second heart sound with further accentuation of its pulmonic component, a right ventricular S₄ gallop, and varying degrees of tricuspid regurgitation. Even at this stage of the disease, however, physical findings can be deceptive, particularly for physicians unfamiliar with the physical diagnostic manifestations of pulmonary hypertension and in patients who are obese or have coexisting cardiopulmonary disease. As right ventricular failure ensues, elevated jugular venous pressure with a prominent v wave, a right-sided S₃, lower-extremity edema, hepatomegaly, ascites, and cyanosis develop. The intensity of the tricuspid regurgitant murmur may diminish as the tricuspid annulus dilates and the transvalvular pressure gradient decreases.

The presence of pulmonary flow bruits is an important physical diagnostic finding in approximately 30% of patients with chronic thromboembolic disease.⁸ The bruits appear to result from turbulent flow across partially obstructed central pulmonary vascular segments. These bruits are not unique to chronic thromboembolic disease, having been described in other disease states associated with focal narrowing of large pulmonary arteries, such as congenital branch stenosis and large vessel pulmonary arteritis. They have not, however, been described in primary pulmonary hypertension, the most common competing diagnostic possibility.

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