A 47-year-old man presented with a history of a gradual worsening hemoptysis over more than a decade, with shortness of breath for the most recent 2 years. Two years prior, he had been diagnosed with pulmonary tuberculosis and was treated with medications for tuberculosis for 6 years without significant improvement. His past medical history, family history, and social history were noncontributory. His physical examination findings were normal. His complete blood count, comprehensive metabolic panel, C-reactive protein, erythrocyte sedimentation rate, HIV testing, and urinalysis yielded mostly normal results, except for an elevated erythrocyte sedimentation rate (22 mm/h) and D-dimer concentration (1137.86 mg/L).
Echocardiography results were consistent with PH and right ventricular dysfunction, with an estimated pulmonary arterial systolic pressure of 78 mm Hg. The echocardiogram also identified turbulent blood flow within the left atrium (Video 1). Color Doppler demonstrated 2 high-velocity continuous jets originating from the right and left PVs, indicative of PV stenosis (Fig. 1). A ventilation/perfusion scan demonstrated diffusely decreased perfusion to the right lung, most prominent in the right upper lobe. Contrast-enhanced computed tomography (CT) of the thorax revealed multiple soft-tissue shadows, mediastinal lymphadenopathy, and calcification, findings consistent with FM (Fig. 2). The CT also revealed bilateral patchy ground glass opacities suggestive of pulmonary edema with occlusion of the left and right superior PVs and stenosis of the inferior PVs (Figs. 2 and 3). In addition, pulmonary consolidation was observable (Fig. 3).
A right heart catheterization was performed, during which an increased PA pressure of 69/29 mm Hg (mean, 42 mm Hg) was observed with a wedge pressure of 16 mm Hg. His cardiac index was 3.52 L/min/m2. Cardiac angiography revealed no contrast flow into the left atrium through the left and right superior PVs; a small amount of contrast reached the left atrium through the left and right inferior PVs (Video 2). It also demonstrated superior PV occlusion and inferior PV stenosis. Together with the history and physical examination, these observations led to a clinical diagnosis of chronic FM with PV occlusion and stenosis leading to PH.
In a discussion of the potential treatment options, the surgeon suggested that efforts to strip away the fibrotic soft tissue could result in rupture of the PVs, and thus recommended a catheter-based intervention for the PV stenosis. However, the interventional radiologist thought that endovascular stenting of the stenotic lesions was likely to result in recurrent stenosis. After comprehensive consideration of the risks of surgery and a catheter-based intervention, the patient chose to proceed with conservative management and was discharged with a diuretic therapy plan aimed at reducing his pulmonary edema.
In September 2016, he was admitted to the hospital for a pulmonary infection and was discharged after showing improvement. At a follow-up visit in November 2016, the patient confirmed that he had adhered to the diuretic regimen, but felt no significant improvement in his symptoms. At present, his condition is stable.
Chest CT (pulmonary window) in patient with MT. Note the findings of pulmonary consolidation (arrow) and pulmonary edema (asterisk).