Table 5.

Clinical, echocardiographic, laboratory, radiographic, and histologic features of PTTM.

Symptoms of cough and progressively worsening dyspnea

Physical examination notable for PH* and/or congestive heart failure†

Hypoxemia

PH on echocardiography or right heart catheterization (elevated mean PAP; normal PAOP; increased PVR)

Anemia, thrombocytopenia, elevated LDH, elevated D-dimer on laboratory analysis

Chest CT findings of inter- and intra-lobular septal thickening, lymphadenopathy, nodules, and GGO

Pulmonary tissue histology revealing pulmonary arteriopathy +/– pulmonary venopathy +/– involvement of lymphatics

Presence of malignancy (carcinoma most common)

^*PH on exam includes split S2 with a loud P2 component; LDH, lactate dehydrogenase.

^†Heart failure on exam includes jugular venous distension, S3 heart sound, lower extremity edema, and pulsatile liver at right upper quadrant.

PAP, pulmonary artery pressure; PAOP, pulmonary artery occlusion pressure; PVR, pulmonary vascular resistance; GGO, ground-glass opacification.