Table 2.

Radiological Features of Computed Tomography of the Chest in Pulmonary Arterial Hypertension Cases Analyzed as Part of the Imaging Substudy

	BMPR2 (n = 34)	No Causal Variant Identified (n = 143)	TBX4 (n = 8)	P Value	Available (Total n)
Vascular features
Axial MPA diameter, mm	34.2 (30.9–37.4)	34.6 (31.4–38.3)	34.0 (30.7–38.5)	0.674	185
PA to Ao ratio	1.23 (1.12–1.40)	1.14 (0.99–1.29)	1.18 (0.94–1.32)	0.083	185
Neovascularity	7 (23.3)	10 (7.69)	—	0.048	167
Lung and mediastinal features
Ground-glass opacities	18 (52.9)	62 (43.4)	—	0.016	185
Centrilobular pattern				0.088	185
None	17 (50.0)	90 (62.9)	8 (100)
Subtle	4 (11.8)	21 (14.7)	—
Present	13 (38.2)	32 (22.4)	—
Nonspecific mosaic pattern				0.441	180
None	31 (91.2)	130 (90.9)	3 (100)
Subtle	—	7 (4.90)	—
Present	3 (8.82)	6 (4.20)	—
Fibrosis				1.000	185
None	34 (100)	137 (95.8)	8 (100)
Present	—	6 (4.2)	—
Pleural effusion
None	33 (97.1)	132 (92.3)	8 (100)	1.000	185
Subtle	—	3 (2.10)	—
Present	1 (2.94)	8 (5.59)	—
Interlobular septal thickening				0.157	179
None	27 (87.1)	122 (87.1)	5 (62.5)
Subtle	3 (9.68)	9 (6.43)	1 (12.5)
Present	1 (3.23)	9 (6.43)	2 (25.0)
Mediastinal lymphadenopathy				0.721	185
None	29 (85.3)	122 (85.3)	6 (75.0)
Present	5 (14.7)	21 (14.7)	2 (25.0)
Airway/acinar features	11 (32.4)	48 (33.6)	7 (87.5)	0.009	185
Bronchial wall thickening				0.051	179
None	26 (83.9)	126 (90.0)	6 (75.0)
Subtle	3 (9.68)	11 (7.86)	—
Present	2 (6.45)	3 (2.14)	2 (25.0)
Emphysema				0.026	185
None	32 (94.1)	120 (83.9)	5 (62.5)
Subtle	—	13 (9.09)	—
Present	2 (5.88)	10 (6.99)	3 (37.5)
Air trapping				0.040	185
None	28 (82.4)	121 (84.6)	4 (50.0)
Subtle	4 (11.8)	7 (4.90)	1 (12.5)
Present	2 (5.88)	15 (10.5)	3 (37.5)
Suspected PVOD	1 (3.03)	8 (5.59)	1 (12.5)	0.500	184

Definition of abbreviations: Ao = aortic; MPA = main pulmonary artery; PA = pulmonary artery; PVOD = pulmonary venoocclusive disease; TBX4 = T-BOX transcription factor 4.

Data are presented as n (%) or median (interquartile range).