Table 2.

Characteristics of patients who died during follow-up

Characteristic	Postoperative PAH	Idiopathic PAH	P
Died	13 (6.5)	43 (26.4)	< 0.001
Time from diagnosis to death, y	2.1 (0.7, 4.4)	2.6 (0.2, 4.1)	0.687
Median age at death, y	21.7 (9.7, 32,2)	9.4 (6.0, 12.9)	0.001
Children	5 (38.5)	39 (90.7)	< 0.001
Cause of death
Heart failure	3 (23.1)	14 (32.6)	0.515
Sudden death∗	3 (23.1)	9 (20.9)	0.869
Hemoptysis	0	4 (9.3)	0.072
PHC	3 (23.1)	5 (11.6)	0.301
Cerebral embolism	0	1 (2.3)	0.579
Respiratory distress	1 (7.7)	0	0.066
Non-disease-related	0	4 (9.3)	0.254
Unknown	3 (23.1)	6 (14.0)	0.433
On PAH therapies at death	7 (53.8)	31 (72.1)	0.217

Values are n (%) or median (interquartile range).

PAH, pulmonary arterial hypertension; PHC: pulmonary hypertensive crisis.

^∗“Sudden death” was defined as the sudden, natural unexpected death of unknown or cardiac cause outside the hospital—last seen alive and functioning normally 24 hours before being found.³⁸