Cause of death during follow-up
| Cause | n (%) |
| Total deaths | 18 (100) |
| Deaths attributed to PAH | 6 (33) |
| Right-ventricular failure | 3 (17) |
| Sudden cardiac event | 3 (17) |
| Deaths not directly attributed to PAH | 9 (50) |
| Respiratory failure | 2 (11) |
| Acute respiratory infection | 3 (17) |
| Malignancy | 3 (17) |
| Other | 1 (6) |
| Unknown | 3 (17) |
PAH: pulmonary arterial hypertension.