Table 3. Clinical characteristics.

	Patient 1	Patient 2	Patient 3	Patient 4	Patient 5	Patient 6	Patient 7	Patient 8
PAH-onset	Adult	Adult	Adult	Adult	Adult	Child	Child	Child
Initial Diagnosis	Familial	Familial	Idiopathic	Idiopathic	Idiopathic	CHD	Idiopathic	Idiopathic
Gender	Female	Female	Female	Male	Male	Male	Female	Female
Follow-up, years	11	4	9	2.1	5	16	8	1.5
Final status	Alive	Alive	Transplant	Death	Alive	Alive	Alive	Alive
Radiological Diagnosis	ILD	PAH	PVOD	PVOD	PAH	PAH	PAH	ILD
Skeletal Disorder	Absent	Absent	Absent	Absent	Absent	Arthritis	Growth delay	Absent
CHD	Absent	Absent	Absent	Absent	Absent	OS ASD	Absent	PDA, PFO
Neurologic/ psychomotor deficits	Absent	Absent	Absent	Absent	Absent	Absent	Developmental delay Nystagmus	Hearing loss
DLCO, % predicted	34	29	55	32	61	49	NA	NA
VFC, % predicted	56	98	86	115	58	70	NA	NA
FEV1, % predicted	53	82	66	104	140	70	NA	NA
mPAP, mmHg	50	49	75	38	49	61	36	39
PCWP, mmHg	8	7	24	9	7	10	12	11
CO, l/min	3.8	2.8	3.5	3.45	6.3	3.1	2.9	1.6
CI, l/min/m2	2.3	1.99	2.02	1.95	3.4	1.5	4.9	4.7
PVR, WU	11	15	15	8.4	6.7	16.5	4.9*	6*
AVT	Negative	Negative	Negative	Negative	Positive	NA	Positive	Positive
Treatment	ERA + PDE5i + syst PC	ERA + PDE5i + Syst PC	ERA + PDE5i + syst PC	ERA + PDE5i	CCB + ERA	ERA + PDE5i + syst PC	ERA + PDE5i + CCB	PDE5i + syst PC

DLCO: Diffusing Capacity of the Lung for Carbon Monoxide; VFC: Vital Forced Capacity; FE1V: Forced Expiratory Volume in 1 second; mPAP: mean Pulmonary Artery Pressure; PCWP: Pulmonary Capillary Wedge Pressure; CO: Cardiac Output; CI: Cardiac Index; PVR: Pulmonary Vascular Resistance; WU: Wood Units; AVT: Acute Vasoreactivity Testing; ERA: Endothelin Receptor Antagonist; PDE-5i: Phosphodiesterase Type 5 Inhibito; CCB: Calcium Channel Blocker; PAH: Pulmonary Artery Hypertension; PVOD: Pulmonary Venooclusive Disease; CHD: Congenital Heart Disease; Syst PC: systemic prostacyclin; PDA: Patent Ductus Arteriosus; PFO: Patent Foramen Ovale; ILD: Interstitial Lung Disease

* Indexed PVR, UW.m2